Cerebral blood volume measured using near-infrared spectroscopy and radiolabels in the immature lamb brain.

Near-infrared spectroscopy (NIRS) is a technique that is increasingly being used for the noninvasive measurement of cerebral blood volume (CBV) in newborn infants, but it has not been fully validated against established methods. These experiments in immature lambs (gestation 92+/-1 d, mean+/-SEM) compared CBV measured using NIRS-derived estimates of oxygenated Hb (n = 5) with CBV estimated with radiolabeled indicators (125I-labeled serum albumin and 51Cr-labeled red blood cells, n = 10). Total brain CBV (mL/100 g tissue) measured using NIRS was 2.5+/-0.2 compared with 2.5+/-0.2 using radiolabels (NS). Regional tissue plasma, red blood cells, and whole blood volumes from radiolabels varied significantly (p < or = 0.05) throughout the brain. Whole blood volume (mL/100 g tissue) was largest in choroid plexus (16.2+/-2.1) and least in white matter (0.7+/-0.1) with a significant hierarchy evident among regions: choroid plexus > cerebellum > cortex > brain stem = midbrain > white matter. Regional plasma and red blood cell distributions were similar to whole blood, being highest in choroid plexus (13.0+/-1.6 and 3.2+/-0.9, respectively), and least in white matter (0.8+/-0.1 and 0, respectively). These data from the immature lamb brain indicate that total CBV measured with NIRS is essentially identical with the volumes obtained using intravascular radiolabels. Among cerebral regions, white matter contributes little to the global blood volume measured with NIRS because its red blood cell content is very low.

Long-term outcome of 51 liveborn neonates with non-immune hydrops fetalis.

To search for an efficient method of management of non-immune hydrops fetalis (NIHF), the clinical outcome of 51 newborns with NIHF was retrospectively assessed in a single centre. As the short-term outcome, the mortality rate was mainly dependent on the causes of NIHF and the presence of pleural effusion. The survival rate of the patients with pleural effusion (7/28; 25%) was significantly lower than that of those without it (14/23; 61%) (p < 0.02). All 7 survivors with pleural effusion were diagnosed antenatally after 29 weeks of gestation and were delivered after 31 weeks of gestation. With respect to the long-term outcome, 13 (68.4%) of 19 patients who survived beyond 1 y of age showed normal development, 2 mild developmental delay at 1 y of age and 1 mental retardation at 8 y of age, while 3 (15.8%) had severe psychomotor retardation with marked growth failure. Two of these three patients were born as very-low-birthweight infants. The follow-up results indicate that pleural drainage in utero and prevention of premature delivery should be proposed to improve the outcome of NIHF patients.

Maturational changes of VEPs in normal premature neonates: a longitudinal study.

In order to clarify the maturational changes of visual evoked potentials (VEPs) in normal premature neonates, longitudinal recordings were obtained for 11 normal premature neonates at low risk, at intervals of mainly 1 week and occasionally 2 weeks. Positive waves around 200 ms, P200, negative waves around 300 ms, N300, and positive waves around 400 ms, P400, were recognized. Since P200 and P400 exhibited more variability in latency and appearance than N300, maturational changes in the N300 component were analyzed. The latency of monophasic N300 showed transient prolongation from 32 to 35 weeks gestational age (GA) and re-shortening after 35 weeks GA, or slow regression from 32 to 35 weeks GA and a steady decrease after 35 weeks GA. The bifid N300 showed a shift of dominancy from the first negative peaks to the second peaks, and after 35 weeks GA latency shortening of the second peaks. These findings suggested that the decrease in N300 latency in the premature period may comprise two stages, a slow regression rate or reverse stage from 32 to 35 weeks GA, and a fast regression rate stage from 35 to 38 weeks GA, and that N300 may consist of two components, early and late ones, with the same polarity. This study indicates that careful attention should be paid to evaluation of VEPs in premature neonates.

Prune-belly syndrome in a female, complicated by intestinal malrotation after successful antenatal treatment of hydrops fetalis.

The authors report a female case of prune-belly syndrome having an antenatal sonographic diagnosis of hydrops fetalis and treated prenatally with transplacental digitalis administration. The findings of this case suggest that the clinical entity includes various phenotypes or aberrants having different origins.

Granulocyte-colony stimulating factor levels in cord blood and neonatal peripheral blood.

We measured the granulocyte-colony stimulating factor (G-CSF) levels in cord blood and peripheral blood obtained from full-term or pre-term infants during the first 3 days of birth. The mean G-CSF level among cord blood (17.2 pg/mL) was similar to that of peripheral blood on day 0 (18.3 pg/mL) and day 1 (13.6 pg/mL), while that of peripheral blood on day 0 was significantly higher than on day 2 (10.9 pg/mL) and day 3 (8.8 pg/mL; both P < 0.05). There was no correlation between neutrophil counts and G-CSF levels. No difference was found in neutrophil counts or G-CSF levels between infants who weighed more or less than 2500 g at birth. These results suggest that the neonatal neutrophil count depends on regulatory factors other than G-CSF.

