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We sought to ascertain the long-term outcome and genotype-phenotype correlations available for primary hyperoxaluria type 1 in a large retrospective cohort study. We examined the clinical history of 155 patients (129 families primarily from Western Europe, North Africa, or the Middle East) as well as the enzymatic or genetic diagnosis. The median age at first symptom was 4 years, and at diagnosis 7.7 years, at which time 43% had reached end-stage renal disease. Presentations included: (1) early nephrocalcinosis and infantile renal failure, (2) recurrent urolithiasis and progressive renal failure diagnosed during childhood, (3) late onset with occasional stone passage diagnosed in adulthood, (4) diagnosis occurring on post-transplantation recurrence, and (5) family screening. The cumulative patient survival was 95, 86, and 74% at ages 10, 30, and 50 years, respectively, with the cumulative renal survival of 81, 59, 41, and 10% at ages 10, 20, 30, and 50 years, respectively; 72 patients had undergone a total of 97 transplantations. Among the 136 patients with DNA analysis, the most common mutation was p.Gly170Arg (allelic frequency 21.5%), with a median age at end-stage renal disease of 47 years for homozygotes, 35 years for heterozygotes, and 21 years for other mutations. Our results underscore the severe prognosis of primary hyperoxaluria type 1 and the necessity for early diagnosis and treatment, as well as confirm a better prognosis of the p.Gly170Arg mutation. [ABSTRACT FROM AUTHOR]

Percutaneous renal biopsy is frequently used in diagnosis of renal dysfunction. Nevertheless, complications, mostly vascular, may occur. Treatment must be minimally invasive with nephron preservation, especially in kidney failure. We present an interesting therapeutic approach with highly selective catheterization under gadolinium and glue embolization, leading to total cure of iatrogenic fistula without renal deterioration. [Copyright &y& Elsevier]

Nedosiran is an investigational RNA interference agent designed to inhibit expression of hepatic lactate dehydrogenase, the enzyme thought responsible for the terminal step of oxalate synthesis. Oxalate overproduction is the hallmark of all genetic subtypes of primary hyperoxaluria (PH). In this double-blind, placebo-controlled study, we randomly assigned (2:1) 35 participants with PH1 (n = 29) or PH2 (n = 6) with eGFR ≥30 mL/min/1.73 m 2 to subcutaneous nedosiran or placebo once monthly for 6 months. The area under the curve (AUC) of percent reduction from baseline in 24-hour urinary oxalate (Uox) excretion (primary endpoint), between day 90-180, was significantly greater with nedosiran vs placebo (least squares mean [SE], +3507 [788] vs -1664 [1190], respectively; difference, 5172; 95% CI 2929-7414; P < 0.001). A greater proportion of participants receiving nedosiran vs placebo achieved normal or near-normal (<0.60 mmol/24 hours; <1.3 × ULN) Uox excretion on ≥2 consecutive visits starting at day 90 (50% vs 0; P = 0.002); this effect was mirrored in the nedosiran-treated PH1 subgroup (64.7% vs 0; P < 0.001). The PH1 subgroup maintained a sustained Uox reduction while on nedosiran, whereas no consistent effect was seen in the PH2 subgroup. Nedosiran-treated participants with PH1 also showed a significant reduction in plasma oxalate versus placebo (P = 0.017). Nedosiran was generally safe and well tolerated. In the nedosiran arm, the incidence of injection-site reactions was 9% (all mild and self-limiting). In conclusion, participants with PH1 receiving nedosiran had clinically meaningful reductions in Uox, the mediator of kidney damage in PH., (Copyright © 2022 International Society of Nephrology. Published by Elsevier Inc. All rights reserved.)

