Low-dose ACTH therapy for West syndrome: initial effects and long-term outcome.

BACKGROUND: Most Japanese pediatric neurologists attempt other treatments before using adrenocorticotropic hormone (ACTH) therapy for West syndrome (WS), and even then, they use only a low-dose synthetic ACTH to avoid serious adverse effects. In this multi-institutional study, the authors analyzed the initial effects, adverse effects, and long-term outcome in patients treated with low-dose synthetic ACTH in Japan. METHODS: The medical records of 138 patients with WS, who were treated with low-dose synthetic ACTH therapy for the first time at the authors' institutions between 1989 and 1998, were analyzed. RESULTS: At the end of ACTH therapy, excellent effect on seizures was noted in 106 of 138 (76%) patients, good effect in 23 (17%), and poor effect in 9 (7%). Initial effects on EEG were excellent in 53 of 138 (38%) patients, good in 76 (55%), and poor in 9 (7%). As for seizure prognosis at the time of follow-up, 51 of 99 (52%) patients were seizure-free, whereas 48 (48%) patients had seizures. Mental outcome was normal in 6 of 98 (6%) patients, mild mental retardation in 16 (16%), moderate mental retardation in 26 (27%), and severe mental retardation in 50 (51%). The initial effects of ACTH on seizures and long-term outcome were not dose dependent (daily dosage 0.005 to 0.032 mg/kg, 0.2 to 1.28 IU/kg; total dosage 0.1 to 0.87 mg/kg, 4 to 34.8 IU/kg). The severity of adverse effects correlated with total dosage of ACTH, and the severity of brain volume loss due to ACTH correlated well with the daily dosage and total dosage of ACTH. CONCLUSION: Low-dose synthetic ACTH therapy is as effective for the treatment of WS as the higher doses used in previous studies. The dosage of synthetic ACTH used in the treatment of WS can be decreased as much as possible to avoid serious adverse effects.

[Historical notes on anatomy of the transversalis fascia].

In the early 19th century, the tissue of the peritoneum was regarded as the duplicature of membrane. In the middle of the century, however, it was considered to be one of serous membranes, because histology had been developed. Between the peritoneum and the abdominal muscles, there are two fasciae, the subperitoneal and the transversalis fascia. But, Sir Astley Cooper reported that there was only the transversalis fascia, because he considered the subperitoneal fascia to be a double covering of the peritoneum, that is, a part of the duplicature of the peritoneum. In this century, Cooper's report has been interpreted through histology. Consequently, it is the general opinion that there is no other fascia than the transversalis fascia between the peritoneum and the abdominal muscles, in a view which disregards the subperitoneal fascia.

Urinary selenium excretion in infancy: comparison between term and preterm infants.

We evaluated the urinary excretion of selenium (Se), an essential component of glutathione peroxidase, in infants aged 1 week and 1, 4, and 7 months and examined the relationship between urinary Se and renal function. Daytime spot urine samples from a total of 80 infants were analyzed. The Se concentration in urine was measured by fluorometry, while the beta 2-microglobulin content, an index of renal tubular function, was determined by radioimmunoassay. In healthy term infants, the urinary Se excretion showed a peak level at 1 month of age. In contrast, the urinary Se concentration rapidly decreased in preterm infants from 1 week to 7 months postnatally. The level at 1 week in preterm infants was significantly higher than that in term infants, although the mean levels at 1, 4, and 7 months were lower in preterm infants. The level of urinary Se did not correlate significantly with the beta 2-micro-globulin concentration, either in term or preterm infants at any age investigated. Our results suggest that the renal excretion of Se probably reflects the body stores of Se and is not related directly to the renal function in infants. Urinary Se, a noninvasive marker of the Se status, may be used for diagnosis and follow-up of Se deficiency in infants.

Bone mineral status in preterm-born children: assessment by dual-energy X-ray absorptiometry.

To elucidate the long-term consequences of osteopenia of prematurity, lumbar spinal bone mass was measured in 21 preterm-born children aged 3-4 years by dual-energy X-ray absorptiometry. Their mineral intake remained low during early life, and all 11 infants previously studied were osteopenic at term postconception. At the age of 3-4 years, however, all 21 children were found to have normal bone mineral content and density with slightly elevated serum osteocalcin levels. Our results show that in preterm-born children spontaneous resolution of lumbar spinal osteopenia occurs during early childhood.

Pediatric total colonoscopy.

One hundred fifty-one total colonoscopies were performed in 105 children ranging from 1 month to 15 years of age. The cecum was reached successfully in 136 examinations without complications, generally within 30 minutes. Our major indications for total colonoscopy were abnormal findings in a contrast enema, undiagnosed rectal bleeding, and possible organic lesion causing intussusception. All procedures were performed using sedation only. The pediatric gastroscope was employed for total colonoscopy on infants less than 6 years of age; the cecum was reached successfully in 98 of 106 procedures with this instrument. Our total colonoscopy series contributed to the diagnosis of various disorders in 86 children and of normality in the 19 remaining children. The results suggest that, if performed with proper precautions and techniques, total colonoscopy is a safe and useful procedure with which to examine the entire colon of infants and children.