Preventive effect of ursodeoxycholic acid on parenteral nutrition-associated liver disease in infants.

INTRODUCTION: Parenteral nutrition-associated cholestasis is well recognized phenomenon in the term and preterm infant receiving long-term parenteral nutrition. OBJECTIVES: The aim of this study was to evaluate the effect of ursodeoxycholic acid (UDCA) use on cholestasis in newborns on prolonged TPN. METHODS: A total of 56 infants were enrolled in this retrospective study: control group consisted of lower (1500 g) birth weight infants (n = 30), as well as the group of pediatric (n = 11) and surgical patients (n = 15) treated with UDCA. Blood chemistries were obtained two times weekly. RESULTS: All of 56 newborns developed cholestasis but duration of parenteral nutrition (PN) before onset of cholestasis was significantly longer in UDCA treated patients. Average duration of PN before the onset of cholestasis in control group of patients was 25 days in distinction from treated pediatric and surgical patients (39 and 34 days, respectively).The peak serum conjugated bilirubin (CB), AST, ALT and alkaline phosphatase (AP) levels were significantly lower in the treated groups.There was no significant difference among treated pediatric and surgical patients and between lower and higher birth weight infants considering the CB, ALT, AST and AP peak. Duration of cholestasis was significantly decreased in all treated groups.There was a significant difference in time needed to achieve complete enteral intake between pediatric and surgical patient group. CONCLUSION: Cholestasis developed significantly later in treated groups than in the controls. UDCA appears to be very successful in reducing the symptoms of cholestasis. The difference in efficacy of UDCA treatment between lower and higher birth weight infants could not be proven.

Complex case of urethral duplication with megalourethra.

Urethral duplication and megalourethra are very rare anomalies and their concomitant presence is extremely rare, with only a few published cases. We present a complex case of complete urethral duplication with dorsal megalourethra that was severely stenotic in its bulbar part and meatus, with the ventral urethra atretic distally and dilated proximally. Both the corpus spongiosum and the cavernosum were missing. He had associated upper urinary tract abnormalities. Urethral patency was restored successfully by meatoplasty, staged buccal mucosa graft urethroplasty, and tailoring of the megalourethra. This report is unique regarding the use of a buccal mucosa graft for urethral reconstruction in patients with associated urethral duplication and megalourethra.

[Congenital hiatus hernia associated with reflux esophageal stenosis]. / Kongenitalna hijatusna hernija udruzena s refluksnom stenozom jednjaka.

A small group of three patients presented in our study represents extraordinary and very complicated problem of congenital hiatus hernia in infant period from 6th to 9th month of life, associated with gastroesophageal reflux and consecutive esophageal stenosis. There are two very rare and delicate entities within differential diagnosis, in the domain of the same pathology: congenitally short esophagus and congenital esophageal stenosis; with completely different surgical options for their treatment. That is why an optimal diagnostics and an adequate operative technique are extremely important for the treatment of hiatus hernia. The uppergastrointestinal barium radiography is definitely the method of diagnosing hiatus hernia, which provides typical, almost pathognomonic image of hiatus hernia accompanied by the esophageal stenosis. Nissen fundoplication is the technique of choice for its surgical treatment, with gastrostomy for probable postoperative esophageal dilatation. The results are more than satisfying: early peroral feeding, with spontaneous resolving of esophageal stenosis, which significantly diminishes the need for esophageal bougienage.

[Initial antibiotic therapy of neonatal sepsis]. / Inicijalna antibiotska terapija neonatusne sepse.

It is certain that in the past the types of bacterial agents responsible for neonatal sepsis and their sensitivity to antibiotics were not the same in all historical periods. However, the reports confirming the conclusion have been published only in the last three years. According to these facts, the bacterial causes of neonatal sepsis were analyzed in patients treated at the University children's hospital in Belgrade (S&M) as well as their sensitivity to antibiotics to determine the most effective initial therapy. Between January 2001 and June 2004, 35 neonates, aged from 1-30 days, with positive blood culture were treated. Gram-negative bacteria were the cause of sepsis in 57% of patients (Pseudomonas--20%, Klebsiella--20%, E. coli--8.5%, Acinetobacter--8.5%), gram-positive in 43% (coagulase-negative Staphylococci--14%, Staphylococcus epidermidis--14%, Staphylococcus aureus--9%, Streptococcus group B--3%, Listeria monocytogenes--3%). The bacteria were the most sensitive to carbapenems (85-89%), amikacin (68%), third-generation cephalosporins (47-50%), while the sensitivity to gentamicin was less than expected (48.5%). Sensitivity to ampicillin (8%) confirmed a high level of resistance to this antibiotic. All isolated Staphylococci were sensitive to vancomycin, and the overall methicillin resistance was 46%. Combined cefotaxime and amikacin therapy was the most effective of all suggested initial combinations of antibiotics (74%). The sensitivity to all other combinations of antibiotics was 51-71%. The most adequate initial combination of antibiotics for the treatment of neonatal sepsis is cefotaxime plus amikacin. The most adequate antibiotic for the treatment of nosocomial neonatal sepsis is carbapenem.

[Surgical management of developmental lung anomalies]. / Hirursko lecenje razvojnih anomalija pluca.

Prenatal diagnosis allows for insight into the evaluation of fetal lung anomalies. Serial ultrasonographic studies of fetuses helped in evaluation and definition of the natural course of these lesions as well as necessity for fetal therapy. It has been found that the overall prognosis depends on the size of the lung mass and the secondary derangement of normal lung tissue and cardiovascular system. Although much is known about the prenatal course of these anomalies, little is known about the postnatal course of asymptomatic patients. Infants who are symptomatic at birth require early surgical treatment. During the period from 1984-2003, 23 patients with congenital lung anomalies were treated, out of whom 19 were diagnosed postnatally and 4 prenatally. All postnatally diagnosed patients (9 congenital lobar emphysema, 5 congenital cystic adenomatoid malformations, 3 pulmonal cysts, 1 bronchopulmonary sequestration and 1 arteriovenous malformation) underwent surgical excision (lobectomy or sequestrectomy) after becoming symptomatic (main symptoms were infection or respiratory distress). Prenatally diagnosed patients (2 bronchopulmonary sequestrations and one enteric mediastinal cyst) underwent elective surgical interventions (sequestrectomy and excision of the cyst) in infancy. Postoperative course was uneventful in all patients. One patient probably had spontaneous involution of the mass. We believe that elective resection is indicated in asymptomatic neonates with congenital lung anomalies, because of the potential risk of infection and occult malignant transformation. Early resection also maximizes compensatory lung growth. This approach eliminates any risk of pulmonary infection, lung abscess formation or malignant transformation. In addition, subsequent exposure to radiation will be avoided.

[Persistent hyperinsulinemic hypoglycemia of the neonate]. / Perzistentna hiperinsulinemijska hipoglikemija novorodjenceta.

Persistent hyperinsulinemic hypoglicemia of the neonate is a rare heterogenous disease (clinically, histologically, metabolically and genetically), which is characterized by inadequatly high insuline rates in the presence of severe hypoglicemia. Hyperinsulinism, rather a syndrome than a disease, of which the main metabolic feature is hypoglicemia and decreased concentration of free fatty acids and ketones in serum (insulin inhibits lypolisis and synthesizes ketonic bodies), presents a major diagnostic and therapeutic chalenge. The disease is often followed by brain atrophy contributed by the attacks of hypoglicemia. It is inherited as an autosomally recessive and autosomally dominant disease. The genetic defects is located on the short arm of the chromosome 11. The authors report a successfully applied conservative treatment in a neonate with persistent hyperinsulinemic hypoglicemia.