Congenital adrenal hyperplasia: transitional care

Hughes, Ieuan A.

doi:10.1016/j.ghir.2004.03.015

Cited by 21 publications

(9 citation statements)

References 63 publications

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“…A variety of clinics involving close interaction between paediatric, reproductive, adult endocrinologists, and clinical psychologists experienced in psychosexual counselling will be necessary for patients with CAH21-24).…”

Section: Discussionmentioning

confidence: 99%

Long-term Follow up of Congenital Adrenal Hyperplasia Patients with Hyponatremia

Jun

Lee

Kim

et al. 2007

Electrolyte Blood Press

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Congenital adrenal hyperplasia (CAH) caused by 21-hydroxylase deficiency is an autosomal recessive disease, which leads to cortisol and aldosterone deficiency and hyperandrogenism. Typical medical treatment includes oral glucocorticoid and mineralocorticoid administration to suppress adrenal androgens and to compensate for adrenal steroid deficiencies. However, some patients stopped taking medicine without the doctor's consent. Among these patients, four cases of CAH patients showing the presence of hyponatremia as an initial electrolyte disorder were found with adrenal adenoma. Hypersecretion of adrenocorticotrophic hormone and chronic poor compliance to therapy appears to be associated with the development of the adrenal tumor. Two cases were managed with adrenalectomy because of increasing adrenal tumor size and virilization. Whereas the other two cases did not increase in size and were observed without adrenalectomy. Therefore, it is important that patients with CAH maintain steroid medication to avoid the appearance of adrenal tumor.

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Section: Discussionmentioning

confidence: 99%

Long-term Follow up of Congenital Adrenal Hyperplasia Patients with Hyponatremia

Jun

Lee

Kim

et al. 2007

Electrolyte Blood Press

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“…8,9 Recommendations for monitoring glucocorticoid and mineralocorticoid therapy in men and women with CAH due to 21-OH deficiency appear in a recent clinical practice guideline of the Endocrine Society. 11 While less has been written on health care transition for AYAs with DSD due to conditions other than 21-OH deficiency, some important information is known about this more general group.…”

Section: Limited Knowledge About Health Care Transition Of Ayas With Dsdmentioning

confidence: 99%

Transition from Pediatric to Adult Care for Adolescents and Young Adults with a Disorder of Sex Development

Hullmann

Chalmers

Wisniewski

2012

Journal of Pediatric and Adolescent Gynecology

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“…Lebenstag mit Bestimmung von 17-OH-Progesteron im Vollblut werden die betroffenen Kinder frühzeitig entdeckt und einer adäquaten Therapie zugeführt. Die frühzeitige Diagnose, eine konsequente Therapie und eine qualifizierte ärztliche Betreuung sollten die päd-iatrischen Therapieziele wie ein normales Gedeihen, die Verhinderung von Salzverlustkrisen, ein normales Längenwachs-tum, eine normale Pubertätsentwicklung und das Erreichen einer normalen Endgröße sicherstellen [26,36]. Nach den Empfehlungen der Fachgesellschaften ist Mittel der 1.…”

Section: Prognoseunclassified

Adrenogenitales Syndrom und Wachstumshormonmangel

Schöfl

2009

Internist

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Children with chronically endocrine diseases should be treated as young adults by adult endocrinologists. To optimize the transfer from the pediatric to adult endocrinologist, the model of a common transition clinic has been developed. Within this setting it should be possible to exchange experiences, extend the knowledge and understanding of the disease with the other side, and to provide for the patient an optimal outpatient care. This model, however, has only been sporadically realized to date. To set an example for the problems of the transition into adult endocrinology, we used two different endocrine diseases, the classical congenital adrenal hyperplasia due to 21-hydroxylase deficiency, and the childhood-onset growth hormone deficiency. Specific problems for this transfer to adult care are the fixation of the patients to their pediatricians and the lack of comprehension in the need of a long term and continuous therapy. The consequence is a dramatic impairment in the quality of the therapy.

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Congenital adrenal hyperplasia: transitional care

Cited by 21 publications

References 63 publications

Long-term Follow up of Congenital Adrenal Hyperplasia Patients with Hyponatremia

Long-term Follow up of Congenital Adrenal Hyperplasia Patients with Hyponatremia

Transition from Pediatric to Adult Care for Adolescents and Young Adults with a Disorder of Sex Development

Adrenogenitales Syndrom und Wachstumshormonmangel

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