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.

Caudal block with analgosedation - a superior anaesthesia technique for lower abdominal surgery in paediatric population.

Aim To compare intraoperative hemodynamic and respiratory stability and postoperative emergence delirium between two anaesthesia regimens in children (caudal block with intravenous continuous analgosedation versus general endotracheal anaesthesia) and intensity of postoperative pain and quality of postoperative analgesia. Method Forty children aged 2-6 years who underwent lower abdominal surgery were randomized depending on performed anaesthesia into two groups: caudal block with analgosedation (group CB) and general endotracheal anaesthesia (group GA). Intraoperative hemodynamic and respiratory stability were evaluated measuring systolic blood pressure (SBP), diastolic blood pressure (DBP), mean arterial blood pressure (MAP), heart rate (HR) and arterial oxygen saturation (SaO2) in preinduction (t0), at the moment of surgical incision (t1), 10 minutes after surgical incision (t2) and at the time of skin suturing (t3). Postoperative emergence delirium was evaluated using Paediatric Anaesthesia Emergence Delirium score (PAED). Postoperative pain was evaluated by Children's and Infants' Postoperative Pain score (CHIPPS). Both scores were recorded every 5 minutes during first half hour postoperatively, additionally after 60 minutes postoperatively for CHIPPS score. Results SBP, DBP and MAP were lower at t1 (p<0.0001), t2 (p<0.05) and t3 (p<0.001) in the group CB. HR was lower at all studied time points (p<0.005) in the group CB. SaO2 was lower in the CB group but comparable with the GA group. PAED and CHIPPS scores were lower at 5, 10, 15, 20 and 25 minutes postoperatively (p<0.001) in the CB group. Conclusion Caudal block with analgosedation provides better control of intraoperative hemodynamic conditions, postoperative emergence delirium and postoperative pain than general endotracheal anaesthesia.

Predictors and incidence of hospitalization due to respiratory syncytial virus (RSV)-associated lower respiratory tract infection (LRTI) in non-prophylaxed moderate-to-late preterm infants in Bosnia and Herzegovina.

Prematurity is a risk factor for respiratory syncytial virus (RSV)-associated lower respiratory tract infections (LRTIs), due to immature humoral and cell-mediated immune system in preterm newborns, as well as their incomplete lung development. Palivizumab, a humanized monoclonal antibody against the F glycoprotein of RSV, is licensed for the prevention of severe RSV LRTI in children at high risk for the disease. This study is a part of a larger observational, retrospective-prospective epidemiological study (PONI) conducted at 72 sites across 23 countries in the northern temperate zone. The aim of our non-interventional study was to identify common predictors and factors associated with RSV LRTI hospitalization in non-prophylaxed, moderate-to-late preterm infants, born between 33 weeks and 0 days and 35 weeks and 6 days of gestation, and less than 6 months prior to or during the RSV season in Bosnia and Herzegovina (B&H). A total of 160 moderate-to-late preterm infants were included from four sites in B&H (Sarajevo, Tuzla, Mostar, and Banja Luka). We identified several significant intrinsic and extrinsic factors to be associated with the risk of RSV LRTI hospitalization in the preterm infants, including: comorbidities after birth, shorter hospital stay, admission to NICU/PICU while in the maternity ward, household smoking, low maternal age, breast feeding, number of family members, and history of family/paternal atopy. Overall, our results indicated that the risk of RSV LRTI in preterm newborns can be associated with different environmental and social/cultural factors, and further research is needed to comprehensively evaluate these associations.

Phenylketonuria screening and management in southeastern Europe - survey results from 11 countries.

