Neonatal Resuscitation Practices in Turkey: A Survey of the Turkish Neonatal Society and the Union of European Neonatal and Perinatal Societies.

OBJECTIVE: Optimal care in the delivery room is important to decrease neonatal morbidity and mortality. We aimed to evaluate neonatal resuscitation practices in Turkish centers. MATERIALS AND METHODS: A cross-sectional survey consisted of a 91-item questionnaire focused on delivery room practices in neonatal resuscitation and was sent to 50 Turkish centers. Hospitals with <2500 and those with ≥2500 births/year were compared. RESULTS: In 2018, approximately 240 000 births occurred at participating hospitals with a median of 2630 births/year. Participating hospitals were able to provide nasal continuous-positiveairway-pressure/high-flow nasal cannula, mechanical ventilation, high-frequency oscillatory ventilation, inhaled nitric oxide, and therapeutic hypothermia similarly. Antenatal counseling was routinely performed on parents at 56% of all centers. A resuscitation team was present at 72% of deliveries. Umbilical cord management for both term and preterm infants was similar between centers. The rate of delayed cord clamping was approximately 60% in term and late preterm infants. Thermal management for preterm infants (<32 weeks) was similar. Hospitals had appropriate equipment with similar rates of interventions and management, except conti nuous-positive-airway-pressure and positive-end-expiratory-pressure levels (cmH2O) used in preterm infants (P = .021, and P = .032). Ethical and educational aspects were also similar. CONCLUSIONS: This survey provided information on neonatal resuscitation practices in a sample of hospitals from all regions of Turkey and allowed us to see weaknesses in some fields. Although adherence to the guidelines was high among centers, further implementations are required in the areas of antenatal counseling, cord management, and circulation assessment in the delivery room.

Intravenous Immunoglobulin Use in Hemolytic Disease Due to ABO Incompatibility to Prevent Exchange Transfusion.

Introduction: Intravenous immunoglobulin (IVIG) has been widely used to treat the hemolytic disease of the newborn (HDN). Although it has been shown that IVIG treatment reduces the duration of phototherapy and hospitalization, the use of IVIG in hemolytic disease due to ABO incompatibility has been controversial in recent years. This study aimed to investigate the role of IVIG in the prevention of exchange transfusion in infants with ABO HDN who presented with bilirubin levels at or above the level of exchange transfusion. Materials and Methods: This study evaluated the data of infants with ABO HDN in the Turkish Neonatal Jaundice Online Registry. The infants with ABO HDN who met the total serum bilirubin level inclusion criteria (within 2-3 mg/dL of exchange transfusion or even above exchange transfusion level) were included in the study according to the guidelines from the American Academy of Pediatrics and the Turkish Neonatal Society. All patients were managed according to the unit protocols recommended by these guidelines and received light-emitting diode (LED) phototherapy. Infants who only received LED phototherapy, and who received one dose of IVIG with LED phototherapy were compared. Results: During the study period, 531 term infants were included in the study according to inclusion criteria. There were 408 cases in the phototherapy-only group, and 123 cases in the IVIG group. The demographic findings and the mean bilirubin and reticulocyte levels at admission were similar between the groups (p > 0.05), whereas the mean hemoglobin level was slightly lower in the IVIG group (p = 0.037). The mean age at admission was earlier, the need for exchange transfusion was higher, and the duration of phototherapy was longer in the IVIG group (p < 0.001, p = 0.001, and p < 0.001, respectively). The rate of re-hospitalization and acute bilirubin encephalopathy (ABE) was higher in the IVIG group (p < 0.001 and p = 0.01, respectively). Conclusion: In this study, we determined that one dose of IVIG did not prevent an exchange transfusion nor decrease the duration of phototherapy in infants, who had bilirubin levels near or at exchange transfusion level, with hemolytic disease due to ABO incompatibility.

Hypernatremic Dehydration Risk Factors in Newborns: Prospective Case-Controlled Study.

