Further delineation of the acrocallosal syndrome.

The acrocallosal syndrome (ACS) was recognized by Schinzel in 1979 as a specific entity, characterized by the association of craniofacial anomalies, total or partial agenesis of corpus callosum, polysyndactyly and mental retardation. The inheritance is autosomal recessive, based on instances of recurrence in siblings and cousins and parental consanguinity. A large inbred kindred with recurrent ACS is presented. This family further strengthens the hypothesis of autosomal recessive inheritance for this syndrome. The array of clinical manifestations in this sibship and those previously reported exemplify the phenomenon of inter- and intrafamilial variability that must be considered when defining ACS. Based on a review of published reports and the present family, essential, additional and occasional findings are distinguished. Attention is drawn to geographical clustering of the families.

Behcet disease: long-term follow-up of three children and review of the literature.

Behcet disease is rare in children. There are only two reports of Behcet disease in childhood, describing seven patients. Three pediatric patients are described, in whom the age of onset ranged from 6 to 11 years. Aphthous stomatitis and arthritis were present in all of the patients; genital ulcers, iridocylitis, erythema nodosum, and CNS involvement were present in two patients. Other manifestations included Stevens-Johnson-like eruption, fever of unknown origin, and testicular involvement. All of the patients responded to glucocorticoids; two were also treated with colchicine and one was treated with chlorambucil. In two patients, follow-up of more than 10 years was done, with complete cure in one patient and benign course of illness in the other. Because of the rarity of the disease in childhood and the difficulty in making the diagnosis, there is not enough awareness by pediatricians concerning this disease.

[Thyroid function disturbances in an infant following maternal topical use of polydine].

A male infant who had symptoms of transient sinus tachycardia and increased concentrations of blood total and free T4 is reported. The mother had used Polydine (P; povidone iodine) for vaginal douching during pregnancy and during the first few weeks after delivery, during which time the infant was fed mother's milk only. Follow-up showed transient elevation of TSH levels in the infant, but with no signs of hypothyroidism. 2 months after the mother stopped using P, the infant's functions had become normal. P should not be used in the perinatal period.