BACKGROUND: We aimed to assess the current state of PKU screening and management in the region of southeastern Europe. METHODS: A survey was performed involving all identified professionals responsible for the PKU management in the 11 countries from South-Eastern region of Europe (Albania, Bulgaria, Bosnia and Herzegovina, Croatia, Kosovo, Macedonia, Moldova, Montenegro, Romania, Serbia, Slovenia). The questionnaire was designed to assess the characteristics regarding PKU management in three main areas: nation-wide characteristics, PKU screening, and characteristics of the PKU management in the responding centre. It consisted of 56 questions. The distribution and collection of the questionnaires (via e-mail) was taking place from December 2013 to March 2014. RESULTS: Responses from participants from 11 countries were included; the countries cumulative population is approx. 52.5 mio. PKU screening was not yet introduced in 4 of 11 countries. Reported PKU incidences ranged from 1/7325 to 1/39338 (and were not known for 5 countries). National PKU guidelines existed in 5 of 11 countries and 7 of 11 countries had PKU registry (registries included 40 to 194 patients). The number of PKU centers in each country varied from 1 to 6. Routine genetic diagnostics was reported in 4 of 11 countries. Most commonly used laboratory method to assess phenylalanine levels was fluorometric. Tetrahydrobiopterine was used in only 2 of 11 countries. Most frequently, pediatricians were caring for the patients. Dietitian was a member of PKU team in only 4 of 11 countries, while regular psychological assessments were performed in 6 of 11 countries. Patient's PKU society existed in 7 of 11 countries. CONCLUSIONS: The region of southeastern Europe was facing certain important challenges of PKU screening and management. Neonatal PKU screening should be introduced throughout the region. Furthermore, PKU management was falling behind internationally established standards-of-care in many aspects.

Newborn screening in southeastern Europe.

The aim of our study was to assess the current state of newborn screening (NBS) in the region of southeastern Europe, as an example of a developing region, focusing also on future plans. Responses were obtained from 11 countries. Phenylketonuria screening was not introduced in four of 11 countries, while congenital hypothyroidism screening was not introduced in three of them; extended NBS programs were non-existent. The primary challenges were identified. Implementation of NBS to developing countries worldwide should be considered as a priority.

The effects of antenatal corticosteroids and surfactant replacement on neonatal respiratory distress syndrome.

The goal of this study was to determine the effects of antenatal corticosteroids and surfactant replacement on the severity and frequency of Respiratory Distress Syndrome (RDS) in a cohort of premature infants born in Sarajevo, Bosnia and Herzegovina, from 2005 to 2007. The cohort consisted of 172 premature neonates with estimated gestational age between 26 and 34 weeks. Babies with IUGR, babies of diabetic mothers and babies with major congenital defects were excluded. Out of 172 neonates, 80 were treated antenatally with corticosteroids (single course of dexamethasone) and 92/172 were not. There was no statistical difference (p>0,5) in average gestational age (31,2 vs. 31,0 GW) and male/female ratio between investigated groups; there were significantly more male patients (p<0,05) in both groups. Frequency of RDS was significantly lower in the corticosteroid group (24/80) in relation to the control group (54/92) (p<0,001). Severe RDS was significantly (p<0,01) more frequent in the control group 34/53 (62,96%) then in the corticosteroid group 6/24 (25,0%). Bovine surfactant (Survanta) was given as a rescue therapy to 78 babies with clinical and radiological signs of RDS who required FiO2>0,40 and mechanical ventilation. Early surfactant administration within six hours after birth appeared to be effective at reducing mortality then later surfactant administration (p<0,005). In the group of babies requiring FiO2> or =0,6 at the time of surfactant replacement, the mortality rate was significantly higher (p<0,05). In conclusion, we confirm the efficacy of antenatal corticosteroid treatment and early surfactant treatment in a cohort of premature infants born in Sarajevo.

[Antenatal corticosteroids in respiratory distress syndrome prevention: efficacy in relation to treatment--delivery interval]. / Antenatalni kortikosteroidi u prevenciji respiratornog distres sindroma: ucinkovitost u odnosu na tretman-porod interval.

Antenatal corticosteroids given to women, who are 24 to 34 weeks pregnant and may deliver within the next 24 hours to 7 days, are associated with significant reduction in rates of respiratory distress syndrome, intraventricular hemorrhage and mortality of pre-term babies. The aim of this study is assessment of antenatal corticosteroid effectiveness in reduction of RDS incidence in optimal delivery-treatment interval, in comparison to babies delivered before and after the optimal treatment interval has elapsed. This investigation included 80 pre-term babies between 26 and 34 gestational weeks whose mothers received corticosteroids before delivery. Control group consisted of 92 children of the same gestational age, whose mothers did not received corticosteroids antenatally. Babies of diabetic mothers, babies with IUGR and babies with congenital abnormalities were excluded. RDS was significantly less frequent in babies antenatally treated by corticosteroids (x2 31,473 p < 0.0001 coefficient contingency 0.366) then in babies whose mothers did not received corticosteroids before delivery. The majority of babies, 54.67% (p < 0.01) were born in optimal interval, 24 hours to 7 days from the beginning of the treatment, 32.0% (24/75) children were born within 24 hours and 13.3% (10/75) were born more then 7 days after the start of treatment. Comparing the incidence of RDS between groups of children born in optimal treatment-delivery interval (1 -7 days) and in the group of children born within 24 hours or after 7 days from the beginning of the treatment, no significant difference was found. The effect was clinically comparable, which suggests the possibility of reduction treatment-delivery interval in acute clinical conditions.

