Background. The significant advancement in the treatment of respiratory distress syndrome can be attributed to prenatal identification of high risk pregnancies, prevention of illness through antenatal care, prenatal administration of glucocorticoids, advancement in respiratory support and surfactant therapy. These measures resulted in the reduction of mortality and morbidity rates in preterm infants. Patients and methods. We analyzed data of 78 preterm babies with respiratory distress syndrome hospitalized in the NICU of the Pediatric Clinic, KCU Sarajevo. All children included in the study were mechanically ventilated and treated with one or more doses of bovine surfactant (Survanta) as rescue therapy. Surfactant was given to children with clinical and radiological signs of RDS, who required FiO2>0,40. We used the standard procedure of giving surfactant therapy to intubated children in sterile conditions, after we confirmed, by X-ray, correct tube placement. Results. We investigated the clinical efficacy of surfactant in relation to time of administration, O 2 requirement and necessity of one or more doses of surfactant. We found that early treatment with surfactant replacement- within 6 hours of birth- is more effective, and resulted in a significant reduction of mortality rate (p<0,01). Treatment with multiple doses is more effective in comparison to one dose, although there was not a significant difference (p<0,20) between the treated groups. There is a significant difference (p<0,01) between groups related to O 2 requirement. In the group of babies which required 60% or more O 2 concentration in inhaled air at the time of surfactant replacement, mortality rate was significantly higher (p<0,01). Conclusion. Our study confirmed the benefits of surfactant therapy in preterm babies with respiratory distress syndrome. We confirmed the advantages of early treatment vs. late treatment, but we could not confirm the obvious advantage of multiple over single doses. So, a reasonable recommendation is to treat the infants as soon as clinical signs of developing respiratory distress appear with an individual dose for each infant.

Wolf-Hirschhorn Syndrome: Report of two Cases in Bosnia and Herzegovina Wolf-Hirschhorn syndrome (WHS) is a rare developmental disorder caused by a partial deletion of the short arm of chromosome 4 (4p-). The main phenotypic characteristics of WHS are: intrauterine growth retardation, mental retardation, typical facial dysmorphism, microcephaly and midline fusion defects (cleft lip or palate, cardiac septal defects). Other abnormalities, such as agenesis of the corpus callosum, dysplastic kidneys, iris coloboma and skeletal abnormalities have occasionally been described. We describe two female newborn babies with a 4p deletion, who have a majority of the main phenotypic features of WHS. Prenatal diagnosis of the syndrome is very important, because dysmorphologic features are associated with profound mental retardation. Postnatal recognition of the syndrome requires genetic counseling of the parents and supportive multidisciplinary treatment.

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.

Pena-Shokeir syndrome is a rare lethal disorder which clinical phenotype is the result of a deformation sequence caused by fetal akinesia/hypokinesia. In approximately 50 % cases an autosomal recessive mode of inheritance were documented. Main clinical characteristics of Pena-Shokeir syndrome are: multiple ankylosis, pulmonary hypoplasia and facial dysmorphism. We have described female newborn with pulmonary hypoplasia, distal joint contractures and facial characteristics of Pena Shokeir syndrome. Our patient had hip and knee ankyloses, ulnar deviation of the hands, aberrant hand position--similar to that of trisomy 18, absent dermal ridges on the palms, talipes valgus and facial dysmorphism (prominent eyes, micrognathia, poorly folded and posteriorly rotated ears). During pregnancy polyhidramnion was documented. Among CNS abnormalities, agenesis of corpus callosum was found. Because of eventration of right hemidiaphragm, weakness of intercostal muskles and pulmonary hypoplasia, child was artificially ventilated from the birth, and died due to pulmonary complication in the second month of the life. Although the majority of those live-borne with Pena Shokeir syndrome die within the first month of life, postnatal recognition requires genetic counseling of parents and obtaining early prenatal diagnosis in next pregnancy.

