During the 10-yr study period, 16 patients were diagnosed with childhood-onset SLE. Five of them (31%) had pulmonary involvement including acute lupus pneumonitis, invasive pulmonary aspergillosis, cytomegalovirus pneumonia and pulmonary haemorrhage (in two patients). These 5 patients with lupus lung disease are presented in more detail.

Acquired Bartter-like syndrome (BLS), characterized by hypokalemic metabolic alkalosis, hypomagnesemia, hypocalcemia, and normal kidney function, can be induced by diuretics or antibiotics. It is a very rare condition and only anecdotal cases mostly in adults were reported. Although tubulopathy associated with colistin was reported in adults, to the best of our knowledge, colistin-associated BLS neither in adults nor in children has been reported in the literature. We here report a-28-week, 740 g female preterm infant who developed BLS just after colistin treatment for Acinetobacter baumannii infection and recovered few days after the drug cessation, and discuss the possible association of colistin and tubulopathy. More research on colistin pharmacokinetics and pharmacodynamics in critically ill patients and preterm infants is needed to guide adequate colistin dosing at the least toxicity.

This study was performed to investigate the attack-free complaints of patients with familial Mediterranean fever (FMF) and the impact of colchicine on these symptoms and on subclinical inflammation. A questionnaire that includes information about the disease course and symptoms during the attack-free period was administered to the parents of 50 FMF patients. For evaluation of the attack-free period, questions were asked about four items concerning daily activities of the children-weakness, lack of appetite, sleep problems, and decreased activity. The respondents rated the items and the total score was taken as the sum of all of the specific items. The laboratory values were noted from the patients' files. During the attack-free period, patients with mild disease had higher total scores, higher weakness, and decreased activity scores than patients with moderate disease. When we compared the daily activity scores before and after colchicine therapy, a statistically significant increase was observed in the total scores and in all of the specific item scores. Also a significant decrease was seen in the erythrocyte sedimentation rate and white blood cell counts, and a significant increase was seen in the hemoglobin levels during the attack-free period after colchicine usage. Regression of inflammation together with improvement in daily activities were observed. FMF patients seem to have complaints during the attack-free period that may be related to subclinical inflammation. Moreover, colchicine besides preventing the FMF attacks and the dangerous complication of amyloidosis also seems to hinder the symptoms of the attack-free period in children with FMF.

Antenatal hydronephrosis (AHN), defined as dilatation of renal pelvis and/or calyces, is the most frequently detected antenatal abnormality. However, postnatal management of AHN is controversial. The purpose of this study was to describe the clinical outcomes of infants with AHN and to contribute to the definition of the postnatal evaluation of these patients. One hundred and thirty-six infants with AHN were prospectively followed up to 18 months. Patients were divided into two groups according to the degree of sonographic hydronephrosis (HN) on days 5-7: group I (n = 87, 64%) included patients who had grades 1 and 2 (64%) and group II (n = 49, 36%) included patients who had grade 3 and above HN. The grade of HN was found to be correlated with the increased risk of urologic pathologies. Frequency of vesicoureteral reflux was found to be significantly lower in patients with mild HN (6%) as compared to patients with severe AHN (29%) (p = 0.005). In addition, the risk of urinary tract infection increases with increasing grades of HN (10% vs. 29%, p = 0.006). The frequency of spontaneous resolution in patients with mild AHN (64%) was also significantly higher than in patients with severe HN (29%) (p < 0.001). The degree of AHN can be used for making decision about further diagnostic imaging and treatment. Our results strongly suggest that low-grade HN is a relatively self-limited condition and needs minimal investigation. In contrast, the outcome of more severe degrees of AHN needs clarification.

A total of 65 women with ureteral stone who underwent ureteroscopy were divided randomly into two groups between April 1993 and March 1994. URS was performed under general anaesthesia in the first group (35 patients), and under local anaesthesia with sedation analgesia in the second group (30 patients). Only 2 patients of the second group did not tolerate the operation. The success rate was 88% in the first group and 83.3% in the second. There was no significant difference between the two groups. Since URS under local anaesthesia is time-saving, cost-effective, and highly tolerable in women, it is a good choice for ureteral stones.

Background: Vesico-ureteral reflux (VUR) is considered to be a risk factor for recurrent febrile urinary tract infections and impaired renal transplant survival. Methods: An online survey supported by the European Society for PaediatricNephrology was designed to evaluate current management strategies of VUR in native and transplanted kidneys of recipients aged <18 years.Results: Seventy-three pediatric transplant centers from 32 countries contributed to the survey. All centers performed urological evaluation prior to pediatric kidney transplantation (KTx) with subsequent interdisciplinary discussion. Screening for VUR in native kidneys (30% in all, 70% in selected patients) led to surgical intervention in 78% (11% in all, 89% in selected patients) with a decided preference of endoscopic intervention over ureterocystoneostomy. Following KTx, continuous antibiotic prophylaxis was applied in 65% of the patients and screening for allograft VUR performed in 93% of selected patients. The main management strategies of symptomatic allograft VUR were continuous antibiotic prophylaxis (83%) and surgical treatment (74%) (endoscopic intervention 55%, redo ureterocystoneostomy 26%).Conclusions: This survey demonstrates the high variability in the management of VUR in pediatric KTx recipients, points to knowledge gaps, and might serve as a starting point for improving the care for patients with VUR in native and transplanted kidneys.

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