The effectiveness of intermittent low-dose trimethoprim-sulfamethoxazole (TMP-SMZ) for the prophylaxis of recurrent urinary infection is well established in adults. The present study assessed the effectiveness and safety of intermittent low-dose TMP-SMZ in 35 children (24 boys, 11 girls, aged 1 month to 9 years, median age 5 months) with vesicoureteral reflux; 18 children had bilateral reflux. A total of 53 refluxing ureters were graded as I in 2, II in 16, III in 19, IV in 14, and V in 2 cases. The children were given 1 mg/kg body weight of trimethoprim together with 5 mg/kg of sulfamethoxazole at bedtime every other day for 6 – 50 months (mean±SD, 22.9±11.7 months). None of the boys had a recurrence of urinary infection, while 2 of the 11 girls had a total of 7 recurrences during the prophylaxis period, with a recurrence rate of 0.027 per patient month in girls. Both girls were over 3 years and had a mildly unstable bladder. Transient neutropenia (<1,000/μl) developed in 2 infants during the prophylaxis period, but disappeared spontaneously. Intermittent low-dose TMP-SMZ seemed very effective for the prevention of recurrent urinary infection in children with ureteral reflux even of higher grades.

骨格系の代謝の基本は破骨細胞による骨吸収と骨芽細胞による骨形成の繰り返しである。これらの細胞は全身性因子の統御の下，様々な局所因子を分泌しauto-crine/paracrine 的に互いの機能調整を行っている。 本稿では，その広範な生理的役割が注目されている一酸化窒素 (NO) が骨組織における強力な局所メディエータであることを支持する最近の研究成果を紹介した。とくに，成長期ラットの骨発育においてinducible型のNO合成酵素活性が重要な役割を果たすことを証明したわれわれの研究について詳述した。最後に，NOの骨代謝調節機構を対象とする研究の将来の方向性に触れた。

有意なVURの診断におけるエコー検査の有用性をprospectiveに検討した。初めて尿路感染症を発症した乳児27人 (男24人，女3人) を対象とした。エコー検査により，腎盂のballooningを4人の5腎で，尿管遠位部の4mm以上の拡張を10人の13尿管で，水腎水尿管症を2人の2腎で認めた。これらの所見を認めた10人にVCGを行い，6人の8腎にVURを認めた (IV度3腎，III度2腎，II度3腎)。ballooningを認めた5腎ではいずれもIII度以上のVURを認めた。II度の3腎のうち，2腎では尿管の拡張のみを認め，1腎では全く異常を認めなかった。VCGを行わなかった17人のうち，3人は排尿時のエコーによる観察ができず，尿路感染症が再発したのはこのうちの1人だけであり，再度のエコー検査によりballooningを，VCGによりVURを認めた。尿路感染症を発症した乳児のVURの一次診断として排尿時のエコー検査は極めて有用であった。

The statistical behavior of disease names referred by physicians with the natural language in a large hospital information system is little known despite the theoretical and practical interest. To address this issue, we reviewed and investigated the usage-frequencies of 18,274 disease names, 10,288 for outpatient care and 7986 for inpatient care, referred from October 1983 to June 1992 with the notation of the natural language in Japanese by use of the registration-retrieval system of disease names at Fukui Medical School, Japan. Consequently, we found that the investigated distributions did not conform to the Poisson distribution, but conformed well to the Polya-Eggenberger distribution in both case of outpatient and inpatient care. It implies that the disease names with the natural language are possibly referred by physicians with some interrelations.

A 4-year-old girl was diagnosed as having acute renal failure due to tubulointerstitial nephritis. The girl presented with remittent fever, vomiting and non-oliguric acute renal failure with sterile pyuria and tubular reabsorptive dysfunction. Ultrasound examination revealed that the kidneys were markedly enlarged with diffuse hyperechogenicity in the cortex when the abnormal renal function was present and were restored in size and echogenicity when the renal function normalised. A diagnosis of Yersinia pseudotuberculosis infection was based on a rise in haemagglutination titres against the organism.