Management of acute liver failure (ALF) and acute on chronic liver failure (ACLF) in the pediatric population can be challenging. Kidney manifestations of liver failure, such as hepatorenal syndrome (HRS) and acute kidney injury (AKI), are increasingly prevalent and may portend a poor prognosis. The overall incidence of AKI in children with ALF has not been well-established, partially due to the difficulty of precisely estimating kidney function in these patients. The true incidence of AKI in pediatric patients may still be underestimated due to decreased creatinine production in patients with advanced liver dysfunction and those with critical conditions including shock and cardiovascular compromise with poor kidney perfusion. Current treatment for kidney dysfunction secondary to liver failure include conservative management, intravenous fluids, and kidney replacement therapy (KRT). Despite the paucity of evidence-based recommendations concerning the application of KRT in children with kidney dysfunction in the setting of ALF, expert clinical opinions have been evaluated regarding the optimal modalities and timing of KRT, dialysis/replacement solutions, blood and dialysate flow rates and dialysis dose, and anticoagulation methods., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Raina, Sethi, Filler, Menon, Mittal, Khooblall, Khooblall, Chakraborty, Adnani, Vijayvargiya, Teo, Bhatt, Koh, Mourani, de Sousa Tavares, Alhasan, Forbes, Dhaliwal, Raghunathan, Broering, Sultana, Montini, Brophy, McCulloch, Bunchman, Yap, Topalglu and Díaz-González de Ferris.)

Introduction: Recently, prolonged intermittent renal replacement therapies (PIRRT) have emerged as cost-effective alternatives to conventional CRRT and their use in the pediatric population has started to become more prominent. However, there is a lack of consensus guidelines on the use of PIRRT in pediatric patients in an intensive care setting., Methods: A literature search was performed on PubMed/Medline, Embase, and Google Scholar in conjunction with medical librarians from both India and the Cleveland Clinic hospital system to find relevant articles. The Pediatric Continuous Renal Replacement Therapy workgroup analyzed all articles for relevancy, proposed recommendations, and graded each recommendation for their strength of evidence., Results: Of the 60 studies eligible for review, the workgroup considered data from 37 studies to formulate guidelines for the use of PIRRT in children. The guidelines focused on the definition, indications, machines, and prescription of PIRRT., Conclusion: Although the literature on the use of PIRRT in children is limited, the current studies give credence to their benefits and these expert recommendations are a valuable first step in the continued study of PIRRT in the pediatric population., (© 2020 International Society for Hemodialysis.)

Background: Sequential liver-kidney transplantation (SeqLKT) from the same living donor has shown excellent results in children with primary hyperoxaluria type 1 (PH1), yet its experience is limited due to the invasiveness of two major procedures for liver-kidney procurement in a single donor. Despite laparoscopic nephrectomy and hepatic left lateral sectionectomy (LLS) being considered standard procedures in living donation, the sequential use of the two laparoscopic approaches in the same living donor has never been reported. Methods: Herein, we present the first two case series of laparoscopic liver-kidney procurement in the same living donor for SeqLKT in children with PH1 and review of the current literature on this topic. Results: In the first case, a 15-month-old boy received a SeqLKT from his 32-year-old mother, who underwent a laparoscopic LLS and, after 8 months, a laparoscopic left nephrectomy. In the second case, a 34-month-old boy received a SeqLKT from his 40-year-old father who underwent laparoscopic LLS followed by hand-assisted right nephrectomy after 4 months. Both donors had uneventful postoperative courses and were discharged within 5 days from each surgery. The first recipient had no complication; the second child after liver transplantation developed a partial thrombosis of the inferior vena cava, which did not preclude the sequential kidney transplantation. After 12 months, donors and recipients displayed normal liver and renal functions. Conclusions: Sequential laparoscopic liver-kidney procurement in the same living donor is safe and feasible, and might be considered as a possible strategy to promote SeqLKT in children with PH1 from the same living donor.

Background: The past few decades have witnessed a tremendous development in the field of genetics. The implementation of next generation sequencing (NGS) technologies revolutionized the field of molecular biology and made the genetic information accessible at a large scale. However, connecting a rare genetic variation to a complex phenotype remains challenging. Indeed, identifying the cause of a genetic disease requires a multidisciplinary approach, starting with the establishment of a clear phenotype with a detailed family history and ending, in some cases, with functional assays that are crucial for the validation of the pathogenicity of a mutation., Methods: Two hundred Lebanese patients, presenting a wide spectrum of genetic disorders (neurodevelopmental, neuromuscular or metabolic disorders, etc.), sporadic or inherited, dominant or recessive, were referred, over the last three and a half years, to the Medical Genetics Unit (UGM) of Saint Joseph University (USJ). In order to identify the genetic basis of these diseases, Whole Exome Sequencing (WES), followed by a targeted analysis, was performed for each case. In order to improve the genetic diagnostic yield, WES data, generated during the first 2 years of this study, were reanalyzed for all patients who were left undiagnosed at the genetic level. Reanalysis was based on updated bioinformatics tools and novel gene discoveries., Results: Our initial analysis allowed us to identify the specific genetic mutation causing the disease in 49.5% of the cases, in line with other international studies. Repeated WES analysis enabled us to increase the diagnostics yield to 56%., Conclusion: The present article reports the detailed results of both analysis and pinpoints the contribution of WES data reanalysis to an efficient genetic diagnosis. Lessons learned from WES reanalysis and interpretation are also shared.

