PubMed · 394289
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R Rappaport. 1979-10-11. [Congenital androgenic adrenocortical hyperfunction].. https://pubmed.ncbi.nlm.nih.gov/394289/
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We report a 3-month-old boy who suffered an out-of-hospital cardiac arrest. During resuscitation, the medical team was informed that he was receiving hydrocortisone treatment. The possibility of adrenal insufficiency with hyperkalemic cardiac arrest prompted the administration of calcium, which resulted in the return of spontaneous circulation. The infant's diagnosis of congenital adrenal hyperplasia was not spontaneously mentioned by the parents. This case illustrates the importance of obtaining adequate parental information and considering hyperkalemia as a possible cause of cardiac arrest.
BACKGROUND: Women with normogonadotrophic normo-estrogenic oligomenorrhoea often disclose a variety of clinical symptoms. Many of these individuals are obese with features of pseudo-hypercortisolism. In the current study, 11-deoxycortisol and cortisol concentrations were determined in this group and compared with ovulatory controls. METHODS AND RESULTS: Twenty-six women with clomiphene citrate-resistant infertility, 12 lean and 11 obese ovulatory controls were studied. Women with infertility had the highest 11-deoxycortisol concentrations (mean +/- SD: 4.1 +/- 1.5 ng/ml) compared with obese and lean controls (3.1 +/- 1.4 and 2.4 +/- 0.9 ng/ml) (P < 0.01), but similar morning cortisol concentrations (0.47 +/- 0.15, 0.45 +/- 0.16 and 0.47 +/- 0.18 nmol/l). Baseline 11-deoxycortisol/cortisol ratios (>90th percentile of ovulatory controls) were elevated in 23/26 infertile women (88%), and in 3/26 women (12%) after adrenocorticotrophic hormone (ACTH) stimulation. Three out of six lean infertile women had elevated baseline 11-deoxycortisol/cortisol ratios, but none of these women had elevated ratios after ACTH stimulation. Stepwise regression analysis, after exclusion of testosterone, revealed significant correlations between the groups (lean controls, obese controls, infertility) and ACTH-stimulated 11-deoxycortisol/cortisol ratio (P < 0.05), but not with fasting glucose, insulin, cortisol, 11-deoxycortisol and baseline 11-deoxycortisol/cortisol ratios. CONCLUSIONS: Congenital adrenal hyperplasia was not observed in the majority of infertile women. The data indicate that extra-adrenal factors were involved in most of the infertility syndromes that were studied.
We report on a newborn with a severe salt-wasting crisis due to congenital adrenal hyperplasia (CAH) with 21-hydroxylase deficiency, in spite of prenatal diagnosis of CAH and awareness of this diagnosis by the parents and the gynecologic and pediatric practitioners. Due to the diagnosis of CAH in the older sister, prenatal treatment with dexamethasone (Dexa) was initiated. Prenatal diagnosis showed an affected male fetus and the prenatal treatment was stopped. The parents and the involved physicians were informed about the diagnosis, treatment, follow-up, and possible complications. Amniotic infection led to preterm delivery in another hospital. Due to perinatal asphyxia, the male newborn received Dexa during mechanical ventilation. Neonatal CAH screening was unsuspicious. An acute salt-wasting crisis with metabolic acidosis at the age of 3 weeks finally led to the correct diagnosis. This experience emphasizes the need to obtain a careful medical history. Furthermore, this case illustrates that neonatal screening for CAH is falsely negative in the event of neonatal Dexa treatment.