Masami Shimada

Trace elements and mineral requirements for very low birth weight infants in rickets of prematurity

To establish mineral and trace element requirements for very low birth it is important to prevent bone mineral disorder. Those infants fed mothers milk only are thought to be at higher risk of this disorder. Both calcium and phosphorus supplementation were thought to be needed to prevent it. Copper and zinc are important as cofactors of major enzymes involved in the synthesis of collagen. These trace elements especially zinc may not be enough for very low birth weight infants fed mothers milk. At present however the relationship between these trace elements and minerals, and bone metabolic disease in preterm infants is not completely clear.

Nitric Oxide and Endothelin 1 during Postnatal Life

Nitric oxide (NO) is a potent vasodilator produced by endothelial cells. Endothelin 1 (ET-1), another agent made by endothelial cells, is the most potent vasoconstrictor known to date. Endogenous NO and ET-1 may play a part in the normal physiological pulmonary vascular changes during the postnatal period. However, the changes of NO and ET-1 in healthy neonates have not been defined. We determined serum NO metabolites, i.e., nitrites and nitrates, and plasma ET-1 in 19 healthy neonates at birth (cord blood) and at ages 5 and 30 days. The sums of serum nitrite and nitrate (NOx) levels were 27.5 +/- 12.8, 53.8 +/- 14.2, and 38.3 +/- 13.2 mumol/l at birth, age 5 days, and age 30 days, respectively. The plasma ET-1 concentrations were 3.9 +/- 1.6, 1.1 +/- 0.2, and 1.1 +/- 0.2 x 10(6) mumol/l at birth, age 5 days, and age 30 days, respectively. These changes in healthy neonates suggest the presence of active physiological roles for NO and ET-1 in circulatory adaptation to extra-uterine life.

Plasma concentration of granulocyte‐colony‐stimulating factor in neonates

We determined the plasma concentration of granulocyte‐colony‐stimulating factor (GCSF) and the neutrophil count in 108 infants (gestational age 23–41 weeks; birthweight 478–4935 g). The GCSF levels in the very low birthweight infants without infection were comparable to those in the full‐term infants. Infants as premature as 23 weeks of gestation showed similar GCSF levels to mature neonates. GCSF levels decreased significantly by day 7 after birth. The levels were not significantly correlated with the neutrophil count. The mean plasma level of GCSF increased significantly when infection developed and was significantly higher in the infants with sepsis than in those with non‐septic infections (p < 0.01). The results suggest that GCSF may be the major determinant of neutrophil kinetics both during fetal life and after birth.

Prediction of transfusions in extremely low-birthweight infants in the erythropoietin era.

Background: The purpose of the present paper was to detect the clinical factors most predictive of red blood cell (RBC) transfusion in extremely low‐birthweight (ELBW) infants in the recombinant human erythropoietin era.

Autologous cord blood transfusion in an infant with a huge sacrococcygeal teratoma.

Abstract We describe a case of cord blood harvest for autologous transfusion in a neonate weighing 3,992 g with a giant sacrococcygeal teratoma. The umbilical vein was pierced with an 18-gauge needle, and placental blood was withdrawn into two 50-ml syringes filled with 4 ml of citratephosphate-dextrose solution. Resection of the sacrococcygeal teratoma was performed on day one. During the operation the infant lost 46 ml of whole blood, more than 15% of the estimated total blood volume, and thus underwent autologous transfusion with 27.8 ml of packed red cells obtained from autologous cord blood. Consequently, she could avoid homologous blood transfusion during the hospital stay. This case highlights the safety of this procedure, with no evidence of consumption coagulopathy, hemolysis or bacterial infection.

USING POLYMERASE CHAIN REACTION FOR RAPID SEX-DETERMINATION OF A CASE OF SIRENOMELIA

Abstract A rare case of sirenomelia with its autopsy findings is reported. The 1188 g infant was delivered by cesarean section at the 36th week of gestation, and died at 4 h of life. Physical and pathologic findings included sirenomelia (a single lower extremity mass), lung hypoplasia, bilateral renal agenesis, blind‐ending colon, severe deformity of the bony pelvis, an imperforate anus, absence of the bladder, and a single umbilical artery. Because of the difficulty in determing the sex of the infant, PCR directed against the SRY gene was performed. The PCR‐based sex determination is a rapid technique in patients with ambiguous genitalia.

Marshall‐Smith Syndrome: Report of a Case and Review of the Literature

ABSTRACT Marshall‐Smith syndrome is a rare disorder characterized by accelerated skeletal maturation, bullet‐shaped proximal and middle phalanges, dysmorphic facial features, and failure to thrive, and is often associated with mental retardation of variable degree. We describe an 8‐month‐old female with this syndrome, who has a hypoplastic corpus callosum and extreme upper airway obstruction requiring tracheostomy. Also, we review the previous reports of this syndrome since 1971 (Marshall et al., 1971).

BACTERIAL CHANGES IN NEONATAL INTENSIVE CARE UNIT

Organisms routinely cultured from throat swabs and infectious agents of sepsis and/or meningitis were reviewed. During the last 12 years, Klebsiella pneumoniae and Escherichia coli have been replaced by Staphylococcus aureus and Pseudomonas aeruginosa as the predominant isolates from throat swabs after admission. These change in the etiologic pattern of infectious agents of sepsis and/or meningitis, i.e., K. pneumoniae, E. coli, S. aureus, P. aeruginosa and staphylococcus epidermidis, were in agreement with the organisms isolated from the throat swabs after admission. The S. aureus isolated from throat swabs after admission showed a decrease in the bacterial activity of cloxacillin, cephazolin and cefotaxime since 1978.

A Case Report of Lethal Multiple Pterygium Syndrome

We report a patient with a lethal multiple pterygium syndrome. The patient was the first child born to a 28‐year old mother. The family history was unremarkable; no consanguinity was reported. The patient was the product of a 37‐week gestation by cesarian section, and admitted to our intensive care unit after resuscitation. He weighed 2,158 g, and measured 33cm (CHL). The patient had hydrops, cystic hygroma on the back of the neck, hypertelorism, a flat upturned nose, a highly arched cleft palate, micrognathia, low set ears and multiple pterygia. In addition, the finger showed slender and hypoplastic ridges and creases. The feet showed rocker‐bottom deformities. Furthermore, the genitalia was normally formed male with bilateral cryptorchism. The spine showed scoliosis and lordosis with vertebral bony fusions, separations and reduced intervertebral spaces. The ribs were 11 pairs and gracile. The lung was hypoplastic, and the heart was small but normal in structure. Chromosomal examination revealed a normal male karyotype (46, XY). The infant died within two hours after birth.