Warung Bebas

Wednesday, 22 September 2010

Haemophilia part 2 (Diagnostic and therapy)


 Until now the family history is still the best way to do the first riddle of the cases of haemophilia, although there are 20-30% of cases of haemophilia, found X chromosome due to spontaneous mutation in the gene encoding F VIII / F IX. A boy - he allegedly suffered from haemophilia, if there is a history of recurrent bleeding (hemartrosis, hematoma) or a history of bleeding after trauma or stretching certain actions with or without family history.

Antenatal diagnosis can actually be done on pregnant women with risk. Examination of factor VIII activity and antigen levels of F VIII in the blood of the fetus in the second trimester fetus can determine the status of the vulnerability of haemophilia A. Identification of F VIII gene and the gene marker for better and more advisable.
A woman suspected of being the bearer of the nature of haemophilia (career) if he has more than one boy or haemophilia patients have one or more brothers and a young boy or his father's haemophilia patients with haemophilia patients. Detection of haemophilia A career can be done by calculating the ratio F VIIIc activity with antigen F VIIIvW. If the value is less than one has accuracy in determining the career of about 90% of haemophilia, but the heart - the heart on the state of pregnancy, hormonal contraceptive use and the presence of liver disease as it can increase the activity factor VIIIc. F VIII activity - average 50% on a career, but sometimes - sometimes <30% and bleeding can occur after trauma or surgery. Genetic analysis using DNA probes, namely by finding polymorphic loci on chromosome X will provide more precise information.
Laboratory abnormalities found in hemostasis testing in disorders, such as lengthening clotting time (CT) and activated partial thromboplastin time (aPTT), thromboplastin generation test abnormality, with the bleeding and the prothrombin (PT) within normal limits.
Definitive diagnosis established by reduced activity of factor VIII / factor IX, and if facilities are available can be performed cytogenetic examination examination seventh marker gene F / F IX. Activity of F VIII / F IX is expressed in U / ml with a mean activity of clotting factor in 1 ml of normal plasma was 100%. Normal value of F VIII activity / F IX was 0.5 to 1.5 U / ml or 50-150%. Must remember is to distinguish haemophilia A with Von Willebrand diseases, by looking at the ratio of F VIIIc: F VIIIag and activity F VW (ristosetin test) lower.


• Haemophilia A and B with a deficiency of factor XI and XII
• Haemophilia A with Von Willebrand disease (especially the Normandy variant), acquired factor VIII inhibitor and a combination of factor VIII deficiency and factor V congenital.
• Haemophilia B with liver disease, the use of warfarin, vitamin K deficiency, F IX inhibitors is very rarely obtained.


Rational treatment of haemophilia is to normalize the levels of factor antihaemophilia. But there are some things that must be considered
• Perform both avoid injury prevention / conflict
• Plan and maintain a surgical level of clotting factor activity approximately 30-50%
• To overcome the acute bleeding that occurs then the first action that rest, ice, compresio, elevation (RICE) at the location of bleeding
1. Corticosteroids
Very helpful to eliminate the acute inflammatory process in sinovitis hemartrosis which happened after the attack. Prednisone 0.5 to 1 mg / kg / day for 5-7 days can prevent the occurrence of residual symptoms such as stiff joints (artrosis) that interfere with daily activities and reduced quality of life for haemophilia patients
2. Analgesic.
Analgesic usage is indicated in patients with severe pain hemartrosis, and should be selected analgesic that does not interfere with platelet aggregation (to avoid usage of aspirin and anticoagulants)
3. Medical Rehabilitation. 
Should be performed as early as possible in a comprehensive and holistic in a team, because the delay would cause the management of both physical disability and disability, occupational and psychosocial and medical edukasi.Rehabilitasi arthritis, haemophilia include: training active / passive, cold and heat therapy (liver - liver), ortosis use, psychosocial therapy and therapeutic recreation also education. 
4. Replacement therapy Clotting Factor
Giving clotting factors are given three times a week to avoid a physical disability (especially joints) so that patients can do normal activities. But to achieve that goal is needed of factors antihemofilia (AHF) is quite a lot with the high cost. Clotting factor replacement therapy in cases hemofili done with F VIII or FIX, either recombinant, concentrates, or blood components that contain quite a lot of factors - such clotting factors. The provision made in a few days until the wounds or swelling improved, and particularly during physiotherapy.
Concentrated F VIII / F IX
Haemophilia A severe or mild and moderate to serious bleeding episodes requiring clotting factor correction with high levels should be treated with F VIII concentrate that has been attenuated virus. Factor IX is available in two forms namely prothrombin Complex Concentrates (PCC) which contains F II, VII, IX, X, and purified FIX Concentrates which contain a number of F IX without other factors. PCC can cause paradoxical thrombosis and coagulation due to intravenous spread by a number of other clotting factor concentrates. Risks can be increased in the provision of factor IX concentrate repeated so purified F IX is more desirable.

A. AHF Cryopresiptat
Is one of the non-cellular blood components that are specific plasma concentrates containing F VIII, fibrinogen, factor Vonwillebrand. May be granted if the F VIII concentrates is not found. One bag contains Cryopresiptat 80-100 UF VIII. One bag containing 100 UFCryopresiptat
B. One-deamino 8-D Arginine vasopressin (DDAVP) or desmopresin
Synthetic anti-diuretic hormone (DDAVP) to stimulate increased levels of F VIII activity in plasma up to four times, but it is temporary. Until now the mechanism of DDAVP is not known entirely, but was recommended to be given to mild and moderate haemophilia A and also on women's careers are symptomatic. Giving can be administered intravenously at a dose of 0.3 mg / kg bw in the 30-50 0.9% NaCl for 15-20 minutes with eight-hour work period. Effect on the granting of this peak reached in 30-60 min. In the year 1994 has been issued in the form of concentrates DDAVP intranasal spray. The recommended dose for patients with body weight <50 kg 150 mg (one spray), and 300 mg for patients with BW> 50 kg (twice a spray), with peak effects after 60-90 minutes. Giving DDAVP for the prevention of bleeding events should be performed every 12-24 hrs. Side effects that may occur in the form of tachycardia, flushing, thrombosis and hyponatremia. Angina can also occur in patients with coronary heart disease
C. Antifibrinolitik
Used on the B haemophilia patients to stabilize the clot / fibrin fibrinolysis by inhibiting the process. This proved very helpful in the management of haemophilia patient with bleeding, especially in cases of oral mucosal bleeding caused by the extraction of teeth because saliva contains an enzyme firinolitik. Epsilon Aminocaproic Acid (EACA) can be given orally or intravenously with an initial dose of 200 mg / kg, followed by 100 mg / kg body weight every six hours (maximum of five grams of each delivery). Tranexamic acid is given a dose of 25 mg / kg body weight (maximum 1.5 g) was administered orally or 10 mg / kg BW (maximum 1 g) intravenously every 8 hours. Tranexamic acid can also be reconstituted with 10% of parenteral fluids, especially the normal saline. 
D. Gene Therapy
The study of gene therapy using retrovirus vectors, adenovirus, and adeno - associated virus provides new hope for patients with haemophilia. Currently under intensive research by moving vivo adenovirus vector carrying the gene into liver cells antihaemophilia. F VIII gene are relatively more difficult than the F IX gene, because of its size (9kb) is bigger, but the end of 1998, the experts succeeded in moving the plasmid - based ex vivo factor VIII into the fibroblasts.
E. FFP (the Fresh Frozen Plasma)  clotting blood cell that has been taken from the red blood transfusion of FFP usually  Perform weight depending on whether haemophilia suffered. If patients with severe levels of the patients had to perform a transfusion of FFP 3x a week, but particularly if it was bleeding that resulted kebengkakan specific area then the transfusion should be done every day until the swelling has become lost. One bag containing the plasma, clotting factors, the complement , and plasma protein, the volume of a bag of FFP is 200-250 ml given six hours after the withdrawal, ABO match, 4-6 units could enhance coagulation factor 20-30%.

Arthropathy hemophilia: the accumulation of intra-articular blood who settled with the result of degeneration of cartilage and bone joints progressively. This causes a decrease until the destruction of joint function. Hemartrosis that are not managed properly can also cause sinovitis chronic synovial tissue inflammation due process is not endless. Joints that often develop complications are knee joints, ankles and elbows.
Prolonged bleeding due to medical action is often found if not done by providing preventive therapy for hemophilia blood clotting factor and weight are in accordance with the kind of medical practice itself (tooth extraction, circumcision, appendectomy, surgery thoracal / intra-abdominal). While bleeding due to trauma are common in everyday situations such as hemartrosis, intramuscular hemorrhage and hematoma. Intracranial hemorrhage is rare, but if  happened, it would be fatal

Friday, 17 September 2010

Hemophilia part 1

Patient asks to Mbah Dukun Bagong: Mbah, i had a friend, he was died 2 months ago, he got bleeding after circumcised, is circumcised dangerous?
Mbah Dukun Bagong answers: "mmmm i see, circumcised is not dangerous, its "sunnah" God commanded Abraham to circumcise his son, Ishmael. However, there is condition, which person can't be able to circumcised, its called HEMOPHILLIA

Hemophilia is a bleeding disease due to deficiency of blood-derived clotting factors (hereditary) by sex - linked recessive on chromosome X (XH). Although hemophilia is a hereditary disease but approximately 20-30% of patients had no family history of blood clotting disorder, so that spontaneous mutation is thought to occur due to endogenous or exogenous environment.

