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We report the case of an infant with Sturge-Weber syndrome whose regional cerebral blood flow (rCBF) showed an increase and a subsequent decrease. As compared with simultaneous CT and MR findings, we speculate that the transient increase in rCBF reflects the neuronal reaction to hypoxia caused by congestion.

We experienced a case of acute necrotizing encephalopathy of childhood with a good outcome. The disease continued for about three weeks, and the patient recovered with a sequela of mild left-hemiparesis. On the brain magnetic resonance imaging, abnormal intensity areas in the thalamus and putamen reduced size at an early stage of the disease. After 1 year, his mental and motor development seemed to be almost normal, but brain single photon emission computed tomography and electroencephalography were abnormal. Subclinical functional abnormalities persisted longer than had been expected.

Background: Clarification of the present status of periventricular leukomalacia (PVL) in Japan., Methods: Questionnaires were mailed to the leading neonatal intensive care units (NICU) and rehabilitation centers for children., Results: The incidence of PVL in the group of surviving preterm infants of gestational ages less than 33 weeks was 4.8-4.9% on ultrasound and 7.7-7.9% on magnetic resonance imaging and/or computed tomography. The incidence of PVL did not decrease between 1990 and 1994. The incidence of PVL in the NICU varied from 0 to 47.1%. Infants from multiple pregnancies had a higher incidence (9.1%) of PVL than those from single pregnancies (6.2%). The proportion of PVL in all patients in rehabilitation centers with cerebral palsy (CP) has increased recently., Conclusions: It was roughly calculated that about 750 cases of CP with PVL occurred annually in Japan, thus accounting for about one-third of the total number of cases of CP.

A review was conducted to determine the trends in exchange transfusion (ET) of newborn infants at the Yodogawa Christian Hospital during the past 18 years. At that hospital in 1957, the first ET was performed on a term infant with severe hemolytic jaundice caused by rhesus factor (Rh) incompatibility. By 1989, ET had been performed in more than 1400 newborn infants. These cases of newborns who had had ET were retrospectively reviewed, with a focus on every 3 year period from 1974 to 1992. The total number of infants requiring ET noticeably decreased from 68 cases (14.0% of total admissions) in 1974 to 19 cases (6.1% of total admissions) in 1992. (chi 2, P < 0.001) There were three major significant changes in ET during those years. The first was a change in the subjects for ET. The incidence of ET for term infants showed a marked decrease, while the incidence of ET for preterm infants, especially for very low birthweight (VLBW) infants (< 1500 g), noticeably increased. The second was a change in indications for ET. There was a marked decrease in the need for ET as a result of hyperbilirubinemia, while the incidence of ET because of other etiologies, such as septicemia and/or disseminated intravascular coagulopathy, noticeably increased. The third was a change in the technical methods of ET. Now at the Yodogawa Christian Hospital, 100% of the infants are given ET with an automated peripheral two-site method, instead of the Diamond method. Although ET might still be a useful treatment for severe hyperbilirubinemia and other acute problems, the total number of ET noticeably decreased in accord with a decrease in the number of severe hyperbilirubinemia in term newborns. On the other hand, the incidence of ET in preterm infants increased relatively, accompanied by an increase in the survival of VLBW infants. The automated two-site method is the preferred technique for ET at the Yodogawa Christian Hospital, rather than the Diamond method. Further changes in ET might occur in accord with new alternative measures in future.

Recent advances in medical technology, such as an assisted ventilation, have made a big impact on pediatrics. With such a progress, many children with intractable diseases have survived intact. On the other hand, chronically ill children with handicaps have also been increasing. Some of them have been artificially supported by a respirator to maintain life in a hospital or at home. Under these social conditions, we should establish a system for total care of these children, to promote their quality of lives, in collaboration with medicine, health & welfare, and education.