The effects of antenatal corticosteroid treatment on IVH-PVh of premature infants.

Intraventricular-periventricular hemorrhage (IVH-PVH) is the most frequent type of intracranial hemorrhage in premature infants and the major cause of neurodevelopmental disabilities in children too. The objective of this work is to evaluate the effects of prenatal corticosteroid treatment on the incidence of IVH-PVH in premature infants. The study enrolled 163 prematures of 26-34 weeks' gestation. They have been divided into two groups: the experimental group (80/163), who have been treated with corticosteroids prenatally and control group (83/163), who have not received such treatment. There is statistically significant difference in IVH-PVH incidence between the experimental group (18/80) and control group (32/83) (chi2 =5,616, p<0,05). There is no statistically significant difference in Apgar score after 5 minutes between the experimental group and control group of IVH-PVH prematures, t= 0,121. There is no statistically significant difference in mean gestation age between the experimental group (30,74 weeks) and control group (29,97 weeks) of IVH-PVH prematures, t= 1,299. There is no statistically significant difference in mean birth weight between the experimental group (1479,44 grams) and control group (1379,37 grams) of IVH-PVH prematures, t= 0,913. Antenatal corticosteroid treatment of premature infants reduced the incidence of IVH-PVH significantly. There is no statistically significant difference in Apgar score after 5 minutes, mean gestation age and mean birth weight between the experimental and control group of IVH PVH prematures.

Congenital malformations of the central nervous system: clinical approach.

Central nervous system (CNS) malformations represent important factor of morbidity and mortality in children. The aim of the study was to determine the incidence, type and clinical features of CNS malformations in children who were admitted at the Neonatal and Child Neurology Department, Neonatal Intensive Care Unit and Paediatric Intensive Care Unit of Paediatric Clinic, University of Sarajevo Clinics Centre, from January 1st, 2002 to December 31st, 2006. There were total of 16520 admissions at the Paediatric Clinic over the studied period. CNS malformations, solitary or multiple, have been diagnosed in 100 patients (0,61%). The total number of various CNS malformations was 127. Lethal outcome was established in 9/100 cases (9%). The most frequent CNS malformations were neural tube defects 49/127 (38,6%). Hydrocephalus was seen in 34/127 (26,8%), microcephaly in 24/127 (18,9%), agenesis of corpus callosum in 10/127 (7,9%), Dandy Walker malformation in 6/127 (4,7%) and other CNS malformations in 4/127 (3,1%). In 20/100 of patients neural tube defect was associated with hydrocephalus (20%). CNS malformations were prenatally diagnosed in 13/100 of patients (13%). Primary prevention of CNS malformations can be improved in our country by better implementation of preconceptional folic acid therapy for all women of childbearing age. Secondary prevention by prenatal diagnosis requires advanced technical equipment and adequate education of physicians in the field of foetal ultrasonography. In our circumstances, prenatal diagnostics of CNS malformations is still not developed enough.

[Stroke in newborn infants]. / Mozdani udar u novorodenackoj dobi.

PURPOSE: Stroke is related to many different cerebrovascular events, with different ethiology and pathogenesis which are not clear enough. Frequency of the stroke in newborn infants is 1:4000 live births. Term infants are affected more frequently; clinical presentation typicaly is manifested by multifocal seisures between 1st and 4th day of life. Laboratory tests include analyses of prothrombotic factors which increase risk of thromboembolism. WORK METHOD: We descriptively showed a term newborn with of middle cerebral arthery infarction. CASE REPORT: A firstborn female neonate was received in intensive Care Unit because of meconial aspiration syndrome (MAS). In 3rd day of life the child had generalised seizure. Routine brain ultrasonography raised a high suspicion of middle certebral arthery infarction. The diagnosis was confirmed by CT, MRI and MR angiography. Child had not disturbances in coagulation status or other possible risk factors related to occurance of the stroke. CONCLUSION: In case of seizures between 1st and 4th day of life ,especially in term infants, cerebrovasular insult is one of possibilities, and adequate laboratory and neuroradiologic diagnostics should be done. Suspicion on middle cerebral arthery infarction can be made on the basis of brain ultrasonography, but for a definite diagnosis CT, MRI and AR angiografy are needed.

