Gulay Can

Neonatal suppurative parotitis: a vanishing disease?

A case of neonatal suppurative parotitis due toKlebsiella pneumoniae is described. This is the first case reported in the last 20 years. Infection with unusual microorganisms should be taken into account when planning antibiotic treatment, especially in hospital acquired infections of the salivary glands in the newborn.

Vein of Galen aneurysmal malformation: prenatal diagnosis and early endovascular management.

A prenatally diagnosed patient was referred to our hospital at 35 weeks of gestation. Antenatal ultrasonography demonstrated cardiomegaly and aneurysm of the vein of Galen. A 3,290 g male baby was delivered by elective cesarean section at 37 weeks of gestation. Physical examination was remarkable for tachypnea, hyperdynamic precordium with a continuous murmur, cranial bruit and bounding carotid pulses. Magnetic resonance angiography confirmed vein of Galen aneurysmal malformation and demonstrated the vessels feeding the aneurysm. Postnatal management included aggressive medical treatment of cardiac failure and transarterial embolization of the vessels feeding the aneurysm at 3 days of age. Posthemorrhagic hydrocephalus developed after embolization, and a ventriculo-peritoneal shunt was placed at 29 days of age. Presently, the patient is 4 years of age and has no neurological abnormality at follow-up. Vein of Galen aneurysmal malformations can cause severe morbidity and mortality in neonates. However, careful obstetric follow-up and early postnatal endovascular treatment of these malformations may lead to a favorable outcome.

Gaucher disease associated with congenital ichthyosis in the neonate

Sir: A small number of cases with Gaucher disease who are symptomatic in the neonatal period and who show ichthyotic skin changes have been described [1, 2, 5]. This association appears to be typical for neonatal Gaucher disease [4, 5]. We wish to report another case. A 2580 g female infant was born vaginally at 37 weeks gestation to a 23-yearold, gravida 3, para 1 mother who was a second degree relative of the father. Her first pregnancy ended in spontaneous abortion. Her second pregnancy resulted in the delivery of a boy who had ichthyosis, hepatosplenomegaly, cardiac defects and died 2 days after birth. The present pregnancy, labour and delivery were uneventful. The baby had a collodion skin and ectropion, eclabinm, generalized joint contractures, gross hepatosplenomegaly, jaundice, petechiae and purpura. Laboratory investigations revealed thrombocytopenia, conjugated hyperbilirubinaemia and elevated liver enzymes. The patients clinical condition deteriorated on the 3rd day and she died on the 8th day with severe septicaemia and disseminated intravascular coagulation. At postmortem examination, large macrophages, histochemically and morphologically consistent with Gancher cells were found in the liver, spleen, lymph nodes, bone marrow and thymus (Fig. 1). The parents were later tested for glucocerebrosidase activity which was 50% of normal in both. To our knowledge eight cases of neonatal Gancher disease associated with ichthyosis have been reported [1-5]. Although the reason for this association is not clear, it appears that the enzyme deficiency may be directly responsible for the skin changes in these neonates [5]. Thus, Gaucher disease should be considered in the differential diagnosis of congenital ichthyosis. In our case the diagnosis was established after the death of the second child. Recognition of this distinct Gaucher Fig. 1 A typical Gaucher cell in the lymph node parenchyma (H & E x 770)

Neonatal hyperbilirubinemia and G71R mutation of the UGT1A1 gene in Turkish patients

