Ana Vujic

Pseudo-Bartter Syndrome in an Infant with Congenital Chloride Diarrhoea

INTRODUCTION Pseudo-Bartter syndrome encompasses a heterogenous group of disorders similar to Bartter syndrome. We are presenting an infant with pseudo-Bartter syndrome caused by congenital chloride diarrhoea. CASE OUTLINE A male newborn born in the 37th gestational week (GW) to young healthy and non-consanguineous parents. In the 35th GW a polyhydramnios with bowel dilatation was verified by ultrasonography. After birth he manifested several episodes of hyponatremic dehydration with hypochloraemia, hypokalaemia and metabolic alkalosis, so as Bartter syndrome was suspected treatment with indomethacin, spironolactone and additional intake of NaCl was initiated. However, this therapy gave no results, so that at age six months he was rehospitalized under the features of persistent watery diarrhoea, vomiting, dehydration and acute renal failure (serum creatinine 123 micromol/L). The laboratory results showed hyponatraemia (123 mmol/L), hypokalaemia (3.1 mmol/L), severe hypochloraemia (43 mmol/L), alcalosis (blood pH 7.64, bicarbonate 50.6 mmol/L), high plasma renin (20.6 ng/ml) and aldosterone (232.9 ng/ml), but a low urinary chloride concentration (2.1 mmol/L). Based on these findings, as well as the stool chloride concentration of 110 mmol/L, the patient was diagnosed congenital chloride diarrhoea. In further course, the patient was treated by intensive fluid, sodium and potassium supplementation which resulted in the normalization of serum electrolytes, renal function, as well as his mental and physical development during 10 months of follow-up. CONCLUSION Persistent watery diarrhoea with a high concentration of chloride in stool is the key finding in the differentiation of congenital chloride diarrhoea from Bartter syndrome. The treatment of congenital chloride diarrhoea consists primarily of adequate water and electrolytes replacement.

Henoch-Schönlein Purpura Nephritis in Children: Prognosis and Treatment Experiences

Abstract Henoch-Schönlein purpura is a type of systemic small vessel vasculitis. The dominant manifestation is the cutaneous component, the illness is self-limiting in nature, and the prognosis and outcome depend mostly on renal manifestations. We analysed the associations among clinical and laboratory parameters with the prognosis and outcome of HSP in children hospitalised at the Paediatrics clinic, Clinical Centre, Kragujevac between January 2011 and January 2012. Children who developed nephritis were older on average and all manifested with arthritis, abdominal complaints, microhaematuria, elevated D-dimer levels in the serum, and significant proteinuria and microalbuminuria (≥ 300 mg/L), and two children had pre-existing allergic conditions. All three children with repeatedly positive proteins in the morning sample urine test had significant proteinuria (≥0,5 g/24 h) and microalbuminuria (≥ 300 mg/L). These children had more bursts of rash and more severe and lasting abdominal pain and arthritis compared to children with normal urine tests. They were therefore treated with glucocorticoids and an angiotensin-converting enzyme inhibitor. The glomerular filtration rate measured by determining creatinine clearance was normal in all patients. These patients were diagnosed with Henoch-Schönlein purpura nephritis, and their condition was regularly monitored. Analysis of this group of patients demonstrated that the average age of 8 years and abdominal complaints were indicative of nephritis development.. Because both of these parameters are easily noted at disease onset, we suggest careful monitoring of disease course in these children.

Experience with developing antibiotic stewardship programmes in Serbia: potential model for other Balkan countries?

AIM Antimicrobial resistance and inappropriate use of antibiotics in children are important issues. Consequently, there is a need to develop comprehensive stewardship programs even in hospitals with limited resources starting with childrens hospitals. METHODS Retrospective observational analysis of antimicrobial utilization and resistance patterns over 5 years in a tertiary care childrens hospital in Serbia. RESULTS Cumulative antimicrobial resistance decreased but was still high, with high cumulative resistance rates among the most widely used antibiotics in the hospital. Total antibiotic use decreased from 2010 to 2014 although there was still high prescribing of reserved antibiotics. CONCLUSION Concerns with inappropriate use and high resistance rates among some antibiotics used in the hospital are being used to develop guidance on future antibiotic use in this hospital, building on the recently introduced antibiotic stewardship program, as well as encourage other hospitals in Serbia to review their policies.

Pneumococcal meningitis associated with glomerulonephritis: A case report

Introduction. Streptococcus pneumoniae is the second most common cause of meningitis in children, producing more serious complications than other bacteria. Streptococcus pneumoniae infections are a rare trigger of glomerulonephritis. We presented a case of glomerulonephritis developing concurrently with meningitis in a young male child. Case report. Gross haematuria, significant proteinuria, hypertension and decreased level of C3 alongside the signs of central nervous system involvement occurred in a male patient of 5 years and 3 months of age. Spontaneous resolution of renal affliction parameters followed the successful treatment of meningitis. The disease course was strongly suggestive of postinfectious glomerulonephritis, although it manifested at the same time as meningitis. The absence of the latent period might point to the development of IgA nephropathy, but since the renal function was stable, without any abnormalities in urine tests documented during follow-up, our opinion is that this was rather the case of postinfectious nephropathy. Conclusion. The presented case is a unique clinical form of postinfectious glomerulonephritis. An accurate diagnosis of this entity should ensure the adequate treatment and follow-up of these patients.