Clotting mechanism is divided into three basic stages:
1) The formation of plasma thromboplastin intrinsic trombopatogenesis also called, began with the work of platelets, especially TF3 and other clotting factors on foreign surfaces or contact with collagen. Clotting factor is factor IV, V, VIII, IX, X, XI, XII and factor III and VII.
2) Changes in prothrombin into thrombin is catalyzed by thromboplastin, F IV, V, VII, and X
3) Changes in fibrinogen into fibrin by thrombin catalyst, TF 1 and TF 2.5

Hemostasis is good progress within a certain time limit so that not only shaped thromboplastin, thrombin or fibrin are important, but also the formation of each Hemophilia is an inherited blood clotting disorder is first recognized and has been well known since 1911. At that time the disease known as hemophilia due to congenital blood clotting disorders in males are derived by a healthy woman. The term hemophilia there is only limited understanding of massive bleeding in boys with a prolonged period of freezing. It turned out that the definition or restriction is not appropriate that it experiences a change, because not all hemophiliacs who accompanied the memanjang.Hal freezing is caused because the examination of blood clotting time is not sensitive.

Until recently recognized 2 types of hemophilia are reduced sex - linked recessive namely: 
  1. Hemophilia A (classical hemophilia), due to deficiency or dysfunction of coagulation factor VIII (F VIII c)

  2. Hemophilia B (Christmas disease) due to deficiency or dysfunction of coagulation factor IX (christmas factor)

Gene factor VIII and factor IX is located on the X chromosome and is recessive, then the disease is carried by women (career, XXh) and manifests clinically in male - male (patient XhY); may manifest clinically in women when both X chromosomes in women there are abnormalities (XhXh).

This disease was first recognized at the Judah family that is around the second century AD in the Talmud. In the early 19th century, modern history new haemophilia, genealogy begins with the British royal family about this disease by Otto (1803). Since it is known as hemophilia blood clotting disorder which reduced X-linked recessive, about half a century before Mendel's laws were introduced. Next, Leggpada 1872 successfully distinguish the disease from the disease hemophilia and other blood-clotting disorders based on clinical symptoms, ie, a disorder which is derived with bleeding tendency muscles and joints that lasts a lifetime. At the beginning of the 20th century, hemophilia was diagnosed based on family history and blood clotting disorders. In the year 1940 - 1950 new experts successfully identify F VIII - FIX deficiency in hemophilia A and B. In 1970 the F VIII were isolated from the carrier protein in plasma, namely von Willebrand factor (vW F), so that now can be distinguished between hemophilia A and von Willebrand disease.
Entering the 21st century diagnostic approaches with advanced technology and providing the necessary coagulation factors capable of carrying patients with hemophilia do other healthy activities such as other people without problems.

Haemophilia derived from Ancient Greek, which consists of two words namely Haima, which means blood and philia, which means love or affection. Hemophilia is a bleeding disease due to deficiency of blood-derived clotting factors (hereditary) by sex - linked recessive on chromosome X (XH). Blood on a hemophiliac can not be frozen by themselves normally. The process of blood clotting in a patient with hemophilia is not as fast and as much as any other normal person. He will require more time for blood clotting process. Patients most susceptible to interference, bleeding under the skin, such as if a little bruising clash, or a bruise incurred by itself if the patient has been doing heavy activity; swelling in the joints, like knee, ankle or elbow. The suffering of the people with hemophilia can cause him any harm if bleeding occurs in the vital organs such as bleeding in the brain.

Patient asks to Mbah Dukun Bagong: Mbah, tell me more about Hemophillia!
Mbah Dukun Bagong answers:

Both types of hemophilia, hemophilia A lack of factor VIII and factor IX deficiency hemophilia B, caused by permanent changes are made to the structure of genes. Genetic mutations play a major role in making proteins needed for blood clotting and that one mutation can cause damage in the process of blood clotting.

Bleeding is a typical clinical signs and symptoms are often seen in cases of hemophilia. Bleeding can occur spontaneously or from trauma mild to moderate and can arise when babies begin to learn to crawl. Clinical manifestations depend on the severity of haemophilia (clotting factor activity). Signs of bleeding that is often encountered in the form hemartrosis, hematoma subcutaneous / intramuscular, oral mucosal bleeding, intracranial bleeding, epistaxis, and hematuria. Often, too, found that continuous postoperative bleeding small (circumcision, dental extraction).
Hematrosis most commonly found (85%) with consecutive locations as follows, knee joints, elbows, ankles, shoulder, wrist, and so forth. Hinge joints more often experience joint hemartrosis than ball and sockets, because of his inability to resist rotary motion and angular at the time of voluntary or involuntary movement, while the ball and socket joints better able to withstand the movement because of its function.
Intramuscular hematoma occurred in the muscles - the flexor muscles, particularly in the calf muscle, muscle - the region of the iliopsoas muscle (often in the pelvis) and forearm. Hematoma is often causes significant blood loss, compartment syndrome, nerve compression and muscle contractures. Intracranial bleeding is a major cause of death, can occur spontaneously or after trauma. Retroperitoneal bleeding and airway retrofaringeal dangerous to life threatening. Massive hematuria often found and can cause renal colic but not life threatening. Postoperative bleeding is often continued for several hours to several days, which is associated with poor wound healing.

Tuesday, 14 September 2010

Myasthenia Gravis


When Mbah Dukun surving on internet, he gets message from Nilla Gustian. Nilla wants information about Myasthenia Gravis.

What is Myasthenia Gravis?
Myasthenia Gravis (MG) is a chronic, autoimmune disease that causes muscle weakness and excessive muscle fatigue. The name comes from Greek and Latin words meaning "grave muscle weakness." But most cases of MG are not as "grave" as the name implies.

The disease can vary in severity and distribution of weakness between individuals, and in any one patient the symptoms fluctuate with relapses and remissions. MG can resolve spontaneously, but for most patients MG persists for life. It can be life threatening, but 90% of patients become symptom-free with modern treatments.
MG produces progressive weakness and fatigue and sporadic abnormal in skeletal muscles, which get worse after exercise and repetition of movement, but can be corrected with drugs antikholineterase.
Usually, this disorder that attacks the muscles controlled by cranial nerves (face, lips, tongue, neck, and throat) but can also attack other muscles.
MG followed the explosion of anger and periodic remissions that can not be predicted. Treatment was not known, but drug therapy can improve symptoms and allow patients to live relatively normal, except during the outburst occurred.
When the disease involves the respiratory system, it can endanger lives.


Patient asks to Mbah Dukun Bagong: Who is at risk of developing MG and  Is MG hereditary?
Mbah Dukun Bagong answers:

MG affects all races and can develop at any age from childhood to extreme old age. Young patients are more commonly women, whereas older patients, over 50 years, are more often men. People who inherit a tendency to develop autoimmune disease are at increased risk of developing MG, so a patient with MG may have another autoimmune disease, such as diabetes or thyroid disease, or have a relative with autoimmune disease. Occasionally MG develops in patients with rheumatoid arthritis who are given the drug penicillamine. In these cases, the MG symptoms usually disappear when the drug is stopped.
MG is not an inherited disease and does not usually occur in families. This is in contrast to the congenital myasthenic syndromes that are genetic disorders (see below). However, it is thought that an individual’s genetic make-up is one factor, of perhaps many, that leads them to develop MG, and it may occasionally be found in more than one family member. 
Estimates of the number of people affected by MG vary, ranging from 5 to 14 people per 100,000. It occurs in all ethnic groups and both sexes. It most commonly occurs in: 
• young adult women (under 40) 
• older men (over 60)MG can occur at any age. Children sometimes develop it as well. MG is not passed down through the family. You can’t “catch it” from some-one who has it. The disease rarely occurs in more than one member of the same family. If a woman with MG becomes pregnant, sometimes the baby gets antibodies from the mother and has MG symptoms for a few weeks or months after birth. This is called neo-natal myasthenia. The symptoms can be treated and the baby does not have per-manent MG. A group of rare disorders called congeni-tal myasthenic syndromes (CMS) can pro-duce symptoms similar to MG. Unlike MG, these disorders are not autoim-mune disorders but are caused by defec-tive genes. Because of these genes, the nerve-muscle junctions in the body cannot work properly. CMS usually starts at or near birth. But it can start in childhood or even adulthood.