Objective. Nonphysiologic hyperbilirubinemia of unexplained cause is prevalent among Turkish newborns, suggesting that there might be genetic risk factors in this population. Mutation of the UGT1A1 gene, glycine to arginine at codon 71 (G71R), is related to the development of neonatal jaundice in East Asian populations but the frequency of this mutation is rare among Caucasian populations. There are insufficient data on the G71R mutation in Turkish newborns with hyperbilirubinemia. The aim of this study was to investigate the genotypic distribution of the G71R mutation and its relationship with nonphysiologic hyperbilirubinemia of unexplained cause in Turkish newborns. Methods. Polymerase chain reaction, restriction fragment length polymorphism and agarose gel electrophoresis techniques were used for detection of G71R mutation in 109 newborn infants: 39 with hyperbilirubinemia and 70 without hyperbilirubinemia. Results. The genotypic distribution for the mutation was 70 G/G, 32 A/G, 7 A/A genotypes and the mutated allele frequency was 0.22. The frequency of G71R mutation was 33.3 % (n = 13) A/G, 7.7% (n = 3) A/A in the hyperbilirubinemia group and 27.1% (n = 19) A/R, 5% (n = 4) A/A in the nonhyperbilirubinemia group. The difference between the groups was not statistically significant. Conclusions. Our results suggest that G71R mutation of UGT1A1 is not rare; however, an association between G71R mutation and hyperbilirubinemia of unexplained cause has not been shown in Turkish newborns.

Cord Blood Cardiac Troponin T and Nonprotein-Bound Iron Levels in Newborns of Mild Pre-Eclamptic Mothers

Background: In hypoxic newborns, cardiac troponin T (cTnT) was shown to be an indicator of cardiac damage and increased levels of nonprotein-bound iron (NPBI), an indicator of increased free radical production and perinatal brain damage. Objective: The aim of this study was to determine cord blood cTnT and NPBI levels in neonates of mild pre-eclamptic mothers. Methods: The study included 50 babies of mild pre-eclamptic mothers and 50 babies of healthy mothers. cTnT and NPBI levels were measured in cord blood. Results: The mean gestational age in the pre-eclamptic and healthy groups were 36.1 ± 3.5 and 38.1 ± 1.9 weeks, mean birth weights were 2,456 ± 945 and 3,059 ± 493 g. Cord blood median cTnT level was significantly higher in the pre-eclamptic group (0.024 vs. 0.015 ng/ml). Serum cTnT in the 95th percentile was 0.047 ng/ml in the healthy group. cTnT levels of preterm babies in the pre-eclamptic group was found to be significantly higher compared to term babies in the control group (0.038 vs. 0.013 ng/ml). It could not be demonstrated whether there is a statistically significant relation between cTnT levels and respiratory distress, gestation, type of delivery, sex and birth weight. The median NPBI level was higher in the control group (3.26 vs. 1.86 µmol/l). Conclusions: Increased levels of cTnT may be a biochemical marker of cardiac involvement in babies of mild pre-eclamptic mothers. In this study, no correlation was found between cTnT levels and NPBI levels.

Elevated ghrelin levels in preterm born children during prepubertal ages and relationship with catch-up growth

BACKGROUND Ghrelin, the natural ligand of the GH secretagogue receptor, has potent orexigenic effect. Ghrelin levels are negatively associated with insulin secretion, increased in anorexia, and reduced in obesity. Increased ghrelin levels may be associated with early postnatal growth in preterm born children. OBJECTIVE Aim of this study was to evaluate ghrelin and insulin levels at prepubertal ages in preterm born children born appropriate for gestational age (AGA) or small for gestational age (SGA) and relationships with catch-up growth (CUG) in a prospective cross-sectional study. METHODS Eighty-four preterm born children grouped as preterm SGA (n=28) and preterm AGA (n=56) were evaluated at age 4.7+/-0.2 and 4.7+/-0.1 years with respect to their ghrelin and insulin levels. Their data were compared with that of body mass index matched term SGA (n=35) and term AGA (n=44) children of age 4.6+/-0.2 and 3.8+/-0.1 years. All children had height appropriate for their target height. CUG was defined as the difference between birth size and recent size and expressed as Delta height and Delta weight SDS. RESULTS Preterm SGA and preterm AGA children had similar ghrelin levels (1717.0+/-166.9 and 1656.5+/-103.8 pg/ml), although Delta height and Delta weight SDS in preterm SGA were significantly higher than in preterm AGA children (P<0.001). Ghrelin levels in both preterm groups were higher than term SGA (469.2+/-132.5 pg/ml) and term AGA children (659.6+/-143.3 pg/ml; P<0.001 for all). Delta Height and Delta weight SDS of the term SGA children were similar to that of preterm SGA children. Ghrelin did not have correlation with CUG but had inverse correlation with recent anthropometric indices. Insulin was significantly higher in term SGA children than other groups (P<0.001). CONCLUSIONS Preterm children have higher ghrelin levels at prepubertal ages regardless of the magnitude of their CUG. Term SGA children, on the other hand, behave differently and have lower ghrelin levels than preterm children at prepubertal ages, which may be related to elevated insulin levels in this group.

