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PubMed2004

Neonatal diabetes mellitus and neonatal polycystic, dysplastic kidneys: Phenotypically discordant recurrence of a mutation in the hepatocyte nuclear factor-1beta gene due to germline mosaicism.

Mutations in the gene coding for hepatocyte nuclear factor-1beta (HNF-1beta) have been known to cause a form of maturity-onset diabetes of the young (MODY5), which is usually characterized by dominantly inherited adolescence-onset diabetes mellitus associated with renal cysts. This report, however, describes recurrence of a novel missense mutation in the HNF-1beta gene, S148W (C443G), in two sibs, one with neonatal diabetes mellitus and the other with neonatal polycystic, dysplastic kidneys leading to early renal failure. The former patient had only a few small renal cysts with normal renal functions, and the latter had only a transient episode of hyperglycemia, which resolved spontaneously. Interestingly, both parents were clinically unaffected, and PCR restriction fragment length polymorphism analysis showed that the mother was a low-level mosaic of normal and mutant HNF-1beta, which suggested that the recurrence was caused by germline mosaicism. This is the first report of permanent neonatal diabetes mellitus caused by a mutation of the HNF-1beta gene as well as the first report of germline mosaicism of this gene. In addition, the two cases described here show that additional factors, genetic or environmental, can have a significant influence on the phenotypic expression of HNF-1beta mutations.

Child↗
PubMed1997

Magnesium sulfate in labor and risk of neonatal brain lesions and cerebral palsy in low birth weight infants. The Neonatal Brain Hemorrhage Study Analysis Group.

OBJECTIVES: We tested the hypothesis that administration of magnesium sulfate in labor protects against the development of neonatal brain lesions and cerebral palsy (CP) in low birth weight infants. METHODS: Magnesium exposure was ascertained in a population-based cohort of 1105 infants weighing 2000 g or less through review of medical records of maternal magnesium sulfate administration and, where available, elevated maternal serum magnesium levels. Neonatal germinal matrix/intraventricular hemorrhage and parenchymal brain lesions were ascertained by a prospective, timed ultrasound scanning protocol in the first week of life. CP was ascertained at 2 years of age by clinical examination in 80% of survivors and by interview and medical record review in another 6% and was classified as disabling or nondisabling. RESULTS: No significant reduction in risk of nondisabling CP (adjusted odds ratio [OR], 1.00; 95% confidence interval [CI], 0.53 to 1.88) or disabling CP [DCP] (adjusted OR, 0.63; 95% CI, 0.32 to 1.24) CP with magnesium exposure was found in a logistic regression model that controlled for gestational age, fetal growth, gender, multiple birth status, mode of delivery, amnionitis, and hypertensive disorders. In a small subset of infants, those with onset of parenchymal lesions at 7 days of age or later (n = 29), magnesium exposure was associated with a significantly reduced risk of DCP (OR, 0.10; 95% CI, 0.02 to 0.65). Magnesium sulfate exposure was not associated with germinal matrix/intraventricular hemorrhage (adjusted OR, 0.89; 95% CI, 0.64 to 1.25) or with parenchymal brain lesions (adjusted OR, 0.83; 95% CI, 0.53 to 1.30). CONCLUSIONS: The hypothesis that magnesium sulfate use reduces the risk of neonatal brain lesions or CP in low birth weight infants was not statistically supported in this study, although a modest reduction in risk of DCP cannot be excluded. The data further suggest that magnesium exposure may be associated with reduction in risk of CP in low birth weight infants who have late-onset brain lesions, but this unpredicted observation requires confirmation in another data set.

Adult↗
PubMed1981

Combined effects of neonatal thyroxine treatment and neonatal castration on sebum production in adult male rats.

Newborn Sprague-Dawley rats were castrated within 24 h of birth and control animals were sham-operated. Intact female rats were also included for comparison. One-half of the rats in each group was treated with 28 micrograms thyroxine (t4) daily for the first week of life. The secretion rate of sebum was measured at the age of 80 days from the amount of skin-surface lipids that could be extracted with acetone and which had been produced during 2 days. Castration of rats at birth decreased the rate of sebum secretion and neonatal castration plus neonatal thyrotoxicosis produced an even lower level of sebum which more nearly approached that seen in female rats with neonatal T4 treatment. It was concluded that a reduction in the response of the sebaceous glands in adult rats made thyrotoxic with high doses of T4 in the early stage of life is not due to a decreased secretion of gonadal steroids.

