A case of collapsing glomerulopathy associated with febrile illness.

Collapsing glomerulopathy (CG) is a distinct clinicopathological entity characterized by high levels of nephrotic range proteinuria, rapidly progressive renal failure, marked parenchymal injury, and poor response to present therapeutic regimens. Growing awareness has led to the identification of associated conditions other than human immunodeficiency virus (HIV) and idiopathic. We report a case of CG from India in a HIV-negative young female, presenting with heavy proteinuria and rapidly progressing renal failure preceded by a febrile illness.

Progression of chronic pulmonary tuberculosis in mice intravenously infected with ethambutol resistant Mycobacterium tuberculosis.

Purpose: Ethambutol (EMB) is an important first line drug, however little information on its molecular mechanism of resistance and pathogenicity of resistant isolates is available. Present work was designed to study virulence of the EMB resistant M. tuberculosis strains and the host responses in-vivo on infection of EMB resistant M. tuberculosis using Balb/c mouse model of infection. Methods: Three groups of Balb/c mice (female, age 4-6 wk; 21 mice in each group) were infected intravenously with 106 CFU of M. tuberculosis H37Rv and two EMB resistant clinical isolates. Age and sex matched control animals were mock inoculated with Middlebrook 7H9 broth alone. At 10, 20, 30, 40, 50, 60, and 70 days post-infection three animals from each group were sacrificed by cervical dislocation and lung tissue was collected for further analysis. Results: Infection with EMB resistant M. tuberculosis led to progressive and chronic disease with significantly high bacillary load (p=0.02). Massive infiltration and exacerbated lung pathology with increased expression of IFN-gamma and TNF-alpha was observed in lungs of mice infected with EMB resistant strains. The present study suggests that infection with EMB resistant M. tuberculosis leads to chronic infection with subsequent loss of lung function, bacterial persistence with elevated expression of TNF-alpha resulting in increased lung pathology. Conclusion: These findings highlight that EMB resistant M. tuberculosis regulates host immune response differentially and its pathogenicity is different from drug sensitive strains of M. tuberculosis.

Primary Sjogren's syndrome: rarity in India.

OBJECTIVE: Primary Sjogren's syndrome (SS) is rarely reported from India. We have studied the clinical spectrum and immunological profile of patients with primary SS. METHODS: A prospective analysis of patients with primary Sjogren's syndrome fulfilling San Francisco criteria, seen at our clinic in the last 10 years was carried out. RESULTS: The study included 26 patients, 21 being women. The presenting symptoms included dry eyes, dry mouth, and arthritis/arthralgia. Extra-glandular manifestations were glomerulonephritis, vasculitis, renal tubular acidosis and peripheral neuropathy. The important laboratory abnormalities were hypergammaglobulinaemia (16/20), antinuclear antibodies (18/26), anti-La (11/19) and anti-Ro (10/19). Minor salivary gland provided a definitive diagnosis in 16/26 (60%). CONCLUSION: The prevalence of primary Sjogren's syndrome is rare even in tertiary care rheumatology clinics. The clinical and immunological profile as seen here is similar to that reported in Western countries.

Stones associated renal pelvic malignancies.

BACKGROUND: The clinico-pathological characteristics of renal pelvic malignancies associated with stones were retrospectively analyzed. AIMS: The main objective was to define the biological behavior and prognostic factors for these malignancies. SETTINGS & DESIGN: The tumors were classified according to the pathological types. The clinical data, imaging features and pathological features were analyzed with relation to prognosis. MATERIAL AND METHODS: Eighteen cases of malignancies associated with stone disease were retrospectively studied. The institute review board permitted the study. RESULTS: High incidence (15/18) of squamous cell carcinoma (SCC) was noted. The prognosis in this group of patients was uniformly poor. The median survival time was 3.6 months in the SCC group, 7.5 months in the Transitional Cell Carcinoma (TCC) group and 24 months in the Adenocarcinoma (AC) group. Infectious and systemic symptoms were noted in the majority of the patients. Preoperative Imaging techniques revealed tumor in only 2 cases. Both underwent radical extirpation and the median survival is 18 months till date. In the other 16 patients, where the initial diagnosis was made only on histological analysis of incomplete nephrectomy specimens, the survival was 3.56 months. All patients had prolonged history of staghorn stone disease with associated non-functioning kidney. We found that the main prognostic factor was the stage of the disease. CONCLUSIONS: Malignancies associated with stone disease have insidious onset of clinical symptoms and need a high degree of suspicion to identify them pre-operatively. The grave prognosis associated with incomplete excision makes it imperative to diagnose them earlier.

Parathyroid carcinoma.

INTRODUCTION: Parathyroid carcinoma is a rare tumor with an incidence of 0.5 to 4%. Preoperative differentiation from benign adenoma is very difficult. Several features, though nonspecific, have been used in differentiating parathyroid carcinoma from the adenoma. MATERIAL AND METHOD: A retrospective analysis during the period of nine years, four cases of parathyroid carcinoma were diagnosed on the basis of per-operative local invasion and on histological evidence of capsular invasion and vascular invasion. RESULT: We have different observations to make. Diagnosis of parathyroid carcinoma which was done on the basis of intra operative suspicion only. One patient died on 6th post operative day due to development of pancreatitis. At the follow-up of two years there was no local recurrence or distant metastasis. CONCLUSION: It is preferable to have a high index of suspicion for parathyroid carcinoma when these features are present than to miss the opportunity for surgical cure by failing to consider it in the differential diagnosis.