Objective: To investigate whether the haemodynamic effects of the standard 2-3 h blood transfusion increases the risk for intraventricular haemorrhage (IVH) and patent ductus arteriosus (PDA) in very low birthweight infants., Methodology: In a randomized controlled study, haemodynamic changes using slow and rapid transfusion were compared. Twenty-seven very low birthweight infants were divided between 12h (n = 14) and 3h (n = 13) transfusion groups. Blood pressure, ejection fraction (EF), anterior cerebral artery pulsatility index (PI), blood gases, serum electrolytes and haematocrit were measured pre- and post-transfusion. Infectious status was also monitored., Results: Blood pressure (48.1/25.5 vs 55.7/30.2 mmHg) and EF (0.68 vs 0.73) increased significantly during rapid transfusion (P<0.01) but remained stable with slow transfusion. Serum potassium, base excess and incidence of infection did not increase in either group., Conclusions: Slow transfusion causes less haemodynamic disturbance than rapid transfusion, thereby preventing the potential risk for IVH and PDA.

Changes in auditory brainstem responses (ABR) were studied and followed in hyperbilirubinemic newborns before and after exchange transfusion (ET), in order to check their usefulness in the early detection of acute and chronic bilirubin encephalopathy. ABR were measured in 10 newborns with marked hyperbilirubinemia (total bilirubin concentration [TBC] > or = 20 mg/dL, direct bilirubin concentration < 2 mg/dL) before and after ET. The means of birthweight, gestational age, and day of life on admission were 3267 g, 38.2 weeks and 3.4 days, respectively. The ABR measurements were performed before ET and 6.0 days (the mean) after the ET. The follow-up of ABR was performed at 3 months of life. In comparison with the control values, the mean latencies of ABR were significantly prolonged (I, I-III, and I-V: P < 0.05) and the mean amplitudes were significantly decreased (I, III and V: P < 0.001) before ET. Significant improvement of ABR was noticed after the ET, especially in the shortening of the latency of wave I (P < 0.02) and in increasing the amplitudes of wave III and V (P < 0.02 and (P < 0.01, respectively), though the recovery of the latency of I-V (P < 0.02) and the amplitudes of I, III and V wave (P < 0.001, P < 0.001 and P < 0.01, respectively) were delayed in comparison to the control. The follow-up data of ABR showed that, in two of nine infants (one was lost from the follow-up), there were still abnormal findings at 3 months of age. Only one of these, who prolonged the recovery of ABR until 5 years of age, developed a border intelligence. Though ET is effective for improvement of acute bilirubin encephalopathy with impaired ABR, a complete recovery of the ABR might be delayed in marked hyperbilirubinemia. The delay in improvement of ABR abnormalities might be possibly used as an early predictor for following chronic bilirubin encephalopathy.

Children who were dependent upon home mechanical ventilation (HMV), suffered in various ways from the disastrous Hanshin-Awaji earthquake disaster. The earthquake abruptly cut the supplies of water, gas and electricity, causing intense anxiety for those families. Through loss of the respirator function, some of them experienced an unexpected catastrophe. In the disaster area, there were children who were dependent upon HMV (19 cases) and children who were preparing for HMV in hospitals (nine cases). Information was gathered from questionnaires about the disaster, communication and correspondence with families. None of the 28 cases died or were injured. Nineteen cases had a variety of problems. In eight cases, respiratory support problems were acute. Nevertheless, all of them survived the crisis successfully even in the midst of such a catastrophic situation. An organization of HMV children's families, called the Baku-Baku Club, helped families with HMV problems by supplying water, food, oxygen and compressed air cylinders among other things. Additional outside batteries for portable respirators are essential equipment for HMV, especially for emergencies. A manual for clarifying the system for support in the Baku-Baku Club and a registration system for public medical service should be established in preparation for such a crisis.

