Hirofumi Urashima

Efficacy of Bromocriptine Administration for Selective Pituitary Resistance to Thyroid Hormone

The relation between thyroid-stimulating hormone (TSH) and triiodothyronine (T3) was evaluated in a girl with the selective pituitary type of thyroid hormone resistance for more than 7 years to clarify whether bromocriptine was an effective treatment or not. Levels of T3 (before: 2.44 +/- 0.64 nmol/l, mean +/- SD) and TSH (4.81 +/- 2.52 mU/l) were significantly decreased during therapy (T3: 2.15 +/- 0.44 nmol/l; TSH: 1.59 +/- 0.78 mU/l). T3 x TSH, calculated as one of the indices of pituitary resistance, on bromocriptine therapy (3.229 +/- 1.255 mU/l x nmol/l) was significantly (p < 0.005) smaller than the product before the administration (11.298 +/- 5.891 mU/l x nmol/l). The results suggest that bromocriptine should be one of the agents initially considered for the treatment of pituitary resistance to thyroid hormone.

Partial monosomy 10q and partial trisomy 9q with anal atresia due to maternal translocation: t(9;10)(q32;q26)

We report a case of a boy with a partial deletion of the long arm of chromosome 10 and partial duplication of the long arm of chromosome 9 due to maternal balanced translocation. The karyotype was 46, XY,‐10, +der(10)t(9;10)(q32;q26)mat. Our patient had imperforate anus and as this finding is usually not observed in association with trisomy 9q, it suggests that partial monosomy of the long arm of chromosome 10 might be associated with anogenital anomalies.

Excessive Food Aversion, Compulsive Exercise and Decreased Height Gain Due to Fear of Obesity in a Prepubertal Girl

A case of a 7-year-old girl with a remarkable food aversion and excessive weight reduction caused by fear of obesity, which has been demonstrated in pubertal girls with symptoms partly similar to anorexia nervosa, is reported. Although the patients weight was reduced to the upper limits of the normal range with diet and exercise, she reduced her food intake more strictly and did not at all eat food consisting of carbohydrates. Exercise was performed longer than before. Her weight continued to decrease and height velocity lowered from 6.0 to 4.1 cm/year (mean +/- SD of the age-matched normal girls: 5.5 +/- 0.74 cm/year). Her eating behavior was normalized without specific psychotherapy for anorexia nervosa. It is suggested that food aversion with weight loss and decrease in height gain due to fear of obesity may occur in prepubertal children as well as in adolescent girls.

Maternal perception of children's weight in relation to eating disorders

Abnormal perception of body weight in girls has been reported previously (1), and distorted perception of body shape is cited as one of the pathogenetic factors in eating disorders such as anorexia and bulimia nervosa (2,3)‐ Parent‐child relationships should also be considered as one of the factors possibly contributing to the onset of the eating disorders (3,4). In the present paper we shall examine whether mothers perceive the weight of their sons and daughters correctly or not, and we shall also compare the results with the perceptions of the children themselves, which we have reported previously in this journal (1). Five outline drawings (‐20% to 20% overweight), used in the previous study (1), were shown to the mothers, who were asked to select the drawings which they considered to represent the body weight of their sons (n=130) and daughters (n=125), who were aged 6 to 18 years. The mean body weights of the boys and girls in the study sample were 6.0% and 6.6% overweight, respectively. The mothers perceived the weight of their sons to be significantly (P < 0.01) less than the actual weight of these boys. The mean difference between the perceived weight and the actual weight was 10.4%. The mothers’ perception of their daughters’ weight was also 7.9% less than the actual weight (P < 0.05) (Fig. 1).

Synthesis of linear-type chondroitin clusters having a C8 spacer between disaccharide moieties and enzymatic transfer of d-glucuronic acid to the artificial glycans

Newly designed linear-type glycoclusters were synthesized which involve a chondroitin repeating disaccharide ligand and a hydrophobic octyl ether spacer. The spacer mimics the corresponding disaccharide unit. Repeating elongation of the pseudo-tetrasaccharide that was derived from the common cluster unit [-->8)-octyl-(1-->3)-beta-D-Gal-NAc-(1-->4)-beta-D-GlcA-(1-->] allowed the syntheses of up to the pseudo-decasaccharide analog of chondroitin. An enzymatic D-GlcA transfer at the non-reducing end of the synthesized artificial glycans by GlcATase II was observed.

Long QT syndrome with insulin‐dependent diabetes mellitus: contiguous gene syndrome on chromosome 11p

Abstract. 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.

Usefulness of a stature-based standard of skinfold thickness, especially for short children

Skinfold thicknessess (SFT) were measured at ulnar, triceps, subscapular and suprailiac sites in 730 boys and 724 girls (age 3–12 years) whose stature ranged from 100 to 150 cm and whose weight was within ±20% of the average. Means and standard deviation (SD) were calculated after logarithmic transformation of the original skinfold readings to demonstrate stature‐based standards of SFT in Japanese children. The means of SFT exhibited nadirs (boys/ girls: ulnar 5.1/5.9 mm, triceps 7.9/9.5 mm, subscapular 4.9/6.1 mm, suprailiac 4.5/6.2 mm) in subjects 110–115 cm tall except for ulnar SFT in girls. SFT values increased as children increased in stature. Standard deviations of SFT at the four sites in short children (staturte < mean −1 SD) were estimated using the stature‐based standard as well as an age‐based standard. Susms of the SDs assessed by the age‐based standard were significantly smaller than those assessed by the stature‐based standard in boys (P < 0.05) and girls (P < 0.01) with short stature, suggesting that SFT in short children was falsely understimated by the age‐based standard. Thus, the stature‐based standard is beneficial for the assessment of SFT, especially in children whose stature is below the mean −‐1 SD.

Fat areas on the extremities in normal weight and overweight children and adolescents: Comparison between age-related and weight-related changes in adiposity

The purpose of the study was to compare changes in fat distribution in association with obesity and puberty in adolescent boys and girls. Fat areas at the ulnar, triceps, thigh, and calf regions were measured in normal weight prepubertal children (P) and adolescents (N), and overweight adolescents (O). There were significant differences in fat areas at the four sites between N and O of both sexes, especially in the proximal extremities (triceps, thigh) in boys. On the other hand, fat areas on the extremities of N boys and girls were not significantly different from those of P children except for thigh fat area in girls. It is suggested that pubertal maturation in girls, but not in boys, is associated with increased adiposity on the legs, particularly in the proximal regions, and that there are sex differences between changes in fat distribution associated with puberty and obesity.