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Publikacije (35)

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Aida Omerctahić-Dizdarević, E. Saracević, F. Jonuzi, V. Mišanović, D. Anić, I. Kalkan, Amra Cengić

BACKGROUND Congenital lobar emphysema is characterized by overinflation of pulmonary lobe and is caused by localised bronchial obstruction. The disease may result in severe respiratory distress in early infancy. METHODS Records of children, with congenital lobar emphysema, who were treated at our department between 1997-2003, were reviewed. RESULTS Three males, aged 16 days till 1 year, were diagnosticed as congenital lobar emphysema. Presenting symptoms were dyspnea in 2 patients, cyanosis in 2 patients, wheesing in 2 patients, recurrent respiratory tract infection in 2 patients. Chest x rays and computerized tomography scans showed hyperinflation of the affected lobe in all patients. The affected sites were left upper lobe in 2 patients and right upper lobe in 1 patient. All patients underwent lobectomy. CONCLUSION It is necessary to suspect on this uncommon anomaly in early infancy. The diagnosis was established by chest x rays and CT scans. Intensive therapy and urgent lobectomy, in severe respiratory distress, are condition for successfully treatment.

Known as D trisomy, Patau syndrome is the third chromosomopathy according to frequency. One of the 5000 newborn carries the trisomy 13. In over 80% cases there is fresh mutation with non separation in myeosis of older mother. The mosaic or translocation forms are not rare. The mail newborn with Patau syndrome is shown in this article. We notice: microcephalia, dolihocephalia, microphthalmia, cheilognatopalatoshisis, polydactilia, and found ultrasound changes at the brain, hearth and genitourinary system. Cytogenetic finding show: mail cariotype with aberrations 47, XY + 13, Sy Patau.

E. Zecević-Cemerlić, D. Miličić, V. Mišanović

Hypoproteinaemia, proteinuria and edema are the main features of Nephrotic syndrome (NS) and are the result of greater permeability of glomerular basal membrane for proteins. It is difficult to predict course of disease and outcome. During 1989-1998 year 18 children were followed up, 9 boys and 9 girls, at the age of 2 to 14 years and diagnosis of NS. Therapy with pronison was initiated in all children according to the protocol of ISKDC. Children were then divided in two groups, depending on success of therapy. In the first group (13 patients, 72.22%) were the patients that had remission, and the second group consisted of 5 patients that did not have remission. In the first group relapses occurred in 7 patients (53.84%), because of side effect of pronison therapy was discontinued in 3 patients. Those 3 patients along with 5 patients from second group were then turned to immuno-suppressive therapy. One patient responded well to cyclophosphamide, rest of them with regular follow up. Two years latter two of them had relapse but responded well on pronisone therapy in the full dose. Biopsies that were done showed that 2 had minimal changes, 2 had focal segmental sclerosis and one membranous proliferative glomerulonephritis. One patient developed chronic renal insufficiency. We conclude that our experience shows that if there is not favorable effect of corticosteroid therapy cyclosporine A is the first choice in NS, without regarding of the patho-hystological findings.

D. Miličić, E. Cemerlić-Zecević, V. Mišanović, D. Anić

Acute renal failure (ARF) is not rare in hospitalized children, especially in newborns and infants. Sudden disorganization of a glomerular and tubular function is the most often transitory and require a quick make of diagnosis and early therapy in order to obtain a adequate kidney function recovery and to reduce mortality. In this article 51 children with ARF were included from newborn period to 16 years of age (25 female: 26 male). We described the most often causes of ARF, diagnosis procedures and nondialytic treatments. The diagnosis was made by history, physical examination, laboratory and radiology findings. Causes of ARF were divided on prerenal, renal and postrenal. The treatment of ARF in all cases was conservative with good success in 45 patients and in 6 children followed death because of the nature of primary diseases. The results of study show that the accent is on the prevention, early diagnosis and early therapy of diseases which lead to ARF. We conclude that a multidisciplinary therapeutic approach is imperative for successful management of ARF.

E. Hasanbegović, R. Terzić, S. Sabanović, V. Mišanović

This paper reports a very rare case of 5-years old girl with a tumor of the right kidney and metastatic changes in the inferior vena cava and right heart. Following the nefrectomy Wilms' tumour diagnosis was established. There was no an adequate answer on chemotherapy according to SIOP i.v. protocol. Three months later metastatic changes spreaded to lungs, pelvic bones and distant lymphonodes of the neck and axillas. The patient in a very severe condition was evacuated to England (Alder Hay Children's Hospital, Liverpool). After numerous tests the diagnosis of Wilms's tumor was excluded and diagnosis of Ewing's sarcoma (soft tissue) was established.

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