Hereditary defects of coenzyme Q10 biosynthesis cause steroid-resistant nephrotic syndrome (SRNS) as part of multiorgan involvement but may also contribute to isolated SRNS. Here, we report 26 patients from 12 families with recessive mutations in ADCK4. Mutation detection rate was 1.9% among 534 consecutively screened cases. Patients with ADCK4 mutations showed a largely renal-limited phenotype, with three subjects exhibiting occasional seizures, one subject exhibiting mild mental retardation, and one subject exhibiting retinitis pigmentosa. ADCK4 nephropathy presented during adolescence (median age, 14.1 years) with nephrotic-range proteinuria in 44% of patients and advanced CKD in 46% of patients at time of diagnosis. Renal biopsy specimens uniformly showed FSGS. Whereas 47% and 36% of patients with mutations in WT1 and NPHS2, respectively, progressed to ESRD before 10 years of age, ESRD occurred almost exclusively in the second decade of life in ADCK4 nephropathy. However, CKD progressed much faster during adolescence in ADCK4 than in WT1 and NPHS2 nephropathy, resulting in similar cumulative ESRD rates (>85% for each disorder) in the third decade of life. In conclusion, ADCK4-related glomerulopathy is an important novel differential diagnosis in adolescents with SRNS/FSGS and/or CKD of unknown origin., (Copyright © 2016 by the American Society of Nephrology.)

We report the case of a Lebanese infant born by normal delivery at 40 weeks of gestation. Weight and height at birth were 3200 grams and 49 cm respectively. Serum creatinine in the first week of life was 323 mmol/l (normal value for age is less than 20 mmol/l). Kidney ultrasound confirmed diagnosis of hypoplastic small kidneys. Conservative treatment of renal failure was initiated from the first days of life. Conservative management of renal failure included careful attention to fluid balance, maintenance of adequate nutrition and correction of hyperkalemia, acidosis, hyperphosphatemia and prevention of renal osteodystrophy by the use of dietary phosphate binders and vitamin D analogs. After a slight decrease of serum creatinine in the first three months of life, creatininemia increased progressively despite conservative treatment associated to hyperkalemia and severe pruritis due to hyperphosphatemia. Faced with the progression of renal failure, we decided to start automated peritoneal dialysis at seven months of life. Weight and height were respectively 6200 g and 63 cm. Serum creatinine was 432 mmol/L. Pruritis improved after starting peritoneal dialysis as well as appetite. At 20 months of life and a weight of 9.7 kg, a familial kidney transplant was performed. Immunosuppressive treatment included SimulectO, on day 1 and day 4, associated to mycophenolate mofetil, tacrolymus and prednisolone. The immediate post-transplant period was complicated by urinay tract infection due to BSLE E. coli. After four episodes of urinary tract infection due to resistant E coli associated to a non obstructive stone of 15 mm in the allograft kidney, we performed two sessions of lithotripsy and placed a double J stent. Biochemical urinary analysis did not reveal any causes for lithiasis. Stone disappeared as well as infection after lithotripsy. Renal function is normal for age one year after the kidney transplant. To our knowledge, this is the first reported case of a successful kidney transplant in a Lebanese infant with kidney failure since birth with kidney transplant performed at less than 10 kg of weight on the transplant day. Peritoneal dialysis is the treatment of choice for infants with chronic kidney failure. Familial or cadaveric kidney transplant should be encouraged and performed even in infants weighing less than 10 kg allowing them to have normal growth as well as social and school integration.

