Congenital discoid lupus in the newborn.

A female infant, born to a 21-year-old mother with systemic lupus erythematosus, had cutaneous discoid lupus at birth. The lesions resolved spontaneously over the first few months and by the age of 1 year the infant's skin was normal. Other possible complications of this maternal disease are discussed and the need for caution in counselling mothers is recommended.     

Congenital malformations in infants born after IVF: a population-based study

The presence of congenital malformations in infants born after IVF was studied from a register consisting of practically all infants born in Sweden after IVF, 1982--1997 (n = 9111). A further 64 infants were studied using only medical records. It is a nation-wide study and has a population-based control group (n = 1,690,577) and relevant potential confounders have been taken into account. There was an excess of congenital malformations registered in the Medical Birth Registry (n = 516, odds ratio = 1.47) but this excess disappeared when confounders were taken into consideration: year of birth, maternal age, parity, and period of unwanted childlessness (odds ratio = 0.89). For some specific conditions, an approximately 3-fold excess risk was seen: neural tube defects, alimentary atresia, omphalocele, and hypospadias (after intracytoplasmatic sperm injection). No excess risk for hypospadias was seen after standard IVF. Various explanations for these findings are discussed. It is postulated that the excess risk for alimentary atresia, like the excess risk for monozygotic twinning after IVF, is a direct consequence of the IVF procedure. The excess risk for hypospadias after ICSI may be related to paternal subfertility with a genetic background. The absolute risk for a congenital malformation in association with IVF is small.     

Ganglioneuroma in a newborn with early inborn syphilis

A case is reported of a retroperitoneal sympathic nodes ganglioneuroma in a newborn with classic early inborn syphilis involving practically all systems of the viscera, skin, placenta. Some peculiar morphological features of the tumour are described. Possible teratogenic and oncogenic effects of treponema pallidus on embryonal fetal structures during intrauterine development are suggested.     

Congenital chloridorrhea in Korean infants

The present paper describes two Korean male infants, 1. 16 year old and newly born neonate from two families who were diagnosed and managed for one of very rare inborn errors of metabolism, congenital chloridorrhea (Darrow-Gamble syndrome). The diagnosis was suggested by one of the authors (HRM) from the unusual combination of metabolic alkalosis with severe gastrointestinal disorder presenting with chronic, profuse watery diarrhea in the newborn period in the first patient; and the maternal polyhydramnios, the appearance of dilated fetal bowel loops on prenatal ultrasonography and profuse watery diarrhea beginning at birth without passage of meconium in the second patient. The diagnosis was confirmed in both patients by examination of the stool chloride concentration which revealed extremely high exceeding the sum of sodium and potassium concentrations. Serum electrolytes and arterial blood gas analyses revealed hyponatremia, hypokalemia and hypochloremia with elevated bicarbonate. With replacement of fluid and electrolyte deficit and adequate dietary supplements of potassium and chloride, both patients remained well although the character of the stools waxed and waned. This is the first reported case of congenital chloridorrhea in korean population.     

Transportation of newborn infants

The benefit of an organized neonatal transport system is well established. Over a 30 month period, January 1987 to June 1989, 172 babies were transported to the Dublin maternity hospitals. Birth weights ranged from 640 to 5,180g 106 (62%) were less than 37 weeks gestation. Indications for transport included respiratory distress syndrome (54), prematurity only (43), convulsions and/or neurologic dysfunction (23), jaundice (11) and apnoea (11). One hundred and sixty-eight were transferred by ambulance and four by helicopter. Twenty travelled more than 100 miles. Forty (23%) received assisted ventilation during transport. On arrival 37 (21%) had temperature less than 36 degrees C; 22 (13%) had blood sugar less than 2.2 m mol/l and 34 (20%) had arterial ph less than 7.25. Fifty per cent of referral letters had incomplete information. Treatment and care given en route was recorded in only 28 babies. Twenty-four babies (14%) died. Infants who died were more likely to have been of low birth weight, travelled a long distance, been hypothermic, had poor arterial gases, had blood sugars less than 2.2 m mol/l, and had poor referral letters. This review indicates that death and morbidity continue to be associated with the present system of postnatal transfer of newborn infants. The urgent need for an organized neonatal transport service remains unmet.     

Congenital abnormalities in newborn lambs after infection of pregnant sheep with Akabane virus.

Akabane virus (a Bunyavirus) has been associated with epizootics of congenital deformities in cattle, sheep, and goats. Experimental studies using mouse-adapted virus inoculated intravenously into pregnant sheep gave an inapparent infection. Neutralizing antibodies were detected on day 5, and peaks in the titer were seen at days 10 and 48. Ewes infected at day 30 to 36 of pregnancy produced five (31% incidence) deformed lambs. Sera from four of these possessed neutralizing antibodies to Akabane virus before ingesting colostrum. Two lambs had arthrogryposis, hydranencephaly, kyphosis, scoliosis, and brachygnathia; one had micrencephaly; and the other two had porencephaly. The two lambs with arthrogryposis and hydranencephaly also had extensive lesions in other tissues. In the spinal cord there was a marked decrease in the number of ventral horn neurones and a depletion of myelin. Skeletal muscles showed marked atrophy. The medulla of the thymus possessed large Hassall's corpuscles and a reduced number of thymocytes in the cortex. It would appear that the pathogenic effects of Akabane virus are related to the gestational age (30 to 36 days) at which the fetus is infected. Akabane virus can now be included in the growing list of teratogenic viruses and provides an interesting system for studying such congenital diseases.     