The umbilical cord alpha-fetoprotein levels for predicting hyperbilirubinemia in term neonates

Abstract Objective: The aim of this study was to investigate the possible relationship between cord bloodalpha-fetoprotein (AFP) level and development of subsequent neonatal hyperbilirubinemia. Study design: The term newborns born between March 2005 and October 2005 were included in the study. Infants with Coombs-positive ABO and/or Rh incompatibility and/or hemolytic jaundice, asphyxia, congenital anomaly and signs of bleeding were excluded from the study. Cord blood AFP levels were measured in 504 full term newborns in this period. Infants were followed-up for possible neonatal hyperbilirubinemia. The capillary bilirubin level (CBL) was examined expeditiously in newborns developing jaundice and in other infants at the time discharge while the screening test was being performed. Results: The mean umbilical cord AFP level was 49.1 ± 44.9 mg/L (range 1.1–396.2 mg/L), mean CBL was 5.8 ± 3.1 mg/dL (range 1–19.4 mg/dL), and the mean bilirubin detection time was 37 ± 23.2 hours (range 12–144 h) of age. Although a significant positive correlation was found between umbilical cord AFP and CBL levels, it was weak (r = 0.187, p < 0.001). Comparison of AFP levels in terms of bilirubin percentile values appropriate for postnatal age also showed a significant weak positive correlation (r = 0.113, p < 0.001). Conclusion: The umbilical cord AFP levels may not be used as a strong predictor for the determination of newborns at risk for hyperbilirubinemia.

Nephrocalcinosis in newborn.

Nephrocalcinosis is uncommon in childhood, and almost always develops outside the newborn period. Over the last decade, nephrocalcinosis due to multifactorial pathogenesis has increased in very low birth‐weight infants. In this report a 23 day old full‐term baby with nephrocalcinosis secondary to distal renal tubular acidosis is described.

Yenidoğan Konvülsiyonlu Olgularımız

Konvulsiyonlar yenidogan unitelerinde en sik rastlanan sorunlardandir Ocak 1988 Aralik 1992 tarihleri arasinda Neonatoloji Bolumu ne kabul edilen hastalar incelendi Bu donem icinde yatirilan 2313 hastadan 142 sine 6 1 yenidogan konvulsiyonu tanisi konulmustu Hastalardan 95 i 67 erkek 47 si 33 kiz idi Gestasyon yaslari ortalamasi 38 3±3 2 hafta 29 44 dogum tartilari ortalamasi 3020±758 866 4750 gram olarak bulundu Konvulsiyonlarin 69 u 98 olgu ilk 72 saatte 31 i 44olgu 4 gun veya sonrasinda baslamisti En sik klonik tipte konvulsiyon gozlendi Etiyoloji degerlendirildiginde perinatal asfiksi infeksiyon ve metabolik bozukluklarin ilk sirayi aldigi goruldu Sadece fenobarbital ile 107 hastada 75 tedaviye yanit alindi Mortalite orani 19 du Makalede bu sonuclar sunuldu ve yenidogan konvulsiyonlari tartisildi Anahtar kelimeler: Yenidogan Konvulsiyonlar

Adrenal hemorrhage: a rare cause of severe neonatal jaundice

Adrenal hemorrhage in newborns is not uncommon. The clinical presentation may be very protean and the diagnosis difficult. Ultrasonography is useful in making the diagnosis. A patient presenting with severe neonatal jaundice due to adrenal hemorrhage is described.