Cytomegalovirus (CMV) remains one of the most important pathogen responsible for the morbidity and mortality of transplantation patients. The impact on recipients depends on the form of CMV infection knowing that 10% to 50% develop symptomatic disease while solid organ involvement if presumed (e.g. CMV nephritis) may have deleterious outcome and requires histopathology testing. Treatment with antivirals IV ganciclovir and valganciclovir is managed according to early diagnostic tools with quantitative nucleic acid testing (QNAT) and antigenemia that will indicate the extent of disease and monitor response to treatment. CMV prevention in particular conditions of high risk patients has proven to be beneficial, resistance to antivirals and CMV vaccines along with novel therapies are thoroughly discussed in this review describing the new perspectives of CMV infection management.

Introduction: During the period from September 1993 to March 2011, 79 kidney transplants in children and adolescents less than 18 years old were performed in our department; fifty-six in the familial group and 13 in the cadaveric group. We report in this study: immunosuppression protocols, medical and surgical complications (including rejection episodes), graft and patient survival., Material and Methods: Immunosuppression protocol has been modified throughout those eighteen years. Induction therapy includes serum anti- lymphocyte + cyclosporine + azathioprine and prednisone. Since 2000, azathioprine was replaced by mycophenolate mofetil and since January 2005 cyclosporine was given as well as tacrolymus according to EBV status. Basiliximab-Simulect® was included in the protocol since 2010., Results: Surgical complications were only two lymphoceles, reversible after surgical drainage in the peritoneum. Medical complications were mainly infections or rejections. Thirty-six episodes of rejection occurred: 7 in the commercial group, 16 in the familial group and 13 in the cadaveric group. At the end of the study, 9 patients returned to dialysis, 5 from the cadaveric group, 2 from the familial group and 2 from the commercial group., Conclusion: After eighteen years experience in kidney transplant, we believe that kidney transplant remains the optimal treatment for terminal renal failure even for children weighing less than 10 kg. The follow-up of pediatric patients with kidney transplant revealed different positive effects on growth, regular school attendance and psychomotor development. At the end of the study: 24 are at school, 12 at universities, 5 are attending specialized schools and 9 are active workers.

Kidney transplantation is now accepted to be the best treatment for end-stage renal disease. Despite the improvement of immunosuppressive therapy, there is still actually substantial loss of allografts, in part due to uncontrolled humoral immunity. For many years, the primary technique for the detection of anti-HLA antibodies was the CDC (complement dependent cytotoxicity). The recent use of solid phase assays, mainly the Luminex technology allowed detection of antibodies at much lower levels, and it has been shown that these antibodies have negative impact on the graft survival. We herein review the principal techniques for anti-HLA detection and the different presentations of humoral rejection.

Kidney transplantation is the treatment of choice of end stage kidney disease. Over the years, kidney transplantation progressed tremendously, mainly by the improvement of immunosuppressive drugs used in the prevention of acute rejection. Since the introduction of cyclosporine in the 80s, many immunosuppressive protocols have been established. These protocols are characterized by two strategies: with or without induction. The agents used in induction therapies can be polyclonal or monoclonal antibodies. The decision of using induction therapy relies mainly on the evaluation of the immunological risk in the recipient. Even if protocols with induction have improved early results concerning acute rejection, the protocoles without induction seem justified in some candidates. The optimal immunosuppressive protocol is not yet established, and individualization of immunosuppressive treatment is necessary.

Familial hyperkalemic hypertension (FHHt) is a Mendelian form of arterial hypertension that is partially explained by mutations in WNK1 and WNK4 that lead to increased activity of the Na(+)-Cl(-) cotransporter (NCC) in the distal nephron. Using combined linkage analysis and whole-exome sequencing in two families, we identified KLHL3 as a third gene responsible for FHHt. Direct sequencing of 43 other affected individuals revealed 11 additional missense mutations that were associated with heterogeneous phenotypes and diverse modes of inheritance. Polymorphisms at KLHL3 were not associated with blood pressure. The KLHL3 protein belongs to the BTB-BACK-kelch family of actin-binding proteins that recruit substrates for Cullin3-based ubiquitin ligase complexes. KLHL3 is coexpressed with NCC and downregulates NCC expression at the cell surface. Our study establishes a role for KLHL3 as a new member of the complex signaling pathway regulating ion homeostasis in the distal nephron and indirectly blood pressure.