Animals↗
PubMed1991

Colfosceril palmitate. A review of the therapeutic efficacy and clinical tolerability of a synthetic surfactant preparation (Exosurf Neonatal) in neonatal respiratory distress syndrome.

Colfosceril palmitate (dipalmitoylphosphatidylcholine) is the primary surface-active agent of natural lung surfactant and the major constituent of exogenous surface replacement preparations. Exogenous surfactants derived from either natural (i.e. animal and human) or synthetic sources are indicated for the prophylaxis and treatment of neonatal respiratory distress syndrome. One of the synthetic surfactants, Exosurf Neonatal, is the focus of this review. This preparation is composed of colfosceril palmitate plus cetyl alcohol and tyloxapol, which facilitate rapid spreading and adsorption of the surface-active agent at the air-alveolar interface. For review purposes, this preparation is referred to only as colfosceril palmitate. Comparative trials with air placebo have shown that colfosceril palmitate improves clinical outcome in infants weighing greater than 700g at birth by reducing mortality and increasing the number of infants who survive without bronchopulmonary dysplasia. It also reduces the number of deaths from respiratory distress syndrome and decreases the incidence of air leak events such as pulmonary interstitial emphysema and pneumothorax. Although colfosceril palmitate itself is very well tolerated and does not increase the incidence of most complications of prematurity or of respiratory distress syndrome, its use is associated with a higher incidence of apnoea of prematurity and pulmonary haemorrhage compared with air placebo, possibly because of earlier extubation of surfactant-treated infants following an improved clinical course and decreased pulmonary vascular resistance secondary to improved ventilation, respectively. Colfosceril palmitate thus has an established efficacy in the prophylaxis and treatment of premature infants with respiratory distress syndrome. Ongoing trials may identify whether prophylactic or rescue administration of the surfactant preparation is the preferred approach and whether different dosage regimens or different administration techniques impart greater therapeutic efficacy. Importantly, it also remains to be determined whether any of the available surfactant preparations, including Exosurf Neonatal, will provide distinct therapeutic advantages over the others.

1,2-Dipalmitoylphosphatidylcholine↗
PubMedDate not supplied

[Assessing the efficacy of the recombinant human granulocyte colony-stimulating factor "rhG-CSF" in the treatment of early neonatal sepsis in premature neonates]

OBJECTIVE: To evaluate the efficacy of the recombinant human granulocyte colony-stimulating factor (rhG-CSF) in the treatment of early-onset neonatal sepsis among premature infants.MATERIALS AND METHODS: A double-blind, randomized, placebo-controlled trial was performed among forty-four preterm neonates who had "clinical diagnosis" of early-onset sepsis. The treatment group (n=22) received 10 micro g/kg/d of rhG-CSF, IV once daily for three consecutive days, and the placebo group (n=22) received the same volume of a visually-indistinguishable vehicle. Prior to the first dose, and prior to the second and third doses, and again 10 days after the first dose, we measured tumor necrosis factor-a, interleukin-6, granulocyte-macrophagocyte colony-stimulating factor, G-CSF, leukocyte count, absolute neutrophil count, immature/total neutrophil ratio, platelet count, and hemoglobin concentration. A bone marrow aspiration was performed seven days after the first dose, and both the neutrophil storage pool (NSP) percent and the NSP/NPP (neutrophil proliferative pool) ratios were tabulated.RESULTS: The treatment and placebo groups were of similar gestational age (29-/+ 3 vs 31-/+ 3 weeks) and birth weight (1376 -/+ 491 vs 1404 -/+ 508 grams). They had similar Apgar scores and 24 hour SNAP scores. No deaths occurred during the first week of life among the treatment group while three deaths occurred in the placebo group. RhG-CSF treatment did not alter the serum concentrations of the cytokines measured (except for G-CSF). Serum G-CSF levels, blood leukocyte counts, absolute neutrophil counts, NSP percentages, and NSP/NPP ratios were higher in the treatment group 24 hours and 72 hours after dosing. The occurrence of a subsequent infection over the two week period following dosing was significantly lower in the treatment group (n=2) than in the placebo group (n=9; p<0.02, RR 0.19 [0.05-0.78]). The overall mortality rate during the entire hospitalization was not different between treatment and placebo groups.CONCLUSIONS: Administration of rhG-CSF to premature neonates with the clinical diagnosis of early-onset sepsis was associated with lower incidence of nosocomial infection over the ensuing three weeks period, but it did not change the overall mortality rate.

