Primary pigmented nodular adrenocortical disease with synaptophysin immunoreactivity in two thai children.

This paper reports the cases of two Thai children diagnosed with primary pigmented nodular adrenocortical disease (PPNAD). The first was a thirteen and a half year old male who presented with Cushing syndrome for three years and severe osteoporosis. He had hypercortisolemia, a non-suppressible dexamethasone suppression test, and low serum ACTH. A CT scan showed slight enlargement of both adrenal glands. A bilateral adrenalectomy was performed. The second case was a thirteen-year old female who presented with mild Cushing syndrome for one year with paradoxical response to high-dose dexamethasone suppression test. An MRI revealed suspected microadenoma of the left adrenal gland. Blood sampling showed a higher cortisol level from the left adrenal vein than from the right. A left adrenalectomy was performed, followed by a right adrenalectomy four months later. The pathologies were compatible with PPNAD. Immunostaining for synaptophysin was done in both patients. The cells in the adrenocortical micronodules of both cases were stained intensely with antiserum for synaptophysin, whereas the surrounding adrenal cortex did not.

Etiologies of central diabetes insipidus in children : 15 years experience in Songklanagarind hospital, Thailand.

Central diabetes insipidus (DI) is a rare disease in children. The authors retrospectively reviewed the records of children with central DI identified at Songklanagarind Hospital from 1985 to 2000. Of the total 29 patients identified, 16 patients were males and 13 were females. All patients received computed tomography or magnetic resonance imaging of the brain to differentiate the etiologies of central DI. The median age at diagnosis was 6.6 years (range 1.5-14.9). The etiologies of central DI were intracranial tumors in 7 patients (24.1%), histiocytosis in 3 patients (10.3%), septooptic dysplasia in 1 patient (3.5%), empty-sella syndrome in 1 patient (3.5%), pituitary abscess in 1 patient (3.5%), and idiopathic in 16 patients (55.1%). All patients with idiopathic central DI were followed-up for a median duration of 4.5 years (range 1.3-15.5). Three of 16 patients (18.8%) were found to have intracranial tumors at 1.3, 2.3, and 3.5 years of follow-up. It was also observed that the patients whose age at presentation was less than 5 years (histiocytosis was excluded) were less likely to have intracranial tumors than those older than 5 years, (0% vs 55%), with significant statistical difference (p<0.01). It is concluded that: 1) the common etiologies of central DI are intracranial tumor and idiopathic, 2) patients initially diagnosed with idiopathic central DI need to have long-term follow-up by magnetic resonance imaging to identify any occult intracerebral tumor.