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Hypercalcemia common in young children with congenital adrenal hyperplasia raises risk for renal complications
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Most children aged 6 years and younger with classic congenital adrenal hyperplasia experience at least one episode of hypercalcemia, increasing their risk for developing hypercalciuria, nephrocalcinosis and hypertension, according to a retrospective analysis of medical records.
“We have observed the anecdotal occurrence of hypercalcemia in children with [congenital adrenal hyperplasia]; however, its prevalence has not been established,” Melissa J. Schoelwer, MD, of the department of pediatrics at Riley Hospital for Children in Indianapolis, and colleagues wrote. “To our knowledge, only one prior study reported classic [congenital adrenal hyperplasia] as a cause of hypercalcemia in two children, and no studies describe hypercalciuria or nephrocalcinosis.”
In a retrospective cohort study, Schoelwer and colleagues analyzed medical records data from 40 children (73% white) diagnosed with classic congenital adrenal hyperplasia (21-hydroxylase deficiency) before age 2 years and at least one documented calcium measurement at Riley Hospital for Children between January 1994 and March 2014. Researchers extracted data from patient records up to age 6 years based on observations of high calcium concentrations. Hypercalcemia was defined as a serum calcium of at least 10.5 mg/dL; hypercalciuria was defined based on published normative data for children based on age.
Within the cohort, 90% were reported to have salt-wasting congenital adrenal hyperplasia; 33 patients (82.5%) had at least one elevated serum calcium concentration. Of those, 12 had one elevated serum calcium measurement recorded; 21 (53%) had more than one recorded high serum calcium measurement.
Of the 126 elevated serum calcium concentrations, the median was 10.9 mg/dL; median age at the last elevated calcium was 5 months. Serum calcium concentration was inversely related to age (r = –0.124; P = .004).
In instances of hypercalcemia, patients were treated with glucocorticoids 83% of the time and with mineralocorticoids 79% of the time.
Researchers found that, overall, calcium positively correlated with 17-hydroxyprogesterone (17OHP) concentration after adjusting for age (r = 0.132; P = 0.05); however, patients had hypercalcemia with both high and low 17OHP concentrations. Serum calcium concentration also was positively related to glucocorticoid (r = 0.185; P = .018) and fludrocortisone (r = 0.204; P = .009) doses, after adjustment for age.
Urine calcium was measured in seven patients; six had at least one documented period of hypercalciuria. Three patients had nephrocalcinosis documented by renal ultrasound, all of whom had hypertension. Aside from IV fluids for admitted patients along with appropriate glucocorticoid and mineralocorticoid treatment, patients did not undergo specific medical treatment for hypercalcemia, according to researchers.
“This study demonstrates that hypercalcemia is a common finding in young children with classic [congenital adrenal hyperplasia] and may place children at risk for serious renal complications, such as hypercalciuria and nephrocalcinosis,” the researchers wrote. “Calcium monitoring during routine [congenital adrenal hyperplasia] laboratory testing may be worthwhile in children with [congenital adrenal hyperplasia], particularly during the first year of life. Recognition of hypercalcemia should be followed by appropriate evaluation to determine etiology and to detect possible hypercalciuria and nephrocalcinosis.” – by Regina Schaffer
Disclosure: The authors report no relevant financial disclosures.
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