Ruža Grizelj

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.

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.

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.