Saturation with oxygen for ductal dependent congenital heart diseases before and after the prostaglandin therapy.

Ductal dependent congenital heart diseases represent 14-20% of all congenital heart diseases. A primary goal of the treatment of these diseases is to retain ductus open until the final cardiosurgical treatment. Prostaglandins are presently the only medicaments, which have a capability to keep ductus open. By means of a retrospective study in a period from January, 2000 until December, 2002 at the Paediatric clinic of the Clinical centre of the University in Sarajevo, 14 patients (treated with prostaglandins) diagnosed with ductal dependent congenital heart diseases were analyzed. In our sample, there are 9/14 male patients (64.3%), 11/14 (78.6%) were full-term newborns, while 10/14 (71.4%) were eutrophic at birth. An average saturation increase, after the prostaglandin therapy, measured in blood from the capillaries is 29, and measured transcutanlly is 32 units. Duration of prostaglandin therapy in our study was on average 17.2 days. The most common cause of death was insufficientia cardiorespiratoria (4 out of 11), but sepsis/infection (3 out of 11) and insufficientia renalis were also common. 78.6% (11 out of 14) patients died partly because of the complexity of these diseases, but also because a cardiosurgical treatment is delayed. A goal of this study is evaluation of saturation with oxygen before and after the prostaglandin therapy.

[Neonatal hyperbilirubinemia: evaluation and treatment]. / Neonatalna hiperbilirubinemija: evaluacija i tretman.

We have analyzed 132 newborns with hyperbilirubinemia (BW < 2500 g, GW > 37 weeks) hospitalized in Neonatal Unit of Paediatric Hospital during 2001. In 78 out of 132 (59.1%) newborns, causal diagnosis were established. Rh isoimmunization caused hyperbilirubinaemia in 2.8% (3/132) cases, ABO isoimmunization in 15.95% (21/32), infections in 9.15% (12/32) and other (polycitaemia, kephalhaematoma, loss of weight > 8%, jaundice related to the brestafeeding) in 31.8% (42/132). Maximal serum bilirubin level was reached between fourth and seventh day of life. In 47/132 newborns (40.9%) non-specific hyperbilirubinaemia were diagnosed. All babies were successfully treated by fototherapy. Blood exchange transfusion was performed in only 2 cases. Rh isoimmunisation was not significant factor in etiology of hyperbilirubinemia because of global prevention of Rh negative mothers. Hyperbilirubinemia of "healthy full-term newborn" was most often seen in 40.9% cases. Diagnostic criteria for hyperbilirubinemia were revised, and fottherapy, as safe, non-invasive method, replaced blood exchange transfusion, as an expansive procedure with low but constant mortality.

[Incidence of early-onset neonatal sepsis caused by group B Streptococcus at the Pediatric Clinic of the University Clinical Center in Sarajevo]. / Incidenca rane neonatalne sepse streptokokom grupe B na Pedijatrijskoj klinici KCU Sarajevo.

Group B Streptococcus (GBS) is the main ethiological agent of neonatal sepsis in the developed countries. Because of high mortality rate American College of Obstetrics and Gynaecologists and Centers for Disease Control published recommendation for prevention of neonatal GBS infection. Program recommends screening of all pregnant women for anogenital GBS infection including intrapartum treatment of pregnant women at high risk, with penicillin or ampicillin. Clindamicin or erythromicin may be used for women allergic to penicillin. Since there is no detailed informations of early-onset neonatal GBS infection in our country, we investigated its incidence in Pediatric Clinic in Sarajevo, from December 1999 (when first case of early-onset GBS infection in last decade was diagnosed) to August 2002. During that period 7 neonates with early-onset GBS infection were identified (incidence 0.52 per 1000 livebirths). Four of seven sick newborns died, 2/7 survived with severe neurological sequelae and only one female newborn survived without deficits. In the same period we registered 36 (2.4%) GBS colonised neonates out of 1530 admitted neonates. The number probably is higher because of discharge GBS colonized asymptomatic neonates from Maternity as a healthy to home. Results of this study intend the necessity of implementation guidelines for GBS prevention since early onset GBS infection is becoming as an increasing problems in our population.