Type III collagen disorders: A case report and review of literature.

Collagen type III is a normal component of interstitium and blood vessels. Collagenofibrotic glomerulopathy (CG) and nail patella syndrome (NPS) are the diseases of abnormal type III collagen deposition. In spite of these curved frayed structures with a periodicity of 45–60 nm are deposited in subendothelium and mesangium in CG, they are found only in the basement membrane in NPS. The clinical features of CG are confined to the kidney, NPS has associated extra‑renal manifestations. Electron microscopy is essential to make the renal diagnosis in both these rare diseases. Both the entities considered to be systemic diseases evidence to suggest similar deposition in other organs, understanding etiopathogenesis and disease progression await research.

Giant cell arteritis: A clinical and pathological study.

Background. Giant cell arteritis is a vasculitis affecting large- and medium-calibre vessels. It is not uncommon in the West and there are many large series in the literature. However, there are very few reports of giant cell arteritis among Indian patients. Methods. We did a retrospective study of 9 Indian patients (5 men and 4 women; age range 59–81 years [mean and median 70 years]) who had had a temporal artery biopsy for suspected giant cell arteritis at a tertiary care hospital. Results. Eight patients had biopsy-proven giant cell arteritis. The common presenting features were pyrexia of unknown origin (4), headache (6) and blurring of vision (2). The erythrocyte sedimentation rate was elevated and ranged from 25 to 120 mm at the end of the first hour (mean 96, median 105). The C-reactive protein level, which was available in 5 cases, was raised. Giant cells and inflammatory cells were seen in 7 of 8 temporal artery biopsies; a transmural lymphocytic and neutrophil infiltrate without giant cells was present in 1 case. All patients were treated with steroids and they responded well. Conclusion. Temporal arteritis is probably under-recognized in India. Pyrexia is a common presenting feature of the disease; temporal arteritis should be considered in the differential diagnosis of elderly patients with pyrexia of unknown origin.

An autopsy report of a rare pediatric lung tumor: pleuropulmonary blastoma.

An autopsy report of pleuropulmonary blastoma (PPB) is described in a two-and-a-half-year-old male child who died within a few days of starting chemotherapy. Autopsy revealed a large tumor almost occupying the whole of left hemithorax with widespread extension to pleura. The diagnosis was confirmed to be PPB, type III on autopsy.