Subcutaneous fat was measured using an ultrasonic method (SF-US) in girls with Ullrich-Turner syndrome (UTS) and in height and weight-matched controls. The method is not influenced by changes in cutaneous compressibility, which is frequently abnormal in UTS especially at a younger age. SF-US in the arms was significantly less in UTS patients (ulnar SF-US: P < 0.05; triceps SF-US: P < 0.005) than in the control girls. Results in suprailiac SF-US measurement was also similar (P < 0.05). Though the mean SF-US in the subscapular region was less in UTS patients than the control girls, there was no statistically significant difference. It is demonstrated that subcutaneous fat in UTS patients is less in their arms than in girls without UTS and that trunkal fatness is not increased but rather decreased in the UTS. The ratio of lean body mass to weight seems higher in UTS patients than in girls with similar weight and height.

Responses of plasma vasoactive intestinal polypeptide (VIP) to methacholine inhalation and to exercise loading were studied in asthmatic patients to clarify a significant role of the peptide. The mean of basal VIP in asthmatics was not significantly different from the normals. The levels were increased after FEV (1.0) (forced expiratory volume in a second) decreased to 80% of the baseline following methacholine inhalation and were returned to values similar to the baseline when FEV (1.0) recovered to 100%. Thirty minutes after the exercise, mean VIP was significantly lower in patients with the lowest FEV (1.0). These results suggest that VIPergic system contributes, at least partly, to relax constricted bronchioles in some patients with bronchial asthma.

A case of hypodipsic hypernatremia in a 16-month-old Japanese boy is reported. Partial antidiuretic hormone deficiency was present. Computed tomography of the brain revealed absence of septum lucidum. No ophthalmological abnormality could be found. He had hyposmia, which has not been reported previously in association with hypernatremia due to hypodipsia. Forced fluid administration and nasal 1-deamino-8-d-arginine vasopressin treatment could maintain serum electrolyte levels within normal ranges. However, episodes of hypernatremia could not be completely avoided while he was treated with 1-deamino-8-d-arginine vasopressin and ad libitum oral fluid.

Long QT syndrome (Romano-Ward syndrome) and insulin-dependent diabetes mellitus (IDDM) have been documented as being linked with gene(s) on chromosome 11p although concurrence of the two disorders has not been reported. Our case is a 13-year-old boy with Romano-Ward syndrome accompanied by IDDM. The long QT syndrome seemed to be transmitted in an autosomal-dominant mode because the Q-T intervals of his father and paternal grandfather were longer than normal. There was no family member with an abnormally high level of blood glucose except the patient. The human leucocyte antigen (HLA) haplotypes of the patient and the father were DR4/DR9 and DR2/DR9, respectively. This study suggests that in our patient IDDM, as well as Romano-Ward syndrome, is linked with chromosome 11p in the presence of HLA-DR4. The results support the previous study that chromosome 11p encodes a gene implicated in HLA-DR4-dependent diabetes susceptibility.

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