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Pediatric Endocrinology, Diabetes and Metabolism 2008

Congenital hypoaldosteronism associated with nails hypothrophy - case report and review of the literature.

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Beata Wikiera
Ewa Głab
Ewa Barg
Anna Noczyńska

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Abstrait

We report 15 years follow-up of a girl born with nails hypothrophy and primary hypoaldosteronism. The failure to thrive, troubles with feeding and vomiting were observed since the first weeks of life. The results of the laboratory examination confirmed isolated hypoaldosteronism. The growth velocity was diminished and short stature was present during first 4 years of life. Thyroid and pituitary disturbances were excluded. At the age of 4 the treatment with fludrocortisone was gradually discontinued without clinical manifestation of aldosteron deficiency. The child was on high sodium chloride diet. The growth velocity improved. At the age of 7,5 years pubarche, lipid and carbohydrate disturbances occurred. The bone age was adequate to chronological age. The treatment with Cortineff and diet modification were recommended. Further improving in growth velocity and lowering in salt consumption were observed. Menarche occurred at the age of 12 years 8 months with subsequent monthly bleedings. She finished growth with height SDS -2.52 (target height SDS -1.71), and BMI SDS 1.83. The nails hypothrophy still maintains.

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