Pediatric nephrology is not a priority medicine in developing countries, but it should improve along with the development of both preventive and curative simple measures since the mortality rate is still important. This can be applied to the management of urinary tract infection, acute nephritis and some other cases of acute renal failure. The identification and management of chronic kidney diseases is mainly based on blood pressure measurement, proteinuria screening and plasma creatinine assessment. However renal replacement therapy (i.e., dialysis and transplantation) is highly expensive and its access is therefore limited to selected growing countries. The improvement in the care of renal children therefore requires better medical knowledge, nurse training and population information.

Purpose: The purpose of this study was to describe the skeletal manifestations of primary hyperoxaluria type 1 (PH1), the most common of the primary hyperoxalurias., Methods: We clinically and radiographically reviewed 12 consecutive patients diagnosed with PH1, aged between 2 and 17 years. All patients had evidence of some type of renal involvement, 4 of whom were at end-stage renal disease (ESRD) and were under dialysis., Results: The main symptom was skeletal pain and was present only in the 4 severely involved patients and appeared during the second year of dialysis. The 2 most severely involved patients had evidence of pathological fractures. Radiological signs were present in patients with or without symptoms. These radiological signs were of two distinct types: those almost specific of oxalosis, such as dense and radiolucent metaphyseal bands and vertebral osteocondensations, which are found mainly in the severely involved individuals, and those less specific, such as signs of renal osteodystrophy, which are also found in less severely involved patients. Interestingly, our study revealed the presence of spondylolysis in 25% of cases. This latter finding is unique and has not previously been reported in the literature., Conclusions: The skeletal manifestations of PH1 include specific and less specific radiological signs, with some patients being asymptomatic, and others presenting with bone pain and pathological fractures, as well as spondylolysis.

Ureteric valves represent a very rare etiology of ureteral obstruction. We experienced an unusual case of bilateral distal ureteric valves that presented as bilateral primitive obstructed megaureters with anuria at the age of 40 days. To our knowledge, this is the second case of bilateral involvement of distal ureteric valves reported in the literature. Bilateral ureteral valves should be included in the differential diagnosis of bladder outlet obstruction, as well as bilateral primitive obstructed megaureters in children. Excision and ureteral reimplantation is curative.

Objectives: Diagnosis, initial workup and immediate prognosis in congenital or acquired heart diseases revealed by cardiogenic shock (CS) in a Lebanese paediatric multicentric study., Material and Methods: During a six-year period (1st January 1999 to 31 December 2004), 56 paediatric patients aged between 0 and 7 years have been admitted to the emergency departments or neonatal and intensive care units in ten different Lebanese hospitals, for high degree of CS suspicion with underlying cardiac disease. All diagnoses were confirmed by echocardiography., Results: All children were initially in a critical condition with signs of CS. Congenital heart diseases (CHD), specially left obstructive diseases (coarctation of the aorta, critical aortic stenosis, hypoplastic left heart syndrome) were the main etiologies found in newborns, followed by dilated cardiomyopathies in infants and children. Mortality was higher in the following two groups of patients: intubation, no diuresis after the first hour of admission despite therapeutic measures (prostaglandines in newborns, dopamine, dobutamine and furosemide). Beside those pathologies necessitating specific treatment, all the CHD (excluding hypoplastic left heart syndrome, one case of critical aortic coarctation and one case of congenital mitral regurgitation) were transferred to the catheterization laboratory or to surgery in a good hemodynamic condition., Conclusion: CS remains a notable medical problem observed in the emergency and paediatric intensive care units in Lebanon. Immediate prognosis is related to: immediate recognition of the CS, nature of the cardiopathy and initial response to therapeutic procedures such as rapid improvement of respiratory status and rapid diuresis. Echocardiography is a reliable, quick and non-invasive procedure for initial diagnosis.

Salmonella species are a rare cause of urinary tract infections in children. They have been associated with a higher incidence of structural abnormalities or immunosuppressive status. We report the case of an 11-year-old girl with urinary tract infection (UTI) secondary to Salmonella typhi and associated with urolithiasis. A review of the subject is then discussed.