Serum erythropoietin levels in term and preterm infants during the first year of life.

PURPOSE: To evaluate the kinetics of erythropoietin (EPO) production and address the pathogenesis of anemia of prematurity, we measured EPO levels in infants during the first year of life. PATIENTS AND METHODS: Using a radioimmunoassay, serum EPO levels were measured in 97 infants classified into three groups according to weight: group A, n = 40, < 1,500 g; group B, n = 19, 1,500-2,499 g; and group C, n = 38, > or = 2,500 g. RESULTS: The serum EPO level ranged widely during the early neonatal period from days 0 to 6 (group A, < 5 to 307 mU/ml; group B, 10-340 mU/ml; and group C, 9-108 mU/ml). EPO reached its lowest level (< 20 mU/ml) between days 7 and 50 in all groups. The hemoglobin concentration reached its nadir between days 51 and 150 in all groups, with the lowest concentration observed in low birth weight infants. In contrast, the EPO level during the anemic phase was approximately 20 mU/ml and was independent of birth weight. A negative correlation between serum EPO level and hemoglobin concentration was observed only in group C (r = -0.54, p < 0.05). The negative slope of the regression equation in group C exceeded that of groups A and B (p < 0.05). When the relationship between EPO and Hb was evaluated over periods of 7-50 days, 51-100 days, and > 101 days, respectively, we noted a significant correlation between values on days 7 and 50 in group A (r = -0.53, p < 0.05) and between days 51 and 100 in group B (r = -0.76, p < 0.05). CONCLUSIONS: These data suggest the appreciable EPO production in premature infants, but its insufficient response to the depressed hemoglobin level, implying the need to administer exogenous EPO to infants with anemia of prematurity.

A case of neonatal spinal cord injury: magnetic resonance imaging and somatosensory evoked potentials.

This is the first case report on the diagnosis of spinal cord injury due to hemorrhage during the neonatal period using magnetic resonance imaging (MRI). Somatosensory evoked potentials are also helpful in the functional demonstration of this lesion. When discrepant signs, alert consciousness and intact cranial nerves are observed in newborn babies with flaccid extremities and respiratory disturbance, the immediate carrying out of MRI is mandatory in order to differentiate spinal cord injury from hypoxic-ischemic encephalopathy, cerebral hemorrhage, and neuromuscular disease.

Abnormal behavioral patterns in the human fetus at term: correlation with lesion sites in the central nervous system after birth.

We describe three fetuses at term that demonstrated abnormal behavioral patterns in utero when observed using real-time ultrasound. The abnormal patterns turned out to have neurologic correlations after birth. In case 1, despite a normal breathing pattern, no movement in any of the four extremities was observed, thus suggesting the existence of a spinal cord lesion at the rostral end, located at C4 or C5. In case 2 a lack of breathing movement was noted repeatedly, together with the abnormal finding that alternation of periods of rapid eye movement (REM) sleep with those of non-rapid eye movement (NREM) sleep was not present. These findings implied a lesion involving the pons and/or medulla oblongata. In case 3 movement of the four extremities, breathing movement, and alternating periods of REM sleep and NREM sleep were found to be within the normal range. The concurrence of regular mouthing and the NREM sleep period was not observed, however, suggesting impairment in the brain function responsible for NREM sleep located from the pons through the thalamocortical connection to the cerebral hemisphere.

Cardiac beriberi (shoshin beriberi) caused by excessive intake of isotonic drink.

A 21 month old female had voluntarily ingested 0.5-1.51 of isotonic sports drink daily from 10 months of age. She developed hyponatremia and beriberi heart disease, which resulted in metabolic acidosis and cardiogenic shock (shoshin beriberi). Mechanical ventilation was applied for pulmonary edema. Right heart failure was improved after administering vitamin B1. However, 5 days after the shock, hypoxemia and diffuse radiographic infiltrates progressed, and a diagnosis of adult respiratory distress syndrome (ARDS) was made. After the occurrence of an air leak, the patient died of respiratory failure. The cardiogenic shock and pulmonary edema due to cardiac beriberi may have triggered the ARDS.

Right-sided diaphragmatic hernia following group B streptococcal pneumonia and sepsis.

A case of right-sided diaphragmatic hernia following group B streptococcal pneumonia and sepsis is reported herein. The clinical course was characteristic. The position of the right hemidiaphragm was initially normal. After an antecedent group B streptococcal infection, an abnormal shadow indicating either pneumonia or a pleural effusion on the chest x-ray was recognized and an elevation of the bowel and liver into the right hemithorax gradually appeared. Repair of the hernia was indicated and the postoperative result was excellent. The relationship between a delayed-onset diaphragmatic hernia and a group B streptococcal infection is still unknown. Increased intrathoracic pressure caused by mechanical ventilation coupled with an abnormal lung compliance due to inflammation may have resulted in the delayed herniation. Among various methods for diagnosis applied, chest x-ray and ultrasonography were noninvasive and useful.

