Clinical Characteristics Of Endocrinopathies In Patients Wit
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Hereditary haemochromatosis (HH) is a genetic disorder characterized by systemic iron overload and can lead to end?organ failure. However, very few data on this disorder, especially those on endocrine gland involvement in Chinese populations, are currently available. This study aimed to analyze the clinical features of endocrinopathies in patients with HH to generate concern among endocrinologists and improve the management of this disorder.

Chinese patients with HH?related endocrine dysfunction were enrolled at Peking Union Medical College Hospital.

Results:
-- A total of six patients were enrolled in this study, comprising five men and one woman; average age was 36.5 ± 13.3 years.

-- Mean serum ferritin concentration was 4508.8 ± 1074.3 ng/mL, and median transferrin saturation was 97.9% (96.6%–110.0%).

-- Endocrine gland involvement associated with HH included the pancreas (5/6 patients), the adenohypophysis (5/6 patients), and the bones (1/6 patients); secondary endocrinopathies consisted of diabetes mellitus, hypogonadism, adrenal insufficiency and osteoporosis.

-- Based on phlebotomy and iron chelation therapy, five patients were treated with exogenous insulin preparations, and three patients were treated with exogenous sex hormone replacement therapy.

-- The clinical symptoms of five patients improved although one patient died of hepatic encephalopathy and multiple organ failure.

Conclusively, HH can cause multiple endocrinopathies. The possibility of HH should be carefully considered in patients with endocrine gland dysfunctions and concomitant elevated serum ferritin levels. Endocrine gland function should also be assessed and followed up in patients with a clear diagnosis of HH.

Source: https://onlinelibrary.wiley.com/doi/10.1002/dmrr.3448?af=R
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