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Chronic fibrosing pancreatitis in childhood: report of a case and literature review.

Chronic fibrosing pancreatitis in childhood is an uncommon condition of unknown etiology with a variety of clinical presentations, histopathologic features, and outcomes. The diagnosis is one of exclusion (of hereditary or secondary pancreatitis), which should include histological assessment. The histological features of this condition have been described, to our knowledge, in nine published cases. We report a case in a 13-year-old male, who presented with obstructive jaundice and subsequently had evidence of endocrine and exocrine pancreatic insufficiency, despite a surgical decompression of the pancreatic-biliary duct system.

Adolescent↗

PubMed1996

Spirometrically controlled high resolution computed tomography - quantitative assessment of density distribution in patients with diffuse fibrosing alveolitis.

STUDY OBJECTIVE: Lung density assessed by high resolution computed tomography (HRCT) is sufficiently sensitive for the diagnosis of interstitial lung disease, but may be hampered by uneven disease distribution. We determined the mean and subpleural density values in patients with diffuse fibrosing alveolitis (FA) and evaluated the diagnostic accuracy of both parameters, expressed as post-test odds ratios. MATERIALS AND METHODS: Pulmonary HRCT was performed on 21 FA patients and compared to scans of 27 healthy volunteers. The HRCT procedure was standardized by taking 3 scans at the carina +/- 5 cm, and by defining inspiration levels at 50% vital capacity. Mean lung density (MLD) and subpleural lung density (SLD) were calculated for all participants. RESULTS: MLD and SLD values for healthy subjects were significantly higher compared with the patient group. Odds ratios were 7.8:1 and 3.9:1 for SLD and MLD, respectively, demonstrating a superior discrimination power of SLD in the diagnosis of FA. CONCLUSION: Diagnostic accuracy of quantitative HRCT measurements in FA was improved by the separate evaluation of subpleural lung density, which is a better indicator of the presence or absence of lung fibrosis than is mean lung density.

Adult↗

PubMed1998

Cryptogenic fibrosing alveolitis: diagnosis and treatment.

Cryptogenic fibrosing alveolitis is increasingly common, and often affects elderly males. High resolution computed tomography is the optimum means of diagnosis. Most patients try steroids but only 10-20% show an objective response. Alternative medical treatment is available, but the results are poor. Single lung transplantation may be offered to suitable patients with life-threatening disease.

Cyclosporine↗

PubMed1953