11β-hydroxylase deficiency natural history, complications and prognosis

Congenital adrenal hyperplasia due to 11β-hydroxylase deficiency Microchapters
Home
Patient Information
Overview
Historical Perspective
Classification
Pathophysiology
Causes
Differentiating Congenital adrenal hyperplasia due to 11β-hydroxylase deficiency from other Diseases
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications and Prognosis
Diagnosis
History and Symptoms
Physical Examination
Laboratory Findings
CT
MRI
Ultrasound
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Surgery
Prevention
Cost-Effectiveness of Therapy
Future or Investigational Therapies
Case Studies
Case #1
11β-hydroxylase deficiency natural history, complications and prognosis On the Web
Most recent articles
Most cited articles
Review articles
CME Programs
Powerpoint slides
Images
American Roentgen Ray Society Images of 11β-hydroxylase deficiency natural history, complications and prognosis All Images X-rays Echo & Ultrasound CT Images MRI
Ongoing Trials at Clinical Trials.gov
US National Guidelines Clearinghouse
NICE Guidance
FDA on 11β-hydroxylase deficiency natural history, complications and prognosis
CDC on 11β-hydroxylase deficiency natural history, complications and prognosis
11β-hydroxylase deficiency natural history, complications and prognosis in the news
Blogs on 11β-hydroxylase deficiency natural history, complications and prognosis
Directions to Hospitals Treating Congenital adrenal hyperplasia due to 11β-hydroxylase deficiency
Risk calculators and risk factors for 11β-hydroxylase deficiency natural history, complications and prognosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ammu Susheela, M.D. [2]

Overview

If left untreated, patients with congenital adrenal hyperplasia due to 11β-hydroxylase deficiency may progress to develop malignant hypertension. Common complications of congenital adrenal hyperplasia due to 11β-hydroxylase deficiency include muscle weakness, metabolic alkalosis, and azoospermia. Prognosis is generally good with treatment.

Natural History

If left untreated, patients with congenital adrenal hyperplasia due to 11β-hydroxylase deficiency may progress to develop malignant hypertension.

Complications

Complications of Hypertension

• Vascular hemorrhage
• Renal insufficiency
• Left ventricular hypertrophy
• Hypertensive retinopathy
• Stroke

Complications of Hypokalemia

• Muscle weakness
• Metabolic alkalosis
• Paralytic ileus

Other Complications

• Azoospermia
• Oligospermia
• Infertility

Prognosis

• The prognosis of congenital adrenal hyperplasia due to 11β-hydroxylase deficiency is generally good with treatment.
• A small percentage of children and adults with infancy or childhood onset congenital adrenal hyperplasia die of adrenal crisis, even after diagnosis and initiation of treatment.
• When a person is well, missing a dose or even several doses, may not worsen the immediate symptoms. However, glucocorticoid needs are increased during illness and stress.