Journal Article↗
PubMedDate not supplied

[Neonatal reflexes variability in the normal full term neonate during the neurological exam].

It has been stated that findings in neonatal neurological examinations can play a role as a pointer to adverse developmental outcome. The description of the examinations, and their presence or absence differed among authors without clear reference to their physiological variability ranges. We approached the study of some neonatal behaviours and reflexes in 143 examinations made on 113 healthy newborn babies from the maternity wards by looking at the influence that perinatal environmental factors which are considered normal, can play in these examinations. The results showed that the flexion answer of the Babinski reflex increased significantly between the first and third day of life (c2= 4.4478; p= 0.03495) in the same way there was an increase in the stepping reflex (c2= 3.999; p= 0.04552) in the righting reflex (c2= 4.9342; p= 0.02633) and in the supporting reaction (c2= 11.7874; p= 0.0006). This was significantly reduced in the caesarean section deliveries (c2= 7.1209; p= 0.0076). Head reaction was only detectable during the Brazelton behavioural states 1 to 3 (c2= 3.8911; p= 0.04854) and the same thing happened with the stepping reflex (c2= 4.9370; p= 0.02629). We discuss the utility of neonatal reflexes scoring scales in predicting the neurodevelopmental outcome of the newborn baby.

Female↗
PubMed2003

Unexpected persistence of platelet hyporeactivity beyond the neonatal period: a flow cytometric study in neonates, infants and older children.

Previous studies, using flow cytometry, have reported a lower platelet reactivity in neonates compared to adults. Only few studies were carried out in older children, and results were controversial in terms of age to reach adult platelet function. We studied a total of 125 healthy neonates, infants and older children, and 15 adults. alphaIIbbeta3 expression on resting and activated platelets was lower in all children, with an impaired capability of alphaIIbbeta3 activation (PAC1 and bound fibrinogen). This defect was observed until the age of fifteen with a gradual recovery with age. In neonates, we observed a defect of GPIalpha internalization, and demonstrated that this defect persisted in older children as well. In contrast with alphaIIbbeta3 integrin activation, we did not observe a gradual age-dependent recovery. These unexpected results point out the need for reference values in childhood.

Adolescent↗
PubMedDate not supplied

The neonatal morbidity and mortality of low birth weight neonates.

During the period between January 1, 1987 and March 31, 1991, a total of 454 low birth weight neonates, classified if below 2,500 grams, admitted to this hospital, 450 of them could be evaluated retrospectively. Among the 450 cases, 41 (9.1%) were transferred from outside hospitals within 24 hours of birth; and 409 were original hospital inpatients. The sex distribution was quite even (M/F = 227/223), but more than half of them were small-for-date (56.4%). Multiple pregnancy accounted for 22.9%, and toxemic mother, for 11.6%. Neonatal mortality rates were 23.9% for those with birth weight below 1,500, and 45.5% for those with birth weight below 1,000 grams, respectively. Respiratory distress syndrome occurred in 15.9% of the low birth weight neonates, and the incidence increased stepwise as birth weight decreased. Symptomatic patent ductus arteriosus could be detected in 11%. Fundoscopical evaluation, a routine for all with birth weight below 1,500 grams or below 2,000 grams with prolonged ventilator support, demonstrated retinopathy of prematurity in 5.6% of all cases or 50% in those below 1,000 grams. Routine brain echo done in all those below 2,000 grams within the first three days of age, and at least once every week thereafter, revealed intracranial hemorrhage in 10.2%, but only 2.4% belonged to grade III-IV. Respiratory distress syndrome and outborn were the two major determinants affecting the severity and occurrence of intraventricular hemorrhage significantly.(ABSTRACT TRUNCATED AT 250 WORDS)

Female↗
PubMed2006

[Cardiac catheterization in neonates: experience in 26 neonates].

