Cinacalcet sustainedly prevents pancreatitis in a child with a compound heterozygous SPINK1/AP2S1 mutation.

Familial hypocalciuric hypercalcemia is an autosomal dominant genetic disorder characterized by hypercalcemia associated with inappropriate hypocalciuria and normal parathyroid hormone levels. Acute recurrent pancreatitis (ARP) is rare in children. Predisposing factors include hypercalcemia and mutations in the serine protease inhibitor Kazal-type 1 (SPINK1) gene. The disease carries a heavy morbidity and preventive treatment options are scant. Here, we report a child with a novel genetic/metabolic form of ARP associated with compound heterozygous SPINK1/AP2S1 (adaptor protein-2 σ1-subunit) mutations, recurrence of which was completely abrogated for 6 years by cinacalcet treatment.

Haemolytic uremic syndrome surveillance in children less than 15 years in Belgium, 2009-2015.

BACKGROUND: The Haemolytic Uremic Syndrome (HUS) is the most severe manifestation of infection with Shiga toxin-producing Escherichia coli (STEC). In Belgium, the surveillance of paediatric HUS cases is conducted by a sentinel surveillance network of paediatricians called Pedisurv. In this article, we present the main findings of this surveillance from 2009 to 2015 and we describe an annual incidence of HUS. METHODS: For each case of HUS <  15 years notified by the paediatricians, clinical, microbiological and epidemiological data were collected by a questionnaire. National hospital discharge data with ICD-9 code 283.11 were used to calculate the incidence of HUS in children < 15 years. RESULTS: From 2009 to 2015, 110 cases were notified to the Pedisurv network with a mean annual notification rate of 0.8/100,000 in children < 15 years. Death occurred in 2.5% of all patients and the median number of days of hospitalization was 10 days. One third (35.4%) of the HUS cases were confirmed positive STEC, with a majority of STEC O157. The mean annual incidence based on the hospital discharge data was 3.2/100,000 in children < 15 years and 4.5/100,000 in children < 5 years. CONCLUSION: The incidence of paediatric HUS in Belgium is high compared to other European countries. Its surveillance in Belgium is quite comprehensive and, although less effective than monitoring all STEC infections to detect the emergence of outbreaks, is important to better monitor circulation of the most pathogenic STEC strains. In this context, efforts are still needed to send samples and STEC strains from HUS cases to the National Reference Centre.

Presentation and management of mycotic pseudoaneurysm after kidney transplantation.

Pseudoaneurysms (PAs) developing at the site of vascular anastomosis after organ transplantation are a rare but serious complication. We report a series of 3 cases of PA observed in a single center over a period of 18 years. The mode of presentation was acute bleeding in 2 cases. In the third patient, who underwent combined kidney and pancreas transplantation, the PA on the renal graft was discovered by chance. Graft removal associated with iliac artery ligation and extra-anatomic femoro-femoral bypass represents the standard treatment. However, interposition of a venous homograft may allow preservation of inferior limb perfusion and possibly graft salvage.

[Pediatric nephrology from the fetal kidney to the kidney graft]. / La néphrologie pédiatrique du rein foetal au rein greffé.

The department of pediatric uro-nephrology was created in 1977 in Brugmann hospital. Since then, various sectors have been developed including: hemodialysis and peritoneal dialysis, kidney transplantation, urological and genital surgery, antenatal screening and rapid management of uronephropathies, treatment of voiding dysfunction and neurogenic bladder, management of tubular and glomerular diseases. The progress in genetics, medical imaging, obstetrics, neonatology and surgery has allowed us to take care of our young patients within a multidisciplinary framework. The most original contributions of the department are related to the performance of combined liver-kidney transplantation in primary hyperoxaluria, to the determination of the natural history of several congenital anomalies of the kidney and urinary tract, to the assessment of the role of genetic mutations on tubular and glomerular diseases, to the usefulness of radioisotopic tracers in the measurement of renal function in infants, and to the study of experimental tolerance of allografts. The transition of young renal patients from pediatric to adult care is actually well organized due to our 30 years experience and the excellent collaboration with the adult nephrologists.

[The medico-surgical department of uro-nephrology, dialysis and renal transplantation in the Children's Hospital of Brussels]. / Le département médico-chirurgical d'uro-néphrologie, dialyse et transplantation rénale à l'HUDERF.

The department of pediatric uro-nephrology was created in 1977 in Brugmann hospital. Since then, various sectors have been developed including: hemodialysis and peritoneal dialysis, kidney transplantation, urological and genital surgery, antenatal screening and rapid management of uronephropathies, treatment of voiding dysfunction and neurogenic bladder, management of tubular and glomerular diseases. The progress in genetics, medical imaging, obstetrics, neonatology and surgery has allowed us to take care of our young patients within a multidisciplinary framework. The most original contributions of the department are related to the performance of combined liver-kidney transplantation in primary hyperoxaluria, to the determination of the natural history of several congenital anomalies of the kidney and urinary tract, to the assessment of the role of genetical mutations on tubular and glomerular diseases, to the usefulness of radioisotopic tracers in the measurement of renal function in infants, and to the study of experimental tolerization of

[Immunosuppressive agents in pediatric renal transplantation]. / Le traitement immunosuppresseur en transplantation renale pédiatrique.

Advances in immunosuppressive therapy over the past decade have led to dramatic improvements of patient and graft survival. The immunosuppression that is used is constantly evolving. The goal remains to find the best combination that will optimize long-term graft survival, while minimizing the adverse effects. It is likely that in the near future the results will even be improved further by the development of new medications with a better therapeutic index and the induction of transplant tolerance.