Urinary sediment in storage diseases: differential diagnosis of Nieman-Pick disease by cytologic means.

Renal involvement in lipid storage diseases is well recognized. Electron microscopy or chemical analysis of urinary sediment has been used for the diagnosis of these diseases. Urine cytology, supplemented by cytochemistry, polarization, and autofluorescence, helped us in the diagnosis of Niemann-Pick disease in an infant. The unique findings are described, and the differential diagnosis of storage cells by cytochemical stains is discussed.

Disseminated strongyloidiasis: report of two fatal cases.

Strongyloides infection is usually asymptomatic but disseminated infection is a cause of morbidity and mortality in immunodeficient patients. Two fatal cases of strongyloidiasis are reported here, with rare manifestations of gastric ulcers with hematemesis and acute renal failure respectively.

Atypical mesoblastic nephroma. A potentially malignant variant of congenital mesoblastic nephroma. Review of literature & report of four cases.

Atypical Mesoblastic Nephroma (AMN) is a rare infantile renal tumour which may behave aggressively, in contrast to conventional Mesoblastic nephroma. During a 10 year period we encountered four cases of AMN. The mean age of presentation was three months. Size varied from 9-18 cms. All were soft and fleshy with a weight varying from 290-1200 gms. Three cases revealed cystic changes. Hemorrhage and necrosis was seen in all four cases. One case had associated Nephroblastomatosis Complex. The capsule and ureter were involved in one case each. Two of the four patients died of distant metastasis. Thus a close periodic follow up by non-invasive imaging techniques is indicated for early detection of recurrence and or metastasis in cases of AMN.

Di-George's syndrome with multiple infections (an autopsy report).

Di-George's syndrome is a rare condition of congenital immunodeficiency. An infant presented as hypocalcemic tetany with multiple infections. Autopsy confirmed the hypoplasia of thymus and thyroid associated with other anomalies. A brief of the relevant literature is presented.

Human pulmonary dirofilariasis in India: a case report.

Dirofilaria immitis is a common parasite of the canine cardiovascular system. In dogs, it often produces pulmonary lesions and rarely dilatation of portal vessels. Most of the human cases reported showed pulmonary lesions. We describe here a child manifesting as portal cavernoma with pulmonary dirofilariasis detected at autopsy. This is the first case of pulmonary dirofilariasis reported from India.

Congenital leukemia--organ involvement in six autopsy cases.

Six cases of congenital leukemia were encountered in pediatric autopsies carried out over a period of 7 years. The postmortem findings of these cases were analysed and presented along with antemortem peripheral and bone marrow smear. All the cases were diagnosed as acute myeloid leukemia. Gross changes were observed in lungs, liver, spleen and kidneys. Histological abnormalities were detected in these organs as well as the heart, pancreas and intestine. Lymph node follicles were well preserved in all. The thymus showed a normal lobular pattern with interstitial infiltrate. Bone marrow showed myeloid blast cells with depletion of the erythroid and megakaryocytic cells.

Histiocytosis-X: role of histology and S-100 protein in grading the severity of disease.

Histiocytosis-X encompasses a wide clinical spectrum from a benign localised lesion to acute generalised disease with malignant behaviour. We have reviewed material from our 18 retrospective cases comparing histological features with clinical data. Degree of positivity for S-100 protein was studied by immunoperoxidase method. Our results indicate that diagnosis of histiocytosis-X necessitates histological confirmation but grading of histological criteria and S-100 positivity do not allow prediction of severity and extent of the disease.

Neurogenic tumor of the urinary bladder (a case report).

Neurogenic tumors of urinary bladder are rare. A ganglionure fibroma arising from the trigons of urinary bladder in two year old boy presenting with hematuria, is described here.

Malignant melanoma of the nasal cavity (a case report).

A case of malignant melanoma of the nasal cavity is reported. The rarity of its occurrence warrants its mention.

Fibromatosis of thyroid gland (a case report).

Fibromatosis of thyroid gland is a rare condition encountered in surgical practice. Clinically presenting as goitre, it is treated with near total thyroidectomy with good results. We had one such case under our care at the K.E.M. Hospital, Bombay. Rarity of this condition prompted us to publish the data, the literature is briefly reviewed.