BACKGROUND: The prevalence of hypernatremic dehydration (HND) has increased in recent years most likely due to insufficient intake of breast milk as the most important factor. This study aimed to investigate risk factors of HND. METHODS: In this study, 47 neonates whom were diagnosed to have HND were included in the study group and 96 healty neonates whom were included in the control group. While demographic data of the patients were recorded, mothers were asked to fill out the sociodemographic/ psychosocial data form, Edinburgh Postpartum Depression Scale(EPDS) and STAI I and II State and Trait Anxiety Scale. Breast milk sodium concentrations were studied from mothers of all infants. The relationship between the development of neonatal HND and risk factors affecting this condition were evaluated. RESULTS: Being the first-born baby of the family was found to be a significant risk factor for HND. Breast milk sodium concentration was 25.8±7.9 mmol/L in the HND group which was significantly higher than the control group. Median depression score was similar in both groups and there was no statistical difference in terms of groups. The anxiety score was higher in the control group compared to the study group. There was no difference in terms of other sociodemographic / psychosocial data of mothers. CONCLUSION: Primiparity or insufficient breastfeeding may result in elevated breast milk sodium levels and related neonatal HND. Breastfeeding support should principally target primiparous women to improve breastfeeding outcomes like as especially HND.

Prenatal smoke exposure is associated with increased anogenital distance in female infants: a prospective case-control study.

OBJECTIVES: To investigate the effects of maternal smoking during pregnancy on newborn infants' anogenital distance (AGD). METHODS: Fifty-six female and sixty-four male newborn infants from mothers who smoked during pregnancy were included in this study. A control group for each sex was selected from infants whose mothers had no active or passive (in either the household or the workplace) smoke exposure before or during pregnancy. Questionnaire data on maternal demographic characteristics and information about cigarette use were collected. We assessed genital anthropometry which included AGD for both male and female neonates, and stretched penile length (SPL), penile girth for males within the first 48 h after birth. AGD measurements were also normalized according to birth weight (AGD/weight in grams), length (AGD/height in millimeters), and ponderal index [AGD/(weight in grams/height in cubic centimeters)]. Anogenital index (AGI) was calculated by dividing the AGD by cube root of birth weight. RESULTS: In female infants, prenatal smoke exposure was associated with significantly increased weight-adjusted AGD (p=0.03). There was also a significant correlation between mothers' daily smoking rates and weight-adjusted AGD (r=0.27/p=0.03). In male infants, fetal smoke exposure was not associated with any AGD measurements, SPL and penile girth. CONCLUSIONS: A significant increase in weight-adjusted AGD in female infants exposed to maternal smoking may be an indicator of antenatal androgen exposure and may pose a risk for short and long-term endocrine, metabolic and behavioral problems.

Colonización e infección por un microorganismo poco frecuente en una unidad de cuidados intensivos neonatales: tres recién nacidos prematuros con Elizabethkingia meningoseptica / Colonization and infection with a rare microorganism in a neonatal intensive care unit: three preterm infants with Elizabethkingia meningoseptica

Elizabethkingia meningoseptica es un bacilo gramnegativo ampliamente distribuido en la naturaleza pero poco frecuente en humanos que se presenta en infecciones intrahospitalarias. Los avances en las instalaciones de cuidados intensivos neonatales y el uso de dispositivos médicos sofisticados fortalecen la capacidad infecciosa invasiva del microorganismo. Las manifestaciones clínicas suelen incluir bacteriemia primaria, meningitis, neumonía intrahospitalaria, bacteriemia relacionada con el uso de catéteres intravasculares e infecciones gastrointestinales y de las vías biliares. Es posible que, en entornos con recursos limitados, la falta de sistemas de diagnóstico mejorados sea una de las causas por las que no se notifican todas esas infecciones. Resulta bastante difícil distinguir entre colonización e infección, y el patrón de sensibilidad a los antibióticos es diferente. Por consiguiente, los médicos deben realizar el diagnóstico preciso para así evitar el tratamiento incorrecto. En este artículo, describimos tres casos de recién nacidos con diagnóstico de infección y colonización por E. meningoseptica con el objetivo de destacar la importancia del diagnóstico y el tratamiento oportunos de esta bacteria poco frecuente pero letal que ya está presente en las unidades de cuidados intensivos

Elizabethkingia meningoseptica is a widespread gram-negative bacillus in the environment, but a arely reported human pathogen presenting mostly as nosocomial infections. Advances in neonatal intensive care facilities and usage of sophisticated medical devices strengthen the invasive infectious potential of the microorganism. Clinical manifestations usually include primary bacteremia, meningitis, nosocomial pneumoniae, intravascular catheter-related bacteremia and gastrointestinal and biliary tract infections. Lack of improved diagnostic systems in resource constrained settings, might be a cause of underreporting of such infections. Discrimination between colonization and infection is quite difficult, and it has an unusual antibiotic susceptibility pattern. Therefore clinicians should pay special attention to accurate diagnosis in order to prevent mistreatment. Here we report three newborn cases with the diagnosis of E. meningoseptica infection and colopnization, with the aim of drawing attention to the diagnosis and management of this rare but lethal bacteria that is already present in the intensive care unit environment

Colonization and infection with a rare microorganism in a neonatal intensive care unit: three preterm infants with Elizabethkingia meningoseptica. / Colonización e infección por un microorganismo poco frecuente en una unidad de cuidados intensivos neonatales: tres recién nacidos prematuros con Elizabethkingia meningoseptica.