Cardiac catheterization is rarely performed in neonates in China. The medical records of 26 neonates who underwent cardiac catheterization in Shanghai Children's Medical Center from June 1994 to June 2004 were reviewed. These infants weighed 2300 to 4500 g (mean 3 400 +/- 500 g). Postnatal age ranged from 5 to 28 days (mean 15.2 +/- 8.7 days). Right heart ventricle angiography was performed in 24 cases, left heart ventricle angiography in 20 cases and pulmonary angiography in 6 cases. Balloon aortic septostomy (BAS) was performed in 10 cases. Twenty cases were diagnosed with complex cyanotic cardiovascular malformations and 4 with acyanotic heart disease. The implantation of a temporary cardiac pacemaker was performed in 2 cases. All diagnostic and interventional procedures were successfully performed. The catheterization-related complications occurred in 15.3% of the 26 cases, but without mortality. In conclusion, cardiac catheterization is an accurate and safe method for diagnosing complex congenital heart diseases in neonates. Interventional catheterization is useful in the treatment of complex congenital heart malformations.

Cardiac Catheterization↗
PubMed1991

[The incidence of a neonatal bacterial infection syndrome with maternofetal transmission in the Neonatal Department of the Baia Mare District Maternity Hospital in 1983-1987].

In a retrospective study extending on a 5-year period, authors have analysed the incidence of the bacterial infectious syndrome with maternofetal transmission in the newborns admitted to the above-mentioned department. Among 20,674 live-born infants, 241 cases (1.16%) with diseases of an infectious character with vertical transmission were recorded. The incidence of the neonatal infectious syndrome was marked by a yearly increase, from 0.86% in the first year of the study to 1.24% in the last year, with a peak of 1.43% in 1986. The infection risk by maternofetal transmission was more than 10 times higher in the group of premature children. During the study period, of the total of 128 deaths, 31 (24.22%) were due to the neonatal infectious syndrome; of these 31 deaths, 21 occurred in premature children, hence 2/3 of all the failures were recorded in premature infants. Authors conclude that the prevention of the neonatal bacterial infectious syndrome could surely exert a favorable influence on the course of perinatal mortality and on the incidence of premature births in maternities.

Adult↗
PubMed1990

[Neonatal surgery: experience of a neonatal obstetrical and surgical unit].

A prospective and retrospective study of the consequences of neonatal surgery started recently in a neonatal intensive care unit. After a prenatal diagnosis of a surgical malformation has been established, the importance of a meeting with parents, neonatologists and surgeons is stressed. The relationships between the family and the neonatal team were good, although sometimes the surgeons seemed to be "forgotten". The infant's pain was usually prevented. Infants were actively managed before surgery and after surgery their rhythms were better respected.

Female↗
PubMedDate not supplied

Preparation for neonatal emergencies: a neonatal emergency medication sheet.

Safe and timely administration of emergency medications and infusions is crucial when caring for critically ill neonates. A neonatal emergency medication sheet can enhance preparation, calculation, and administration of medications during emergency situations in neonatal intensive care units (NICUs).

Drug Therapy↗
PubMed1989

The geographical variation in neonatal and post-neonatal mortality in South Africa, 1978-1982.

Early neonatal, neonatal and post-neonatal mortality rates were systematically calculated according to the statistical regions of South Africa for the white and coloured populations over a 5-year period. The geographical variation is wide and the statistical significance in these mortality rates for coloured infants in rural areas of the Cape and the Orange Free State are particularly high and call for urgent attention.

Black or African American↗
PubMed1987

[Relation between size of fetal and neonatal adrenal glands and steroid levels in maternal and neonatal serum].

Fetal adrenal gland is known to develop till term and decrease in size after birth. In this study, the size of the adrenal glands was evaluated by real time ultrasonography and steroid levels in the maternal and neonatal peripheral veins were measured by Gas chromatography-Mass spectrometry with application of deutrated steroids as internal standards. One hundred normal pregnant women at 28-40 weeks of gestation and ten cases of cesarean section, not labor, infants were selected for this study. The size of the fetal adrenal glands increased as gestation advanced. Levels of maternal 16DHA-S, P5-S and E3 correlated well with the length of the fetal adrenal glands. No correlation between the size of fetal adrenal gland and maternal DHA-S levels was noticed, except at 36-40 weeks of gestation. On the 7th day after birth, the neonatal adrenal glands were about 20% longer than at delivery. Levels of DHA-S, 16DHA-S, P5-S and 16P5-S in neonatal blood were 85%, 49%, 71% and 50% lower than at delivery during this period. In the present study, the relation between the adrenal steroid levels in maternal/neonatal serum and the size of the fetal/neonatal glands was demonstrated for the first time.