Ciclosporine was launched in therapeutic use in 1978. It marked the transplantation era due to the success of organ transplantation. Ciclosporine is actually used in bone marrow transplantation and in the transplantation of slid organs such as kidney, liver, heart, lungs, lung-heart, pancreas and bowel. It was proven also efficient in the treatment of auto-immune diseases especially in the treatment of psoriasis and nephrotic syndrom. Pharmacokinetic and drug interactions of ciclosporine have to be taken in consideration in order to allow an optimal use of the medication. However, the drug presents a long list of side effects: hypertension, increased level of serum creatinine, hypomagnesemia, severe gingivitis, maxillary modification, which can restrict the therapeutic use of the drug.

Renal biopsy may be safely performed on selected pediatric patients in an ambulatory care setting. In order to verify the safety of this new procedure, we performed 51 renal biopsies on an ambulatory care basis over a five-year period (from December 1993 to December 1998): twenty-eight biopsies on transplanted kidneys and twenty-three of native kidneys. During the same period, 56 renal biopsies were performed during a regular hospitalization of a minimum of 24 hours. We compared the two groups: Ambulatory procedure group and overnight hospitalization group. There were no significant differences between the two groups in mean age, adequacy of renal samples and complications such as post-biopsy pain or hematuria. After this encouraging experience, we are proposing the ambulatory procedure to all pediatric patients in our unit. Parents will be informed that if macroscopic hematuria is noted in the immediate post-biopsy period, hospitalization might be necessary.

Background: The aim of our study was to establish a standard growth curve of renal, hepatic and splenic dimensions with respect to age, and to find if any relationship existed between these different curves., Patients and Methods: One hundred and fifty abdominal ultrasound studies were obtained on 62 male and 88 female normal children free of any chronic disease, whose ages ranged from 0 to 15 years. Hepatic, splenic and renal dimensions were obtained in a similar and reproducible fashion for all patients. A statistical study of the measurements obtained compared to the age was performed by dividing the subjects into five groups according to age, and after calculating the mean size and variance., Results: Standard growth curves for the liver, kidney and spleen were constructed. Compared to age, splenic size follows the same growth as that of the kidneys, with a constant ratio. The growth pattern of the liver parallel the renal curve with a mean difference of 2.72 cm., Conclusion: A moderate enlargement of spleen and liver is difficult to evaluate only by clinical examination. Ultrasound may detect it by using the kidney size as a reference.

A retrospective study was conducted in June 1997 concerning all Lebanese children with end stage renal failure. This study was able to recognize 20 children and infants (age less than 15) dialysed regularly in 8 hemodialysis centers. The other 23 centers are not following children. Thirteen children are dialysed on bicarbonate and seven on acetate. Children receive erythropoietin occasionally; 30% of them are polytransfused, 60% of them suffer from anemia. Four children have never received immunization against hepatitis B and three are seropositive for hepatitis C. None of these children has regular school attendance and psychological support for the child and his family do not exist. We conclude that the situation of children on hemodialysis in Lebanon is alarming. Solutions are possible and available; they need to be undertaken urgently.

We report the case of a 10-year-old girl who received a cadaveric kidney transplant for oxalosis after a period of 12 months on hemodialysis. The donor was a 6-year-old child. Cold ischemia was four hours. Diuresis occurred immediately in the operating room. Mean daily diuresis was maintained at 8 liters: first by i.v. perfusion, then by nocturnal continuous nasogastric hydration. In addition to the usual immunosuppressive drugs, she received pyridoxine, sodium citrate, phosphate, hydrochlorothiazide and magnesium. Daily hemodialysis was performed from Day 1 to Day 9 and four additional sessions every other day. The postoperative course was satisfactory. Oxaluria was elevated initially at 1074 mg/24 h (normal < 50 mg/24 h). One year later, mean daily diuresis is still 8 liters, renal function is normal and oxaluria is at 296 mg/24 h. Repeated graft sonography showed no nephrocalcinosis, but mild oxalate deposits are noted on renal biopsy. Isolated renal transplantation was successful in our patient. It allowed us to stop hemodialysis and to avoid extra-renal accumulation of oxalate. Despite this success, we are convinced that long term prognosis is uncertain and liver transplantation should be realized to correct definitely the biochemical defect.