Elizabethkingia meningoseptica is a widespread gram-negative bacillus in the environment, but a rarely reported human pathogen presenting mostly as nosocomial infections. Advances in neonatal intensive care facilities and usage of sophisticated medical devices strengthen the invasive infectious potential of the microorganism. Clinical manifestations usually include primary bacteremia, meningitis, nosocomial pneumoniae, intravascular catheter-related bacteremia and gastrointestinal and biliary tract infections. Lack of improved diagnostic systems in resource constrained settings, might be a cause of underreporting of such infections. Discrimination between colonization and infection is quite difficult, and it has an unusual antibiotic susceptibility pattern. Therefore clinicians should pay special attention to accurate diagnosis in order to prevent mistreatment. Here we report three newborn cases with the diagnosis of E. meningoseptica infection and colopnization, with the aim of drawing attention to the diagnosis and management of this rare but lethal bacteria that is already present in the intensive care unit environment.

Elizabethkingia meningoseptica es un bacilo gramnegativo ampliamente distribuido en la naturaleza pero poco frecuente en humanos que se presenta en infecciones intrahospitalarias. Los avances en las instalaciones de cuidados intensivos neonatales y el uso de dispositivos médicos sofisticados fortalecen la capacidad infecciosa invasiva del microorganismo. Las manifestaciones clínicas suelen incluir bacteriemia primaria, meningitis, neumonía intrahospitalaria, bacteriemia relacionada con el uso de catéteres intravasculares e infecciones gastrointestinales y de las vías biliares. Es posible que, en entornos con recursos limitados, la falta de sistemas de diagnóstico mejorados sea una de las causas por las que no se notifican todas esas infecciones. Resulta bastante difícil distinguir entre colonización e infección, y el patrón de sensibilidad a los antibióticos es diferente. Por consiguiente, los médicos deben realizar el diagnóstico preciso para así evitar el tratamiento incorrecto. En este artículo, describimos tres casos de recién nacidos con diagnóstico de infección y colonización por E. meningoseptica con el objetivo de destacar la importancia del diagnóstico y el tratamiento oportunos de esta bacteria poco frecuente pero letal que ya está presente en las unidades de cuidados intensivos.

A rare vascular lesion of newborn: cutis marmorata telangiectatica congenital.

Cutis marmorata telangiectatica congenita is a rare, benign, sporadic and cutaneous vascular disease. A newborn female baby whose mother was aged 29 years and used propylthiouracil during pregnancy was hospitalized because of varicose lesions on the skin of the lower extremity and on the back, which were present at birth. It was observed that the lesions did not disappear, although appropriate room temperature was provided. The patient was diagnosed as having cutis marmorata telangiectatica congenita and screened for additional anomalies. She had no additional anomalies, and she was discharged and monitored. At the postnatal sixth month, the cutaneous vascular lesions disappeared spontaneously. Cutis marmorata telangiectatica congenita, which is a rare condition, should be kept in mind in the differential diagnosis of physiologic cutis marmoratus, which occurs frequently in the neonatal period.

Kutis marmorata telenjiektatika konjenita; nadir görülen, selim, sporadik, deriyi tutan vasküler bir hastaliktir. Yirmi dokuz yasinda hipertroidi nedeni ile propiltiyourasil kullanan anneden dogan kiz bebek, her iki alt ekstremitede ve sirtta yerlesim gösteren renk degisikliginin eslik ettigi variköz lezyonlar nedeniyle yatirildi, lezyonlarin uygun ortam sicakligi saglanmasina ragmen düzelmedigi saptandi. Kutis marmorata telenjiektatika konjenita tanisi konan olgu; eslik edebilecek ek anomaliler açisindan tarandi; ek anomali saptanmayan olgu taburcu edilerek izleme alindi, postnatal altinci ayda kutanöz vasküler lezyonlarin kendiliginden kayboldugu görüldü. Yenidogan döneminde sik olarak karsimiza çikan fizyolojik kutis marmoratus ayirici tanisinda, ender görülen bir durum olan kutis marmorata telenjiektatika konjenita da akilda tutulmalidir.