Serum unbound bilirubin concentrations (UBC) and serum total bilirubin concentrations (TBC) were measured serially in 138 low birthweight (LBW) infants treated with phototherapy for non-hemolytic hyperbilirubinemia. We attempted to assign the suitable critical UBC levels for predicting bilirubin encephalopathy into two different birthweight groups: a very low birthweight (VLBW) group (birthweight < 1,500 g) and an LBW group (birthweight between 1,500 g and 2,499 g). Twelve infants were diagnosed as 'at risk' for kernicterus, of whom 11 had signs of acute bilirubin encephalopathy and received exchange transfusion. One VLBW infant had neurological sequelae at a 3 year follow-up, although exchange transfusion was not carried out because of low TBC. Sensitivity and specificity for predicting kernicterus were calculated at different UBC levels between 0.6 microgram/dl and 1.5 micrograms/dl and TBC levels between 8 mg/dl and 26 mg/dl. The receiver-operating characteristic (ROC) curves plotted for UBC as a predictor of kernicterus were clearly shifted up and to the left compared with the curves for TBC in the VLBW and LBW groups. Thus, the UBC measurement may well provide a more rational basis for evaluating the risk of kernicterus in LBW infants. The optimal cut-off points were derived from these curves. In the VLBW group, the sensitivity was 100% and the specificity was 96% for a UBC of 0.8 microgram/dl, and 80% and 64% for a TBC of 11 mg/dl. In the LBW group, the sensitivity was 100% and the specificity was 98% for a UBC of 1.0 microgram/dl and 71% and 78% for a TBC of 16 mg/dl.(ABSTRACT TRUNCATED AT 250 WORDS)

We report on 2 patients with de novo terminal deletion of 6q. The first was a 4-month-old boy whose karyotype was 46,XY,del(6)(q24.3); the second a 2-year-old girl whose karyotype was 46, XX, del(6)(q25.3). The main anomalies in both patients included mental retardation, minor craniofacial and cerebral anomalies, and cardiac defects. The characteristic manifestations were imperforate anus in the first patient, and retinitis proliferans and a triatrial heart in the other. Comparison of clinical findings of our 2 patients with those of 18 previously reported patients with similar phenotypes suggests that terminal deletion of the 6q23 or 6q25 band is critical in producing the main anomalies of del(6q) syndrome.

The prognostic significance of cerebral myelination was evaluated with magnetic resonance imaging (MRI) in very low birth weight infants. Myelination was graded in two specified sites, optic radiation and corpus callosum, based on the stages of normal term babies and healthy premature infants. The subjects were 30 preterm infants weighing less than 1,500 gm at birth. MRI was performed at 4 to 7 months (corrected age). The normal myelination stage was seen in 18 cases, while a delayed stage was noticed in 12 cases. In the normal myelination group, only 1 case (6%) had handicaps. In the delayed myelination group, 8 cases (67%) had handicaps. Our results showed that delayed myelination was closely related to a poor prognosis. We believe that MRI would be a very good imaging modality for predicting the outcome of very low birth weight infants, particularly in terms of evaluation of myelination.

Twenty-eight drugs, including cephem antibiotics and anti-inflammatory agents currently used or considered potentially useful in neonatal intensive care nurseries in Japan, were examined to determine their influence on albumin and bilirubin interaction by means of a glucose oxidase - peroxidase method, using an automated analyzer (Arrows) for unbound bilirubin (U.B.). The apparent binding constant for drugs to the high-affinity site on albumin (KD) was determined. Of cephem antibiotics, latamoxef sodium (LMOX) and cefazolin sodium (CEZ) were found to displace bilirubin from albumin (KD = 5.9 x 10(3) M-1 and 4.5 x 10(3) M-1, respectively) as strongly as Na salicylate (KD = 6.8 x 10(3) M-1). Mephenamate and indomethacin, which are used for medical closure of patent ductus arteriosus in premature infants, were also found to be stronger bilirubin displacers (KD = 1.3 x 10(5) M-1 and 1.2 x 10(5) M-1, respectively) than sulfisoxazole (KD = 1.6 x 10(4) M-1). Maximal displacement factors (MDF's) were also estimated in reference to protein binding (%) and effective serum concentration (M) of each drug in human adults. Of these drugs, mephenamate showed a higher risk of bilirubin displacement (MDF = 3.79) than sulfisoxazole (MDF = 2.58) and LMOX had a higher risk of displacement (MDF = 1.97) than Na salicylate (MDF = 1.85). On the other hand, indomethacin and CEZ showed minimal effects on displacement at therapeutic levels (MDF = 1.03 and 1.00, respectively). At therapeutic serum levels, mephenamate and LMOX may possess the potential for displacing bilirubin from albumin and increasing the risk of bilirubin encephalopathy, in a manner similar to sulfisoxazole.