Background: Hypomagnesemia-hypercalciuria and nephrocalcinosis is a rare inherited syndrome which is characterized by persistent hypomagnesemia despite supplementation, hypercalciuria, nephrocalcinosis and progressive renal failure., Observations: Case 1. A girl was referred at the age of 18 months because of polyuria, polydipsia and vitamin-resistant rickets. There was hypomagnesemia, hypercalciuria and mild renal insufficiency; ultrasonography showed nephrocalcinosis. For two years, she received hydrochlorothiazide and the course of the disease was marked by a significant reduction of urine output and hypercalciuria, recurrent urinary tract infections and a progression toward chronic renal failure. Case 2. The brother of this child was investigated at the age of nine months because of polyuria and polydipsia. He also had hypomagnesemia, hypercalciuria and nephrocalcinosis. Renal function was initially normal. After two years on continuous treatment with hydrochlorothiazide, hypercalciuria decreased without deterioration of renal function. No signs of rickets were noted and nephrocalcinosis remained stable., Conclusion: To our knowledge, these two patients are the youngest reported in the literature. The long-term deterioration of renal function is hazardous but rickets may be avoided by early administration of hydrochlorothiazide.

A retrospective study was conducted on all kidney transplantations performed between January 1993 and June 1996 in our multidisciplinary pediatric department. Thirteen children with a mean age of 6 years (3.5-12) were transplanted during the study period after an average waiting time of 2 years on dialysis for cadaveric transplants and 3.5 months for living related ones. No urologic complication was noted; however, intra-abdominal approach was decided to be performed only in children less than 9 kg. This decision was taken after the occurrence of one intestinal intussusception and two ileal obstructions. During these 3.5 years, five rejection episodes were treated: three were steroid responsive, two were resistant but responded respectively to plasmapheresis and to OKT3. The actuarial survival of the grafts and recipients were 100% for an average mean time of follow-up of 18 months. The latest serum creatinine and creatinine clearance averages were respectively 66 mumol/l and 105 ml/min/1.73 m2. In seven transplanted children for more than 18 months growth was satisfactory. Eleven children returned to school. Kidney transplantation is the optimal treatment for children and infants with chronic renal failure; however, this technique needs a highly experienced team and a permanent close follow-up. Some children with end stage renal disease can directly have preemptive transplantation which is becoming our recent choice to avoid the hemodialysis strain.

Pseudotumor cerebri is a syndrome characterized by intracranial hypertension (intracranial pressure >200 mmH2O) and a normal ventricular system. The diagnosis should be made as early as possible to prevent impairment of vision. Several diseases have been reported in association with pseudotumor cerebri in pediatric patients, and have been occasionally also noted with chronic renal failure, heart and renal transplantation. We report a 7-year-old boy who complained of severe headaches and visual impairment 2 years after hemodialysis for renal hypoplasia. Pseudotumor cerebri was suspected and, despite treatment with corticosteroids, acetazolamide, and lumboperitoneal diversion, visual impairment worsened. Bilateral optic nerve sheath decompression (ONSD) was performed without success and the child completely lost his vision within 2 weeks. He was successfully transplanted 2 months later. Two years post transplantation, the blind child has a normal renal function and school performance. Pseudotumor cerebri must be rapidly suspected in a child with renal failure suffering from headaches and papilledema. Visual loss may progress rapidly and ONSD seems to be the best surgical treatment when medical treatment fails. In this patient renal transplantation was well tolerated, with no deterioration in the neurological status over 2 years of follow-up.

Percutaneous renal biopsy in children is a safe procedure. It is used for definitive diagnosis, prognosis and evaluation of the response to therapy. Repeated percutaneous biopsies are currently performed especially in allograft kidneys and in native kidneys as well. No major complications were noted in our series. The use of the new automated technique with small disposable needles in a well sedated child and experienced operator minimizes the complication rate. The important clinical benefit of the percutaneous renal biopsy overcomes the minimal risk of the procedure. Mesangiocapillary glomerulonephritis and focal segmental glomerulosclerosis are the predominant histological findings in our series. More data from other centers are necessary to evaluate the real incidence of the different entities of renal diseases in our country.