Biomedical subjects
Publications and source records attributed to R K Vashishta.
We describe a case of plexiform variant of vascular transformation of lymph nodes sinuses in association with myelodysplastic syndrome. The patient had repeated bacterial infections and terminal fungal infection and dies after a protracted illness of seven years.
BACKGROUND: Congenital hepatic fibrosis (CHF) is an uncommon cause of portal hypertension in children. So far, there is no report of this from the subcontinent. We have studied the clinical spectrum of CHF in North Indian children. METHODS: Fifteen children were diagnosed with CHF on the basis of their liver histology over a period of 6.5 years. Their clinical details were recorded. Oesophagogastroduodenoscopy and abdominal ultrasonography were performed in all cases. All siblings were examined clinically; and ultrasonography, endoscopy and liver biopsy were performed if there was firm hepatomegaly. Children with variceal bleeding were managed by endoscopic sclerotherapy. The median age of these children was 8 years with a male to female ratio of 1.5:1. RESULTS: Only one sibling (of 33) was diagnosed as having CHF. The predominant presentations were variceal bleeding in six, abdominal distension in seven and incidental detection of organomegaly in two. Hepatomegaly was present in all patients and splenomegaly in all but one. Liver function and renal function tests were normal in all children, except for a raised serum alkaline phosphatase in six. Two children had associated renal cysts, two had choledochal cysts, one each had Caroli's disease and biliary atresia and two children had portal vein thrombosis. Variceal obliteration was achieved in five children after an average 4.8 sclerotherapy sessions and one required a mesocaval shunt. On follow up (median 41 months, range 1-80 months) all are doing well. CONCLUSIONS: Congenital hepatic fibrosis is mainly sporadic in India and associated renal lesions are uncommon. Endoscopic sclerotherapy is effective in controlling variceal bleed and the prognosis is universally good in the absence of renal diseases.
A malformation is a morphological defect of an organ, or a larger region of the body resulting from an intrinsically abnormal developmental process. In this analysis of 1421 neonatal autopsies performed between 1984 and 1993, 243 (17.1%) cases showed malformations. The data was analysed to find external cues to internal malformations. Twenty three (85%) of the 27 neonates with various facial abnormalities had associated internal malformations in the form of cardiac (n = 11; 40%), renal (n = 7; 25%), or gastrointestinal (n = 3; 11%) abnormalities and diaphragmatic hernia (n = 2). Sixty seven neonates had neural abnormalities. These were associated with cardiac (n = 7; 10%), renal (n = 12; 18%) or gastrointestinal (n = 7; 10%) abnormalities; 43 cases, however, did not have any associated malformations. Renal malformations (n = 66) had highest association with skeletal abnormalities (n = 9; 22%). As many as 45(76%) of the 59 cases with cardiac abnormalities had no external anomalies. Five cases of hypoplastic lungs were seen, all associated with external malformations.
A 48 year old woman presented with obstructive jaundice 10 years back. Upper gastrointestinal endoscopy revealed a growth infiltrating the ampulla of Vater, which was confirmed to be adenocarcinoma on cytology. At laparotomy, a large nodular growth was seen in the head of pancreas. Surgical resection could not be done because of encasement of superior mesenteric vessels, hence a cholecystojejunostomy was performed. The patient remained asymptomatic for 9 years, when she developed cholangitis. Duodenoscopy at this stage revealed an ulcerated growth at the ampulla and biopsy from the growth confirmed a well differentiated adenocarcinoma. A straight flap 10 F stent was placed in the common bile duct. Thereafter the patient has remained asymptomatic for more than a year.
Primary amebic meningoencephalitis and granulomatous amebic meningoencephalitis are central nervous system infections caused by free-living amebae. We describe the neuroimaging findings in 5 such cases on CT and MR imaging. A spectrum of findings was seen in the form of multifocal parenchymal lesions, pseudotumoral lesions, meningeal exudates, hemorrhagic infarcts, and necrosis in the brain. Familiarity with the imaging findings is important for the diagnosis and management of this nearly universally fatal disease.