Early Initiation of Steroid-sparing Drugs in Idiopathic Pulmonary Hemosiderosis

Idiopathic pulmonary hemosiderosis is conventionally treated withsteroids, prolonged usage of which maybe deleterious anddisease often recurs on tapering. We initiated hydroxy-chloroquine and azathioprine early in treatment along withsteroids in seven children with idiopathic pulmonaryhemosiderosis, and observed that early introduction of secondline immunosuppressants helped in reducing disease flare andsteroid toxicity without serious adverse effects.

Dengue Associated Hemophagocytic Lymphohistiocytosis: A Case Series.

Hemophagocytic lymphohistiocytosis is a rare complication of dengue. We present 8 cases of dengue associated hemophagocytic lymphohistiocytosis diagnosed in our hospital during the dengue outbreak of 2012. All the cases were treated with a short (4 weeks) course of steroids along with supportive measures, and showed an excellent response.

Macrophage Activation Syndrome in Kawasaki Disease.

Background: Kawasaki disease is an acute febrile vasculitis of childhood. Macrophage activation syndrome is a rare life threatening complication. Case characteristics: 4-yearold boy with Kawasaki Disease treated with intravenous immunoglobulins. Observation: He developed encephalopathy, hepatosplenomegaly and pancytopenia. Blood investigations and bone marrow aspiration suggested macrophage activation syndrome. Outcome: Good response to pulse methylprednisolone (30 mg/kg/d) for 5 days. Message: Macrophage activation syndrome may complicate Kawasaki disease.

Infantile Systemic Hyalinosis.

Infantile systemic hyalinosis is a rare disorder characterized by widespread deposition of hyaline. They usually present with skin lesions, joint contractures, and intractable diarrhea. We report a 2 year 4 month old boy with growth retardation, typical facial appearance, gingival enlargement, generalized stiff skin, joint contractures, and intermittent diarrhea. Skin biopsy revealed deposition of hyaline.

Benign angiopathy of central nervous system.

Benign angiopathy of central nervous system (BACNS) is rare in children. We report a seven-year-old boy presenting with sudden severe headache and progressive external ophthalmoplegia. Magnetic resonance angiography (MRA) showed diffuse segmental narrowing of major cerebral arteries. Following a course of glucocorticoid, there was complete resolution of vascular lesions and follow-up MRA did not show any evidence of new lesions.

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.

Congenital tuberculosis: late manifestation of the maternal infection.

Tuberculosis in pregnancy though not uncommon, congenital tuberculosis continues to be a rare entity. A case of congenital tuberculosis where the mother manifested the disease 3 months after it was diagnosed in the newborn is reported considering its rarity.