medwireNews: The results of a long-term study indicate that managing Cushing’s disease (CD) in children is “broadly similar” to managing it in adults, say the researchers.

But they add that “growth is an important additional parameter, which must be assessed and treated appropriately.”

Nineteen of the 21 patients in the study were assessed for growth hormone deficiency at an average of 0.5 years after undergoing trans-sphenoidal pituitary surgery (TSS) or pituitary radiotherapy, at which point 81% were deficient. Nine were reassessed at an average of 8.5 years after treatment of whom four (44%) remained deficient.

Three-quarters of the deficient patients received growth hormone treatment and, for the 15 with follow-up height data, average height standard deviation score (SDS) improved from –1.6 to –0.99, which was 1.01 below their target height SDS.

The patients were aged an average of 12.1 years at diagnosis. All presented with weight gain and changed facial appearance, while 95% had growth retardation. Emotional lability and fatigue were common, in 71% and 62% of patients, respectively, with other presenting features including hypertension, acne, hirsutism, headaches and striae.

TSS alone, or combined with radiotherapy for five patients and bilateral adrenalectomy for one, resulted in remission for 16 patients. Additional treatments led to remission for a further three patients, with the other two having abnormal cortisol circadian rhythm and Nelson’s syndrome.

However, over an average 10.6 years of follow-up after definitive treatment, Helen Storr (Queen Mary University of London, UK) and colleagues identified a “small but important prevalence of recurrence”.

Three patients had recurrence, at 2.0, 6.0 and 7.6 years after treatment, they report in Pituitary, illustrating the need for lifelong follow-up.

“Even though relapse is usually expected during the first 5 years following definitive treatment, it may occur later and the percentage of relapsed cases increases with length of observation”, say the researchers.

Average body mass index SDS improved from +2.9 to +0.58, and bone mineral density also improved although it did not return to normal in all patients, “suggesting some individuals may be at future risk of osteopenia.”

And there were other long-term effects, including multiple pituitary hormone deficiencies in four patients and psychiatric comorbidities in five. Nine patients had delayed onset or slow progression of puberty, with six requiring hormone replacement, and four had hypogonadotropic hypogonadism as adults.

“Close follow-up of pubertal development and early identification of pituitary–gonadal axis deficiency following remission of pediatric CD is important”, says the team.

By Eleanor McDermid

Pituitary 2016; Advance online publication

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