A male infant with scaphoid megalourethra, which is a rare congenital anomaly, is presented. At birth he exhibited a grossly swollen, malformed penis and cryptorchidism. The corpus spongiosum was not palpable. The infant had congenital renal failure. Retrograde urethrography revealed a crescent-shaped dilatation of the anterior urethra, and the diagnosis of scaphoid megalourethra was made. Subsequent uroradiological examinations showed that there were also severe urogenital anomalies, i.e. renal hypoplasia-dysplasia, hydronephrosis, hydroureter and vesicoureteral reflux. This disorder is frequently associated with other urogenital anomalies. Therefore, once the diagnosis is reached, a careful investigation for other associated abnormalities should be undertaken.

A 5-year-old boy with short stature, hepatomegaly and motor weakness due to hepatic phosphorylase kinase deficiency is described. Laboratory data showed mild hypoglycaemia and metabolic acidosis, hepatic dysfunction, and a low insulin-like growth factor-I level. Mild hypoglycaemia, marked ketosis and insufficient growth hormone secretion were revealed at night. Serum total and free carnitine levels were low and the acyl/total carnitine ratio was high. Urinary acylcarnitine profile using fast atom bombardment and tandem mass spectrometry showed increased excretion of acetylcarnitine and dicarboxylylcarnitines. These endocrinological and metabolic abnormalities and clinical symptoms were improved with uncooked cornstarch treatment.

To identify patients with nonclassic steroid 21-hydroxylase deficiency, 137 Japanese women who visited our clinics because of infertility and/or hyperandrogenism were tested with adrenocorticotropin (ACTH) stimulation. In women with serum 17α-hydroxyprogesterone (17 OHP) values 60 min after ACTH administration higher than 5 ng/mL, ratios of 17-hydroxypregnanolone (5α) (17OH-PO) (5α) to the sum of tetrahydrocortisone (THE) and tetrahydrocortisol (THF) in 24-hour urine after ACTH-Z administration were determined. None of the women investigated showed any evidence of having the nonclassic form of this deficiency.

Bone mineral density (BMD) of the lumbar spine was measured by dual energy X-ray absorptiometry (DXA) in nine Japanese children with growth hormone (GH) deficiency. Before hGH therapy, BMDs of the GH-deficient patients were lower than those of controls. When their bone ages were substituted for their chronological ages, however, almost all BMDs of the patients were distributed within the normal range. After 12 months of hGH therapy, the BMDs increased in 8 of 9 GH-deficient patients; they were still lower as compared to controls. The relation between BMDs and bone ages suggested delayed skeletal maturation in GH-deficient patients.

We retrospectively studied 12 Japanese children (8 boys, 4 girls) with idiopathic membranous nephropathy (IMN), aged 2.9–15.8 (mean 7.7) years at onset. All patients were identified through either screening or a routine urinalysis; proteinuria was present in all, haematuria, which was macroscopic in 4, in 11. Three had nephrotic syndrome (NS) at or soon after onset. Stages on electron microscopy, performed in 10 patients, were I in 3, II in 5 and III in 2. Steroids alone or with cyclophosphamide were administered to 5 patients, including the 3 patients showing NS. Complete remission of proteinuria occurred in 8 patients 0.3–1.6 (mean 0.6) years after onset, and proteinuria did not recur. After a follow-up of 1.6–11.6 (mean 5.9) years, these 8 patients were in complete remission and the remaining 4 had only mild proteinuria; none had hypertension or impaired renal function. Thus, we infer that IMN in Japanese children may have a better course and outcome than IMN in non-Japanese children. Based on a comparative study of Japanese (previously reported cases added to ours) and non-Japanese (mostly Caucasian) children with IMN, this was confirmed: it is possible that steroid therapy in Japanese patients is more effective in inducing remission of NS and preserving renal function.

Dual x-ray absorptiometry (DXA) (Hologic QDR-1000/W; Hologic, Inc.) was used to measure lumbar vertebral bone mineral density (BMD) in 83 healthy Japanese infants and children (55 boys and 28 girls) aged 0–17 years, and the values obtained were correlated with age, body weight and body height. The lumbar BMD (average of L1–L4 values) increased with age, with a nearly twofold increase found from preschool age to adolescence. It also increased with body weight and body height. Our results on normal Japanese infants and children appear almost similar to those reported in French and American studies. Because of its great precision and accuracy, low radiation exposure and rapid scanning, DXA may be the most suitable for use in infants and children. With normal Japanese data now available with this technique, pediatricians can better detect metabolic bone diseases in infants and children and follow the bone response to medical intervention in patients with these conditions.