Clinical patterns and linear growth in children with congenital adrenal hyperplasia, an 11-year experience

Abstract

Objective: An important goal in treating children with congenital adrenal hyperplasia (CAH) is to achieve a normal final adult height (FH). The aim of this study was to describe the clinical presentations and evaluate linear growth and possible factors affecting it in children with CAH. Methods: This is a retrospective study of 56 patients with CAH followed up in a tertiary center for 11 years. Patient's data including demographics, clinical, anthropometric, and laboratory information at presentation and during follow-up period were collected from medical records. Results: Fifty-six children (31 females) with CAH were seen at KAMC-Jeddah over 11-year period and 91% were 21-hydroxylase deficient. Of these, 46.4% had hyponatremia and 28.6% had hyperkalemia (21.4% had hyponatremia and hyperkalemia) at presentation. Positive family history was documented in 53.6%. Ambiguous genitalia were present in 72% of females and the majority required corrective surgery. Males had significantly decreased HtSDS versus females and females had significantly higher body mass index. The HtSDS of children who had had higher 17OHP or salt-losing crisis during treatment was significantly lower than those who had normal 17OHP and those who did not have salt-losing crisis, respectively. Conclusion: The final height outcome in our patients with CAH treated with glucocorticoids is lower than the population norm. Proper control of the disease clinically and biochemically through strict compliance to medical therapy as well as close clinical and laboratory monitoring is an important key to achieve normal final adult height in these patients. Side effects, including overweight, obesity, and hypertension are true risk associations and need timely diagnosis and early management.

The validity of the Bayley-Pinneau method in predicting final adult height at the onset of puberty in patients with classic congenital adrenal hyperplasia

“…The weights and BMIs in our study cases tended to be high. Our findings agree with those of Al Shaikh et al [29], who concluded that children and adults with CAH are at an increased risk of developing obesity. Sarafoglou et al showed that higher glucocorticoid dosage and advances in CA and BA were associated with an elevated BMI and obesity [30].…”

Caracterización de una cohorte de pacientes pediátricos con Hiperplasia Suprarrenal Congénita

“…El metaanálisis en 18 centros realizado por Eugster et al, reportó una puntuación media de talla final en -1,37 DE, resultado significativamente mejor en pacientes identificados tempranamente 28 . Publicaciones más recientes muestran que con un manejo adecuado se puede obtener una talla final dentro del potencial genético 29 .…”

Clinical patterns and linear growth in children with congenital adrenal hyperplasia, a 11 year experience

“…I read with interest the distinguished study by Al Shaikh et al[1] published in May-June 2019 issue of Indian Journal of Endocrinology and Metabolism . The authors studied the clinical pictures and evaluated linear growth and possible factors affecting it in Saudi children with congenital adrenal hyperplasia (CAH).…”

“…The HtSDS of children who had had higher 17-hydroxy progesterone (17OHP) or salt-losing crisis during treatment was significantly lower than those who had normal 17OHP, and those who did not have salt-losing crisis, respectively. [1] The authors addressed two limitations that might pose some suspicions on the results of this study. These included the retrospective nature of the data and relatively small number of the studied CAH patients ( n = 56).…”

“…These scores have been released for the precise assessment of nutrition and growth in the clinical settings and researches. [3] I wonder why Shaikh et al[1] referred to WHO standard instead of the national Saudi standard to evaluate linear growth of the studied cohort. I assume that if they referred to the national standard in the study methodology, different results might be yielded.…”