Hemolytic Uremic Syndrome.

Context: Hemolytic uremic syndrome (HUS) is a severe acute disease, sometimes with long-term sequelae. The diarrhoea-unrelated forms are particularly associated with a poor prognosis. The aim of this paper is to review current evidence regarding etiology and management, and explore methods by which the outcome may be optimized. Evidence acquisition: An internet search of Medline, Medscape, MDConsult and Cochrane databases for publications related to HUS from 1998 onwards was performed. A review of articles pertaining to etiopathogenesis and management was undertaken. Results: HUS is now classified according to cause. New assays and gene studies allow exact diagnosis of many of the atypical forms. Post-exposure prevention of diarrhoea associated HUS with vaccines and toxin-binding agents, remains in the experimental stages. Specific directed therapies aimed at replacing deficient factors can improve the outcome of atypical HUS. Conclusions: Supportive care remains the cornerstone of management of HUS. The infection-unrelated forms should in addition be treated rapidly with plasma therapy. Efforts should be made to make an exact etiological diagnosis in all patients, as long-term treatment and prognosis is affected. Prevention of diarrhea-associated HUS by improving sanitation and proper attention to food hygiene is a practical goal.

Reactive haemophagocytic lymphohistiocytosis.

We present a series of five cases diagnosed and treated as reactive haemophagocytic lymphohistiocytosis (HLH) in three tertiary referral centers of Kolkata, within a time frame of 3 months. The initial presentations were very variable, the most prominent clinical feature being - acute renal failure in the first patient, convulsions in the second, encephalopathy the third, marked cervical lymphadenopathy in the fourth and polyserositis in the fifth. All had a history of prolonged fever preceding admission and hepatosplenomegaly on examination. Investigations revealed multi-organ involvement with pancytopenia;- haemophagocytosis was eventually diagnosed by bone marrow examination. These cases highlight the diagnostic challenge posed by infection associated haemophagocytosis and the need for maintaining a high index of suspicion to promptly diagnose and treat this potentially life threatening condition.

Management of steroid sensitive nephrotic syndrome: revised guidelines.

JUSTIFICATION: In 2001, the Indian Pediatric Nephrology Group formulated guidelines for management of patients with steroid sensitive nephrotic syndrome. In view of emerging scientific evidence, it was felt necessary to review the existing recommendations. PROCESS: Following a preliminary meeting in March 2007, a draft statement was prepared and circulated among pediatric nephrologists in the country to arrive at a consensus on the evaluation and management of these patients. OBJECTIVES: To revise and formulate recommendations for management of steroid sensitive nephrotic syndrome. RECOMMENDATIONS: The need for adequate cortico-steroid therapy at the initial episode is emphasized. Guidelines regarding the initial evaluation, indications for renal biopsy and referral to a pediatric nephrologist are updated. It is proposed that patients with frequently relapsing nephrotic syndrome should, at the first instance, be treated with long-term, alternate-day prednisolone. The indications for use of alternative immunosuppressive agents, including levamisole, cyclophosphamide, mycophenolate mofetil and cyclosporin are outlined. The principles of dietary therapy, management of edema, and prevention and management of complications related to nephrotic syndrome are described. These guidelines, formulated on basis of current best practice, are aimed to familiarize physicians regarding management of children with steroid sensitive nephrotic syndrome.

Wasp stings with multisystem dysfunction.

The insect order of Hymenoptera includes bees, hornets, wasps and fire-ants. Their stings are not usually life threatening, causing mainly local reactions and rarely anaphylaxis. This is a report of an 18 month old child who survived after an unusually severe envenomation, resulting in encephalopathy, hypertension, coagulopathy, intra-vascular hemolysis, hepatic and renal failure, following multiple wasp stings.

Steroid resistant nephrotic syndrome.

Steroid Resistant-Nephrotic Syndrome (NS) is a chronic, progressive disorder affecting upto 10% of all children with NS. It causes morbidity and mortality due to persistent edema, hypertension, hyperlipidemia, thrombosis and infection. Progression to renal failure was thought to be inevitable in survivors. Recent insights into the pathogenesis of the disease has identified several responsible genes and proteins. Studies have shown that long term aggressive therapy with combinations of steroids, alkylating agents and cyclosporine, cause complete or partial remission in 20-80% patients. The use of nonspecific renal protective agents such as the angiotensin converting enzyme inhibitors, angiotensin 2 receptor blockers, and anti-lipid agents retard disease progression. Although these are indications of significant improvement in outcome, further multicentre controlled studies are required to determine the optimum drugs and regimens to be used.