Prevalencia de microorganismos patógenos respiratorios durante dos temporadas consecutivas de virus sincicial respiratorio en un centro de atención de alta complejidad / Prevalence of respiratory pathogens during two consecutive respiratory syncytial virus seasons at a tertiary medical care center

Objetivo. Determinar el perfil etiológico de la infección de vías respiratorias bajas (IVRB) en los neonatos durante la temporada de virus sincicial respiratorio (VSR) y definir las características de esta y otras infecciones. Métodos. Estudio retrospectivo con neonatos hospitalizados por IVRB durante dos temporadas de VSR, con reacción múltiple en cadena de la polimerasa en tiempo real. Se revisaron los datos relevantes y se compararon las características de la infección por VSR con las de otros microorganismos. Resultados. Entre 224 pacientes, 160 (71 %) fueron positivos para, al menos, un agente causal potencial. Entre ellos, el 65 % tenía VSR y el 15 %, más de un agente (coinfección). En el grupo con VSR, hubo más dificultad respiratoria (p < 0,01), radiografías de tórax anormales (p < 0,01), requerimiento de atención en terapia intensiva (p < 0,01) y más días de oxigenoterapia (p < 0,01). No obstante, hubo menos casos de fiebre al ingreso y menos días de antibioticoterapia (en ambos, p < 0,01), y la duración de la hospitalización no fue más prolongada. Los pacientes con coinfección requirieron atención en terapia intensiva en mayor medida que los demás (25 % contra 6,5 %, p < 0,01). Conclusión. Este estudio demostró que el VSR fue más frecuente en los neonatos hospitalizados por IVRB durante la temporada y condujo a una evolución más grave que otros microorganismos patógenos detectados. Posiblemente la gravedad de la infección por VSR se viera aumentada por la presencia de una coinfección y radiografía de tórax anormal.

Aim: To determine the etiological profiles of lower respiratory tract infection (LRI) in neonates during respiratory syncytial virus(RSV) season, and to define the clinical features of RSV-related infection and others. Methods: The retrospective study included newborn infants who were hospitalized for LRI during the two consecutive RSV seasons, and then tested for possible etiological agent by multiplex real-time polymerase chain reaction. All relevant data were reviewed, and the clinical characteristics of RSV-related infection were compared to those of others. Results: Of 224 patients, 160 (71 %) were positive for at least one potentially causative agent. Of them, 65 % had RSV, and 15 % had more than on ecausative agent (co-infection). The RSV group had more the findings of respiratory distress (p< 0.01), abnormal chest radiography (p< 0.01), need for intensive care (p< 0.01), and duration of oxygen requirement (p< 0.01) but less fever on admission and duration of antibiotic use (for both, p< 0.01), and no longer hospital stay. Need of intensive care nursery was more common in patients with co-infection than others (25 % vs. 6.5 %, p< 0.01). Conclusion: This study highlighted that RSV was the most frequent agent in neonates hospitalized for LRI during the season, with a more severe clinical course than other detected pathogens. The disease severity of RSV infection may have seemed to be increased by the presence of coinfection and abnormal chest radiography.

Prevalence of respiratory pathogens during two consecutive respiratory syncytial virus seasons at a tertiary medical care center. / Prevalencia de microorganismos patógenos respiratorios durante dos temporadas consecutivas de virus sincicial respiratorio en un centro de atención de alta complejidad.

AIM: To determine the etiological profiles of lower respiratory tract infection (LRI) in neonates during respiratory syncytial virus(RSV) season, and to define the clinical features of RSV-related infection and others. METHODS: The retrospective study included newborn infants who were hospitalized for LRI during the two consecutive RSV seasons, and then tested for possible etiological agent by multiplex real-time polymerase chain reaction. All relevant data were reviewed, and the clinical characteristics of RSV-related infection were compared to those of others. RESULTS: Of 224 patients, 160 (71 %) were positive for at least one potentially causative agent. Of them, 65 % had RSV, and 15 % had more than on ecausative agent (co-infection). The RSV group had more the findings of respiratory distress (p< 0.01), abnormal chest radiography (p< 0.01), need for intensive care (p< 0.01), and duration of oxygen requirement (p< 0.01) but less fever on admission and duration of antibiotic use (for both, p< 0.01), and no longer hospital stay. Need of intensive care nursery was more common in patients with co-infection than others (25 % vs. 6.5 %, p< 0.01). CONCLUSIONS: This study highlighted that RSV was the most frequent agent in neonates hospitalized for LRI during the season, with a more severe clinical course than other detected pathogens. The disease severity of RSV infection may have seemed to be increased by the presence of coinfection and abnormal chest radiography.