Patient asks to Mbah Dukun Bagong: Tell me pathology of Myasthenia Gravis?
Mbah Dukun Bagong answers:
The voluntary muscles of the entire body are controlled by nerve impulses that arise in the brain. These nerve impulses travel down the nerves to the place where the nerves meet the muscle fibers. Nerve fibers do not actually connect with muscle fibers. There is a space between the nerve ending and muscle fiber; this space is called the neuromuscular junction.
When the nerve impulse originating in the brain arrives at the nerve ending, it releases a chemical called acetylcholine. Acetylcholine travels across the space to the muscle fiber side of the neuromuscular junction where it attaches to many receptor sites. The muscle contracts when enough of the receptor sites have been activated by the acetylcholine. In MG, there is as much as an 80% reduction in the number of these receptor sites. The reduction in the number of receptor sites is caused by an antibody that destroys or blocks the receptor site.
Antibodies are proteins that play an important role in the immune system. They are normally directed at foreign proteins called antigens that attack the body. Such foreign proteins include bacteria and viruses. Antibodies help the body to protect itself from these foreign proteins. For reasons not well understood, the immune system of the person with MG makes antibodies against the receptor sites of the neuromuscular junction. Abnormal antibodies can be measured in the blood of many people with MG. The antibodies destroy the receptor sites more rapidly than the body can replace them. Muscle weakness occurs when acetylcholine cannot activate enough receptor sites at the neuromuscular junction. 


Patient asks to Mbah Dukun Bagong: How can we know MG symptoms?
Mbah Dukun Bagong answers:

Dominant symptoms of this disease are skeletal muscle weakness and fatigue. In the early stages, certain muscles are susceptible to fatigue, but no other symptoms. Ultimately, this phenomenon is getting worse and can cause paralysis.Normally, muscles felt strong in the morning and weakened throughout the day, especially after exercise. Short break for a while can restore muscle function. Growing muscle weakness; finally some muscle to not function at all. Symptoms that occur depending on the affected muscle groups; this phenomenon is increasingly becoming the menstrual period and after emotional stress, too long in the sun or the cold or infection.The appearance of symptoms can occur suddenly or from within. In many patients, weak eye closure, the closed eyelids, and double vision is the beginning of this disorder. Patients usually have nasal regurgitation of fluids that often occurs and difficulty chewing and swallowing.Because of this disorder, sufferers are often afraid of choking. Patients also experienced difficulty breathing. Because the eyelids are closed, the patient should raise his head to look toward the back, neck muscles become too weak to support the head without being short.Miastenik crisis patients (respiratory disturbance that appears suddenly) can be susceptible to pneumonia and other respiratory tract infections. This situation can get worse so that it requires mechanical ventilation and airway emergency.


Patient asks to Mbah Dukun Bagong: How to Diagnose?
Mbah Dukun Bagong answers:

The history and examination of the patient can suggest the diagnosis but it is important to confirm the diagnosis by special investigations: Antibodies to the acetylcholine receptor are found in 85% of patients with generalised MG, and 50% of patients with ocular MG. They are detected by a blood test.
Electromyography (EMG) is performed by a specialist doctor and involves measuring the electrical response in the muscle with a very fine needle. An electrical stimulus is applied to a nerve and the response in the muscle is recorded. It is a very sensitive test, showing an abnormality in most patients with MG, but is not available at all hospitals.
Tensilon® test, an injection of Edrophonium is given which results in a rapid but short-lived improvement in symptoms in many patients.
Chest scan should be done to check whether the thymus is abnormal as many patients with MG have an enlarged thymus, and some have a benign tumour.

Treatments for Myasthenia Gravis (MG)

Patient asks to Mbah Dukun Bagong: How to manage patient with Myasthenia Gravis?
Mbah Dukun Bagong answers:

 There is no known cure for MG, but there are effective treatments that allow many-but not all-people with MG to lead full lives. Common treatments include medications, thymectomy and plasmapheresis. Spontaneous improvement and even remission may occur without specific therapy.
Medications are most frequently used in treatment. Anticholinesterase agents (e.g., Mestinon®) allow acetylcholine to remain at the neuromuscular junction longer than usual so that more receptor sites can be activated. Corticosteroids (e.g., prednisone) and immunosuppressant agents (e.g., Imuran®) may be used to suppress the abnormal action of the immune system that occurs in MG. Intravenous immunoglobulins (IVIg) are sometimes used to affect the function or production of the abnormal antibodies also.
Thymectomy (surgical removal of the thymus gland) is another treatment used in some patients. The thymus gland lies behind the breastbone and is an important part of the immune system. When there is a tumor of the thymus gland (in 10-15% of patients with MG), it is always removed because of the risk of malignancy. Thymectomy frequently lessens the severity of the MG weakness after some months. In some people, the weakness may completely disappear. This is called a remission. The degree to which the thymectomy helps varies with each patient.
Plasmapheresis, or plasma exchange, may be useful in the treatment of MG also. This procedure removes the abnormal antibodies from the plasma of the blood. The improvement in muscle strength may be striking, but is usually short-lived, since production of the abnormal antibodies continues. When plasmapheresis is used, it may require repeated exchanges. Plasma exchange may be especially useful during severe MG weakness or prior to surgery.
Treatment decisions are based on knowledge of the natural history of MG in each patient and the predicted response to a specific form of therapy. Treatment goals are individualized according to the severity of the MG weakness, the patient's age and sex, and the degree of impairment. 


1. http://forum.um.ac.id

2. http://www.myasthenia.org

3. http://www.mdi.ie

4. http://www.neurology.org

5. http://www.wrongdiagnosis.com

6. http://jama.ama-assn.org

Monday, 13 September 2010



Patient asks to Mbah Dukun : "Mbah, what are complications of NS?"

Mbah Dukun Bagong answers:

1. Nitrogen balance.

Massive proteinuria in the NS will cause a negative nitrogen balance. Decreased muscle mass is found but these symptoms are often found covered with new symptoms and edema anasarka looked after the edema disappeared. Loss of muscle mass by 10-20% of body mass (lean body mass) are not rare in NS.

2. Hyperlipidemia and Lipiduria.

Hyperlipidemia is a condition that often accompanies SN. Cholesterol levels generally increased, while triglyceride levels vary from normal to slightly rising. Increased cholesterol levels caused by increasing LDL (low density lipoprotein), the major lipoprotein carrier of cholesterol. Trigliserid high levels are associated with increased VLDL (very low density lipoprotein). Also found increased IDL (intermediate density lipoproteins) and lipoprotein (Lp)a, while HDL (high density lipoprotein) cholesterol tends to be normal or low. Mechanism of hyperlipidemia in the NS associated with elevated lipid and lipoprotein synthesis and decreased catabolism of the liver. Originally thought to hyperlipidemia is the result of non-specific stimulation of protein synthesis by the liver. Therefore, no correlation with protein synthesis concluded that hyperlipidemia hyperlipidemia indirectly caused by hipoalbunemia. Hyperlipidemia can be found in the NS with near-normal albumin levels and vice versa in patients with hypoalbuminemia may be a normal cholesterol level. High levels of LDL in the NS due to increased synthesis of heart without interruption catabolism. Improved synthesis and disruption of liver VLDL and IDL conversion into LDL causes high levels of VLDL in the NS. The reduced activity of the enzyme LPL (lipoprotein lipase) are thought to cause reduced VLDL catabolism in the NS. Increased lipoprotein synthesis of the liver caused by pressure or viscosity of plasma. Decrease in HDL cholesterol levels in NS allegedly due to reduced enzyme activity HUW (colesterol acyltransferase lecithin) that function catalyzing the formation of HDL. This enzyme also serves to transport cholesterol and circulation to the liver for catabolism. Decrease in the enzyme activity associated with hypoalbuminemia who allegedly occurred in NS. Lipiduria often found in the SN and is characterized by accumulation of lipids in the cell debris and casts, such as oval fat bodies (oval fat bodies) and fatty casts. Lipiduria more associated with proteinuria than with hyperlipidemia.