Coeliac disease in Indian children: assessment of clinical, nutritional and pathologic characteristics.

Coeliac disease is an important cause of chronic diarrhoea, failure to thrive, and anaemia in children. Little information on the disease is available in India. This study was undertaken to determine the prevalence, clinical, anthropometric and histological profiles of coeliac disease in patients attending a tertiary referral centre in India. Coeliac disease was diagnosed in 42 (16.6%) of 246 children with chronic diarrhoea, failure to thrive, and anaemia. The mean ages at onset of symptoms and at diagnosis were 2.4 (range 0.5-10) years and 8.3 (range 3-14) years respectively, and a mean period of delay in diagnosis was 5.9 (range 1-13.5) years. Of the 42 cases, history of failure to thrive was observed in 38 (90%), chronic diarrhoea in 37 (88%), and anaemia in 6 cases. Short stature, under-nutrition, anaemia, oedema of feet, rickets, clubbing of fingers, features of vitamin A deficiency, and B-vitamin deficiency were found in 42, 26, 38, 9, 8, 6, 3, and 2 cases respectively. Onset of symptoms, such as, chronic diarrhoea and failure to thrive, was earlier in children with subtotal villous atrophy than in those with partial villous atrophy (mean +/- SD; 2.00 +/- 1.46 years vs 3.30 +/- 2.72 years; p < 0.05). Results of the study suggest that coeliac disease is not uncommon in Indian children. Coeliac disease should be considered in the differential diagnosis, particularly in children without any symptoms of diarrhoea.

Squamous cell carcinoma of thyroid gland.

We present the clinical manifestation, management and outcome in two cases of squamous cell cancer arising in the thyroid gland which is a rare entity. Both cases had advanced disease invading adjacent structures. At the followup of three to six months, one patient developed recurrence and died.

Infected Ewing's sarcoma of spine.

An unusual case of extradural, intraspinal Ewing's sarcoma with significant extraspinal extension is reported. In view of associated constitutional symptoms, suggestive signs, leucocytosis, elevated ESR and operative findings, the initial diagnosis of Pott's spine was entertained. The lesion however turned out to be infected Ewing's sarcoma on histopathology and bacteriological examination.

Xanthogranulomatous cholecystitis.

Xanthogranulomatous cholecystitis (XC) is a rare benign disease often confused with carcinoma gall bladder (CaGB). We retrospectively reviewed the clinical, radiological and operative features of 81 patients with XC alone (73) or XC with associated CaGB (8). Patients with XC alone had no gender preponderance, had a mean age 48 years and mean duration of symptoms of 34 months. Most XC patients (75%) had clinical features of chronic cholecystitis. On the other hand, patients with both XC and CaGB had a female preponderance, a mean age of 60 years and mean duration of symptoms of 6 months. In this group, the USG and operative findings often supported the clinical impression/findings. Xanthogranulomatous cholecystitis is a benign condition which is often confused with CaGB. Associated CaGB is however present in a few patients; elderly women with relatively shorter duration of symptoms, have a greater chance of associated CaGB.

Cutaneous metastases in pancreatic adenocarcinoma.

A patient with pancreatic carcinoma who developed disseminated cutaneous and scalp metases is reported. To the authors knowledge scalp metases have hitherto not been reported with pancreatic carcinoma.

Invasive thymoma: cytomorphological features on fine needle aspiration cytology.

Although thymomas are the commonest neoplasms of the anterior mediastinum, distinction between benign and malignant thymomas is controversial on Fine needle aspiration biopsy. We present cytomorphological features of a case of invasive thymoma on computed tomographic guided fine needle aspiration cytology and its basis is discussed in light of current concepts of thymic tumours.

Acute renal failure in lymphoma of the kidney.

Involvement of kidney by lymphomatous process occurs in 30-40% of cases of lymphoma, if the disease is left untreated. Incidence of renal involvement at initial presentation is 2.7%-6%. Mostly it is in the form of insidious renal failure. Acute renal failure as initial manifestation of the disease is very rare. A case of lymphomatous involvement of the kidney, with initial presenting manifestation of ARF is discussed, and the mechanisms responsible for rarity of this is analysed.

Giant cell tumour of the petrous bone.

Giant cell tumours (GCT) are rare in the skull bones. In the cranium the sphenoid bone is the most common site. The petrous bone is a very uncommon location for such tumours. The chance location of GCT at the base of the skull makes total surgery difficult and hazardous. A case of GCT of the petrous bone is presented. The clinical course in the patient and role of primary radiotherapy in GCT in such an unusual site is discussed.

A comparative study of PAP technique and in-situ molecular hybridization for identification of human papilloma viruses in cervical lesions.

The association of Human Papilloma Virus (HPV) with cervical lesions was correlated using peroxidase anti-peroxidase (PAP) technique and in-situ molecular hybridization (MH) with H3 labelled DNA probes (HPV type 6, 11, 16 and 18). The PAP technique to demonstrate capsid antigen and molecular hybridization for DNA sequences was studied in 30 cases. The correlation of HPV with cervical lesions was seen in 29.9% cases with PAP technique unlike 73.4% using M.H. technique, indicating the efficacy of the latter.