[Bartter's syndrome: new classification, old therapy]. / Barterov sindrom: nova podela, stara terapija.

We report on two cases of Bartter's syndrome, together with the review of current literature on the aetiology, development and treatment of Bartter's syndrome. Bartter's syndrome belongs to a group of hypokalaemic renal channelopathies, which are caused by a molecular hereditary disorder of ion channels in renal tubules. These channels are located in the lipid layer of cell membranes where they exist as water channels through which ion transport is performed. Based on the type of genetic disorder and clinical presentation, Bartter's syndrome is classified as neonatal, classical and Gitelman's syndrome. Neonatal form is found in newborns and is characterized by foetal polyuria, premature birth, postnatal episodes of severe dehydration, growth retardation, hypercalciuria and early nephrocalcinosis. It is the result of mutation of a gene responsible for renal tubular Na-K-2Cl cotransport or another gene which controls the ATP-dependant potassium channel (ROMK). Classic form is found in young children with polyuria, hypokalaemia and growth retardation. This type is caused by a defect of a gene for chloride channel (CIC-Kb) in the distal tubule. Gitelman's syndrome is found in late childhood or adolescence. It is caused by mutation in the gene for Na-Cl co-transport in the distal tubule. Children with Gitelman's syndrome occasionally have muscle weakness or tetany, hypokalaemia and hypomagnesaemia. Even though there have been advances in understanding the aetiology and pathogenesis of Bartter's syndrome in the recent years, the possibilities and strategies for its management remained almost the same. Treatment is based on prostaglandin inhibitors, potassium sparing diuretics and substitution therapy.

[Ultrasound in the diagnosis of hypertrophic pyloric stenosis]. / Ultrasvuk u dijagnostici hipertroficne stenoze pilorusa.

The paper deals with the advantages of ultrasound diagnosis of hypertrophic pyloric stenosis in newborns. Ultrasound decreases the number of radiological examinations of gastroduodenum and reduces diagnostic exposure of children to X-rays. In the last three years 107 children had manifested clinical signs of hypertrophic pyloric stenosis. The ultrasound studies revealed hypertrophic pyloric stenosis in 55 patients (51.4%). There were 48 boys (87.3%) and 7 girls (12.7%). Patients were aged 17 to 75 days (average about 40 days). The sonogram finding was typical for hypertrophic pylorus, which made the diagnosis easier. There were no false positive or false negative ultrasound findings. All patients with hypertrophic pyloric stenosis were operated on and diagnosis was confirmed. Ultrasound is reliable in diagnosis of hypertrophic pyloric stenosis. Ultrasound examination is technically easy and fast, what is important for urgent surgical therapy. In children with persistent projectile vomiting, suspected of hypertrophic pyloric stenosis, ultrasound is the method of choice and should replace X-ray examinations.

[Intestinal intussusception in children. Ultrasonic diagnosis]. / Invaginacija intestinuma kod dece. Dijagnostika ultrazvukom.

The aim of the paper is to demonstrate a successful use of ultrasound in the diagnosis of intestinal intussusception in children. Ultrasound decreases the number of irrigographic examinations and reduces diagnostic exposure of children to X-rays. In the last three years 35 children, aged from 3 months to 15 years (average 2 years), had a suspected clinical diagnosis of intussusception. The ultrasound studies revealed intestinal intussusception in 26 patients (74%). There were no false positive or false negative ultrasound findings. In four patients with secondary intussusception the main symptoms were identified (three solid lesions and two Meckel's diverticula). Intraluminal lesions at the apex of intussusception were confirmed by surgery. In 22 patients intussusception was idiopathic. In 15 of these patients (68%), hydrostatic desinvaginations, under combined ultrasound and radioscopic control, were successful. High grade unsuccessful hydrostatic reductions were associated with long persistence of symptoms (2 to 9 days). Ultrasound is reliable in diagnosis of intestinal intussusception and useful in control of hydrostatic reduction. In patients with expected intestinal perforation ultrasound should be combined with fluoroscopy.

[Adrenal gland hemorrhage in neonates--radiologic aspects]. / Hemoragija u nadbubreznoj zlezdi kod novorodjenceta--radioloski aspekt.

INTRODUCTION: The relatively large adrenal glands of the newborn are vulnerable to mechanical trauma during delivery. Great birth weight, difficult labor, perinatal hypoxia and prematurity are predisposing factors of adrenal haemorrhage. Minor adrenal haemorrhage may not cause symptoms. Massive adrenal haemorrhage is uncommon. Symptoms include anaemia and jaundice associated with a suprarenal mass. In cases with severe blood loss acute shock may develop. In 5 to 10 per cent of cases the haemorrhage is bilateral. Ultrasonography has replaced urography in the diagnosis of this condition demonstrating the site and size of the lesion and allowing an accurate follow-up. Within a month after haemorrhage the blood and necrotic adrenal tissue are resorbed and thin calcification appears at the periphery of the gland. Surgery is necessary if haemorrhagic pseudocyst is large and does not resorb spontaneously. MATERIAL AND METHODS: From 1992 to 1996, five patients with neonatal adrenal haemorrhage were treated at the University Children's Hospital in Belgrade. Two of them were females. All patients were born at term by vaginal delivery. Their birth weights ranged between 3200 and 4050 g. At hospitalization infants were aged from 6 hours to 18 days. The first symptom of adrenal haemorrhage was an abdominal mass in three patients. One of them had laparoschisis with guts and stomach protruding out; the surgeon discovered a mass in the right retroperitoneum during operation. Two patients had jaundice associated with anaemia, and sepsis another two. Ultrasonography was done in all patients. We punctured the haemorrhagic pseudocyst (diameter above 5 cm) in three patients and made cystography. Liquid components of pseudocysts were aspirated and sent to bacteriological and cytological analyses. RESULTS: The diagnosis of adrenal haemorrhage was confirmed by ultrasonography in all patients, demonstrating a right adrenal mass (unilateral in all patients), mostly hypoechoic, which displaced the right kidney. Calcification at the periphery of the pseudocyst appeared in one patient. The adrenal haemorrhage disappeared spontaneously in two patients after two months. An attempt to support the adrenal hemorrhagic pseudocystic regression by puncturing and aspirating its content in three patients was successful in one infant. The patient with laparoschisis died because of sepsis and thrombocytopenia. In a patient the haemorrhagic pseudocyst persisted (6 cm in diameter) and was surgically removed. DISCUSSION: Ultrasound is the method of choice in the diagnosis of adrenal haemorrhage, antenatally and neonatally. It also allows diagnosis of coexisting complications such as renal vein or inferior vena cava thrombosis and a proper follow-up. Puncture of pseudocyst and aspiration of liquid components may support involution of large haemorrhagic pseudocysts. If it is unsuccessful, surgery is necessary.