Endocrine manifestations related to inherited metabolic diseases in adults

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Endocrine manifestations related to inherited metabolic diseases in adults

Most inborn errors of metabolism (IEM) are recessive, genetically transmitted diseases and are classified into 3 main groups according to their mechanisms: cellular intoxication, energy deficiency, and defects of complex molecules. They can be associated with endocrine manifestations, which may be complications from a previously diagnosed IEM of childhood onset. More rarely, endocrinopathies ca...

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Hypoglycaemia related to inherited metabolic diseases in adults

In non-diabetic adult patients, hypoglycaemia may be related to drugs, critical illness, cortisol or glucagon insufficiency, non-islet cell tumour, insulinoma, or it may be surreptitious. Nevertheless, some hypoglycaemic episodes remain unexplained, and inborn errors of metabolism (IEM) should be considered, particularly in cases of multisystemic involvement. In children, IEM are considered a d...

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Respiratory manifestations in patients with inherited metabolic diseases.

Growing evidence indicates that inherited metabolic diseases are increasingly being recognised. Life expectancy for many patients is progressively improving because new therapeutic strategies are available. Because most inherited metabolic diseases are systemic disorders, virtually all organs may be involved. Respiratory disease complicates the management of several inherited metabolic diseases...

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Endocrine Dysfunctions in Patients with Inherited Metabolic Diseases

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. ...

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Respiratory manifestations in endocrine diseases

The control mechanisms of respiration as a vital function are complex: voluntary - cortical, and involuntary - metabolic, neural, emotional and endocrine. Hormones and hypothalamic neuropeptides (that act as neurotrasmitters and neuromodulators in the central nervous system) play a role in the regulation of respiration and in bronchopulmonary morphology. This article presents respiratory manife...

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ژورنال

عنوان ژورنال: Orphanet Journal of Rare Diseases

سال: 2012

ISSN: 1750-1172

DOI: 10.1186/1750-1172-7-11