Ozge Altun Koroglu

Histological Chorioamnionitis: Effects on Premature Delivery and Neonatal Prognosis

BACKGROUNDnChorioamnionitis is closely related to premature birth and has negative effects on neonatal morbidity and mortality.nnnMETHODSnIn this prospective study, 43 mothers who delivered earlier than 35 gestational weeks and their 57 infants were evaluated clinically and with laboratory findings. Placentas and umbilical cords were investigated histopathologically for chorioamnionitis and funisitis.nnnRESULTSnThe overall frequency of clinical and histological chorioamnionitis (HCA) was 8.3% and 23.2%, respectively. The frequency of HCA was 47.3% and 83.3% in mothers delivered <32 weeks and <30 weeks, respectively. Maternal demographic and clinical findings and also leukocyte and C-reactive protein values were not indicative of HCA. Infants of mothers with HCA had significantly lower Apgar scores together with higher SNAP-PE-II and CRIB scores. These infants had increased mechanical ventilator and surfactant requirements, higher incidences of patent ductus arteriosus, early sepsis, and bronchopulmonary dysplasia, and higher mortality rates. The effect of HCA on neonatal morbidity and mortality was more prominent than the effect of low birthweight and lower gestational age.nnnCONCLUSIONnChorioamnionitis not only causes premature deliveries, but is also associated with neonatal complications and increased mortality. Clinical findings and infectious markers in mother or infant do not predict the diagnosis of histological chorioamnionitis. Therefore, placental histopathology may have a role in predicting neonatal outcome in premature deliveries, especially those below 30 weeks.

Genetic Factors that Influence Short-Term Neurodevelopmental Outcome in Term Hypoxic-Ischaemic Encephalopathic Neonates

It is difficult to predict outcome in neonates that experience perinatal hypoxic ischaemia. Morbidity and mortality may be affected by genetic factors that augment inflammatory and coagulative responses. This prospective study analysed the effects of proinflammatory cytokine gene polymorphisms (tumour necrosis factor-α [TNFA] 308G>A and interleukin-6 [IL6] 174G>C) and prothrombotic factor gene mutations (prothrombin G20210A, factor V Leiden G1691A and methylenetetrahydrofolate reductase [MTHFR] C677T) on the early neurological prognosis in 40 term hypoxic ischaemic encephalopathic neonates. There were significant relationships for Sarnat and Sarnat staging with electroencephalographic findings, transfontanelle ultrasound (US) results, early neonatal outcome and neurological morbidity. Genetic mutations in the prothrombotic proteins, the TNFA 308G>A polymorphism and the cerebrospinal fluid levels of TNF-α protein were not related to clinical stage, electroencephalography, transfontanelle US or neurological status at discharge or at postnatal months 6 and 12. The IL6 174GC genotype demonstrated a protective role, being significantly correlated with normal electroencephalography, transfontanelle US and normal neurological findings at discharge. In conclusion, the IL6 174GC gene polymorphism seems to play a role in determining the risk and/or severity of perinatal cerebral injury.

Titration of betaine therapy to optimize therapy in an infant with 5,10-methylenetetrahydrofolate reductase deficiency

Betaine therapy was given for 2xa0years to a 2-year-old boy with 5,10-methylenetetrahydrofolate reductase deficiency. Used as a methyl donor to lower homocysteine levels through methylation of methionine, betaine has been reported to be effective in treating homocystinuria. Satisfactory biochemical and clinical responses were obtained with the following regimen: betaine started in the newborn period at increasing doses to reach 1xa0g given six times a day. It is suggested that frequent administration of a moderate dose may provide clinical and biochemical benefit.

Serial Intravesical Pressure Measurements Can Predict the Presence and the Severity of Necrotizing Enterocolitis

BACKGROUND AND AIMSnNecrotizing enterocolitis (NEC) which is accompanied with gastrointestinal ulceration and necrosis is one of the most important problems of preterm infants in neonatal intensive care unit (NICU). Increased intra-abdominal pressure (IAP) is detected among most of the pediatric patients hospitalized in intensive care unit and undergoing surgery or trauma. This pathology, namely, abdominal compartment syndrome, causes ischemia and hypoperfusion of abdominal organs. Recently, the effect of increased IAP on NEC is under focus and this increase is thought to be related with the onset of NEC by leading to intestinal ischemia and necrosis. In this study, we aimed to investigate if serial intravesical pressure (IVP) measurements as an indirect indicator of IAP may help to early diagnosis in NEC and to decision for surgery besides to predict the mortality of NEC.nnnMATERIAL AND METHODnA total number of 61 preterm infants with a birth weight of ≤ 1,500 g hospitalized in NICU were included to the study. IVP values were measured by the same nurse twice daily during their hospitalization through urinary catheter. The IVP values of the preterm infants with and without NEC were compared.nnnRESULTSnTotally 61 premature infants included in the study were grouped as follows: group 0, the control group without NEC (n = 38); group 1, medically treated NEC patients (n = 14); and group 2, NEC patients undergoing surgery (n = 9). The median IVP measurements of group 0 were lower than the other groups (p = 0.001). No statistically significant difference in IVP measurements was detected between group 1 and group 2 (p = 0.155). A 10% of increase in IVP measurement was significant in predicting the development of NEC with consecutive serial measurements. The mean IVP measurements were higher in infants with NEC who died during their follow-up at NICU compared with NEC patients who survived (p = 0.043).nnnCONCLUSIONnSerial IVP measurements may help for early diagnosis and surgery decision of NEC and high IVP levels also may predict mortality in cases with NEC.

