Triterpene Quinone-Methides from Gymnosporia montana.

Iguesterin, pristimerin, tingenone, beta-amyrin, beta-sitosterol and maytenonic acid have been isolated from the light petroleum extract of the stem bark and iguesterin, pristimerin, tingenone, beta-amyrin and beta-sitosterol from the root bark of GYMNOSPORIA MONTANA.     

Shaken baby syndrome masquerading as apparent life threatening event

Disseminated cryptococcosis is a rare and often fatal disease in children. The majority of cases usually occur in individuals with defective cell-mediated immunity, most commonly due to HIV infection. The authors here in report an 8-year-old girl from Nepal who presented with fever, cough, headache, lymphadenopathy, hepatosplenomegaly and cutaneous lesions. Lymph node biopsy revealed multiple granulomas composed of histiocytes and epitheliold cells along with numerous yeast forms of cryptococcus. Cultures of CSF, sputum and urine yielded cryptococcus neoformans. Surprisingly,the immune function in terms of T-cell number, CD4 : CD8 ratio, serum immunoglobulins and HIV serology was normal. After the diagnosis of disseminated cryptococcosis was established, the patient was treated with 5-fluorocytosine (100 mg/kg/day) for initial two weeks and amphotericin B (1 mg/kg/day) for 13 weeks. Patient responded well to the treatment with disappearance of presenting symptoms, cutaneous lesions, and lymphadenopathy, though she still had hepatosplenomegaly, which also decreased. Unfortunately, she developed loss of vision in 10th week of therapy. The patient was discharged on oral fluconazole (6 mg/kg/day) and no recurrence was found during the follow-up period of more than 9 months. This is the first case of disseminated cryptococcosis with no detectable immune deficit, from India.     

Shaken baby syndrome masquerading as apparent life threatening event

A variety of diseases and disorders can present as an acute life-threatening event among which shaken baby syndrome has been recently recognized. A high index of suspicion along with an ophthalmologic evaluation and cranial imaging helps to identify this form of child abuse, which needs multidisciplinary management.     

Juvenile hyaline fibromatosis in siblings

Juvenile hyaline fibromatosis (JHF) is a rare, autosomal recessively inherited disorder. We report two siblings with multiple large tumors on the scalp, translucent papules on the nape of the neck, hypertrophic gingiva, and severe flexural contractures of large joints. The histopathology from the skin lesions showed features characteristic of juvenile hyaline fibromatosis. The cases are being reported on account of the extreme rarity of the condition.     

Successful treatment of pure red cell aplasia associated with systemic lupus erythematosus with oral danazol and steroid

We describe a middle-aged Chinese systemic lupus erythematosus (SLE) patient developing steroid refractory and transfusion dependent red cell aplasia. Oral danazol 200 mg twice per day was started together with low-dose prednisolone therapy. There was no further recurrence of anemia 1 month after this combined therapy.     

Acute iron poisoning: clinical picture, intensive care needs and outcome

In this retrospective study, we examined the prevalence of acute iron poisoning among children attending Pediatric Emergency service of a teaching hospital, and studied their clinical profile, treatment and outcome to define intensive care needs. During the 5 years' study period of 27125 patient visits to Pediatric Emergency, 337 (1.2%) were for accidental poisoning. Of these 21(7%) patients had iron poisoning; 18 were transferred to PICU. Three patients were asymptomatic, others had vomiting (n =15, 83%), diarrhoea (n =13, 72%), malena (n = 8, 44%), and hemetemesis (n=6, 33%) generally within 6 hours of ingestion. Nine progressed to shock and/or impaired consciousness; two had acute liver failure. Dose of ingested iron and clinical signs were most useful guide to iron toxicity and management decisions; serum iron did not help. Gastric lavage yielded fragments of iron tablets in 10 patients. On desferrioxamine infusion Vin-rose colour urine was not seen in 31% even in presence of high serum iron. Shock responded to normal saline (33 +/- 15 mL/kg) and dopamine (10 +/- 4 microg/kg/min) within 4-24 hours in 7 of 9 patients. Presence of shock or acute liver failure with coagulopathy and/or severe acidosis predicted all the four deaths. Desferrioxamine infusion and supportive care of shock was the mainstay.