3. Hypercoagulability.
Thromboembolic complications are often found in the NS due to increased intravascular coagulation. In the NS occurred due to the tendency GNMN renal vein thrombosis is quite high while the NS on GNMP GN Minimal Lessions and small frequency. Pulmonary embolism and deep venous thrombosis (deep vein thrombosis) are often seen in this NS. Antitrombin caused by loss of (AT) III, protein S, C and plasminogen activating factor in the urine and increased factor V, VII, VIII, X, platelets, fibrinogen, increased platelet aggregation, changes in endothelial cell function and the decrease zimogen factor (factor IX, XI).
4. Calcium and Bone Metabolism
Vitamin D is an important element in calcium and bone metabolism in humans. Vitamin D which banned will be excreted through urine protein causing a decrease in plasma levels. Levels of 25 (OH) D and 12.5 (OH2) D also decreased plasma levels of vitamin D, while free is not susceptible to interference. Therefore, kidney function is generally normal in the NS osteomalasia or uncontrolled hyperparathyroidism is rarely encountered. NS also happen to lose on thyroid hormone binding protein (thyroid binding protein) through urine and decreased levels of plasma thyroxine. And free thyroxine stimulating hormone, thyroxine (thyroxine stimulating hormone) remained clinically normal so do not cause interference.
5. Infection
Before the era of antibiotics, infection is often the cause of death in the NS, especially by organism (encapsulated organism). Infections caused by defects in humoral immunity, cellular, and disruption of the complement system. Decrease in IgG, IgA, and gamma globulin are often found in patients with SN , therefore decreased synthesis or increased catabolism and increased in numbers is wasted through urine. The number of T cells in the circulation is reduced which describes cellular immunity disorders. This is associated with the release of transferrin and zinc are required by T cells to function normally.
6. Impaired Renal Function
NS patients have the potential to occur with acute renal failure through various mechanisms. Decrease in plasma volume or sepsis often causes acute tubular necrosis. Another mechanism which is expected to cause acute renal failure intrarenal edema causing compression of the renal tubules. Nephrotic syndrome can be progressive and developed into PGTA. Proteinuria is a risk factor determinants of the progression of SN. Progression of glomerular damage, glomerulosklerosis developments, and damage associated with proteinuria tubulointerstisium. Hyperlipidemia are also associated with the occurrence mechanism and fibrosis glomerulosklerosis tubulointerstisium on SN, although the role of the progression of the disease is not known with certainty.
7. Other complications
Protein-calorie malnutrition can occur in adults, especially if accompanied NS massive proteinuria, less oral intake, and high catabolism process. Possible toxic effects of drugs that are bound to increase due to hypoalbuminemia causing proteinakan OTC levels in plasma higher. Hypertension is not uncommon as a complication of NS mainly associated with sodium and water retention.


Patient asks to Mbah Dukun : "Mbah, How to Diagnose NS?"
Mbah Dukun Bagong answers:

NS diagnosis is made based on clinical and laboratory examination of proteinuri massive (> 3.5 g / dl), hipoalbuminemi (<3 g / dl), edema, hiperlipidemi, lipiduri and hypercoagulabilityvenografi required to diagnose venous thrombosis which can occur due to hypercoagulability. In NS primer to determine the type of renal histopathologic abnormalities that determine the prognosis and response to therapy, renal biopsy is necessary.
 In patients can be diagnosed with NS because:

1. From physical examination found the existence of edema anasarka especially in the second leg, both legs and abdomen.
2. From examination laboratorim can be found:
    * Lipd: elevated triglycerides, total cholesterol, and LDL cholesterol
    *  Urine: proteinuria found the existence of twoThe radiological examination did not reveal any abnormality.


Patient asks to Mbah Dukun : "Mbah, how to treat patient with N?"
Mbah Dukun Bagong answers:

The treatment of NS include specific therapies for disorders or diseases causing kidney foundation (at secondary NS), reduce or eliminate proteinuria, repairing and preventing and overcoming hipoalbuminemi complication. Minimal lesions nephropathy and membranous nephropathy are two disorders that respond well to steroid therapy. Other researchers found that the focal segmental glomerulosklerosis up 40% of patients responded well to steroids with complete remission. Schieppati found that in most patients with idiopathic membranous nephropathy, symptomatic therapy with better kidney function for a long time and can heal spontaneously. Therefore they do not support the use of glucocorticoids and immunosuppressive in this type of nephropathy. Use of corticosteroid regimens in a variety of NS, including prednisone 125 mg once every two days during two months later reduced the dose gradually and stopped after 1-2 months if relapse, therapy may be repeated. Other regimens in adults is prednisone / corticosteroid 1 to 1.5 mg / kg body weight / day for 4 weeks followed by 1 mg / kg of body weight one day intervals for 4 weeks. Up to 90% of patients remission if therapy will be continued until 20-24 weeks, but 50% of patients will experience a relapse after corticosteroid is stopped. Hopper uses doses of 100 mg/48 hours. If there is no progress in 2-4 weeks, the dose was increased to 200 mg per 48 hours and maintained until proteinuri down to 2 grams or less per 24 hours, or until the therapy is considered to no avail. In children given prednisone 60 mg/m2 body surface area or 2 mg / kg body weight / day for 4 weeks, followed by 40 mg/m2 body surface area every two days during four weeks clinical. Respondof corticosteroids can be divided into:

 a. Complete remission
• proteinuri minimal (<200 mg/24 hours)
• Serum albumin> 3 g / dl
• serum cholesterol <300 mg / dl
• Current diuresis and edema disappeared

 b. Partial Remission
 • proteinuri <3.5 g / day
• Serum albumin> 2.5 g / dl
• serum cholesterol <350 mg / dl
• diuresis substandard and still edema

c. Resistant
 • clinical and laboratory showed no change or improvement after four months of treatment with corticosteroids.

Corticosteroids provide a complete remission in 67% cases of minimal lesion nephropathy NS, complete or partial remission in 50% NS membranous nephropathy and 20% -40% in focal segmental glomerulosklerosis. Please note the side effects of long-term corticosteroid use include aseptic necrosis, cataracts, osteoporosis, hypertension, diabetes mellitus. In patients unresponsive to corticosteroids, to reduce proteinuria use symptomatic therapy with angiotensin converting enzyme inhibitor (ACEI), such a low dose of captopril or enalapril, and the dose increased after 2 weeks or non-steroidal antiinflammatory drugs (NSAIDs), such as indomethacin 3 × 50mg. Angiotensin converting enzyme inhibitors reduce glomerular protein ultrafiltration by reducing pressure and improving intrakapiler glomerulus glomerular size selective barrier. Antiproteinuria effects of this drug lasts longer (approximately two months after the drug was stopped). Angiotensin receptor blockers (ARBs) was also able to improve proteinuri because it inhibits inflammation and fibrosis interstisium, inhibiting the release of cytokines, growth factors, adhesion molecules due to the work of local angiotensin II in the kidneys. Combination ACEI and ARB was reported to give greater antiproteinuria effect on primary glomerulonephritis compared with ACEI or ARB use only. Non-steroidal antiinflammatory drugs can be used in patients with membranous nephropathy and focal segmental glomerulosklerosis to reduce prostaglandin synthesis. This causes renal vasoconstriction, decreased glomerular capillary pressure, filtration surface area and reduce proteinuria up to 75%. Besides NSAIDs can reduce the levels of fibrinogen, fibrin-related antigenic and prevent platelet aggregation. However, please note that NSAIDs cause a progressive decline in renal function in some patients. This medicine should not be given if the creatinine clearance <50 ml / min. In patients who frequently relapse with corticosteroids or corticosteroid resistant to other therapies can be used with cyclophosphamide or chlorambucil. Cyclophosphamide gives a longer remission than corticosteroids (75% over two years) with a dose of 2-3 mg / kg bw / day for 8 weeks. Side effects of cyclophosphamide is bone marrow depression, infection, alopecia, hemorrhagic cystitis and infertility if given more than six months. Chlorambucil given at a dose of 0.1 to 0.2 mg / kg bb. / day for 8 weeks. Side effects of chlorambucil is azoospermia and agranulocytosis. Ponticelli and colleagues found that in idiopathic membranous nephropathy, a combination of methylprednisolone and chlorambucil for six months earlier to induce remission and maintain renal function as compared with methylprednisolone alone, but this difference diminished with time (in four years this difference was not significant anymore) . Regimen used was methylprednisolone 1 g / day intravenously for 3 days, then 0.4 mg / kg / day for 27 days followed by peroral chlorambucil 0.2 mg / kg / day one month ago seling. Another alternative therapy membranous nephropathy is cyclophosphamide 2 mg / kg / day plus 30 mg of corticosteroid every 2 days for several months (maximum 6 months). Levamisol a antihelmintic, can be used for minimal lesions nephropathy NS therapy in children with a dose of 2.5 mg / kg body weight every two days at least 112 days. A rare side effect is netropeni, thrombocytopenia and skin rash. Cyclosporine A can be attempted in patients who relapse after being given cyclophosphamide or to prolong remission after administration of corticosteroids. Dose of 3-5 mg / kg / day for six months to one year (after 6 months of reduced doses of 25% every two months). Cyclosporin A can also be used in combination with a corticosteroid in the case of a failed SN in combination with other therapies. Side effects of this drug is hiperplasi gingival, hypertrichosis, hiperurisemi, hypertension and nefrotoksis. Other therapies that have not proven their effectiveness is azatioprin 2 to 2.5 mg / kg / day for 12 months. In the case of NS that were resistant to steroid and drug imunospresan, this time can be given a new immunosuppressive mycophenolate mofetil (MMF), which has the effect of inhibiting the proliferation of B lymphocytes and T lymphocytes, inhibits antibody production of B cells and adhesion molecule expression, inhibit cell proliferation vascular smooth muscle.
Resistance to this diuretic is multifactorial. Hipoalbuminemi Allegedly reduce drug transport to work, while binding by the urine protein is not the main mechanism for this resistance. In such patients can be given intravenous salt-poor human albumin. This therapy is said to increase plasma volume, increased glomerular filtration rate, urine flow and sodium excretion. However, albumin infusion is still doubt its effectiveness as quickly excreted through the urine albumin, other than that can increase blood pressure and even pulmonary edema in patients hipervolemi. Hiperlipidemia in the long term increase the risk of early atherosclerosis. To overcome the barrier can be used hiperlipidemia hidroxymethyl glutaryl co-enzyme A (HMG Co-A) reductase that effectively lower plasma cholesterol. These drugs are said to be most effective with minimal side effects. Gemfibrozil, bezafibrat, klofibrat significantly lower triglyceride levels and slightly lower cholesterol levels. Klofibrat can be toxic at normal levels due to increased levels of free klofibrat cause muscle damage and acute renal failure. Probukol reduce total cholesterol and LDL cholesterol, but minimal effect on triglycerides. Nicotinic acid (niacin) can lower cholesterol and more effective when combined with gemfibrozil. Kolestipol Kolestiramin and effectively lower total cholesterol and LDL cholesterol, but this drug is not recommended because of their effect on intestinal absorption of vitamin D in vitamin D deficiency is exacerbated in the NS. To prevent thromboembolic complications of hypercoagulability that occurs in approximately 20% of cases of NS (most frequently in membranous nephropathy), used dipiridamol (3 x 75 mg) or aspirin (100 mg / day) as an anti-platelet aggregation and deposition of fibrin / thrombus. Besides these drugs can reduce significantly decreased kidney function and the occurrence of end stage renal failure. This therapy is given for patients experiencing nephrotic proteinuri, albumin <2 g / dl, or both. In case of thromboembolism, heparin should be given intravenous / infusion for 5 days, followed by oral warfarin administration until three months or after a healing SN. Giving heparin with activated partial thromboplastin time monitoring (aPTT) 1.5 to 2.5 times control, whereas the effect of warfarin was evaluated by prothrombin time (PT) is usually expressed as International Normalized Ratio (INR) 2-3 times normal. In the event of complications of bacterial infections (pneumococcal pneumonia or peritonitis) are given the appropriate antibiotics and can be accompanied with intravenous immunoglobulin G administration. For pneumococcal vaccine used to prevent infection. The use of immunosuppressive virus infections cause problems such as measles and herpes. Another complication that can occur include hypertension, hypovolaemic shock, acute renal failure, chronic renal failure (after 5-15 years). Handling similar to handling this situation in general. In case of chronic renal failure, in addition to hemodialysis, kidney transplantation can be performed. Dantal et al found in patients with focal segmental glomerulosklerosis who underwent kidney transplants, 15% -55% NS will happen again. Recurrence may be caused by plasma factors (circulating factors) or factors that increase glomerular permeability. Imunoadsorption plasma protein A decrease urine protein excretion in patients with focal segmental NS because glomerulosklerosis, membranous nephropathy and secondary NS because of diabetes mellitus. Imunoadsorpsi suspected release of plasma factors that alter hemodynamic or factors that increase glomerular permeability.

