Epidemiologic and clinical characteristics of selected congenital anomalies at the largest Bosnian pediatric surgery tertiary center.

Congenital anomalies (CA) are any abnormality present at birth, either structural or functional, that may potentially affect an infant's health, development, and/or survival. There is a paucity of studies on clinical characteristics and outcomes of CA in Bosnia and Herzegovina, mainly due to the lack of a nationwide congenital malformations monitoring system. A 5-year hospital-based study was conducted to determine the prevalence at birth and clinical characteristics of selected major CA in Sarajevo Canton, Bosnia and Herzegovina. Ninety-one CA were observed from 2012 to 2016 (the overall prevalence was 39.6 cases/10,000 live births). The mean age of neonates at diagnosis was 3 days. The gastrointestinal tract was the most commonly affected system (76.9%), with esophageal atresia (EA) being the most frequent (17.6% of all CA). Major CA were more prevalent among preterm infants than term infants (P = .001), particularly in males (61.5% vs. 38.5%; P = .028; M:F ratio was 1.59). Multiple CA were seen in 37.4% of neonates. The overall mortality rate of neonates was 11%, and the median length of hospital stay was 19.8 days. Our study revealed the distribution and clinical patterns of common major CA in the largest tertiary care facility in Bosnia and Herzegovina. It also confirmed a relatively high mortality rate, which requires further efforts to improve the quality of neonatal care in the country.

Biochemical predictors of death before discharge in cooled newborns following perinatal asphyxia.

Aim To analyse biochemical markers as possible predictors of death before discharge in cooled newborns following perinatal asphyxia. Methods A total of 91 infants that underwent therapeutic hypothermia after perinatal asphyxia were included. Inclusion criteria for therapeutic hypothermia were Sarnat stage 2 or 3. Data were collected from medical histories regarding gender, gestational age, birth weight, Apgar and Sarnat score; additionally, gas analyses, liver and cardiac enzymes before, and in the first 12 hours after starting therapeutic hypothermia, were evaluated. The patients' characteristics were compared between two groups, survivors and non-survivors. Results Statistical difference was not found between groups regarding gender, gestational age, birth weight, delivery type, 1st and 5th minute Apgar score, seizures, alanine aminotransferase (ALT), creatine kinase (CK), troponin and fibrinogen level. Groups were significantly different regarding acid-base balance (p=0.012), base excess (BE) (p=0.025), lactate (p=0.002), aspartate aminotransferaze (AST), (p=0.011), lactate dehydrogenase (LDH) (p=0.006), activated partial thromboplastin clotting time (aPTT) (p=0.001) and international normalized ratio (INR) (p=0.001). Conclusion Acid-base balance, BE, lactate, AST, LDH, aPTT and INR were significantly higher in the group of cooled newborns after perinatal asphyxia (non-survivors), and can serve as predictors of death before discharge. Combining diagnostic modalities raises a chance for accurate prediction of outcomes of asphyxiated infants.

Lenticulostriate vasculopathy in routine brain ultrasonography in infants: next step?

Lenticulostriate vasculopathy (LSV) is a relatively common fi nding in routine cranial ultrasound examination that has been associated with many infectious and non-infectious conditions. The aim of this review was to provide a better understanding of LSV ultrasound fi nding, as well as the need for further laboratory and imaging examinations in infants. The most of the published studies represented small series, with few prospective long-term studies involving the control groups. Authors have mostly found an association between LSV, especially higher-grade (although there is no universally accepted classifi cation) with congenital cytomegalovirus (CMV) infection, classifying those children as at risk for sensorineural hearing loss. In contrast, some authors pointed out that LSV could be found relatively often, and believe that isolated LSV, especially lower-grade, is not predictive for an unfavourable outcome and a long-term prognosis. Therefore, although 35 years have passed since the first publication of LSV, there is still no consensus among experts on the clinical signifi cance of isolated LSV, but caution is certainly needed given the fact that most infants with congenital CMV are asymptomatic.

The Effects of Ranitidine Treatment on the Risk of Necrotizing Enterocolitis in Preterm Infants: A Case-Control Study.