Objetivo. Determinar el perfil etiológico de la infección de vías respiratorias bajas (IVRB) en los neonatos durante la temporada de virus sincicial respiratorio (VSR) y definir las características de esta y otras infecciones. Métodos. Estudio retrospectivo con neonatos hospitalizados por IVRB durante dos temporadas de VSR, con reacción múltiple en cadena de la polimerasa en tiempo real. Se revisaron los datos relevantes y se compararon las características de la infección por VSR con las de otros microorganismos. Resultados. Entre 224 pacientes, 160 (71 %) fueron positivos para, al menos, un agente causal potencial. Entre ellos, el 65 % tenía VSR y el 15 %, más de un agente (coinfección). En el grupo con VSR, hubo más dificultad respiratoria (p < 0,01), radiografías de tórax anormales (p < 0,01), requerimiento de atención en terapia intensiva (p < 0,01) y más días de oxigenoterapia (p < 0,01). No obstante, hubo menos casos de fiebre al ingreso y menos días de antibioticoterapia (en ambos, p < 0,01), y la duración de la hospitalización no fue más prolongada. Los pacientes con coinfección requirieron atención en terapia intensiva en mayor medida que los demás (25 % contra 6,5 %, p < 0,01). Conclusión. Este estudio demostró que el VSR fue más frecuente en los neonatos hospitalizados por IVRB durante la temporada y condujo a una evolución más grave que otros microorganismos patógenos detectados. Posiblemente la gravedad de la infección por VSR se viera aumentada por la presencia de una coinfección y radiografía de tórax anormal.

The Turkish Neonatal Jaundice Online Registry: A national root cause analysis.

BACKGROUND: Neonatal jaundice (NNJ) is common, but few root cause analyses based on national quality registries have been performed. An online registry was established to estimate the incidence of NNJ in Turkey and to facilitate a root cause analysis of NNJ and its complications. METHODS: A multicenter prospective study was conducted on otherwise healthy newborns born at ≥35 weeks of gestation and hospitalized for only NNJ in 50 collaborator neonatal intensive care units across Turkey over a 1-year period. Patients were analyzed for their demographic and clinical characteristics, treatment options, and complications. RESULTS: Of the 5,620 patients enrolled, 361 (6.4%) had a bilirubin level ≥25 mg/dL on admission and 13 (0.23%) developed acute bilirubin encephalopathy. The leading cause of hospital admission was hemolytic jaundice, followed by dehydration related to a lack of proper feeding. Although all infants received phototherapy, 302 infants (5.4%) received intravenous immunoglobulin in addition to phototherapy and 132 (2.3%) required exchange transfusion. The infants who received exchange transfusion were more likely to experience hemolytic causes (60.6% vs. 28.1%) and a longer duration of phototherapy (58.5 ± 31.7 vs. 29.4 ± 18.8 h) compared to infants who were not transfused (p < 0.001). The incidence of short-term complications among discharged patients during follow-up was 8.5%; rehospitalization was the most frequent (58%), followed by jaundice for more than 2 weeks (39%), neurological abnormality (0.35%), and hearing loss (0.2%). CONCLUSIONS: Severe NNJ and bilirubin encephalopathy are still problems in Turkey. Means of identifying at-risk newborns before discharge during routine postnatal care, such as bilirubin monitoring, blood group analysis, and lactation consultations, would reduce the frequency of short- and long-term complications of severe NNJ.

Effect of unconjugated hyperbilirubinemia on neonatal autonomic functions: evaluation by heart rate variability.

BACKGROUND: Serum bilirubin levels beyond the physiological limits, may lead to alterations in autonomic regulation in a newborn infant. Heart rate variability (HRV), is a noninvasive and quantitative marker of the activity of the autonomic nervous system (ANS). To date, few studies have demonstrated the undesirable effects of severe unconjugated hyperbilirubinemia (UHB) on autonomic functions, and only one study has used HRV as a marker of the autonomic activity. However, the relationship between altered cardiac autonomic functions and UHB by using the HRV derived from 24-hour Holter electrocardiography (ECG) recording has not been investigated previously. OBJECTIVE: We aimed to assess whether a relationship exists between severe UHB and cardiac autonomic dysfunction by evaluating HRV via 24-hour Holter ECG recording. METHODS: This single-center, prospective, case-control study was conducted on 50 full-term newborn infants with severe UHB requiring phototherapy and 50 healthy infants as controls. HRV assessment was performed by using 24-hour Holter ECG recording. RESULTS: There was no significant difference in terms of mean average heart rate, mean maximum heart rate and mean RR duration between the groups. However, mean minimum heart rate was significantly lower in the study group. When 24-hour time and frequency domain parameters were compared, time and frequency domain parameters rMSDD as well as high frequency (HF), which represent parasymphathetic activity, were significantly higher in the study group. Furthermore, low frequency to high frequency (LF/HF) ratio, that serves as an indicator of sympathovagal balance, was significantly lower in the study group. CONCLUSION: Severe UHB may cause cardiac autonomic dysfunction in favor of parasympathetic predominance in jaundiced neonates.

