Hirokuni Negishi

Nonsurgical management of epidural hematoma in neonates

We successfully treated 4 neonates with epidural hematoma by nonsurgical methods. In 3 of these infants, epidural hematoma was complicated by cephalohematoma and skull fracture; however, the epidural hematoma disappeared after aspiration of the dark red blood in the cephalohematoma, resulting in complete recovery without sequelae. In the remaining infant, epidural hematoma was complicated by intraventricular and subarachnoid hemorrhage and disseminated intravascular coagulation; the patient was successfully treated by conservative therapy, including exchange transfusion and repeated lumbar puncture with only mild motor difficulties remaining. Some patients with neonatal epidural hematomas can be managed by nonsurgical, conservative procedures, including cephalohematoma aspiration.

Femoral and spinal bone mineral density in Japanese osteoporotics with hip fracture

In the present study, bone mineral density (BMD) of femoral neck and lumbar spine was compared between 38 Japanese female patients with hip fracture (age 63–89 years, mean±SD 76±7 years) and 162 age-matched female controls (age 62–90 years, mean±SD 75±7 years). BMD was measured in the femoral neck and lumbar spine (L2–4) using dual-photon absorptiometry (Norland model 2600). BMD values of femoral neck as well as lumbar spine were significantly lower in patients with hip fracture than in controls (0.504±0.097 v 0.597±0.101,p<0.01, for femoral neck; 0.661±0.146 v 0.720±0.128,p<0.05, for lumbar spine). Patients with hip fracture and controls were stratified according to their BMD levels at two measuring sites, and the ratio of the number of patients and controls at each BMD level was calculated as an indicator of fracture rate. This ratio showed an exponential increase as the femoral neck BMD declined, but only a gradual increase as the lumbar spine BMD declined. Specificity-sensitivity analysis revealed that BMD values of 0.59 and 0.54 g/cm2 at the femoral neck provided a specificity of 52% and 68% with a sensitivity of 90% and 75%, respectively. These findings suggest that Japanese patients with hip fracture are more osteoporotic than age-matched controls and that the selective measurement of femoral neck would be useful for predicting the risk of hip fracture.

Platelet glucose-6-phosphatase activity in patients with von Gierke's disease and their parents

The present report describes the results of an assay of platelet glucose-6-phosphatase (G-6-Pase) in patients with von Gierkes disease and their parents. The G-6-Pase activity of the patients was found to be about 10–25% of that of controls, while that of the parents showed an intermediate value between that of the patients and that of the controls, which led to the conclusion that the parents were the heterozygotes of the disease. These results suggest a possible method for the diagnosis of G-6-Pase deficiency as well as for detection of the heterozygotes of the disease by a platelet G-6-Pase assay without making a G-6-Pase assay of liver biopsy materials. As for the separation of platelets, a differential centrifugation was undertaken using Na2 -EDTA as an anticoagulant. In the G-6-Pase assay, universally 14C-labelled glucose 6-phosphate ([U-14C]-G-6-P) was used as a substrate and the enzyme activity was measured by the amount of glucose liberated by the platelet suspension after the reaction. The effect of non-specific phosphatase was estimated by using universally 14C-labelled glucose 1-phosphate ([U-14C] -G -6-P) as substrate. In the diagnosis of von Gierkes disease, as well as the detection of the heterozygotes of the disease by the G-6-Pase assay using platelets as material, the effect of non-specific phosphatase was considered to be negligible.

Glycogen phosphorylase kinase deficiency: A survey of enzymes in phosphorylase activating system

Abstract A four year-old Japanese boy with hepatomegaly and hypoglycemia has low activity of hepatic phosphorylase. A survey of enzymes involved in the phosphorylase activating system has revealed that liver phosphorylase kinase is deficient although adenosine 3′,5′-monophosphate (cyclic AMP)-dependent protein kinase and total phosphorylase measured in a mixture supplemented by rabbit muscle phosphorylase kinase show normal activities. The hormone receptor as well as adenyl cyclase system appears to be normal since cyclic AMP increases immediately after intravenous injection of glucagon. His muscle phosphorylase activating system is normal.