Thursday, 9 September 2010

Nephrotic syndrome Part 1


Patient asks to Mbah Dukun: "Mbah, would you explain about Nephrotic Syndrome?"
Mbah Dukun Bagong answers: " Sure, ok read the text below"

Nephrotic syndrome (NS) is one of the clinical manifestations of glomerulonephritis (GN) are marked with anasarka edema, massive proteinuria ≥ 3.5 g / dl, hypercholesterolemia and lipiduria. At the beginning of the process or the mild NS stage, to make the diagnosis, not all symptoms must be found. Massive proteinuria is a typical sign of NS, but in severe NS accompanied low serum albumin concentration, excretion of protein in urine was also reduced. Proteinuria also contributes to various complications that occur in the NS. Not only Proteinuria,hypoalbuminemia, hiperlipidemia and lipiduria also found in NS. Nitrogen balance diorders, hypercoaguloability, disturbances of calcium and bone metabolism, and thyroid hormones are often found in NS. Generally, NS with normal renal function can heal itself and showed a good response to steroid therapy. However, for some cases develop into end stage renal disease or develop inti chronic.


Patient asks to Mbah Dukun: "Mbah, what are cause of NS?"
Mbah Dukun Bagong answers:
Nephrotic syndrome can be caused by primary and secondary Glomerulonephritis.
A. Primary Glomerulonephritis
  • GN with minimal lessions
  • Glomerulosclerotic focal
  • Membranous Glomerulonephritis
  • GN membranoproliferatif
  • Other proliferative
B. Secondary Glomerulonephritis

1. Infections:
  • HIV, Hepatitis B and C virus
  • Syphilis, Malaria
  • TBC, Leprosy
2. Malignancy:
  • Adenosarcoma
  • Lymphoma
  • Multiple Myeloma
  • Renal Carcinoma
3. Connective Tissue Disease:
  • SLE
  • Rhematoid Arthritis
  • Mixed Cinnective Tissue Disease
4. Drugs and Toxins:
  • NSIDs
  • Gold Preparations
  • Penicillamine
  • Probenecid
  • Mercury
  • Captopril
  • Heroin
5. Other:
  • Diabetes Mellitus
  • Amyloidosis
  • Pre-Eclampsia
  • Vesikoureter reflux
  • Primary or idiopathic Glomerulonephritis is the most frequent cause of NS. in Primary GN, histopathological abnormality was found. Due to secondary causes such as infections that are often encountered in post infectious GN Stretococcus or Hepatitis B virus infection, caused by medications such as non steroidal anti-inflammatory drugs or preparations of organic gold, and caused by systemic disease.

Patient asks to Mbah Dukun: "Mbah, tell me about patophysiology of NS?"
Mbah Dukun Bagong answers:

1. Proteinuria
Proteinuria caused by increased capillary permeability to proteins due to glomerulus damage. Normally, Glomerular basement membrane (GBM) has a barrier mechanism to prevent leakage of protein. The first barrier mechanism based on moleculear size (size barrier) and the second based on electrical charge. In the NS, two mechanism are invloved disrupted. Besides the configuration of protein molecules also determine wether the protein passes through the GBM. Proteinuria is divided into selective and non-selective based on size of protein molecules that come out through urine. Selective proteinuria outif a protein consisits of small molecules such as albumin, while non-selective when the protein comes out such a large molecule composed of immunoglobulin. selectivity of proteinuria determined by GBM structural integrity. In the NS which caused by GN Minimal Lessions, found selective proteinuria. Electron microscope examination showed fusion of foot processor glomerular visceral epithelial cells and the released of cells from GBM structure. In Glomerulosclerotic Focal, GBM increased permeability caused by a factor involved in the circulation. These factors lead to glomerular visceral epithelial cells regardless of GBM that permeability increases. In Glomerulonephritis Membranous, GBM structural are damage caused by immune complex deposition in the sub-epithelium.
2. Hypoalbuminemia
Plasma Albumin concetration determined by protein intake, Liver albumin synthesis, and loss of protein through urine. Hypoalbuminemia is caused by masive proteinuria due to reduced plasma oncotic presure. To maintain plasma oncotic pressure, the liver increases production of albumin. Improvement of liver albumin synthesis was not succesfully to block the emergence hypoalbuminemia. High protein diet can improve liver albumin synthesis, but it may encourage increased excretion of albumin in the urine. Hypoalbuminemia may also occur due to increased reabsorption and catabolism of albumin by the proximal tubule.
3. Edema
Edema can be explained by theory of UNDERFILL and OVERFILL.
  • Underfill theory explains that hypoalbuminemia is a factor of edema of NS. Hypoalbuminemia caused a decrease in plasma oncotic pressure so that fluid shifts from intravascular to the network interstitium and edema. Consequent of Oncotic pressure drop due to plasma and plasma fluid shift occurs hypovolemia and to compnsate by increasing renal sodium and water retention. These compensatory mechanisms will improve intravascularvolume but will also excerbates occurence of edema increasingly hypoalbuminemia so continues. 
  • Overfill theory explains that the retention of sodium as a primary renal defect. retention of Sodium by the kidneys causes increased extracelluler fluid causing edema. Decrease of GFR (glomerulus filtrate rate) due to kidney damage would increase the occurence of sodium retention and edema. Both mechanism found together in patients with NS. 

Saturday, 4 September 2010

Theraphy HIV AIDS

Patient ask to Mbah Dukun: “Mbah! I have heard many people talk about AIDS, what is it?”
Mbah Dukun Bagong answers:

Acquired Immune Deficiency Syndrome (AIDS) is a collection of symptoms of disease or abbreviation for acquired imune deficiency syndrome caused by Human Immunodeficiency Virus (HIV).
HIV virus can be found in body fluids, especially in blood, semen, vaginal fluids, breast milk. The virus destroys the human immune system and may decrease or loss endurance, so easily affected by infectious diseases.