The effect of inhaled nitric oxide therapy on thromboelastogram in newborns with persistent pulmonary hypertension.

Studies about the effects of inhaled nitric oxide (iNO) on bleeding time and platelet aggregation in newborns are limited in number and have inconclusive results. Thromboelastogram (TEG) shows the combined effects of coagulation factors and platelet functions. In this preliminary study, we aimed to evaluate the effects of iNO on coagulation using TEG in newborns with persistent pulmonary hypertension (PPH). TEG assays were performed in 10 term infants receiving iNO treatment for PPH and 32 healthy term infants. Samples of the iNO group were collected before and during iNO. Clot reaction time (R), clot kinetics (K), maximum amplitude (MA), and alpha angle were obtained from the TEG tracing. TEG-R values were statistically higher during iNO treatment (7.75u2009±u20093.34) when compared to the values before iNO (4.83u2009±u20091.38) and the healthy controls (3.75u2009±u20090.98). The alpha angle was lower in iNO treated infants at both periods (before iNO, 55.33u2009±u20098.58; during iNO, 42.90u2009±u200918.34) compared to the control group (64.95u2009±u20096.88). MA values before iNO treatment were the lowest (44.43u2009±u200914.09) and improved with the iNO treatment (48.40u2009±u20099.49) despite still being lower compared to the controls (53.67u2009±u20095.56). Conclusion: Both PPH and iNO may negatively effect in vitro coagulation tests. Therefore, newborns with PPH requiring iNO treatment should be closely monitored for coagulation problems.

Neonatal status epilepticus controlled with levetiracetam at Sturge Weber syndrome.

Sturge-Weber syndrome is a rare, sporadic, congenital neurocutaneous syndrome characterized by facial cutaneous vascular malformation, leptomeningeal angioma and eye abnormalities. Seizures develop during the first year of life, may become refractory to multiple anticonvulsants and status epilepticus may develop. A rare subtype of Sturge-Weber syndrome with bilateral facial vascular malformation, unilateral cerebral involvement and neonatal status epilepticus is reported here. Neonatal status epilepticus was successfully controlled with intravenous levetiracetam infusion.

Effects of Two Different Exogenous Surfactant Preparations on Serial Peripheral Perfusion Index and Tissue Carbon Monoxide Measurements in Preterm Infants with Severe Respiratory Distress Syndrome

BACKGROUNDnAdministration of an exogenous surfactant may affect both ventilatory and hemodynamic parameters in preterm infants with respiratory distress syndrome (RDS). Peripheral perfusion may be expected to be influenced, and serial perfusion index (PI) values may show this effect. Noninvasive transcutaneous carbon monoxide (TCO) monitoring may show RDS severity, oxidative and inflammatory stress, and response to surfactant treatment.nnnMETHODSnThis randomized controlled nonblinded study was performed in 30 preterm infants with RDS, treated with poractant alfa (n = 15) or beractant (n = 15); 18 preterm infants without RDS served as a control group. Oxygenation and hemodynamic parameters were recorded and compared through the first 6 hours of treatment. PI and TCO values were measured prior to (Tp), immediately after (T0), and at 5 minutes (T5), 30 minutes (T30), 60 minutes (T60), and 360 minutes (T360) after the bolus surfactant administration. The mean arterial pressure, oxygenation index, pH, and lactate levels were recorded simultaneously.nnnRESULTSnBoth study groups had lower Tp PI and higher Tp TCO levels than controls. Both surfactant preparations improved the PI, TCO, mean arterial pressure, oxygenation index, pH, and lactate levels at the end point of T360. However, the median Tp PI value of 1.3 first decreased to 0.86 at T0 (P < 0.001), and then it increased to 0.99 at T5 (p < 0.001) and to 1.25 at T30 (p = 0.037). The median Tp TCO value of 3 decreased to 2, 1.5, 0, and 0 at T0, T5, T30, and T60, respectively (p < 0.001). PI more quickly recovered to Tp values (30 minutes vs. 60 minutes) and reached the control group values (30 minutes vs. 360 minutes) with beractant compared to that with poractant alfa. TCO recovered to Tp values in both groups at the same time (5 minutes vs. 5 minutes), but reached the control group values more quickly (5 minutes vs. 30 minutes) with poractant alfa than with beractant.nnnCONCLUSIONnPatients with RDS had poor perfusion, and PI improved with both surfactant preparations only following a short decline in the 1(st) minute. The expected improvement of PI occurred earlier in the beractant subgroup. TCO declined in both groups, showing lung improvement and decreased oxidative/inflammatory stress, and it was normalized earlier with poractant alfa.