Immune studies in infants with congenital syphilis.

Seventeen neonates with congenital syphilis were studied to determine the immune response of the fetus following intra-uterine infection with Treponema pallidum. The results were compared with those from healthy controls matched for gestational age, birth weight and sex. B cells, IgM, and circulating immune complexes were significantly elevated in the infected newborns. There were no differences in lymphocyte transformation to phytohaemagglutinin (PHA) and in the CD3, CD4, and CD8 lymphocytes between infants with congenital syphilis and controls. Newborns with congenital syphilis have a heightened humoral response but no quantitative abnormality in cell-mediated immunity. Speculation on the role of the circulating immune complexes is presented.     

Congenital abnormalities in newborn calves after inoculation of pregnant cows with Akabane virus.

A fresh isolate of Akabane virus was inoculated intravenously into 11 seronegative pregnant cows at 62 to 96 days of gestation. Two of the cows were slaughtered 18 days post-inoculation, and the fetuses were examined; the remaining cows were allowed to give birth. All the inoculated cows developed viremia and neutralizing antibody for the virus, indicating that the cows were actually infected with the virus, although fever or any other clinical abnormalities were not noted. The virus further infected the fetuses. This was proved by virus isolation in one of the two fetuses from the slaughtered cows, and polymyositis was noted in both fetuses. Six of seven calves born alive had anti-Akabane antibody in their precolostral sera, indicating that in utero infection with the virus took place in these calves. Some of the in utero-infected calves demonstrated congenital abnormalities such as cerebral defect, hydranencephaly, and arthrogryposis. These findings provide additional evidence that Akabane virus is the etiological agent of epizootic abortion and congenital arthrogryposis-hydranencephaly syndrome in cattle.     

Congenital syphilis in newborn rabbits: immune functions and susceptibility to challenge infection at 2 and 5 weeks of age.

Experiments were performed to further elaborate on our congenital syphilis rabbit model. Attempts were made to determine whether in utero exposure to Treponema pallidum would stimulate immune reactivity and whether this activity would, in turn, affect lesion development upon challenge infection. Newborn rabbits aged 2 or 5 weeks were obtained from control does or from does infected intravenously with T. pallidum during pregnancy. Congenitally infected newborns exhibited increased immunologic functions. Concanavalin A-induced T-lymphocyte proliferation was elevated at both 2 and 5 weeks. In addition, macrophage Ia expression and RPR antibody titers were increased at 5 weeks. In separate experiments, newborn rabbits from control does or from does infected during pregnancy were challenged intradermally with viable organisms at either 2 or 5 weeks of age. Subsequent lesion severity was markedly increased in those newborns previously exposed to treponemes in utero. These observations further strengthen our model for congenital transmission of T. pallidum during pregnancy. We propose that at least some of the tissue pathology in syphilitic infection is associated with activated host defenses.     

Lymphocyte subpopulations in the blood of newborn infants

Assays of lymphocyte subpopulations and function have been applied to cells from the cord blood of twenty-four infants. The results are compared with those obtained in healthy adults. T cells, assayed by spontaneous rosette formation with sheep red blood cells (E rosettes) were present in lower proportion in cord (53%) than in adult blood (65%). There was a higher proportion of lymphocytes bearing stainable immunoglobulin in cord (32%) than in adult blood (22%). From the blood lymphocyte counts it was calculated that both T and B lymphocytes are present in greater numbers in the newborn infants' blood than in adults. Comparison of DNA synthesis showed that cord blood leucocytes had a higher spontaneous rate, but there were only minor differences in the lymphocyte mitotic response to phytohaemagglutinin (PHA). The response of cord blood lymphocytes was slightly lower to a submaximal stimulus and higher to a maximal stimulus. There was a correlation between the submaximal response and the proportion of E rosetting cells.

The most striking differences between infant and adult blood lymphocytes were in their cytotoxic activity against homologous target cells (Chang cells). Antibody-dependent cytotoxicity (K-cell activity) was readily detected using cord blood leucocytes, though it was lower than that of adult cells. PHA-induced cytotoxicity was very low in all cord blood samples, and in many cases was almost unmeasurable. This dissociation between the two types of cytotoxic activity is consistent with other evidence that they may be mediated by different cell types.

The assays were also applied to blood samples taken from five mothers of tested infants immediately after delivery. While some differences from normal adults were found with the mothers' lymphocytes they did not mirror those of the cord blood samples. This suggests that the pattern found for cord blood lymphocytes is not due to maternal factors crossing the placenta.

     

Disseminated herpes simplex in newborn infants

Six cases of disseminated herpes simplex virus infection in newborn infants have been collected. The morbid anatomical and histological findings are described for the various tissues of the body. For the first time, the typical lesions were found in the lung. This and the other findings are discussed from the aetiological point of view.     

Auditory temporal grouping in newborn infants

Adults normally perceive auditory scenes in terms of sound patterns emitted by concurrently active sources. Thus pattern formation is an important process of auditory object perception. The aim of the present study was to determine whether neonates group sounds by repeating pitch patterns. Standard ("S"; p=80%) and deviant tones ("D", p=20%) differing only in pitch were delivered either in a randomized order (random condition) or in a repeating SSSSD pattern (grouped condition). Both event-related brain potentials and gamma-band activity differed between the S and D tones in the random condition but not in the grouped condition. These results suggest that in the grouped condition, the S and D tones were processed as part of the same higher order regularity by the neonate auditory system. Also, for the first time, we observed oscillatory gamma-band activity in neonates, which was sensitive to infrequent pitch changes.