Clinical implications of prenatal diagnosis of aorto-left ventricular tunnel on postnatal treatment and final outcome

Kosutic J, Prijic S, Stajevic M, Kalaba M, Ninic S, Mikovic Z, Vujic A, Popović S. Clinical implications of prenatal diagnosis of aorto-left ventricular tunnel on postnatal treatment and final outcome. Turk J Pediatr 2017; 59: 342-344. There are no more than 20 antenatally diagnosed aorto-left ventricular tunnel cases reported in the literature. In most of them the diagnosis was made indirectly and only after multiple fetal scans based on findings such as thick and dilated left ventricle and grossly dilated ascending aorta. We present a patient in whom a direct tunnel visualization and aorto-left ventricular tunnel diagnosis was made at the 30th gestation week after a single fetal scan using the recently introduced `cockade sign`. Clinical implications of antenatal diagnosis on postnatal treatment and outcome are also discussed.

Laboratory parameters in differential diagnosis of iron deficiency anemia and chronic infections anemia among children

Objective. Aim of this study were to establish laboratory parameters of sideropenic and secondary anemia among children. Methods. From December 1, 2010 to January 31, 2011, 35 children with sideropenic anemia, 35 children with anemia of chronic disease and 35 healthy children were recruited in Hematology infirmary of Pediatric Clinic of Clinical Centre Kragujevac. All children underwent following analyses: count of leukocytes (Le), erythrocytes (Er), platelets (Tr), hemoglobin (Hb), hematocrit (Hct), mean corpuscular volume (MCV), mean corpuscular hemoglobin (MCH), mean corpuscular hemoglobin concentration (MCHC), red blood cell distribution width (RDW), serum iron (Fe), total iron binding capacity (TIBC), unsaturated iron binding capacity (UIBC) and ferritin. For data processing we used t-test, with probability threshold p =<0.05. Results. Leukocytes count values were increased in group with secondary anemia compared to control and sideropenic anemia. Erythrocytes count values showed no difference between groups as well as Hb, Hct, MCHC, Tr and serum Fe between groups, but there was a difference between groups with anemia compared to control group. Values of MCV and MCH differed between groups, with the highest ones in controls, and the lowest value in sideropenic anemia group. Values of RDW, TIBC and UIBC were increased in group with sideropenic anemia, compared to control and secondary anemia group. Ferritin differed between groups, with the highest value in secondary anemia group and the lowest value in sideropenic anemia group. Conclusion: Valid laboratory parameters in differential diagnosis of sideropenic and secondary anemia are Le, MCV, MCH, RDW, TIBC, UIBC and ferritin.

Successful closure of large congenital coronary fistula with an amplatzer vascular plug: a case report and review of the literature.

We describe the case of a 3.5-year-old-girl with large coronary fistula that originated from the proximal left coronary artery and, after a tortuous distal course, drained into the right atrium. The narrowest fistula diameter was 6.7 mm. Fistula was successfully closed with a 12-mm Amplatzer vascular plug type 1 using a retrograde approach without creating an arteriovenous loop. Femoral artery damage that required thrombolytic (streptokinase) therapy was observed after the procedure. Retrograde approach does not require creation of an arteriovenous loop. However, potential benefits have to be considered in the light of potential peripheral artery damage and subsequent thrombolytic therapy.

[GM1 gangliosidosis--case report].

INTRODUCTION Gangliosidoses occur due to inherited deficiency of human beta-galaktosidase, resulting in the accumulation of glicophyngolipides within the lisosomes. Clinical manifestations of lysosomal storage disorders are remarkably heterogeneous, they can apear at any age and each of them can vary from mild to severe conditions. CASE REPORT We present a patient with an early, infintile type of GM, gangliosidosis. The facial features were coarse: hypertelorismus, wide nose, depressed nasal bridge with lingual protrusion. From the very first months of life she had severe generalized hypotonic, delayed development and hapatosplenomegaly. Before she died, when she was 13 months old, she had not had any spontaneus movements, she was deaf and blind, dispnoic, with apnoiccrises, with anemic face, but without seizures and decerebrate rigidity, which often accompanies the terminal stage of this illness. CONCLUSION The absence of beta-galaktosidase enzyme activity at the skin fibroblasts confirmed the definitive diagnosis. There has been no successful treatment so far, but increasingly better results of the gene therapy for other lysosomal storage disorders can make us optimistic.