Is there a relationship between platelet parameters and patency of ductus arteriosus in preterm infants?

Postnatal closure of the ductus arteriosus is a complicated two-phase process involving functional and structural changes. So far, the precise mechanisms regulating this process are not fully understood. A growing body of evidence from recent studies suggests that platelets play a key role in inflammatory processes including ductal closure via interaction with endothelial cells. The aim of this study is to assess whether a relationship exists between the occurrence and/or closure of hemodynamically significant ductus arteriosus (HSDA) and platelet parameters (platelet count, circulating platelet mass, mean platelet volume, platelet distribution width) in preterm newborns. This single-center, retrospective study included 824 premature infants between 24 and 34 gestational weeks, evaluated by echocardiography at postnatal 72-96 h. Infants with and without HSDA (n = 208 vs. n = 616) were compared in terms of platelet parameters recorded within the first 3 days of life. Oral or intravenous ibuprofen was commenced for medical treatment, and echocardiography was repeated 24 h thereafter to determine ductal closure. No statistically significant difference could be demonstrated between the groups in terms of baseline platelet parameters. HSDA was independently associated with early-onset neonatal sepsis. Thrombocytopenia, low circulating platelet mass, high platelet distribution width, or high mean platelet volume could not be demonstrated as a risk factor for HSDA. None of the platelet parameters had an influence on ductal closure after medical treatment. Unlike most reports in the literature, presence of HSDA was not associated with any platelet parameter in our study. We could not demonstrate an association between any platelet parameter and either persistence or closure after medical treatment.

A Histologically Diagnosed Case with Infantile Osteopetrosis Complicated by Hypopituitarism.

Malignant infantile osteopetrosis is a rarely seen severe disorder which appears early in life with general sclerosis of the skeleton. It is caused by functionally defective osteoclasts which fail to resorb bone. Affected infants can exhibit a wide spectrum of clinical manifestations including impaired hematopoiesis, hepatosplenomegaly, visual impairment, and hypocalcemia. With the exception of secondary hyperparathyroidism, involvement of the endocrine system seems to be quite rare. Hypopituitarism is defined as underproduction of the growth hormone in combination with deficiencies of other pituitary hormones. Any lesion that damages hypothalamus, pituitary stalk, or anterior pituitary can cause secondary hypopituitarism. In this report, we presented a rare combination of malignant infantile osteopetrosis and secondary hypopituitarism in a newborn who presented predominantly with endocrinological symptoms. This is the first case report of malignant infantile osteopetrosis accompanied by hypopituitarism secondary to sclerosis of the sella turcica. On the other hand, this is a very interesting case which was diagnosed based on histological examination of bone marrow biopsy specimens despite lack of any clinical suspicion.

Two different patterns of mini-puberty in two 46,XY newborns with 17ß-hydroxysteroid dehydrogenase type 3 deficiency.

We report two newborns with female external genitalia and bilateral inguinal swelling who were diagnosed with 17ß-hydroxysteroid dehydrogenase type 3 deficiency, a rare cause of 46,XY disorder of sexual development. The first case had normal clitoral size and vaginal and urethral openings, palpable gonads in the inguinal region, low testosterone, and low levels of basal and GNRH-stimulated gonadotropin. The second case had similar external genitalia, low testosterone but borderline basal and normal stimulated gonadotropin levels. Low testosterone/androstenedione ratios (0.22 and 0.24, respectively; normal, >0.8) after human chorionic gonadotropin stimulation indicated 17ß-hydroxysteroid dehydrogenase type 3 deficiency. HSD17B3 sequencing revealed a homozygous novel mutation (c.464A>C, p.H155P) in exon 6 in the first case and homozygous c.239G>A (p.R80Q) in exon 3 in the second.