INTRODUCTION: Gastric acidity plays an important role in the protection of infants against various pathogens from the environment. The histamine-2 receptor blockers (H2-blockers) are off-labeled drugs that are frequently prescribed in preterm neonates to prevent stress ulcers. The impact of the H2-blockers on the development of the necrotizing enterocolitis (NEC) in preterm infants is still controversial, particularly in the developing world. MATERIALS AND METHODS: One hundred twenty-two preterm infants were enrolled in the study. The multivariate logistic regression model was used to identify potential postnatal risk factors associated with NEC. RESULTS: Preterm infants (n = 51) with total NEC, medical NEC, and surgical NEC had the highest rate of receiving ranitidine compared with controls (n = 71) (39.2%, 19.6%, and 47.6%, p < 0.05). Logistic regression analysis revealed that ranitidine use and nosocomial infections were significantly associated with NEC development (odds ratios 1.55 and 3.3). CONCLUSIONS: We confirm that ranitidine administration was associated with an increased risk of NEC in preterm infants. H2-blockers use should be only administered in very strictly selected cases after careful consideration of the risk-benefit ratio.

The complete spectrum of pentalogy of Cantrell in one of a set of dizygotic twins: A case report of a rare congenital anomaly.

RATIONALE: Pentalogy of Cantrell (POC) is an extremely rare syndrome with an estimated incidence of 1:65,000 to 200,000 live births. Its complete form includes a midline epigastric abdominal wall defect, defects affecting the lower sternum, anterior diaphragm, diaphragmatic pericardium, and various intracardiac defects. PATIENT CONCERNS: We report a case of complete POC affecting only the first-born of a set of premature dizygotic twins. DIAGNOSIS: A giant omphalocele with an eviscerated liver and bowel on prenatal, obstetric ultrasonography at 24 gestational weeks was observed. At birth, physical examination confirmed a massive (10 × 8 cm) epigastric omphalocele in which a significant part of the liver was seen. A postnatal echocardiogram revealed the presence of an ostium secundum atrial septal defect, perimembranous ventricular septal defect, and moderate pulmonary stenosis. X-ray showed an abnormal intrathoracic positioned stomach, which was confirmed with a plain x-ray of the upper intestinal tract with hydrosoluble contrast. Computed tomography (CT) scan revealed the sternum's absence and a close connection between the pericardial sac and the stomach wall. INTERVENTIONS: The patient underwent surgical intervention at 18 days of age. OUTCOMES: Despite adequate and appropriate postoperative treatment, the baby rapidly deteriorated and died 72 hours after surgery. LESSONS: POC is a complex, high-mortality syndrome whose management requires a multidisciplinary approach and meticulous planning. Despite all efforts, POC carries a poor prognosis, particularly in patients affected by its complete form.

The contribution of morphine sulfate to the development of necrotizing enterocolitis in preterm infants: a matched casecontrol study.

Zvizdic Z, Milisic E, Jonuzi A, Terzic S, Zvizdic D. The contribution of morphine sulfate to the development of necrotizing enterocolitis in preterm infants: a matched case-control study. Turk J Pediatr 2019; 61: 513-519. The aim of the study was to determine whether morphine sulfate administration is associated with an increased risk of necrotizing enterocolitis (NEC) in preterm infants supported by mechanical ventilation due to respiratory failure. The matched case-control retrospective study was carried out at the Neonatal Intensive Care Unit (NICU) of the University Clinical Center Sarajevo, on 122 preterm infants classified into total NEC group and control group. The total NEC group was further divided into medical NEC and surgical NEC subgroups. The association between the use and duration of morphine sulfate infusion and the development of NEC was evaluated in both unadjusted and adjusted analysis. Preterm infants who developed NEC were on mechanical ventilation more frequently compared to premature infants without signs of NEC (Mann- Whitney U test; p=0.0031). A positive correlation between the frequency of receiving morphine sulfate and the development of NEC was observed (Chi square test of independence; p=0.0001). The risk of NEC in preterm infants was increased by the use of morphine sulfate. Validation of this observation in other populations is warranted.

Assessing mortality risk in very low birth weight infants.