Patient ask to Mbah Dukun: “May i know it’s Symptoms?”
Mbah Dukun Bagong answers:

A person who has been infected with HIV is likely to pas through three stages of the disease. Not all individuals experience all of the stages.
1. Acute Retroviral Syndrome
Acute retroviral syndrome is a term used describe a group of symtoms that can resemble mononucleosis. Mononucleosis is like a flu-like infection, include fever, fatigue, muscle aches, loss of appetite, upset stomach, weight loss, skin rash, headache, and swollen lymp nodes. These symptoms occur in 50 to 70 % of all men who are HIV positive and in 45 to 90% of all women with the infection. The symptoms develop between one and six weeks after infection and last for two three weeks.
2. Latency Period
After entering a person’s lymph nodes, the virus becomes latent, latency meansthat the virus is still present in the body, but that there are no signs of infection. Therefore, a person may appear to be perfectly healthy even though blood tests show that the virus is present.
HIV infection has an unusually long latency period. It may for 10 years or more. During this period, the virus contnous to reproduce itself in the lymph nodes. As a result, certain abnormal conditions and symptoms may develop. These include the following:
• Persistent Generilzed Lymphadenopathy (PGL). As HIV continues to reroduce, it can cause swelling of the lymph nodus known as persistent generalized lymphadenopathy. Yhe nodus become larger, but are usually not sore or painful. The lymp nodus most comonly affected are those in the neck, jow, groin, and armpits. PGL affects between 50 to 70 % of all patients during latency.
• Constitutional symptoms. Many patients will develop low-grade fevers, fatigue, and general weakness. The virus may also cause a loss of appetite, a decerase in the body’s ability to absorb food, and an increased rate of metabolism, the process by which the body converts food to energy. These changes result in condition called wasting in which a person contunally loses weight and energy.
• Other Symptoms. At any time during the course of HIV infection, the virus may cause problems with organs and tissues throughout the body. A common problem is a yeast infection in the mouth known as thrusd. Ulcers and open sores can also develop in the mouth. The virus can also damage the digestive system. Patients may develop diarrhea or malnutrition as a result. The virus can also destroy cells in the lungs, kidneys, and nervous system. Damage to the nervous system leads to a general loss of strength, loss of reflexes, and feeling of numness or burning sesations in the feet or lower legs.
3. Late-stage AIDS
Late – Stage AIDS is the periode of HIV infection when the virus has become very active and has started to cause massive damage to the immune system. One sign of late-stage AIDS is a sharp decrease in the number of white blood cells known as CD4 lymphocytes. The patient also begins to have more frequent and more serious medical problems, such as infectious disease and cancers. The infections that occur are called opportunistic infections. That term means that foreign bodeis, such as bacteria, have taken advantage of the bodies weakened immune systems.
CD4 celll counts are an important indication of the course of the HIV infection. Doctors use these counts to determine how far the disease has developed and what treatments to use. About 10 percent of those individuals infected with HIV never reach this final stage of disease
AIDS Dementia complex usually occurs late in the progress of AIDS. It is marked by loss of reasoning ability, loss of memory, inability to concentrate, listlessness, and unsteadiness in walking. There are no treatments for this condition.
Patients in late-stage AIDS may develop inflammation of the muscles, especially in the hip area. They may experience pain in their joints similiar to those tha occur with arthritis. Thrush and ulcers in the mouth continue to occur during the late stages of AIDS. Another common condition of this stage is hairy leukoplakia of the tongue. Hairy leukoplakia is characterized by a white area on the tongue that may be flat or slightly raised.
Patients with late-stage AIDS may develop a form of cancer known as Kaposi’s sarcoma (KS). KS is a form of skin cancer characterized by reddish-purple blotches or patches. The disease may also occur in the digestive tract or lungs. KS is one of the most common causes of death in AIDS patients.

An adult (> 12 years) are considered AIDS when the HIV test shows positive by checking the appropriate strategy to get at least two major symptoms associated with a minor symptoms, and symptoms are not caused by other conditions unrelated to HIV infection:

Major symptoms:
1. Body weight decreased by more than 10% in one month
2. Chronic diarrhea lasting more than 1 month
3. Prolonged fever for more than a month
4. Impairment of consciousness and neurological disorders
5. Dementia / HIV encelopathy

Minor symptoms:

1. Cough settled more than 1 month
2. Generalized dermatitis
3. The existence of multisegmental herpes zooster and recurrent herpes zooster
4. Oropharyngeal candidiasis
5. Herpes simplex chronic progressive
6. Generalized lymphadenopathy
7. Recurring fungal infection in a woman's genitals
8. Cytomegalovirus retinitis

If not available HIV testing facilities so, if there is one of the signs / symptoms below, are reported as AIDS cases, although without laboratory examination:
1. Kaposi's sarcoma
2. Life-threatening pneumonia and recurrent


Patient ask to Mbah Dukun : "Mbah! Would you tell me how can HIV make AIDS?
Mbah Dukun Bagong answers :

HIV infection begins with viral attachment to cells that have CD4 molecules as the main receptor of T lymphocytes, monocytes, macrophages and other dendritic cells. Gp120 which is a surface receptor the virus will bind to CD4. then interact with gp120 will koreseptor embedded in cell membranes and exposed to the peptide of Gp41 and start going fusion between viral and cell membranes. After fusion, the internal virion core is released into the cytoplasm as a complex ribonucleoprotein. Have HIV reverse transcriptase enzyme that will convert viral RNA into DNA. This DNA will enter the host cell nucleus to mRNA to subsequently entered into a translation with structural proteins to form protein, mRNA will produce all viral proteins. Genomic RNA and viral proteins will form the virus particle, which will be attached to the outside of the cell. Through the process of budding at the cell surface, virions would be issued in a state of host cells mature.

Soon after HIV infection, virus-free or partially located within the CD4 T cells that are infected will reach the regional lymph glands and stimulates cellular and humoral immunity among other ways to recruit lymphocytes-lymphocytes. But this collection of lymphocyte-lymphocyte exactly cause CD4 + T cells will be more and more infected. Monocytes and lymphocytes are infected with the virus will spread throughout the body, HIV can also enter the brain via infected monocytes or through endothelial cells.

A few days after HIV infection will occur limfopenia due to a decrease in blood CD4 T. During this initial period of free virus and viral protein p24 can be detected in high levels in the blood and the number of HIV-infected CD4 + T cells increased. In this phase the virus replicates rapidly with little control of the immune response. Then after 2-4 weeks will be a dramatic increase in the total number of lymphocytes caused by the increase in the number of CD8 T cells (cytotoxic cells), which is part of an immune response against viruses. The existence of cytotoxic T cells is a sign of neutralizing antibody stimulation. Antibodies will be formed after the second or third week but sometimes up to several months. Decline in free virus and infected T-cell lysis caused by HIV-infected cells by CD8 T Activated CD8 cells in HIV-infected individuals also produce several soluble cytokines that can inhibit viral replication in CD4 T cells without causing cell lysis. After that the CD4 count will be returned to original levels prior to HIV infection. During the acute phase showed the majority of cases of acute viral symptoms in general, which include fever, lethargy, myalgia, and headache, and symptoms such as pharyngitis, lymphadenopathy and rash.

After the acute phase of infection, a state of asymptomatic for several years despite declining CD4 slowly. Amount of virus in blood and peripheral cells can be detected is low. The decrease in blood CD4 count average of 65 cells / ml every year. Showed damage to the immune system but not latent and can still be improved, especially in limphonodules. Decrease in the number of CD4 T cells during HIV infection may directly affect some reactions immunologic played by CD4 T cells as hypersensitivity slow type, lymphocyte transformation of young cells, and cytotoxic T lymphocyte cell activity. The emergence of HIV strains are more pathogenic and replicate faster on the host was a major factor in controlling the ability of the immune system. It also said that the number and function of cytotoxic T cells will decrease if the number of CD4 cells drops to <200/ml. Because these cells play a role in controlling virus-infected cells and clean the virus at an early stage so that the proposed loss of cell activity is having an impact in increasing the number of viruses. Another possibility is because there is no mutation of the viruses that are not recognized by cytotoxic T cells. The average time from HIV infection to AIDS is 8-10 years.

Patient ask to Mbah Dukun : "Can HIV transmitted?and how?
Mbah Dukun Bagong answers :

 Yes it can transmitted so everybody can infected. HIV spread by mucosal secret, breast feeding, sperm or blood. So it can transmitted by sexual contact, pregnancy and breast feeding, transfusion, accident needle among health care. Remember! Saliva isn't media to spread HIV because it needs 1 gallon saliva to transmit HIV. so don't worry to kiss your partner.

Patient ask to Mbah Dukun : "Can Patient with AIDS be cured?
Mbah Dukun Bagong answers :

The main approach is to improve the provision of antiretroviral immunity. Current ARV regimens recommended by WHO is a combination of three antiretroviral drugs. There are several regimens that are used, with the advantages and disadvantages of each. First-line drug combinations commonly used in Indonesia is a combination Zidovudin (ZDZ), Lamivudin (3TC), with Nevirapin (NVP). Three classes of antiretroviral drugs (ARVs) are as follows:

A. Reverse transcriprase nucleoside inhibitors (NRTIs)
NRTI class of zidovudine (AZT), didanosine (DDL), zalcitabine (ddC), stavudin (d4T), lamivudine (3TC), abacavir (ABC), tenofavir (TDF) and emtricitabine (FTC). NRTIs work by phosphorylation interseluler to form and join triphosphate inhibits further DNA chain elongation of RNA viruses. Each specific drug similar to nucleosides. CNS penetration in both NRTI and zidovudine in AIDS dementia looks useful. This class of drugs primarily eliminated through the kidneys and does not interact with other drugs through the cytochrome P-450.