Can neonatal hepatitis be more fatal than biliary atresia?

BACKGROUND/AIMS: The basic problem in diagnosis of neonatal cholestasis (NC) is to differentiate biliary atresia (BA) from other non-obstructive disorders. Because if bile flow cannot be provided by surgery, BA leads to cirrhosis and death within the first year of life. The aim of the present study is to determine histopathological features that may help to differentiate BA from neonatal hepatitis (NH). MATERIAL AND METHODS: This retrospective study was carried out on 105 liver biopsy specimens of 74 infants with NC who were diagnosed between 2003 and 2012. RESULTS: The mean age was 76.5 ± 40.64 days. The most valuable biopsy findings for the discrimination between NH and BA, in decreasing order of importance, were ductular proliferation (p < 0.001), cholestasis in neoductuli (p < 0.001), fibrosis (p = 0.002), and extramedullar hematopoiesis (p = 0.02). While Kasai operations were performed in 19 cases, liver transplantation was performed in 10 cases. Survival rate among the death cases with BA was longer than the survival time of the death cases with NH (p = 0.023). Currently more children live with a close to normal quality of life with portoenterostomy and/or liver transplantation. On the contrary, NH can be more fatal with associated disorders such as growth retardation, specific infections, respiratory distress, and metabolic or endocrine diseases.

Is there a relationship between maternal periodontitis and pre-term birth? A prospective hospital-based case-control study.

OBJECTIVE: The aim of this study is to verify the existence of an association between maternal periodontal disease and pre-term delivery in an unselected population of post-partum Turkish women. MATERIALS AND METHODS: This case-control study was conducted on 100 women who gave birth in either a special or a government maternity hospital. The case group consisted of 50 mothers who had delivered an infant before 37 weeks' gestation and weighed under 2500 g. The control group included 50 mothers who had given birth to an infant with a birth weight of more than 2500 g and a gestational age of ≥37 weeks. Data of mothers and infants were collected using medical registers and questionnaires. Clinical periodontal examinations were carried out in six sites on every tooth in the mother's mouth. A participant who presented at least four teeth with one or more sites with a PPD ≥4 mm and CAL ≥3 mm at the same site was considered to have periodontal disease. Statistical methods included parametric and non-parametric tests and multiple logistic regression analysis. RESULTS: There were no statistically significant differences between the cases and controls with regard to periodontal disease and pre-term delivery (OR = 1.48; 95% CI = 0.54-4.06). CONCLUSION: The findings indicated that maternal periodontitis was not a possible risk factor for pre-term delivery. Further studies with additional clinical trials are needed to explore the possible relationship between periodontal disease and pre-term birth.

Predictors of postnatal complications and congenital cardiac diseases in infants of mothers with pregestational and gestational diabetes.

AIM: In this study, we aimed to evaluate the postnatal problems of infants of mothers with pregestational and gestational diabetes and the clinical properties of infants who were found to have congenital cardiac disease. MATERIAL AND METHODS: We retrospectively examined the records of 337 newborns who were followed up with a diagnosis of infant of diabetic mother between January 2010 and January 2012 in our Neonatology Unit. The demographic data of the diabetic mothers and their babies, the postnatal problems of the babies of diabetic mothers and congenital heart diseases found on transthoracic echocardiography were examined. RESULTS: The patients were classified as group A, B and C in accordance with the recommendations of The American Congress of Obstetricians and Gynecologists (ACOG) according to the type of diabetes. The most common postnatal problems included hyperbilirubinemia, respiratory distress, hypoglycemia and hypocalcemia. The rate of congenital heart disease was found be 17.3% in group A, 50% in group B and 9% in group C. No correlation was found between congenital heart disease and gender, multiple pregnancy, diabetes type, diet treatment, use of oral antidiabetic drugs and drug usage. A positive significant correlation was found between congenital heart disease and genetic disease, murmur, cyanosis and presence of gestational hypertension. It was shown that use of insulin, genetic disease and presence of gestational diabetes increased the risk of congenital heart disease. CONCLUSIONS: In our study, the overall incidence of congenital heart disease was found to be 24% in infants of diabetic mothers. It should be kept in mind that it is important to investigate the infants of mothers with pregestational and gestational diabetes in terms of the risk of congenital heart disease.

Epidemic adenoviral keratoconjunctivitis possibly related to ophthalmological procedures in a neonatal intensive care unit: lessons from an outbreak.

