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2016 ; 8
(3
): 330-3
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Endocrine Dysfunctions in Patients with Inherited Metabolic Diseases
#MMPMID27086477
Erdöl ?
; Sa?lam H
J Clin Res Pediatr Endocrinol
2016[Sep]; 8
(3
): 330-3
PMID27086477
show ga
OBJECTIVE: Inherited metabolic diseases (IMDs) can affect many organ systems,
including the endocrine system. There are limited data regarding endocrine
dysfunctions related to IMDs in adults, however, no data exist in pediatric
patients with IMDs. The aim of this study was to investigate endocrine
dysfunctions in patients with IMDs by assessing their demographic, clinical, and
laboratory data. METHODS: Data were obtained retrospectively from the medical
reports of patients with IMDs who were followed by the division of pediatric
metabolism and nutrition between June 2011 and November 2013. RESULTS: In total,
260 patients [139 males (53%) and 121 females (47%)] with an IMD diagnosis were
included in the study. The mean age of the patients was 5.94 (range; 0.08 to 49)
years and 95.8% (249 of 260 patients) were in the pediatric age group. Growth
status was evaluated in 258 patients and of them, 27 (10.5%) had growth failure,
all cases of which were attributed to non-endocrine reasons. There was a
significant correlation between growth failure and serum albumin levels below 3.5
g/dL (p=0.002). Only three of 260 (1.1%) patients had endocrine dysfunction. Of
these, one with lecithin-cholesterol acyltransferase deficiency and another with
Kearns-Sayre syndrome had diabetes, and one with glycerol kinase deficiency had
glucocorticoid deficiency. CONCLUSION: Endocrine dysfunction in patients with
IMDs is relatively rare. For this reason, there is no need to conduct routine
endocrine evaluations in most patients with IMDs unless a careful and detailed
history and a physical examination point to an endocrine dysfunction.
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