B. Non-nucleoside reverse Trancriptase inhibitors (NNRTIs)
NNRTI class of nevirapine (NVP), delaviridine (DLV) and efavirenz (EFV). NNRTIs inhibit the enzyme reverse transcriptase through a bond with the enzyme activity. Drugs do not require intracellular activation and was not active against HIV-2. This drug may inhibit or induce cytochrome P-450 activity in the liver that interact with other drugs through the cytochrome P-450. These drugs require attention when combined with other antiretrovirals.

C. Protease Inhibitor (PI)
PI Group of saquinavir (SQV), indinavir (IDV), ritonavir (RTV), nelvinafir (NFV), amprenavir (APV), lopinafir / Kaletra (LPV / r) and atazanafir (ATV). This drug works to prevent the release of post-translational polypeptides into functional viral proteins. This medicine is given in combination 2NRTI can control viral replication in tissue and plasma as well as improve the immune system. PIs inhibit the cytochrome P-450, increases the potential for many drug interactions. Some drugs that interact with the protease inhibitor ritonavir is especially rifampin, midazolam, simvastatin, and certain antihistamines.

D.Stimulation of Blood Cell Production
Many AIDS patients have very low levels of white and red blood cells. People with low red blood cell counts often suffer from anemia a condition that causes weakness, exhaustion, and generally poor health. People with low white blood cell counts are unable to fight off infections. To protect AIDS patients against these conditions, drugs may be given to stimulate the production of both red and white blood cells. 

E. Alternative Treatment
For many years, doctors were able to offer AIDS patients little assistance in treating their disease. As a result, patients became very interested in alternative forms of treatment. Among those treatments were a variety of Chinese and Western herbal medicines and specialized diets designed to strengthen the immune system. Patients also tried nonphysical methods, such as visualization. In visualization, a person tries to imagine what a virus looks like and what kind of battle is going on in his or her body. By this method, the person believes that he or she may have some control over that battle.
Patients have tried a variety of pain control techniques as well. These have included hydrotherapy (the use of water baths and treatments), acupuncture (a Chinese therapy technique where fine needles puncture the body), meditation, and chiropractic (therapy that involves manipulation of the spine). 

F. Very Important
You have treatment all diseases in Patient HIV with AIDS example : Chronic Diarrhoea, Candidasis, Tuberculosis and other chronic diseases. They cause bad condition of patient

Don't ignore Patient with AIDS. Don't be afraid with them. Don't be afraid to infected HIV. They should be afraid about you. They are afraid if you infect them. If they infected disease from you, their life will be threatened. Because, their immune is so weak

1. http://www.faqs.org/health/Sick-V1/AIDS-Treatment.html

Thursday, 2 September 2010



Patient ask to Mbah Dukun : "Mbah! what is the meaning of Shock?
Mbah Dukun Bagong answers :

Shock is a clinical syndrome resulting from hemodynamic and metabolic disorder characterized by circulatory failure to maintain adequate perfusion to the organs - the vital organs of the body. Adequate tissue perfusion depends on three main factors, namely cardiac output, blood volume, and peripheral vasomotor tone. If one of these three determinants is chaotic and other factors can not compensate, there will be a shock. Originally arterial blood pressure may be normal to compensate for increased cardiac output .

If shock persists, decreased cardiac output and peripheral vasoconstriction increases. If hypotension and vasoconstriction settling continues, hypoperfusion cause lactic acidosis, and ileus Oliguria. If arterial pressure is low enough, there is brain dysfunction and cardiac muscle. Shock is resulting from the incident on serious hemostasis disorders.


Patient ask to Mbah Dukun : "How many types of Shock?
Mbah Dukun Bagong answers :

  1.  Hypovolemic shock

  2. Cardiogenic shock

  3. Septic Shock

  4. Anafilatic shock

Patient ask to Mbah Dukun : "Would you explain about them?
Mbah Dukun Bagong answers :

A. Hypovolemic Shock
Hypovolemic shock is caused by disturbance of the circulation system of blood volume in blood vessels is reduced.
 B. Cardiogenic shock
Cardiogenic shock is the inability of sufficient blood flow to the heart of the network to meet the basal metabolic disturbances due to cardiac pump function. The definition here includes a poor cardiac output and evidence of tissue hypoxia with the existence of adequate intravascular volume
C. Septic shock
Septic shock is a situation where there is a decrease in blood pressure (systolic blood pressure less than 90 mmHg or systolic blood pressure decreased more than 40 mmHg) accompanied by signs of circulatory failure, although it has been adequately fluid resuscitation or requiring vasopressor to maintain blood pressure and organ perfusion as a result of septicemia. Septic shock is caused by various types of microorganisms and a large number of mediators play a role in pathogenesis. Generally caused by gram-negative bacteria.
D. Anaphylactic Shock
A Widespread and very serious allergic reaction. Symptoms include dizziness, loss of consciousness, labored breathing, swelling of the tounge and breathing tubes, blueness of the skin, low blood pressure, heart failure, and death.


Patient ask to Mbah Dukun : "What are etiology which cause them?
Mbah Dukun Bagong answers :

 Etiology hypovolemic shock:
1. Bleeding
  • Trauma

  • Subcapsular liver hematoma

  • Aortic aneurysm rupture

  • Gastrointestinal Bleeding

2. Loss of plasma
  • Extensive burns

  • Pancreatitis

  • Desquamation skin

  • Dumping syndrome

3. Loss of extracellular fluid
  • Vomiting (vomitus)

  • Diarrhea

  • Very aggressive diuretic therapy

  • Diabetes insipidus

  • Adrenal insufficiency

Etiology Cardiogenic shock
1. Problem  ventricular ejection
- Acute Myocard Infarc
- Acute Myocarditis
- Mechanical complications
  • Acute mitral regurgitation due to dysfunction of muscle or ruptur papilaris 

  •  Interventriculorum septum ruptured

  • free wall ruptured

  • Left ventricle aneurysm

  • Severe aortic stenosis

  • Cardiomyopathy 

  •  Myocard Contusio

2. Filling Ventricular disruption
  • Heart Tamponade

  • Mitral stenosis

  • Miksoma the left atrium

  • Ball-valve thrombus in the atrium

  • Right Ventricle Infarct

Trigger factor
  1. Ischemic or myocard infarct

  2. Anemia:tachycardia or bradicardi

  3. Infection: endocarditis, myocarditis, or infection outside the heart

  4. Pulmonary embolism

  5. Advantages of liquids or salt

  6. Drugs myocard pressure such as  β blocker

  7. Other: Pregnancy, tirotoksikosis, anemia, stress (physical or emotional), acute hypertension. 

Etiology Septic Shock
infected by organisms

Etiology Anaphylactic Shock

Patient ask to Mbah Dukun : "How can i know about them?"
Mbah Dukun Bagong answers :

You have to know what their symptoms!

Symptoms of hypovolemic Shock
1. Mild ( lost < 20% blood): Cold extremities, Increased capillary filling time, Diaporesis, Veins collapse, Anxious
2: Moderate ( lost 20-40% blood): Symptoms of Mild grade plus Tachycardia, tachypneu, Oligouria, ortotastic Hypotensive
3. Severe (lost > 40% blood): Mild and moderate plus decreased consciousness, Unstable hemodynamic

Symtoms of Cardiogenic Shock
1. Systolic arterial pressure <80 mmHg (determined by measurement of intra-arterial)
2. Production of urine <20 ml / day or disturbance of mental status. Left ventricular filling pressure> 12 mmHg
3. Central venous pressure of more than 10 mmH2O considered removing the possibility of hypovolaemia
4. These situation is accompanied by: anxiety, cold sweat, cold extremity, tachycardia, and others.

Symptoms of Septic Shock
Clinical symptoms of septic shock can be divided into three groups:
1. Blood pressure. Clinically to produce adequate blood flow in coronary and cerebral circulation, blood pressure must be above a certain value,  mean arterial pressure (mean arterial pressure, MAP) 60 mmHg or systolic arterial pressure of 90 mmHg.
2. Signs of perfusion organ / tissue affected, :
  • Skin: Cold and cyanosis

  • Renal: decreased urine production and may lead to kidney failure.

  • Hearts: possible causes hyperbilirubinemia

  • Brain: chaos / confusion and when settling can cause coma.

  • Lungs: respiratory failure syndrome symptoms adults.

When related to multi organ can cause metabolic acidosis by lactic acid in the blood stacked. Elevated levels of blood lactate is a bad sign.
3. Signs / symptoms of a serious systemic infection that underlies the occurrence of septic shock, the clinical signs of severe sepsis (fever, chills, weakness, nausea, vomiting) and laboratory examination showed leukocytosis with a shift to the left, sometimes thrombocytopenia or neutropenia.

