Glomerular basement membrane thickness in normal adults and its application to the diagnosis of thin basement membrane disease: an Indian study.

Microscopic hematuria of non-urologic origin warrants ultrastructural study of renal biopsy. Thinning and variations in the texture of glomerular basement membrane (GBM) are difficult to be recognized under light microscope; transmission electron microscope (TEM) therefore plays a vital role in identifying such changes. Ultrastructural morphometry is a valuable diagnostic aid when GBM is suspected of being abnormally thin. In an effort to determine the normal GBM thickness (GBMT) in Indian adults and to determine the cutoff value of GBMT for a diagnosis of thin basement membrane disease (TBMD), we determined GBM thickness in 25 normal adults. Postmortem biopsies of 25 normal adults (16 males and 9 females) aging between 18-58 years were included in the study. GBM thickness was determined through ultrastructural morphometry on accurately enlarged electron micrographs as harmonic mean of 50 orthogonal intercepts across the GBM in each case. Study revealed a mean GBM thickness of 321 nm with a standard deviation (SD) of 28 nm. Mean-2SD (321-56), that is 265 nm, was fixed as cutoff value of GBMT for the diagnosis of TBMD. A systematic split study of control subjects revealed thicker GBM (329+/-38 nm) in higher age group (35-60 years) as compared to GBMT (316+/-21 nm) in lower age group (18-30 years). Males in higher age group also revealed thicker GBM (males: 343+/-39 nm versus females: 300+/-12 nm). Ten patients with non-urologic hematuria and having GBMT<265 nm were diagnosed as cases of TBMD. Patients with TBMD revealed significantly attenuated GBM as compared to age and sex matched controls (214 +/- 40 nm versus 311 +/- 17 nm; p<0.0005). No overlap was observed in the distribution of GBMT in patients of TBMD and age and sex matched controls. Ultrastructural morphometry is the ultimate and appropriate method for diagnosing TBMD.

Acute intermittent porphyria: an unusual cause of malignant hypertension.

Hypertension is a rare complication of acute intermittent porphyria (AIP) and is related to the sympathetic over-activity seen in this condition. We report a patient with AIP with malignant hypertension that recurred with a subsequent episode. Mechanisms of hypertension and renal damage are discussed.

Visceral leishmaniasis: a rare cause of post-transplant fever and pancytopenia.

Despite the endemic distribution of visceral leishmaniasis in certain parts of our country, there are only a few reports of this infection in renal transplant recipients. We report one renal transplant recipient from non-endemic area with visceral leishmaniasis and graft dysfunction that responded to treatment with stibogluconate. The infection should be considered in the differential diagnosis of a febrile transplant recipient with pancytopenia and allograft dysfunction.

Changing scenario of cryptococcosis in a tertiary care hospital in north India.

BACKGROUND & OBJECTIVES: With the increase in the number of patients of AIDS, the incidence of cryptococcosis is on the rise in India. It was therefore considered important to evaluate the predisposing factors, laboratory investigations and outcome of patients with cryptococcosis in this changed scenario. METHODS: We assessed 58 patients with cryptococcosis retrospectively over a five year period (January 1995-December 1999) at the Nehru Hospital, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh. RESULTS: The annual incidence of cryptococcosis in PGIMER, Chandigarh has increased about 15 fold from 1970-1982 (pre AIDS era) to 1995-1999 (present series). Of the 47 patients studied for predisposing factors, 36 patients were identified with predisposing factors, HIV infection (57.4%) was the commonest followed by haematologic malignancies (6.3%) and renal transplant (4.2%). Forty one patients were diagnosed by isolation of the organism as well as antigen detection in cerebrospinal fluid/serum, 9 by isolation alone and 8 by antigen detection alone. Quantitative antigen titres were done in 38 patients and a significantly higher (P < 0.01) antigen titre (> 512) was observed in HIV positive patients as compared to HIV negative patients. All isolates tested were of Cryptococcous neoformans var neoformans biotype and no resistance to antifungal agents was noted. Twenty of 41 patients receiving treatment improved. The results were compared with other studies available from India. INTERPRETATION & CONCLUSION: The incidence of cryptococcosis is on the rise in this part of north India and this can be attributed to an increase in AIDS cases.

Primary IgA nephropathy: a preliminary report.

A total of 106 renal biopsies were examined. Each biopsy was processed for routine paraffin sectioning and frozen sectioning. Direct immunofluorescence was done by the conventional method. The immunofluorescence patterns were correlated with histopathological changes and clinical presentation. Fourteen biopsies revealed weak to strong IgA staining and out of these, 11 seemed to fulfil the criteria of primary IgA nephropathy. These cases were diagnosed in a brief period of eleven months and there had not been any earlier published series from India.

Invasive pulmonary aspergillosis and nocardiosis in an immunocompromised host.

A case of invasive pulmonary aspergillosis and nocardiosis following high dose prolonged steroid therapy given for suspected rapidly progressive glomerulonephritis is reported. A favourable response was achieved with a combination of amphotericin B and cotrimoxazole. A high index of suspicion and aggressive investigations are necessary for confirmation of diagnosis and early institution of appropriate therapy.

Renal amyloidosis in juvenile rheumatoid arthritis.

We report a child who developed juvenile rheumatoid arthritis at the age of 7 years and nephrotic syndrome due to renal amyloidosis within 2 years of the onset of arthropathy. Literature on the management and prognosis has also been reviewed.

Wegener's granulomatosis: clinical experience with eighteen patients.

Wegener's granulomatosis is being recognised with increasing frequency in India. Our 18, histologically confirmed, patients had a clinical profile similar to that described from developed countries. Delayed diagnosis led to the death of nine patients, usually within days of hospital admission, due to extensive vasculitis and renal failure. Tuberculosis was the most frequently considered diagnosis and 12 patients had been treated for it in spite of progressive clinical deterioration. Those who could be adequately treated with low dose daily cyclophosphamide and corticosteroids did well. Six of seven such patients are alive and well 1-8 years later. We believe that if prompt lung biopsy and ANCA determination are resorted to in patients with "resistant tuberculosis", it will greatly expedite case detection, diagnosis and optimum treatment of this remediable disease.