Moebius syndrome with central hypoventilation and brainstem calcification: a case report.

Moebius syndrome (MS) is described in an infant with central hypoventilation and brainstem calcification. The patient had limb defects and bilateral paralysis of the 6th, 7th, 9th, 10th, and 12th cranial nerves. Mechanical ventilation was continued from birth because of shallow spontaneous respiration. Computed tomography revealed brainstem atrophy and four small calcifications restricted to the dorsal portion of the pons and medulla. Prenatal brainstem injury such as ischaemia may have caused MS and central hypoventilation.

Significance of prenatal diagnosis in a patient with a huge neck tumor.

Due to the great advancements in fetal ultrasonography, the number of prenatal diagnoses are increasing, greatly contributing to improved neonatal surgery. It is now relatively easy to detect huge fetal cystic masses, and we have experienced three cases with a huge neck tumor detected in utero, one case with teratoma and two cases with cystic hygroma. Each case was complicated by neonatal asphyxia, and the neonate needed resuscitation by means of endotracheal intubation. The infant with teratoma unfortunately died of respiratory distress due to compression of the trachea before a perinatal team could be organized. Although the remaining cases with cystic hygroma were treated by a perinatal team, one died 19 hours after birth and the other has survived with the aid of endotracheal intubation in the hospital for three years. In addition, all four cases of cystic hygroma detected antenatally in our institute, which were not delivered, also had fetal hydrops which suggested a general lymphatic derangement. Cystic hygroma detected in utero is considered to be different from that detected after birth, since the former is associated with genetic lymphatic derangement. Prenatal diagnosis enables such patients to survive the perinatal period, but may not improve the prognosis of fetal cystic hygroma so much.

Improved outcome for infants weighting less than 750 grams at birth: effects of advances in perinatal care, infection prevention and maternal transport for fetus.

Between 1980 and 1987, we investigated the outcome for 20 infants with birth weights less than 750 g, admitted to the neonatal unit of Kyushu University Hospital. All infants were delivered at the hospital. Seven infants (35%) survived and were discharged. Comparing the first and second 4 year periods, the survival rate improved from 17% (1 of 6 infants) to 43% (6 of 14 infants). Intensive perinatal care, prevention of infection and early transport of mothers of high-risk babies improved the outcome. In the long-term, among the 7 survivors, 1 had psychomotor retardation, 1 had epilepsy and the other 5 were normal neurologically. Growth in height and weight of these children remained below the -2SD levels until 2 years of age, thereafter they began to catch up. Growth in head circumference increased to a level above the -2SD value from 2 years of age. With intensive perinatal care, the outcome for infants weighting less than 750 g is improving, and good results may be expected.

Transient neurologic abnormalities and BAEPs in high-risk infants.

We examined brainstem auditory evoked potentials in 2 neurodevelopmentally different groups of high-risk premature infants during the first year of life. Our 77 patients were considered at birth to be at risk for neurologic disabilities, but were found to have normal development in the second year of life. The patients were divided into 2 groups on the basis of their neurologic findings during the first year of life; 24 of the 77 patients demonstrated transient neurologic abnormalities (group I) and the remaining 53 demonstrated normal neurologic findings through the first year of life (group II). Normative data of brainstem auditory evoked potentials were obtained from 60 low-risk and neurologically normal infants. Group I patients had prolonged III-V and I-V intervals at 2 months of age or younger and poorly detectable waves VI and VII at 5 months of age or younger, compared with control subjects. Wave VI was poorly detected in group II patients only at 35-39 weeks of conceptional age. Brainstem auditory evoked potentials suggested that the patients with transient neurologic abnormalities had transient dysfunction or maturational delay in the brainstem and upper auditory pathway early in the first year of life.

Measurement of the fetal urine production rate using real-time ultrasound in fetuses with bilateral hydronephrosis.

The fetal urine production rate (FUPR) in 147 normal fetuses after 27 weeks of gestation and 5 with bilateral hydronephrosis was measured using real-time ultrasound. Later, the correlation between FUPR and renal function was evaluated postnatally. In the normal fetuses, FUPR (mean +/- 2 SD) increased gradually from 1.9 +/- 1.6 at 27-28 weeks of gestation to 7.5 +/- 4.5 ml/10 min at 37-38 weeks of gestation. In the 5 cases with bilateral hydronephrosis, FUPR was within normal ranges and increased with advances in gestation. Intrauterine intervention was not performed in any of the cases, and, at present, renal insufficiency is nil.