PURPOSE: Epidemic adenoviral keratoconjunctivitis can spread rapidly among preterm infants who frequently undergo ophthalmological examination. Here we present our experience on a nosocomial outbreak that affected 8 nursery staff members and 26 premature infants. We focus on the presentation and progress of the outbreak, the diagnosis of the disease and the measures taken for its control. METHODS: Data were collected from patients' files and records of the infection control team. Conjunctival swabs were collected to perform direct fluorescent assay (DFA) and viral culture. Diagnosis was made according to clinical evidence and/or detection of the virus. Statistical analysis was performed using SPSS 15.0 statistical software. RESULTS: Infection was introduced to our unit after a laser photocoagulation procedure of a 28-week gestational infant and circulated rapidly within the unit due to direct transmission through contaminated medical equipment, fomites and hands of nursery staff members. Neither the patients, nor the nursery staff members developed systemic symptoms. While DFA tests were positive in seven infants, culture positivity could be demonstrated in only three infants. Contact and droplet precautions were implemented with the recommendation of the infection control team. No recurrence occurred after definition of the last case on the 32nd day. CONCLUSION: Ophthalmologic procedures continue to be a potential source of adenovirus outbreaks. However, negligence of contact measures during routine daily nursing care seems to be a more important contributing factor for rapid spread. Strict adherence to appropriate aseptic procedures is required to prevent this potentially hazardous infection in preterm infants.

Comparison of oral and intravenous Ibuprofen for medical closure of patent ductus arteriosus: which one is better?

OBJECTIVE: Intravenous ibuprofen is an expensive drug that is being used currently for treating and preventing patent ductus arteriosus. Although oral ibuprofen is much cheaper, there is limited data published about its safety and efficacy. The aim of this study was to compare two forms of ibuprofen in terms of safety and efficacy in closure of patent ductus arteriosus. DESIGN: This is a single-center retrospective study. SETTING: Data were collected from patients' files of preterm infants who were hospitalized at the Neonatal Intensive Care Unit of Dr. Behcet Uz Children's Hospital between April 2009 and June 2010. PATIENTS: Six hundred sixty infants were evaluated by echocardiography between 24 and 48 postnatal hours. Clinically and hemodynamically significant ductus arteriosus was defined in 66 infants with gestational age less than 32 weeks and birth weight less than 1500 g. INTERVENTIONS: Oral or intravenous ibuprofen (loading dose: 10 mg/kg on day 1, followed by maintenance dose: 5 mg/kg on days 2 and 3) was administered. OUTCOME MEASURES: Treatment success was defined as a completely closed duct without reopening on follow-up. Drug-associated renal, gastrointestinal, cerebral, hematological, and metabolic side effects were monitored and compared between treatment groups. RESULTS: Ductal closure rates were 100% and 97.6%, respectively, in the oral and intravenous groups. Hypernatremia was the remarkable side effect in the intravenous group, whereas bronchopulmonary dysplasia and septicemia were prominent in the oral group. No statistically significant difference could be demonstrated between the groups in terms of mortality rates. CONCLUSION: Oral ibuprofen therapy is as efficacious as intravenous ibuprofen with some concerns about increased sepsis and bronchopulmonary dysplasia incidence. However, comprehensive and large-scale pharmacokinetic studies are required in order to prove this efficacy. On the other hand, intravenous ibuprofen still remains to be the drug of choice for patent ductus arteriosus but only with meticulous control of serum sodium levels in smaller infants.

Investigation of acquired von Willebrand Syndrome in children with hypothyroidism: reversal after treatment with thyroxine.

BACKGROUND: Acquired von Willebrand Syndrome (AvWS) is a rare bleeding disorder associated with various underlying conditions. Many case reports have been published so far on bleeding tendency in hypothyroidism resembling AvWS. OBJECTIVE: This study was designed to define the relationship between hypothyroidism and AvWS and to investigate the effects of L-thyroxine treatment. SUBJECTS: Twenty four hypothyroid patients were included in the study. Nineteen patients were evaluated during treatment, 5 patients were evaluated before hormone replacement. METHODS: Complete coagulation screening tests including levels of von Willebrand Factor antigen (vWF:Ag) and functional activity (vWF:RCo) were measured by thrombocyte aggregometer. RESULTS: We demonstrated low vWF:Ag and vWF:RCo in 13 patients. Two of the 13 patients were diagnosed as AvWS, while another 2 patients were diagnosed as hereditary vWD Type 1. The remaining patients are still being followed-up. CONCLUSION: We would like to attract the attention of paediatricians to the possibility of bleeding due to decreased activity of vWF in hypothyroid children.