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Haupt R.,Epidemiology and Biostatistics Section | Minkov M.,Childrens Cancer Research Institute | Astigarraga I.,Hospital Universitario Cruces Barakaldo | Schafer E.,Reference Center for Histiocytosis at Hopital Trousseau | And 10 more authors.
Pediatric Blood and Cancer

These guidelines for the management of patients up to 18 years with Langerhans cell histiocytosis (LCH) have been set up by a group of experts involved in the Euro Histio Net project who participated in national or international studies and in peer reviewed publications. Existing guidelines were reviewed and changed where new evidence was available in the literature up to 2012. Data and publications have been ranked according to evidence based medicine and when there was a lack of published data, consensus between experts was sought. Guidelines for diagnosis, initial clinical work-up, and treatment and long-term follow-up of LCH patients are presented. © 2012 Wiley Periodicals, Inc. Source

Sovtic A.,11 Health | Minic P.,11 Health | Bogdanovic R.,Mother and Child Health Institute of Serbia Dr Vukan cupic | Stajic N.,Mother and Child Health Institute of Serbia Dr Vukan cupic | And 2 more authors.
Vojnosanitetski Pregled

Introduction. Infants with cystic fibrosis may fail to thrive despite recommended caloric intake because of electrolyte disurbances caused by salt depletion resulting in hypochloremic metabolic alkalosis or pseudo-Bartter's syndrome. In most patients reported symptoms began in infancy, but it may be an initial presentation of disease in a previously healthy adolescent. Case report. A 15-year-old boy was admitted for evaluation of recurrent episodes of malaise associated with dehydration and acute renal insufficiency. Laboratory analysis showed hypochloremic metabolic alkalosis with hyponatremia and hypokalemia. On admission the boy was obese, with body weight of 95.5 kg (> P 97), height 174 cm (> P 75), and body mass index of 31.2 kg/m 2 (> P 95). Physical examination was inconclusive. Blood pressure holter monitoring proved significant systolic hypertension. Routine urinalysis, protein and electrolyte levels in urine were normal. Plasma renin and aldosteron were normal. Sweat chloride concentration was 63 mmol/L. Genetic testing confirmed the diagnosis of cystic fibrosis. Conclusion. To our knowledge, this is the first reported case of atypical presentation of cystic fibrosis in an adolescent presented with pseudo-Bartter's syndrome and signs of obesity and hypertension. We suggest that every patient with hypochloremic metabolic alkalosis should be evaluated for cystic fibrosis. Source

Djukic M.,University of Belgrade | Stankovic Z.,Mother and Child Health Institute of Serbia Dr Vukan cupic | Vasiljevic M.,University of Belgrade | Savic D.,Mother and Child Health Institute of Serbia Dr Vukan cupic | And 2 more authors.
Clinical and Experimental Obstetrics and Gynecology

Purpose: To determine whether the presence of normal ovarian tissue could assist in the diagnosis of large benign ovarian neoplasms in young females and in choosing the laparoscopic treatment. Materials and Methods: A prospective study of 25 patients treated surgically for a cystic ovarian neoplasm measuring diameter more than ten cm or volume more than 500 ml and having normal ovarian tissue or ovarian crescent sign (OCS). Ultrasonography was performed at six weeks, then at three, six, nine, and 12 months postoperatively. Results: The mean age of patients was 15.3 ± 3.6 years, ranging between 6.5 and 19 years. The mean preoperative volume of the ovarian neoplasm was 1,686 ± 1,380 cm3, ranging between 550 and 6,000 cm3. The presence of OCS was visualized by ultrasonography in all 25 patients and serum tumor markers were negative in 22. No borderline tumors or malignancies were identified. There was a statistically significant difference between the volume of the affected ovary and the contralateral ovary during the first six weeks follow-up, but without significant difference after three months. Conclusions: Postoperative ultrasound revealed that the affected ovary resumed its normal volume within three months after surgery, despite the thinned appearance of the ovarian cortex present on ultrasound as the OCS. Laparoscopic ovarian preservation should be the preferred surgical approach for adolescents to ensure the conservation of the entire ovarian tissue. Source

Sarajlija A.,Mother and Child Health Institute of Serbia Dr Vukan cupic | Djuric M.,University of Belgrade | Kisic Tepavcevic D.,University of Belgrade
Vojnosanitetski Pregled

Background/Aim. Rett syndrome (RTT) is a severe neurodevelopmental disorder primarily affecting females with an estimated incidence of 1: 10,000-15,000 female births. Currently, there is no specific treatment that halts or reverses the progression of RTT. Therefore, management was mainly symptomatic, focussed on optimising patient's abilities. The aim of this study was to investigate factors influencing health-related quality of life (HRQoL) and depression in mothers who care for children with Rett syndrome (RTT) in Serbia. Methods. The cross-sectional study was conducted on 49 mothers giving care to females with RTT. Caregivers HRQoL was assessed by using the SF-36 questionnaire. Clinical severity score (CSS) of RTT patients and Beck Depression Inventory II (BDI -II) scale were used to quantify RTT severity and mothers depression, respectively. Statistical assessment included descriptive statistics, ttest, Pearson correlation coefficient and multiple logistic regression. Results. The age of mothers ranged from 22 to 55 years and of their affected children from 3 to 29 years. Severe depression was observed in 15 (30.6%) participants. CSS and BDI -II scores correlated negatively with all SF- 36 domains and composite scores. Lowest scoring domains of HRQoL in mothers giving care to RTT children were mental health, vitality and role functioning emotional. Multiple linear regression analysis revealed that severity of RTT patients disability (CSS) and caregivers age are factors with strongest influence to HRQoL and depression in care giving mothers. Conclusion. Mothers giving care to children with RTT are at high risk of severe depression and lower HRQoL scores of domains that reflect mental well-being. Results of this study can help in planning subsequent interventions directed at families dealing with Rett syndrome. Source

Djukic M.,University of Belgrade | Stankovic Z.,Mother and Child Health Institute of Serbia Dr Vukan cupic | Vasiljevic M.,University of Belgrade | Savic D.,Mother and Child Health Institute of Serbia Dr Vukan cupic | Djuricic S.,Mother and Child Health Institute of Serbia Dr Vukan cupic
European Journal of Gynaecological Oncology

The authors present a case of a 14-year-old premenarchal girl with a large solid tumor of the left ovary. The rim of normal ovarian tissue was visible around the tumor on ultrasonography scan. Although the levels of two tumor markers, LDH and CA125, were elevated, the authors performed an organ-sparing tumorectomy. The final pathology report revealed foci of immature neural tissue, with a final diagnosis immature teratoma Stage Ia. Source

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