Mitsunori Murata

Pyloromyotomy Versus Atropine Sulfate for Infantile Hypertrophic Pyloric Stenosis

PURPOSE Atropine sulfate (atropine) and pyloromyotomy were compared for managing infantile hypertrophic pyloric stenosis (IHPS). METHODS From 1996 to 1998, cases of IHPS treated surgically (pyloromyotomy; n = 20) or medically (atropine; n = 14) at separate institutions were compared retrospectively with regard to status on presentation, physical symptoms and signs, progress, and costs. Atropine was given orally, then intravenously if ineffective. Refractory cases were referred for pyloromyotomy. RESULTS All subjects were matched for clinical and physiological status on admission. Oral atropine alone was effective in 11 cases, was converted to intravenous atropine in 2 cases, and was terminated in 1 case because of hematemesis. Two cases were referred for pyloromyotomy. All pyloromyotomies were successful. Atropine took on average, 2.6 days to take effect. The difference in time taken for normalization of pyloric muscle thickness between the 2 groups was not significant. Average time to return to full feeding was longer in the atropine group (P<.01). Costs were lower in the atropine group (P<.01). There were 2 wound infections in the pyloromyotomy group, but no adverse effects of atropine. There were no recurrences in either group. CONCLUSION This study provides reasonable evidence to support a trial of atropine in IHPS.

Bone Maturation Reflects the Secular Trend in Growth

The aim of this study was to compare a series of X-rays from the mid–1990s with another taken in the mid–1980s in order to test the possibility that environmental causes affect the skeletal maturation. The first group of subjects included a total of 1,057 girls and 1,055 boys participating in a project for Japan and China health research in 1986. The second group of subjects included a total of 382 girls and 629 boys participating in a project for bone mineral density research in 1996. The skeletal maturity score using the Tanner-Whitehouse 2-RUS method was used as the fundamental datum. This score was used to represent each group. The Wilcoxon’s rank sum test was applied to examine the significance of the difference between the 1986 and the 1996 groups. The 1996 children had not matured more than the 1986 children; children in both groups reached the given scores at almost the same ages. In girls, there was little difference between the groups at 7 years of age, but it declined from 8 years of age onward. Some apparent differences arose at ages 14 and 15, but ceased by age 16 in girls. In boys, no differences were found in those aged from 7 to 17 years, except for 12-year-olds. We did not detect much of a difference in bone maturation between the 1986 and 1996 groups of children, and no differences in height during the same period. Our findings suggest that bone maturation reflects the secular trend in growth.

Cytotoxic T lymphocyte antigen 4 gene polymorphism confers susceptibility to Type 1 diabetes in Japanese children: analysis of association with HLA genotypes and autoantibodies

OBJECTIVE Although the polymorphisms of the cytotoxic T lymphocyte antigen 4 (CTLA4) gene have been shown to be associated with Type 1 diabetes in Caucasians, some conflicting results have been reported among subjects of different ethnic backgrounds. We examined a CTLA4 polymorphism and its relationship to human leucocyte antigen (HLA) genotypes and autoantibodies for glutamic acid decarboxylase 65 (GAD65) and IA‐2 in Japanese children with Type 1 diabetes.

Etiology of 19 infants with apparent life‐threatening events: Relationship between apnea and esophageal dysfunction

There are various identifiable diseases or conditions that can cause apparent life‐threatening events (ALTE; e.g. gastroesophageal reflux (GER) and seizures). Nineteen infants with ALTE (mean age: 4.3 months) were brought to our hospital between June 1986 and August 1991. The causes of these ALTE were investigated. Analysis of laboratory data, radiological studies and esophageal function tests led to the diagnosis of GER in six of 19 infants; pertussis in five; upper respiratory infection in three; vagotonia‐like condition with esophageal dysfunction in two; laryngostenosis with cerebral palsy in two; choking on food or drink in two; and epilepsy in one infant. Two cases (one case of pertussis and one of vagotonia‐like condition) were associated with GER. Some of the cases demonstrate that ALTE in infants may be induced by GER or some esophageal dysmotility. Further studies of ALTE are needed to ascertain how frequently GER or esophageal dysmotility is responsible for ALTE.

Ganglioneuroma of left adrenal gland in a patient with Turner syndrome during growth hormone therapy

We report on a Japanese girl with Turner syndrome (45,XO) who developed ganglioneuroma of the left adrenal gland during growth hormone (GH) therapy. She had received GH replacement therapy from the age of 6.8 years. At the age of 10.3 years, abdominal ultrasonography revealed a mass which occupied the upper area of her left kidney. Computed tomography and magnetic resonance imaging of the abdomen showed a low density mass with a smooth surface located between the upper portion of the left renal vein and the pancreas. Microscopic examination resulted in a diagnosis of ganglioneuroma of the left adrenal gland. At present we cannot conclude that patients who have received GH replacement therapy are at higher risk for developing tumors compared to those without GH replacement therapy.

Exercise performance in children with hyperthyroidism.

An attempt was made to define exercise performance in children with untreated hyperthyroidism using treadmill stress testing. Data were obtained for five female patients and the results were compared with those obtained for 16 normal female subjects. There were no significant differences at rest between the hyperthyroidism group and the control group in oxygen uptake, minute ventilation and respiratory rate. On the other hand, heart rate in the hyperthyroidism group was significantly higher than that in the control group. During exercise, there were significant differences between the two groups in oxygen uptake, heart rate, minute ventilation and respiratory rate. Hyperthyroid patients did not show an abrupt increase in heart rate during the first 30s of exercise. Exercise stress testing can therefore reveal cardiopulmonary abnormalities that are not evident at rest in children with hyperthyroidism.

Changes in leptin and testosterone levels in a girl with congenital adrenal hyperplasia during hydrocortisone therapy

age in both sexes before the onset of puberty. During sexual maturation, leptin levels continue to rise in girls. However, in boys, leptin rises from the prepubertal to the early pubertal stage and then declines in the late pubertal stage. The sexual dimorphism in leptin levels is strongly related to pubertal stage.1 In adults, males have lower leptin levels than females at any level of adiposity; for example, leptin messenger ribonucleic acid expression is lower in obese boys than in obese girls.2 These gender differences in serum leptin concentrations are well established3,4 and in vitro results suggest that testosterone may be an important regulator of leptin secretion.2 A strong inverse association between serum levels of leptin and testosterone is reported in untreated and testosterone-treated hypogonadal men.5 Congenital adrenal hyperplasia (CAH) is an autosomal recessive disorder of steroid metabolism. The most frequent and best known form is due to deficient activity of 21hydroxylase, causing increases 17α-hydroxyprogesterone (17-OHP) and androstenedione and a reduction in cortisol. Clinical symptoms include virilization, most notable in females, and salt wasting in many affected individuals. A markedly elevated testosterone level in the serum is a feature of CAH. The aim of the present investigation was to assess whether manipulation of testosterone in a girl with CAH would alter serum leptin concentrations independently of changes in body fat mass. Case Report