Jurica Vuković

Successful use of recombinant activated FVII and aminocaproic acid in four neonates with life-threatening hemorrhage.

Reports on the use of recombinant activated factor VII (rFVIIa) to counteract hemorrhagic shock in neonates and preterm infants are increasing. rFVIIa enhances thrombin generation in situations with impaired thrombin formation and, since thrombin has a crucial role in providing hemostasis, rFVIIa is regarded as a general hemostasis agent. Full thrombin generation is necessary for the formation of a stable fibrin plug resistant to premature fibrinolysis. Antifibrinolytic drugs are not recommended for the treatment of acute bleeding. We report four neonates (one with massive postsurgical hemorrhage after ileostomy and three with severe pulmonary hemorrhage in the course of mechanical ventilation for meconium aspiration syndrome, congenital heart disease and during postoperative resuscitation after cardiac surgery for congenital heart disease) who were successfully treated with multiple administration of rFVIIa (120 μg/kg per dose) and antifibrinolytic therapy — aminocaproic acid (100 mg/kg per dose). In a fibrinolytic environment therapeutic concentrations of rFVIIa may sometimes be insufficient to produce adequate amounts of thrombin necessary for stable clot structure. Laboratory data in three of our patients with pulmonary hemorrhage (low fibrinogen levels with slightly prolonged prothrombin time) supported this thesis, so we blocked fibrinolysis with aminocaproic acid and achieved a complete clinical and laboratory therapeutic effect.

An unusual outcome in a child with hepatosplenic cat-scratch disease.

ZusammenfassungDie klassische Katzenkratzkrankheit (Infektion mit Bartonella hensalae) ist bei einem immunkompetenten Kind mit der Anamnese eines Kratzers, Bisses oder intimen Kontaktes mit einer Katze vergesellschaftet. Die meisten Patienten entwickeln nach drei bis 10 Tagen einen harten Knoten in der Kratzlinie. Dieser kann wenige Tage, aber auch zwei bis drei Wochen bestehen bleiben. Während der nächsten ein bis zwei Wochen, manchmal auch länger, schwellen die regionalen Lymphknoten, die das Gebiet drainieren, in 10% der Patienten allmählich an und wieder ab. Die Knoten entwickeln ein Erythem, manchmal eitern sie. In wenigen Fällen von Katzenkratzkrankheit ist der Verlauf atypisch: Es kommt dann zu Augen- oder neurologischen Symptomen, Befall der Leber und Milz, Osteomyelitis der Wirbel, Endocarditis und Ähnlichem. Die Infektion mit Bartonella hensalae kann bei immunkompromittierten Individuen zu einer bazillären Angiomatose, bzw. Peliosis und zu einer rezidivierenden Bakteriämie führen. Es gibt verschiedene Berichte über Granulome in der Leber oder Milz die bei immunkompetenten Kindern durch B. hensalae ausgelöst wurden. Wir berichten über einen sechsjährigen Knaben mit einer derartigen hepatosplenalen Form der Katzenkratzkrankheit. Trotz früher Diagnose und Langzeit-Antibiotika-Behandlung konnte bei ihm eine Splenektomie nicht vermieden werden.SummaryTypical cat-scratch disease (Bartonella henselae infection) in an immunocompetent child is usually associated with a history of scratch, bite or intimate contact with a cat. Most patients develop a non-tender papule in the scratch line after three to ten days. This may persist for only a few days or as long as two to three weeks. During the next two weeks or more, regional lymph nodes that drain the area gradually enlarge and then slowly resolve in more than 10% of patients. The nodes develop overlying erythema and may suppurate. Atypical forms of cat-scratch disease occur in a minority of cases and are characterized by ocular or neurological manifestations, hepatosplenic involvement, vertebral osteomyelitis, endocarditis etc. Immunocompromised individuals with B. henselae infection may develop bacillary angiomatosis, bacillary peliosis, and relapsing bacteremia. There have been several reports of hepatosplenic granulomas caused by B. henselae in immunocompetent children. We report a case of a 6-year-old boy with the hepatosplenic form of cat-scratch disease. Despite early diagnosis and long-term antimicrobial treatment, splenectomy could not be avoided.

Cardiopulmonary exercise performance is reduced in congenital diaphragmatic hernia survivors

Congenital diaphragmatic hernia (CDH) is associated with lung hypoplasia. CDH survivors may have pulmonary morbidity that can decrease cardiopulmonary exercise. We aimed to examine whether cardiopulmonary exercise testing (CPET) results differ in CDH survivors versus healthy age‐matched controls and whether CPET results among CDH survivors differ according to self‐reported daily activity.

Giant Mycotic Right Atrial Thrombus Due to Candida dubliniensis Septicemia in a Premature Infant

Rad opisuje slucaj ekstremne mikoticne tromboze desnog atrija u nedonosceta vrlo male rodne mase te izljecenje nakon trombektomije. U prilogu rada je i poveznica na ultrazvucni prikaz flotirajuceg tromba u desnom atriju.

Postoperative Gastric Perforation in a Newborn with Duodenal Atresia

Gastric perforation (GP) in neonates is a rare entity with high mortality. Although the etiology is not completely understood, it mostly occurs in premature neonates on assisted ventilation. Combination of duodenal atresia and gastric perforation is very rare. We present a case duodenal atresia who developed gastric perforation after operetion for duodenal atresia. Analysis of the patient medical record and histology report did not reveal the etiology of the perforation.