An automated blood exchange transfusion (BET) with a two-site technique has been devised by Goldmann et al and by us, using an infusion pump. With this method, we successfully performed exchange transfusions 189 times in the past four years on 110 infants with birth weights ranging from 530 g to 4,000 g. The exchange rate by the automated method was compared with the rate by Diamond's method. Serum bilirubin (SB) levels before and after BET and the maximal SB rebound within 24 hours after BET were: 21.6 +/- 2.4, 11.5 +/- 2.2, and 15.0 +/- 1.5 mg/dl in the automated method, and 22.0 +/- 2.9, 11.2 +/- 2.5, and 17.7 +/- 3.2 mg/dl in Diamond's method, respectively. The result showed that the maximal rebound of the SB level within 24 hours after BET was significantly lower in the automated method than in Diamond's method (p less than 0.01), though SB levels before and after BET were not significantly different between the two methods. The exchange rate was also measured by means of staining the fetal red cells (F cells) both in the automated method and in Diamond's method, and comparing them. The exchange rate of F cells in Diamond's method went down along the theoretical exchange curve proposed by Diamond, while the rate in the automated method was significantly better than in Diamond's, especially in the early stage of BET (p less than 0.01). We believe that the use of this automated method may give better results than Diamond's method in the rate of exchange, because this method is performed with a two-site technique using a peripheral artery and vein.

Children who require prolonged respiratory support often have to spend their whole lives in the hospital. It is important, however, for these children to have time with their families in their own homes. Home mechanical ventilation can make it possible. In Japan, the concept of home mechanical ventilation has just begun to open up. We reported five cases of home mechanical ventilation for children by using a portable respirator (Bennett, Companion 2800). Two cases of these were patients with hypoxic ischemic encephalopathy. The others were cases with neuro-muscular diseases (Werdnig-Hoffmann disease, mitochondrial myopathy, myotubular myopathy). Before taking the patients out, parents received a training in nursing procedures and in skillful use of the ventilator and equipment. The patients went out on excursions 17 times, and were released for home stay a total of 25 times. Initially, 12 excursions were undertaken with doctors or nurses in attendance; later, however, all of the home stays and 5 of the excursions were carried out successfully by the family only. They went to places such as their own home, a park, a zoo, an aquarium and a museum. The average time was 4.6 hours (3-8 hours) for excursions, and 1.8 days (1-7 days) for home stays. Questioning of family revealed that home mechanical ventilation accelerated the social and emotional development of these children whose condition necessitated respiratory support, especially patients with neuro-muscular diseases. It also revealed that family could accept the patient as a member with more familiarity and with more ease.(ABSTRACT TRUNCATED AT 250 WORDS)

Ten babies weighing less than 1500 g at birth subsequently developed chronic respiratory insufficiency and bubbly appearance on chest X-ray during their infancy. The clinical diagnoses were compatible with Wilson-Mikity syndrome. They were compared with very low birth-weight infants who did not show these signs and symptoms. On the first day (mean) of life significantly high serum immunoglobulin M levels were found in the study group. The possibility exists that the chronic respiratory insufficiency seen in these very low birth-weight infants may have been caused by intrauterine infection.

A continuous monitoring of the germinal layer by linear scanning ultrasound has been proposed for the purpose of ascertaining the moment of intraventricular hemorrhage (IVH). Using a VHS videotape, we performed the 48 hours monitoring in 7 immature infants weighing less than 1,000 g who required respiratory support. Four cases of these developed IVH. In one case, which was 755 g in birth weight and 24 weeks in gestational age, the moment of IVH was successfully demonstrated on the ultrasonic monitor. At that moment, there were no significant changes in heart rate and systemic blood pressure. No direct manipulation or treatment, such as an endotracheal suctioning or a heel puncture which might induce a blood pressure fluctuation, was being given at the moment of IVH. About 15 minutes after the episode, abnormal seizure-like movement periodically developed.