 |  H.S. - Star1, DocClotaire n Justice
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| Author | 7 Posts |
doyoudig
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1. Treatment of Acute Episodes in Herediatary Spherocytosis?
2. When do we Give Folic Acid
3. """"""""""""'"Trans fusions
2. When is Splenectomy indicated in H.S.?
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| star1
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The info Given in kaplan note is not precise , Below is the extraction from emedicine site , read them all and you will get your all answer. Great source, if anyone has more info them add to this.
thanks n goodluck
Medical Care: For practical purposes, the treatment of HS involves presplenectomy care, splenectomy, and postsplenectomy complications.
- Neonates with severe hyperbilirubinemia caused by HS are at risk for kernicterus, and these infants should be treated with phototherapy and/or exchange transfusion as clinically indicated.
- Aplastic crises occasionally can cause the hemoglobin level to fall because of ongoing destruction of spherocytes that is not balanced by new RBC production. Red cell transfusions often are necessary.
- Folic acid is required to sustain erythropoiesis. Patients with HS are instructed to take supplementary folic acid (1 mg/d) for life in order to prevent a megaloblastic crisis. During the first 6 years of life, if patients have compensated anemia, are growing well, and can keep up with their peers in most activities, limiting folic acid supplementation to 1 mg/d is prudent.
- Subsequently, depending on the severity of the disease, splenectomy usually is curative, but not always. Some splenectomies fail because of accessory spleen, accidental autotransplantation of splenic tissue into the peritoneum during surgery, another hemolytic disorder, or splenosis. Failure to observe Howell-Jolly bodies may indicate the presence of functional splenic activity.
- Indications for splenectomy are not always clear.
- Little doubt exists that patients with more severe anemia and symptoms and complications of HS should undergo splenectomy. Similarly, splenectomy can be deferred safely in patients with mild uncomplicated HS (hemoglobin level >11 g/dL).
- No good studies have been performed that provide a basis for clinical judgments in patients with moderate asymptomatic HS (hemoglobin level 8-11 g/dL).
- Splenectomy usually is curative, except in the unusual autosomal recessive variant of HS.
- Red cell survival is improved significantly but is not absolutely normal. The MCV usually falls, but the MCHC does not change significantly. Postsplenectomy blood changes include an increased hemoglobin level, decreased reticulocyte count, and the appearance of Howell-Jolly inclusion bodies and target cells. Leukocytosis and thrombocytosis are expected corollaries of splenectomy.
- Fatal sepsis caused by capsulated organisms (eg, Streptococcus pneumoniae, Haemophilus influenzae) is a recognized complication in children who have had a splenectomy. The estimated rate of mortality from sepsis is approximately 200 times greater than that expected in the general population. Although most septic episodes have been observed in children whose spleens were removed in the first years of life, older children and adults also are susceptible.
- A simultaneous cholecystectomy in patients with bilirubin stones may eliminate future complications and the need for a second operative procedure.
- Bilirubin gallstones are found in approximately 50% of patients with HS and frequently are present in patients with very mild disease. Therefore, periodic ultrasonic evaluation of the gallbladder should be performed. If surveillance ultrasound examination findings reveal gallstones, performing a prophylactic laparoscopic cholecystectomy seems reasonable. This procedure helps prevent significant biliary tract disease and, in some patients with mild HS, helps avoid the need for splenectomy.
- Children who are candidates for splenectomy include those with severe HS requiring red cell transfusions and those with moderate HS who manifest growth failure or other signs and symptoms of anemia. Splenectomy for children with HS should be performed when the child is older than 6 years.
- Another interesting approach has been the use of partial splenectomy to retain splenic immunologic function while at the same time reducing the rate of hemolysis.
Surgical Care: Generally, the treatment of HS involves presplenectomy care, splenectomy, and postsplenectomy complications. See Medical Care. A trend toward partial splenectomies exists in the pediatric population, which appears to control hemolysis and, at the same time, preserve splenic function. Consultations: - Those patients with recurring episodes of severe hemolysis should be evaluated by a surgeon for possible splenectomy because splenectomy provides the greatest chance for control and possible cure of their disease.
- Another reason for consulting a surgeon is for the complications of gallstone formation and for the prevention of formation in those patients with continued hemolysis.
- If the diagnosis is unclear after routine testing or the patient has severe episodes of hemolysis, consultation with a hematologist is warranted because the patient may have a variant of HS or more than one hemolytic disease.
- A hematologist also may provide treatment advice directed at iron over load issues for patients who have an extensive transfusion history or for those who have been receiving prolonged oral iron supplementation.
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| doc_clotaire
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1.- Folic acid is required to sustain erythropoiesis. Patients with HS are instructed to take supplementary folic acid (1 mg/d) for life in order to prevent a megaloblastic crisis.
2.- Some litterature say we give transfusion in APLASTIC ANEMIA due to Parvovirus B19 infection (as we previously discussed that before , PV B19 not only can give AA in SS anemia but it is also found in HS ) , Recommendation for transfusion is adviced usually in severe symptomatic anemia with hemoglobin less that 8 g /dl
3.- Indications for splenectomy are not always clear .
- Little doubt exists that patients with more severe anemia and symptoms and complications of HS should undergo splenectomy. Similarly, splenectomy can be deferred safely in patients with mild uncomplicated HS (hemoglobin level >11 g/dL).
- No good studies have been performed that provide a basis for clinical judgments in patients with moderate asymptomatic HS (hemoglobin level 8-11 g/dL).
ANY MORE INPUT IS WELCOME 
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| doc_clotaire
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I did not see Star 1 Post !
Well , anyway it is basically from the same info and source ( emedicine )
Thanks for answering first start 1
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| star1
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 Welcome!!!!
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| doyoudig
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thx so much for ur time
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| Justice
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No need in my opinion since I was gonna say/write the same...
I also like the eMedicine very much... Very clear, well-formatted and regularly-updated articles... Recommend to everyone... BUT, we are still uncertain about many issues like priapism in SS (I am sure I am right since I checked out this issue even with the head of the pediatric hem-onco dept in Univ of Wisconsin, and they have right now a Pt with priapism that gets IV fluids and opiates+oxygen, i.e. no transfusion)... Wish we had a single source of info we could study from...
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