Background. Invasive candidiasis in neonates has become an increasing problem over the past decade in Neonatal Intensive Care Units (NICUs) in the world; it is a relatively common cause of late onset sepsis associated with a high mortality. Risk factors are preterm babies who require invasive diagnostic and aggressive therapeutic procedures, prior antibiotic therapy, congenital abnormalities, gastrointestinal tract diseases, necrotizing enterocolitis and anatomical abnormalities requiring surgery. Prior colonization is the major risk factor for candidemia.Methods. To determine the rate of colonization, risk factors and the possible modes of acquisition of Candida spp. In neonates in NICU, we conducted a prospective cohort study including 48 successive admissions at NICU of Pediatric University Hospital, Sarajevo, during 3 months. Samples (mucocutaneous swabs, urine, stool, blood) were obtained weekly until the time of discharge or death (one infant enrolled in the study exited). Care health worker hands were cultured weekly (55 samples).Results. Candida spp. from various body sites samples were isolated in 7 patients (rate of colonization 14,8%) while 7 samples of health workers hands were positive for C. spp (14,5%). Patients colonizated or infected by Candida spp. In our study were or very preterm newborns (3/7) or compromised term newborns (4/7), with congenital abnormalities (2/7) or gastrointestinal surgical interventions (2/7). Two very preterm neonates were colonized early from the birth, which indicates possible vertical transmission. In other 5 patients, colonization occurred more lately, after at least 15 days stay in NICU, which indicates nosocomial transmission as a predominant mode of acquisition. Among 10 positive patterns from different body sites, Candida albicans was isolated in 6 cases; other 4 cases were C. glabrata (2/10), C. krusei (1/10) and C. famata (1/10).Conclusions. Rather than focusing on methods of identifying of pregnant women with vaginal Candida colonization, attention should be directed to high risk neonates and infection control measures including hands washing by health care workers.

Wolf-Hirschhorn syndrome (WHS) is a rare developmental disorder associated with hemizygous deletion of short arm of chromosome 4. Main phenotype characteristics of WHS are: intrauterine growth retardation, mental retardation, typical facial dysmorphism ("Greek warrior helmet"), microcephaly and midline fusion defects (cleft lip or palate, cardiac septal defects). Other abnormalities, like agenesis of corpus callosum, dysplastic kidneys, coloboma iris, skeletal abnormalities have been described occasionally. We described female newborn baby with 4p deletion, who had most of the phenotype characteristics of Wolf-Hirschhorn syndrome: intrauterine growth retardation, microcephaly, facial dysmorphism, congenital hypotonia and seizures in neonatal period. Other abnormalities included: hypoplastic cervical vertebra C4-C5, renal cyst and partial agenesis of corpus callosum. Patient had not cardiac septal defect. Due to a broad spectrum of possible morphologic abnormalities followed by mental retardation, prenatal diagnosis is very important. Postnatal recognition of the syndrome requires genetic counseling of parents and supportive multidisciplinary treatment.

Although the incidence of intra-periventricular hemorrhage has reduced in recent years, the increasing survival rates for the very low birth babies indicates that the lesion remains to be a big problem. Neuropathologic base of IVH-PVH is subependimal bleeding into germinal matrix, with consecutive dilatation of lateral ventricles and possible development of germinal matrix destruction, posthemorrhagic hydrocephalus and periventricular hemorrhagic infarction. Pathogenesis of IVH-PVH is multifactorial with participation of intravascular, vascular and extravascular factors. Detection of IVH-PVH and its neuropathologic consequences has been facilitated greatly by the introduction of real-time cranial ultrasonography. Long-time prognosis relates to the mechanisms of brain injury, i.e. presence of hypoxic-ischemic injury, posthemorrhagic hydrocephalus and periventricular hemorrhagic infarction. Extensive parenchymal lesions are associated with frequent subsequent motor, less frequent cognitive deficits. Prevention of IVH-PVH remains the most important goal. That includes prenatal interventions, such as prevention of premature birth, transportation of the premature infant "in utero", prenatal administration of phenobarbital and K-vitamin and optimal management of labor and delivery. Postnatal interventions include careful newborn resuscitation, correction of fluctuating cerebral blood velocity, correction of hemodynamic disturbances and pharmacologic interventions. Postnatal administration of different medicaments (phenobarbital, indomethacin, ethamsylate and vitamin E), have showed inconsistent results in reduction of IVH-PVH, thus there is no pharmacologic agent yet to be recommended for routine use in premature infants.