Desferrioxamine treatment of iron overload secondary to RH isoimmunization and intrauterine transfusion in a newborn infant

Intrauterine transfusion is the standard of care in the management of severe Rh isoimmunization. Desferrioxamine has been used for the treatment of iron overload secondary to hemolysis and intrauterine transfusions in Rh isoimmunization cases. Here, we report a preterm infant born at 34xa0weeks of gestational age who had formerly received intrauterine transfusions for Rhesus hemolytic disease and presented with severe hyperferritinemia and elevated liver enzymes in the first week of life. Desferrioxamine treatment was started due to a ferritin level of 28,800xa0ng/ml and continued for 13xa0weeks. Although the treatment was successful, we observed resistant leukopenia which resolved after the cessation of treatment. In conclusion, iron overload secondary to intrauterine transfusions can be treated successfully with desferrioxamine; however, neonatologists must be aware of the possible side effects of this drug which has been used in only a limited number of newborns.

Diagnostic tools of early brain disturbances in an asymptomatic neonate with maple syrup urine disease.

Maple syrup urine disease (MSUD) is a rare inherited metabolic disorder resulting from the defective activity of branched-chain 2-ketoacid dehydrogenase complex. Routine screening of newborn with tandem mass spectroscopy on the third day of life may detect elevated branched-chain amino acids in blood before the appearance of encephalopathic symptoms in MSUD cases. If undiagnosed by such a routine screening test, patients often present with encephalopathy and seizures. Clinical neurologic examination is supplemented by electroencephalography and imaging. Here, we report abnormal amplitude-integrated electroencephalography, electroencephalography, magnetic resonance imaging, and magnetic resonance imaging spectroscopy findings in a neurologically asymptomatic male newborn who was diagnosed with MSUD at the third week of life. These neurologic disturbances disappeared at the fourth month of life with appropriate special diet. Therefore, even in already asymptomatic cases, early neurologic deterioration of brain metabolism and structure can be detected with these early laboratory findings, indicating the importance of early diagnosis and management. Patients may also benefit from these investigations during the follow-up period.

Reference intervals of α-glycosidase, β-glycosidase, and α-galactosidase in dried blood spot in a Turkish newborn population

AbstractInherited lysosomal storage diseases (LSDs) are rare, and diagnosis is often delayed for 7–10xa0years. Since the therapies have become available for a limited number of LSDs, (Fabry, Gaucher, Pompe, and MPS-1), early diagnosis of treatable LSDs can be lifesaving or ameliorating and allows timely treatment before irreversible damage occurs. Recently, the use of dried blood spot test (DBS) for newborn screening of LSDs has been proposed for newborn screening tests. They are noninvasive, sensitive, and specific assays with the further advantage of a fast turnaround time compared to measurement in leukocyte and/or fibroblast culture. We aimed to determine the reference intervals for lysosomal enzyme activities of newborn babies in our population and to investigate the effect of gestational week on enzyme activity. One hundred thirty healthy newborn babies (70 girls, 60 boys) were included into the study. α-Glycosidase, β-glycosidase, and α-galactosidase activities in DBS samples of newborns were determined fluorometrically. Reference intervals were calculated using Dixons rule and percentiles of 2.5–97.5. Cutoff limits (5xa0%) for α-glycosidase, β-glycosidase, and α-galactosidase activities were 0.57, 0.92, and 2.18, respectively. α-Galactosidase activity was higher in girls compared to boys (pu2009<u20090.05). Interestingly, α-glycosidase and β-glycosidase activities of newborns who were delivered before 38xa0weeks were significantly lower than those who were delivered at 39–40xa0weeks.n Conclusion It is of utmost importance to define the reference intervals for lysosomal enzyme activities as well as cutoff limits for newborn babies with regard to gestational age and sex. More studies to clarify the reason for the change in enzyme activity by gestational week will be required.