INTRODUCTION: Preterm birth is the most important univariant risk factor of neonatal mortality. Assessment of risk factors affecting mortality in preterm infants with very low birth weight is important for the treatment of this highly vulnerable population. OBJECTIVE: Detection of risk factors for neonatal mortality in very low birth weight premature infants. METHODS: The current study was conducted in a tertiary research and educational hospital, NICU, Pediatric Clinic KCU Sarajevo, from January 2010 to December 2010. After admission CRIB score was determined to every hospitalized infant with birth weight < 1500 g, born before the full 31 weeks of gestation (30 weeks + 6 days). We also gathered information about the Apgar score in 5th minute, gender, presence of respiratory distress syndrome and hemodynamic stability. 67 infants fulfilled inclusion criteria. RESULTS: Mean birth weight was 1136.4 g +/- 250.9, range 550-1500 g. Mean gestational age was 27.29 weeks +/-1.97, range 22-30 weeks. Mean CRIB score was 3.22, range 0-18. Twenty VLBW infants out of 67 died (29.85%). There was significant difference between groups of survived and dead infants regarding gestational age, birth weight, Apgar score, Crib score, base excess, presence of respiratory distress syndrome and hemodynamic stability at the birth. CONCLUSION: CRIB score, birth weight, gestational age, base excess, Apgar score, respiratory distress syndrome and hemodynamic instability are valuable predictors for a neonatal mortality in population of preterm infants with very low birth weight.

Familiar spastic paraplegia presenting in a boy with Klinefelter syndrome--case report.

In this case report, the boy with familiar spastic paraplegia, the relatively rare genetic disorder and Klinefelter syndrome that was found during investigation, has been presented. The diagnosis of the disease has been established by anamnesis, clinical features and relevant diagnostic procedures, so the criteria for autosomal dominant type of the familiar spastic paraplegia have been fulfilled. The therapeutic possibilities are limited to the physical therapy and orthopedic treatment of feet deformities.

[Fryns syndrome]. / Fryns-ov syndrom.

Fryns syndrome is an extremely rare developmental disorder associated with deletion of long arm of chromosome 16. Characteristics of the Fyns syndrome are: craniofacial dysmorfism, diaphragmatic defects with lung hypoplasia, distal digital hypoplasia, brain and urogenital abnormalities and other developmental disturbances. After the first description in two stillborn sisters by Fryns (1971), new reports appeared with descriptions included disorders which have not described previously. We described a case of female live born with deletion of long arm of chromosome 16. Our patient had a typical craniofacial dysmorfism, brain abnormalities (Dandy Walker malformation), cardiac defects (artial septal defect and persistent ductus arteriosus), renal hypoplasia, gastrointestinal problems, hypotonia and feeding difficulties. Our patient had no diaphragmatic hernia and he survived neonatal period with severe neurological impairment.

[Monitoring oxygen saturation in the pediatric population]. / Monitoring saturacije kiseonikom u pedijatrijskoj populaciji.

Pulsoxymetry is noninvasive technique which is in use for percentage estimation of hemoglobin saturation of oxygen in arterial blood. It represents a therapeutical screening of treatment in paediatric population. The aim of this retrospective study is to evaluate the validity of noninvasive technique for determination of oxygen concentration in cardiac and pulomological patients. The study included 221 patients, with a mean age of 39 months (1.5-192) who were hospitalised during the period of 1.6.2000. till 31.12.2002. at Pulmoalergology and Cardiorheumatology department of Paediatric clinic CCU Sarajevo. Three groups of patients were evaluated: first one, which included 194/221 patients with obstructive and inflammatory lung diseases, 17/221 patients formed II group of pts with congenital heart anomalies (CHA) and the third one which had 10/221 patients with CHA associated with lung diseases. In all patients the diagnostic-laboratory investigations were done including transcutaneous determination of oxygen saturation by pulsoxyimetry (PO) Nellcor and Johnson-Johnson, as well as capillary, with adequate treatment. The mean oxygen saturation (OS) in the first group of patients at admission by PO was 86%, capillary 73.6%, and after treatment OS PO was 91.6%. In the II group the mean OS at admission was 88.5% PO, capillary 83%, after treatment 94.9%. The mean OS at admission in third patient's group was 76%, and after treatment 91.2% which is statistically significant (F = 0.03) and is in concordance with modern therapeutical approach. Pulseoxymetry is noninvasive, painless, simple method which offers a valid continuous data needed for the adequate choice of treatment in paediatric patients.