Neural tube disorders develop as a result of failure of neural tube closure between 3rd and 5th gestational weeks. This failure can cause soft structure anomalies (spina bifida, lumbal meningocela) or possible can contain neural tissue (meningomyeloccla, encephaloccla). Etiology of this disorder is not clear enough, and probably has multifactorial roots. Besides genetic factors, there are impact of some nutritional causes like folic acid. 28 cases with neural tube dysraphism hospitalized during period August 1999 till August 2002. at the Pediatric Clinic KCU Sarajevo were analyzed through retrospective study. 19/28 (67.8%) of newborn were from controlled pregnancy but without folic acid supplementation, 4 of them (14.2%) had prenatal diagnosis. Dysraphic disorder was the most often accompanied by paraplegia 16/28 (57.1%), hydrocephalus 17/28 (60.7%), from which 6/17 (35.2%) with Arnold Chiary malformation. 13/28 (46.4%) had skeletal deformities. Active preoperative treatment was conducted in 20/28 (71.4%) cases, and the rest of them were treated with home palliative care because of parent's rejection of surgery or major accompanied anomalies presence. In order to decrease the incidence of dysraphic disorders it is necessary to conduct periconceptional folic acid prevention, and provide early prenatal diagnosis. Long term treatment of children with meningomyclocele requires multidisciplinary approach that includes surgeons, orthopedists, pediatricians, physical therapists, in order to improve life quality of survived children.

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.

Birth asphyxia is O2i CO2 exchange disorder during the labour, with consequent hypoxia and ischemia. The term "asphyxia" has been used unprecisely quite often. The most frequently used criteria for birth asphyxia have been: fetal bradycardia, meconium stained amniotic fluid, fetal acido-base status with umbilical artery pH value below 7.10, low Apgar score and need for endotracheal intubation. The correct Apgar score quantification depends on the examiner. Fetal acido-base status measured in umbilical artery could be useful biochemical parameter of birth asphyxia. Only if the fetal oxygen supply during the labour is severe and long enough disturbed, the neurological abnormalities will develop later. Our study has enrolled 70 children with various degree of motor impairment, detected during neonatal period and/or infancy. They have been followed up till 24 months chronological age for term neonates and 24 months corrected age for prematures. 34 children out of them have developed clear clinical signs of cerebral palsy. Birth asphyxia as a possible cause of cerebral palsy has been documented in 10 cases, e.g. 29.4%. The criteria for birth asphyxia have been low Apgar score, meconium stained amniotic fluid and clinical signs of hypoxic-ischemic encephalopathy. Fetal blood gas and acido-base measurements obtained from umbilical artery at delivery have been an important parameter of intrapartal asphyxia. Those measurements should be introduced as a routine method in our practice, in the cases of fetal heart deceleration, to asses the extent of fetal acidosis.

Major intraventricular hemorrhage (IVH) and cystic leucomalacia (PVL) are major causes of mortality and neurologic morbidity in premature infants that are strongly associated with long-term neurodevelopmental sequelae. Preterm babies ventilated by intermittent positive pressure ventilation are in higher risk for this type of lesion. We studied incidence of major intraventricular bleeding (with ventricular dilatation and parenchymal involvement) and cystic periventricular leucomalacia in 79 preterm babies who required mechanical ventilation for respiratory-distress syndrome (RDS), admitted on Department of Neonatology of the Pediatric University Hospital in Sarajevo from February 1999. to May 2000. Thirty-five babies were classified as less than 32 weeks of gestation. Major intraventricular hemorrhage and cystic leucomalacia were documented in 5 babies (10.2%) of 49 survivors. Two babies developed posthemmoragic hydrocephalus and required shunting. Severe brain damage was more common in ventilated infants with gestational age with (32 weeks of gestation (23.5%) compared with the babies > 32 weeks of gestation (2.9%). The severity of RDS also correlated with an increased risk of severe IVH.