17-alpha-Hydroxypregnenolone↗
PubMedDate not supplied

[Group B beta-hemolytic Streptococcus: study on colonization of mother-neonate couples observed in January-May 1988 at the obstetrics and gynecology unit and the neonatal section at the Pistoia Hospital].

In a previous study, carried out on women at delivery recovered at the obstetric ward of the Pistoia Hospital, we noted a high incidence of group B Streptococci cultured from vaginal site. Because group B streptococcal infection is very serious in neonates we decided to examine the frequence of vertical and horizontal transmission among the mother-neonate couples. We have also compared their rate of colonisation by doing rectal and vaginal swabs on women and rectal, vaginal and external auditory canal swabs on babies. In fact we known that infants moderately to heavily colonised with group B Streptococci at birth, appear to have a significant higher risk of developing serious disease than do infants only lightly colonised. In our work we haven't observed any acute group B streptococcal infection, but we have identified 18 couples mother-neonate colonised by the same group B Streptococci serotype, which is proof of an easy vertical transmission of group B Streptococci whilst horizontal transmission is very rare (only one case).

Female↗
PubMed1981

Lethal vasculitis of coronary arteries in a neonate and two infants: possible neonatal variant of the MLNS/IPN complex?

An unusual case of fatal myocardial infarction in a neonate due to coronary arteries is compared with two lethal cases of Mucocutaneous Lymph Node Syndrome and/or Infantile Periarteritis Nodosa (MLNS/IPN). The neonatal coronary arteritis was pathologically indistinguishable from typical forms of MLNS/IPN, which cannot be reliably separated and whose strange epidemiologic features as well as their etiology(-ies) remain to be clarified. It is suggested that further research of the etiology of MLNS/IPN should be aware of a possible neonatal variant of this syndrome.

Arteritis↗
PubMed1984

Homologous monoclonal antibodies induce idiotope-specific suppression in neonates through maternal influence and in adults exposed during fetal and neonatal life.

The fine specificity of idiotype suppression induced early in ontogeny was investigated in the murine A/J anti-azophenylarsonate (Ar) response. Suppression was induced with two hapten-inhibitable, homologous monoclonal anti-idiotopic antibodies, AI and MB, that recognize partially overlapping sets of Ar-immune antibodies. Suppression was found to be idiotope-specific when adult mice were exposed to anti-idiotope as neonates; suppression was also idiotope specific when adult mice were exposed to anti-idiotope during fetal (through maternal inoculation) and neonatal life. Of particular interest, anti-idiotope, administered maternally, induced suppression in offspring first immunized with Ar as neonates, and this suppression was idiotope specific too. Thus, AI and MB induce idiotope-specific suppression in mice exposed to anti-idiotope early in ontogeny. These results parallel previous findings in adult mice and suggest that the mechanism of suppression in very young mice is the same as that in adults.

Aging↗
PubMed1981

Neonatal anthropometry: its value in the assessment of nutritional status and neonatal blood glucose homeostasis.

The use and application of neonatal anthropometry in newborns with different body measurements and nutritional evaluation status is reviewed. The neonatal blood glucose level has been used as a model in testing the predictive value of various indices of body size for the capacity of neonatal blood glucose homeostasis. For example, combining weight and length deficit from the expected means for gestational age and relative body measurements such as weight for length and ponderal index not only convey information about the type and magnitude of growth impairment, but are also good predictors of the risk of hypoglycaemia within the different anthropometric groups of growth retarded infants. Efforts have been made to delineate the diagnostic criteria by somatic measurements for identification of the different degrees and types of intrauterine growth deviation. The anthropometric approach to the heterogeneity of the intrauterine growth pattern seems to be a simple and very useful tool in somatic classification and evaluation of the newborn infant. In addition to the assessment of body proportions and nutritional status, the anthropometric approach can be of great help in exploring the relationship of body size and the physiological adaptation to the extrauterine environment.

Adipose Tissue↗