Radial bone mineral content of normal Japanese infants and prepubertal children: influence of age, sex and body size

The present study was performed to measure appendicular bone mass of Japanese infants and children, and to assess the influence of age, sex and body size on bone mass during the period of bone growth. The bone mineral content (BMC) and bone width (BW) at the distal third of the radius were measured by single photon absorptiometry (SPA) in 229 healthy Japanese infants and children aged 0-12 years, and the BMC/BW ratio was calculated to give the bone mineral density (BMD). BMC and BW increased with age until 2 years, while BMD did not obviously change until 2 years. After 2 years of age, the overall effect of aging appeared more prominent in BMC and BMD than in BW. There were no significant differences in BMC, BW and BMD between males and females aged 0-12 years. Age, body height, and body weight were strongly correlated with three parameters of bone mass (BMC, BW, and BMD). Among the three parameters of bone mass, BMC showed the highest Pearson coefficient of correlation with age (r = 0.955), body height (r = 0.957) and body weight (r = 0.966), as compared with BW and BMD. The present cross-sectional study provides normative data of the appendicular bone mass in healthy Japanese children, which may serve as a standard for assessment of bone mineralization in Japanese infants and children with medical problems.

Clinicopathological Differences Between Early-onset and Late-onset Sepsis and Pneumonia in Very Low Birth Weight Infants

We performed clinicopathological studies on early-onset sepsis (5 infants, less than 72 hours of life, EOS) and late-onset sepsis (15 infants, greater than 72 hours, LOS) of very low birth weight, less than 1500 g (VLBW). In EOS, the clinical features mimic the respiratory distress syndrome and hematological changes were not observed. The lungs showed slight interstitial pneumonia with structural immaturity, hyaline membranes, hemorrhage, and minimal infiltration by polymorphonuclear neutrophils (PMNs). The pathogen was group B streptococcus or weakly gram-negative bacilli. In LOS, pneumonia proceeded to sepsis and neutropenia with elevated numbers of circulating immature neutrophils, and increased levels of C-reactive protein were observed at the onset of sepsis. Severe pneumonia with infiltration of numerous PMNs and bacterial colonies and polymicrobial infection by nosocomial pathogens such as Staphylococcus aureus and Pseudomonas aeruginosa were common. The thymus and spleen weights varied but retained normal structure in EOS. The thymus was depleted of lymphocytes, and the spleen was hypertrophic but poorly reactive against infection in LOS. The pathogenesis of EOS is regarded as being more closely correlated with lung immaturity and circulatory disorder in early life, whereas that of LOS is associated with immunological defenses of the host, potency of the pathogens, and terminal multiple organ failure.

Two Cases of Fetal Akinesia/Hypokinesia Sequence

Two cases of fetal akinesia sequence are described. Both showed facial anomalies, arthrogryposis multiplex, bilateral camptodactyly, and pulmonary hypoplasia. One child had degeneration of large motor neurons of the thoracolumbosacral spinal cord and irregular atrophy of diaphragm; the other had left microphthalmia, hemiatrophy of the left temporal lobe with calcification of degenerated neurons, and hypoplasia of the cervothoracic spinal cord with decrease and degeneration of neurons. The iliopsoas and intercostal muscles showed focal myofiber atrophy. These findings suggested that some instances of this fetal akinesia syndrome might be due to neuromuscular dysfunction that occurred in utero and may have various causes.

Arhinencephaly with Normal Karyotype Associated with Hypocalcemic Convulsion

The patient was a male infant who was born at the 39th week of gestation. He had cleft lips and palate, low set ears, myogenic torticollis in the left neck, clubhand in both 5th fingers, dysplasia of the penis, and cryptorchism. Although he had convulsion due to hypocalcemia 9 days after birth, this improved 25 days after birth by the administration of a calcium agent. Five months after birth, cleft lip plastics were performed. Six months after birth, an apnea attack occurred and cyanosis was observed during examination of the optic fundi. Two days later, he died. Chromosomal analyses has revealed a normal chromosome karyotype 17 days and again 4th months after birth. Autopsy findings showed the brain to be normally separated into two cerebral hemispheres but lacking in olfactory bulbi and tracts. Also observed were an atrio‐septal cardiac defect, Meckels diverticulum in the lower small intestine, and enlarged renal pelves. Retinal and choroid defects were seen at the sites where giant papillae were found in examination of the optic fundi.