Hypertension in a neonate with 11 ?-hydroxylase deficiency

Mimouni, M.; Kaufman, H.; Roitman, A.; Morag, Ch.; Sadan, Naum

doi:10.1007/bf00442149

Cited by 42 publications

(19 citation statements)

References 5 publications

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“…Repair early in infancy seems to have led to improved long-term outcomes [81], although the recurrence of stenosis and hypertension in childhood remain significant problems that merit close follow-up with ambulatory BP monitoring [80,81]. Endocrinologic disorders that can produce hypertension in infancy include congenital adrenal hyperplasia with 11-β-hydroxylase or 17-α-hydroxylase deficiency [82][83][84][85]. Other heritable forms of hypertension, including primary hyperaldosteronism [86,87] and hyperthyroidism [88], albeit rare, are also important diseases that should be considered in the setting of normal renin hypertension.…”

Section: Etiologymentioning

confidence: 99%

Hypertension in infancy: diagnosis, management and outcome

2011

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Advances in the ability to identify, evaluate, and care for infants with hypertension, coupled with advances in the practice of Neonatology, have led to an increased awareness of hypertension in modern neonatal intensive care units. This review will present updated data on blood pressure values in neonates, with a focus on the changes that occur over the first days and weeks of life in both term and preterm infants. Optimal blood pressure measurement techniques as well as the differential diagnosis of hypertension in the neonate and older infants will be discussed. Recommendations for the optimal immediate and long-term evaluation and treatment, including potential treatment parameters, will be presented. We will also review additional information on outcome that has become available over the past decade.

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Section: Etiologymentioning

confidence: 99%

Hypertension in infancy: diagnosis, management and outcome

2011

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“…However, patients with NC 11βOHD are more often normotensive or have high-normal blood pressure at the time of diagnosis [16,32,38]. Although hypertension is usually noted at a later age, the earliest age of onset of hypertension in a case of 11βOHD was at birth [39].…”

Section: Hypertensionmentioning

confidence: 99%

Clinical perspectives in congenital adrenal hyperplasia due to 11β-hydroxylase deficiency

2016

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Congenital adrenal hyperplasia due to 11 beta-hydroxylase deficiency is a rare autosomal recessive genetic disorder. It is caused by reduced or absent activity of 11β-hydroxylase (CYP11B1) enzyme and the resultant defects in adrenal steroidogenesis. The most common clinical features of 11 beta-hydroxylase deficiency are ambiguous genitalia, accelerated skeletal maturation and resultant short stature, peripheral precocious puberty and hyporeninemic hypokalemic hypertension. The biochemical diagnosis is based on raised serum 11-deoxycortisol and 11-deoxycorticosterone levels together with increased adrenal androgens. More than 100 mutations in CYP11B1 gene have been reported to date. The level of in-vivo activity of CYP11B1 relates to the degree of severity of 11 beta-hydroxylase deficiency. Clinical management of 11 beta-hydroxylase deficiency can pose a challenge to maintain adequate glucocorticoid dosing to suppress adrenal androgen excess while avoiding glucocorticoid-induced side effects. The long-term outcomes of clinical and surgical management are not well studied. This review article aims to collate the current available data about 11 beta-hydroxylase deficiency and its management.

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“…Repair early in infancy seems to lead to an improved long-term outcome compared to delayed repair [48]. Endocrinologic disorders, particularly congenital adrenal hyperplasia [49,50], hyperaldosteronism [51] and hyperthyroidism [52] constitute easily recognizable clinical entities that are accompanied by hypertension.…”

Section: Clinical Presentation and Diagnostic Approachmentioning

confidence: 99%

Neonatal hypertension: diagnosis and management

Flynn¹

2000

Pediatric Nephrology

161

114

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Hypertension in the term or preterm neonate may be seen in up to 2% of all infants cared for in the modern neonatal intensive care unit. Although the definition of hypertension in this age group has not been completely standardized, recent studies have provided new normative data that may be used to facilitate identification of such infants. Common causes of hypertension in neonates include thromboembolic events related to umbilical catheterization, congenital problems such as aortic coarctation, structural renal malformations and renovascular disease, as well as acquired renal disease and certain medications. A careful history and physical examination will usually identify the probable cause in most cases without the need for extensive laboratory or radiologic testing. Therapy of neonatal hypertension should be tailored to the severity of the blood pressure elevation, and to the underlying cause of hypertension as appropriate. A wide range of therapeutic agents are now available for management of neonatal hypertension in both the acute and chronic settings. In most cases hypertension will resolve, but some infants may require prolonged treatment.

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Hypertension in a neonate with 11 ?-hydroxylase deficiency

Cited by 42 publications

References 5 publications

Hypertension in infancy: diagnosis, management and outcome

Hypertension in infancy: diagnosis, management and outcome

Clinical perspectives in congenital adrenal hyperplasia due to 11β-hydroxylase deficiency

Neonatal hypertension: diagnosis and management

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