The pulmonary complications (PC) during mechanical ventilation were caused by barotrauma, infection, oxygen toxicity and excessive pulmonary extravascular fluid. The most frequent manifestations are: pneumothorax, pulmonary haemorrhage and lobar or segmental athelectasis. This is a retrospective study conducted between 1.1.1990 till 31.8.2000. The aim of this study was to evaluate the frequency of the pulmonary complications during mechanical ventilation in 163 ventilated neonates, as well as the relation towards gestational age, birth weight, primary disease and the outcome. The most common causes for mechanical ventilation were: hyaline membrane disease in 95 (58.3%) preterm babies, perinatal asphyxia in 26 (16%) and less frequent conditions such as: apnea, congenital heart diseases, neonatal infections and surgical cases. 25/163 (15.3%) ventilated pts have developed pneumothorax, 29 (17.7%) athelectasis and 14 (8.5%) pts had pulmonarly haemorrhage. The pulmonary complications were more frequent in preterm newborns with significant influence at the duration of the ventilation and the outcome. However, pulmonary complications could not be completely avoid, so we would like to underline the importance of preventive measures with the aim of decreasing PC incidence.

The high-frequency ventilation (HFV) has been in use in the World since early 70s, on patient (RDS, MPH, PPHN, etc.) who did not respond well as it was expected, on conventional mechanical ventilation (IPPV/IMV). At our Clinic we started to use this type of ventilation two months ago, and throw this work it was presented our first experience. In case report was showed massive pulmonary hemorrhage (MPH) in premature newborn 30 GW, where we applied HFV. Immediately after the bird conventional mechanical ventilation has been applied with positive pressure (IPPV) and surfactant has been given because RDS. After the MPH appeared we successfully applied HFV.

Using a new concept of terminology for sepsis syndrome, septic shock and multiorgan dysfunction/failure syndrome (MODS/MOSF) as a framework, we have evaluated 21 cases of severe sepsis of new admitted newborns in Neonatology Department of Pediatric clinic of Clinical center University of Sarajevo during last 2 years. We found that the most common etiologic agents were gram negative organisms (Klebsiella pn. and E. coli) and staphylococci. We did not observed any streptococcus group B sepsis despite of high incidence of these infections in developed countries. In 12 (60%) of 21 patients with severe sepsis MODS was developed, involving at least two organ systems, 3 of them also had the other acute insult (emergency surgery and asphyxia) as a possible trigger for MODS. The incidence of specific organ failure was: CNS (58%), respiratory (50%), cardiovascular (41%), the other systems were less involved. The overall mortality rate of patients with sepsis was 28%, whereas the overall mortality rate of MODS/MOSF was 50%.

We investigated 185 newborns with hyperbilirubinemia hospitalised in Neonatal Unit of Pediatric clinic during 1997. We identified an apparent cause for the jaundice in 76 newborns (41.0%), but in 109 newborns (58.8%) no cause for the jaundice was found. Contributing factors for this jaundice were: male sex, preterm bith, breast feeding, considerable weight loss, instrumental or induced delivery as well as genetic factors. Exchange transfusion was done in only 2 cases (1.08%) with Rh isoimmunisation and positive Coombs test, which is according to attitude that blood exchange transfusion as an expensive procedure with high mortality and morbidity has largely been replaced by phototherapy because of its relative freedom from complications together with its non-invasive nature. The average duration of hospitalisation of hyperbilirubinemic newborns lasted 4.8 days. We consider to make all efforts to short duration of hospitalisation time, because separation of newborn from the mother during phototherapy has a negative impact on early mother-infant social and emotional interaction.