Symptoms of Anaphyllactic Shock
Clinical manifestations
1. Local reactions: urticaria and local edema

Mild: swollen eyes, nasal congestion, itching of the skin and mucosa, sneezing, usually appear two hours after exposure to allergens.
Moderate: symptoms are more severe than the above symptoms was found bronchospasm, laryngeal edema, nausea, vomiting, usually occurs within two hours after exposure to antigen.
Weight: occurs immediately after exposure to the allergen, the symptoms as a reaction to the above is just more severe bronchospasm, laryngeal edema, stridor, shortness of breath, cyanosis, cardiac arrest, dysphagia, abdominal pain, diarrhea, vomiting, seizures, hypotension, arrhythmias heart, shock and coma.  Deaths by laryngeal edema and cardiac arrhythmias.


Patient ask to Mbah Dukun : "So what I have to do if i find that cases?"
Mbah Dukun Bagong answers :

Treatment Hypovolemic Shock
a. Bleeding
  • Placing patients at higher leg position

  • Keeping the respiratory track, meet the need of oxygen.

  • Put two lines intravenous infusion using a large infusion needle or other means that allow such installation CVP intraarterial route .. Give crystalloid fluids such as RL or 0.9% NaCl or colloids such as dextran with a fast drip. Giving 2-4 liters in 20-30 minutes is expected to restore hemodynamic state.

  • Ongoing blood loss with a hemoglobin level ≤ 10 g / dL with a transfusion need of blood replacement. Give the PRC (packed red cells) with 1-2 units of FFP (fresh frozen plasma) for each four units of blood.

  • Failure of fluid resuscitation with crystalloid is almost always caused by massive bleeding, because it should be considered to take immediate action with surgical hemostasis.

  • In a state of prolonged hypovolemia, inotropes support with dopamine, vasopressin or dobutamin can be considered to have sufficient strength after ventricular blood volume be satisfied first. Giving naloxone bolus 30 Mg / kg in 3-5 min followed by 60 mcg / kg in 1 h in 5% dekstrose can help to increase MAP.

b. Loss of gastrointestinal fluid
  • Give 1-2 liters of NaCl 0.9% in 30-60 minutes, then continue with additional liquid while monitoring vital signs, CVP, and PCWP.

  • Check and correction of electrolyte abnormalities.

Treatment Cardiogenic Shock
  • Make sure the airway remains inadequate, if not conscious intubation should be performed.

  • Give oxygen 8-15 L / min by using a mask to maintain PO2 70-120 mmHg.

  • The pain caused by acute myocardial infarction which can aggravate existing shock must be overcome by administration of morphine.

  • Correction of hypoxia, electrolyte disturbances, and acid-base balance that occur.

  • If there is tachyaritmia must be overcome: Tachyaritmia supraventikular and atrial fibrillation can be treated with digitalis, Sinus bradikardi with heart frequency <50x/minute sulfas must be overcome by giving atropine.

  • Ensure adequate left ventricular filling pressure. The first priority in treating cardiogenic shock is the provision of adequate parenteral fluid using the basic guidelines or PAED PCWP or CVP. Type of liquid used depends on clinical circumstances, but it is recommended to use isotonic fluids. Intravenous fluid tolerance test is a simple way to determine whether giving intravenous fluids useful in the management of cardiogenic shock. How:

  1. If PCWP or PAED <15 mmHg, it is difficult to say the pump failure, so that intravascular fluid administration should be improved with the initial test vol; ume of 100 ml through within 5 minutes. If no response is given an additional 200 ml of fluid within 10 minutes.

  2. When next PCWP remained stable, followed by giving intravenous fluids 500-1000ml/jam.

  3. If at the initial examination of PCWP 15-18 mmHg then given 100 ml of intravenous fluids within 10 minutes.

  4. If PCWP> 20 mmHg may not dilakikan tolerance test and intravenous fluids started treatment with vasodilators.

  5. If PCWP <5 mmHg infus fluids can be given even if there is pulmonary edema. But if there was an increase of lung congestion and the clinical deterioration of fluid infusion was stopped.

  • Patients with inadequate tissue perfusion and adequate intravascular volume have sought possible cardiac tamponade.

  • Handling of pump failure were divided based on hemodynamic subset

  1. TDS> 100 mmHg: nitroglycerin 10-20 mcg / min iv

  2. TDS 70-100 mm Hg and signs / symptoms of shock (-): dobutamin 20-20 mcg / kg / min

  3. TDS 70-100 mm Hg and signs / symptoms of shock (): dopamine 5-15 mcg / kg / min iv

  4. TDS <70 mm Hg and signs symptoms of shock (-); norepinephrine from 0.5 to 30 mcg / min iv

  • The use of thrombolytic 

  • PTCA, cabs, a heart transplant.

Treatment of Septic Shock
  • Broad-spectrum antibiotic. Recent research states that the treatment of early stages of septic shock is to use a third-generation cephalosporins.

  • Fluid therapy to improve oxygen consumption using crystalloid fluids (NaCl, RL) or koloid. Plasma ekspander and albumin is required when Ht is less than 30 vol% and serum albumin less than

  • Dopamine is given when it reached the target fluid therapy, namely MAP 60 mmHg or systolic pressure is 90-110 mmHg.Dosis 2-5μg/kgBB/menit early. If this dose failed to increase MAP according to the target, then the dose can be increased until 20μ/kgBB/menit. If still fail to be restored on 2-5μg/kgBB/minute dose dopamine, but combined with levarterenol (norepinephrine). When a combination of both vasoconstrictor still fails, it means very bad prognosis.

  • Corticosteroids. This treatment is still controversial. Based on several studies, dexamethasone is a corticosteroid which has the highest therapeutic index.

  • Immunoglobulin

  • Correcting metabolic acidosis with sodium bicarbonate until pH normal and improve the provision of electrolyte disturbances electrolyte

  • Correcting hypoxia by giving oxygen equipment

  • Correcting hypoglycemia or hyperglycemia

  • Fix azotemia and Oliguria

  • If there is widespread intravascular coagulation, treatment is basically enough to treat the disease course.

  • During treatment, hemodynamic monitoring,:

  1. Installation of the ECG monitor

  2. Installation of arterial catheter / vein to measure the fluid balance.

  3. Urinary catheterization to measure the amount of urine.

  4. Fitting cuff to measure blood pressure.

  5. Examination of blood gas analysis, electrolytes, lactic acid, blood coagulation tests to assess the metabolic function.

  6. Serial examination of kidney and liver function

  7. Installation of intravenous fluids, O2, and if necessary aggressive oxygen can be mounted Peep .

Treatment of Anaphylactic Shock
  • Epinephrine 1:1000 0.01 ml / kg until reaching a maximum of 0.3 ml subcutaneously and can be given every 15-20 minutes 3-4 x samp0ai if symptoms get worse or from the initial conditions of severe illness, can be given by IM injection and even the occasional dose of epinephrine may be increased up to 0.5 ml throughout the patient did not suffer from heart problems.

  • When originators allergens such as infiltration injections imunotherapy, penicillin, or insect sting, immediately given an injection of epinephrine infiltration 1: 1000 0.1 to 0.3 ml in the former site of injection to reduce the absorption of allergens earlier. If possible install tourniquet proximal from the injection site and loosen every 10 minutes and then off when the situation was under control.

  • Next, two important things that must be considered in providing therapy in patients with anaphylactic shock is

  1. The respiratory system is smooth, so good walking oxygenation
    - Maintaining an adequate airway. When laryngs or spasm of the bronchi, do tracheostomy or puncture the membrane of cricothyroid.
    - Giving oxygen 4-6 l / min is very important both in the respiratory and cardiovascular disorders.
    - Bronchodilators if there is airway obstruction bottom. Solution can be given beta-2 agonist salbutamol or other-0 .25 cc, 5 cc in 2-4 ml of 0.9% Nacl provided through nebulisation or aminophylline 5-6 mg / kg diluted in 20 cc of dextrose 5% or NaCl 0, 9% and given slowly about 15 minutes.

  2. Cardiovascular system must function well so that adequate tissue perfusion
    - Symptoms of hypotension or shock is not succeed by giving epinephrine indicate a hipovolemi. These patients require intravenous fluids rapidly with crystalloid or colloid fluids. It is advisable to give colloidal fluids from 0.5 to 1 liter, the rest of crystalloid.
    - Oxygen is absolutely necessary and the provision of sodium bicarbonate in case of metabolic acidosis.
    - Sometimes it is necessary CVP
    - When blood pressure is still not resolved with fluid administration, the experts agree to give vasopressor via intravenous infusion. By way of dissolving 1 ml epinephrine 1: 1000 in 250 dextrose (concentration of 4 mg / ml) is given by infusion 1-4 mg / min or 15-60 microdrip / min, if necessary, the dose may be increased to a maximum of 10 mg / ml.
    - When the means of the blood vessels are not available, the state of severe anaphylaxis, American Heart Assosiacion recommends granting an endotracheal epinephrine dose given 10 ml of epinephrine 1:10.000 through long needle or a catheter through the endotracheal tube. The above measures are followed by respiratory hyperventilation to ensure rapid drug absorption.


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