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Previous Volume 343:998-1007 October 5, 2000 Number 14 Next

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ABSTRACT

Background Central diabetes insipidus is rare in children and young adults, and up to 50 percent of cases are idiopathic. The clinical presentation and the long-term course of this disorder are largely undefined.

Methods We studied all 79 patients with central diabetes insipidus who were seen at four pediatric endocrinology units between 1970 and 1996. There were 37 male and 42 female patients whose median age at diagnosis was 7.0 years (range, 0.1 to 24.8). All patients underwent magnetic resonance imaging (MRI) and periodic studies of anterior pituitary function. The median duration of follow-up was 7.6 years (range, 1.6 to 26.2).

Results The causes of the central diabetes insipidus were Langerhans'-cell histiocytosis in 12 patients, an intracranial tumor in 18 patients, a skull fracture in 2 patients, and autoimmune polyendocrinopathy in 1 patient; 5 patients had familial disease. The cause was considered to be idiopathic in 41 patients (52 percent). In 74 patients (94 percent) the posterior pituitary was not hyperintense on the first MRI scan obtained, and 29 patients (37 percent) had thickening of the pituitary stalk. Eighteen patients had changes in the thickness of the pituitary stalk over time, ranging from normalization (six patients) or a decrease in thickness (one patient) to further thickening (seven patients) or thickening of a previously normal stalk (four patients). Anterior pituitary hormone deficiencies, primarily growth hormone deficiency, were documented in 48 patients (61 percent) a median of 0.6 year (range, 0.1 to 18.0) after the onset of central diabetes insipidus.

Conclusions Most children and young adults with acquired central diabetes insipidus have abnormal findings on MRI scans of the head, which may change over time, and at least half have anterior pituitary hormone deficiencies during follow-up.

Source Information

From the Departments of Pediatrics (M.M., G.C., F.S., M.A.), Radiology (E.G.), and Biometry–Scientific Direction (C.T.), University of Pavia and Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia; the Department of Pediatrics, University of Rome Tor Vergata, Rome (M.L.M.-B., B.B.); the Department of Pediatrics, Istituto di Ricovero e Cura a Carattere Scientifico G. Gaslini Institute, Genoa (A.C., S.Z.); the Department of Radiology, University of L'Aquila, L'Aquila (M.G.); and the Department of Pediatrics, University of Modena, Modena (S.B.) — all in Italy.

Address reprint requests to Dr. Maghnie at the Department of Pediatrics, University of Pavia, IRCCS Policlinico S. Matteo, 27100 Pavia, Italy, or at maghnie{at}smatteo.pv.it.

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