Massive retroperitoneal haemorrhage in a neonate with severe haemophilia A

A case report of neonate with severe haemophilia A and massive retroperitoneal haemorrhage.

Molecular basis and clinical presentation of classic galactosemia in a Croatian population

Abstract Background: Classic galactosemia is an autosomal recessive disorder of galactose metabolism caused by severely decreased activity of galactose-1-phosphate uridylyltransferase (GALT) due to pathogenic mutations in the GALT gene. To date more than 330 mutations have been described, with p.Q188R and p.K285N being the most common in Caucasian populations. Although acute manifestations can be fully avoided by a galactose-restricted diet, chronic complications, such as neurological ones, cannot be prevented in a significant number of patients despite compliance with the dietary treatment. Methods: A cohort of 16 galactosemic Croatian patients, including one pair of siblings, was studied. Molecular characterization was performed by direct sequence analysis of the GALT gene. Results: Sixteen patients were analyzed and only four different mutations were detected. As expected, p.Q188R and p.K285N were common, accounting for 40% and 37% of unrelated alleles, respectively. The third mutation accounting for 20% of mutant alleles was p.R123X causing a premature stop codon, is thus considered to be severe, which is in accordance with the phenotype presented by the homozygous patient described here. The fourth mutation p.E271D was found in a single allele. More than half of our patients manifested some chronic neurological complications. Conclusions: This is the first report on mutational and phenotypic spectra of classic galactosemia in Croatia that expands the knowledge on the mutational map of the GALT gene across Europe and reveals the genetic homogeneity of the Croatian population.

Detecting Undernutrition on Hospital Admission - Screening Tool Versus WHO Criteria

To prevent the development of malnutrition in hospitalized children, it is important to develope an early identification of nutritional depletion, ideally at the time of admission to the hospital. In 2009 Hulst et al. proposed new guidelines for assessing the nutritional status of hospitalized children called STRONGkids questionnaire (Screening Tool Risk on Nutritional Status and Growth). This study was designed to describe the current prevalence of malnutrition on admission to a pediatric gastroenterology hospital unit and to compare the value and feasibility of STRONGkids scoring system versus anthropometric World Health Organization (WHO) criteria in identifying children at risk of developing malnutrition during hospital stay. The prospective observational study involved 124 children hospitalized at the Department of Pediatrics, University Hospital Center Zagreb. Nutritional status and risk for malnutrition were estimated by STRONGkids questionnaire and anthropometric measurements of subjects. Statistical analysis was performed using the computer program STATISTICS 10, StatSoft. Inc. 1984-2011; using descriptive statistics, Fishers exact (FE) test and non-parametric Kruskal-Wallis (KW) test. Total malnutrition was observed in 18.5% of patients. Larger number of children at risk for malnutrition were identified by STRONGkids questionnaire than by anthropometric measurements (STRONGkids questionnaire: 75.8%; anthropometric measures: 40.3%). Patients that lost weight during hospitalization (33.1%) were further analyzed: 8/41 were not detected to be at risk by either method, 11/41 were identified by STRONGkids and anthropometry, and 22/41 were detected only by STRONGkids (Fishers exact test p=0,08). This study justifies the inclusion of the STRONGkids questionnaire in the initial evaluation of children on admission to the hospital, in order to recognize those who need special nutritional support and thus prevent the development of malnutrition during hospitalization.

PREHEPATIC PORTAL HYPERTENSION: 43 YEARS OF FOLLOW-UP IN SINGLE INSTITUTION AND 20 YEARS AFTER INTRODUCTION OF SCLEROTHERAPY: PH1-23

Introduction: Surgery was treatment of choice for children with complications of prehepatic portal hypertension until sclerotherapy and band ligation has occurred. Introduction of octerotide was another landmark in treatment. We report a 43- year (first 26 retrospective and next 17 prospective) analysis of 64 patients. Methods: The charts of all patients treated before 1987 were reviewed retrospectively and from that year on a prospective evaluation based on database and registry has been started. Results: Between 1960 and Dec 2003 64 children were followed up between 2 and 43 years with a median of 17.4 years. We have divided our patients in two groups. First consisted of all patients (No 20) treated before the introduction of sclerotherapy (1984. in our hospital), and in the second group were remaining 44 patients. First symptom in historical group was hematemesis in 17 of 20 patients (85%). Thirteen palliative surgical interventions, and 4 shunt (spleno-renal) procedures were done in 14 patients (mortality rate 35%), a 6 patients were treated medically (mortality rate 33%). In group which was treated initially with sclerotherapy hematemesis was leading symptom in 54% of patients, and overall mortality rate is 9%. In this group, 20 patients along the course of their disease, due to failure of conservative therapy or other reasons were assigned for 25 operations. Mortality rate in combination therapy group is 12.5%. Rest of the patients (24) were treated only with sclerotherapy or octreotide. Mortality rate in this subgroup is only 5%. In the last 2 years, there was any new case of prehepatic portal hypertension and we are the only hospital in our country equipped for dealing with such a problem. There is a steady decline in incidence that has been observed for years probably due to disappearance of so far unknown etiological factor and improving neonatal practices. Conclusion: There is a decline in incidence of prehepatic portal hypertension. Sclerotherapy and octreotide have improved outcomes considerably, but there is still subset of patients who have to be operated. Our data support thesis that exist subgroup of patients who should be candidates for primary surgical procedure.