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There seems to be a possible link between nephrotic syndrome (NS) and lymphoproliferative syndrome, but it remains poorly understood.This multicentric and retrospective study focuses on children, who developed idiopathic NS and malignant or benign proliferation between 2000 and 2021.Eleven patients were included, with a median age of 4 years. Only one had a steroid-resistant nephrotic syndrome (SRNS). The maintenance therapy before the proliferation was in majority tacrolimus or mycophenolate mofetil (MMF), but three patients did not receive treatments. The proliferation was mainly a Hodgkin's lymphoma (45%) or a lymphoproliferative disease (36%), in a median time after the NS of two years. Viruses were found in seven cases (EBV in five cases and HHV-8 in two).The association between proliferative syndrome and idiopathic NS may not be fortuitous, possibly with a common lymphocytic disturbance. Genetic analyses could improve the comprehension of these manifestations in the future. A higher resolution version of the Graphical abstract is available as Supplementary information.

Clinical practice guidelines (CPGs) are systematically developed statements backed by scientific evidence to assist practitioners in management in clinical practice. An international cross-sectional survey was conducted by the IPNA to examine the perceptions of pediatric nephrologists on guidelines and their usage and to identify important diseases for future clinical practice guidelines (CPGs). The survey found that the majority of pediatric nephrologists find CPGs useful in clinical practice and admitted to using them most of the time. Developing CPGs is challenging and there are standards available to develop trustworthy guidelines. While evidence-based global guidelines are ideal, pediatric nephrologists expressed the desire that they address regional differences. Most respondents (89.2%) to the survey agreed that adult guidelines did not cover the pediatric perspective adequately and 71.4% opined that consensus-based pediatric guidelines can be developed when evidence for the pediatric population is lacking. The development of high-quality practice guidelines requires substantial resources and may not be feasible in resource-poor countries. Adaptation of an existing guideline has been suggested as an alternative and the ADAPTE collaboration provides a systematic approach to adapting guidelines. Several diseases where pediatric guidelines are needed as a priority including IgA and C3 glomerulopathy were identified in the survey. Implementation of guideline-based care is challenging and the survey found that lack of availability of guidelines (43%) and resources (22.8%) are important reasons for poor implementation in lower-middle and low-income countries. Perceived complexity of guidelines, physician attitudes, and lack of training also contribute to non-adherence to guidelines.

The management of infants with congenital nephrotic syndrome (CNS) is very challenging as they are prone to severe complications such as hemodynamic disturbances, infections, thromboses, and impaired growth, and most will develop end-stage kidney disease (ESKD) within a few years. Since the seventies, an "aggressive" approach, including daily albumin infusions, early nephrectomies, dialysis, and transplantation, has dramatically improved survival and morbidity. More recent case-note reviews have reported successful conservative treatment (using optimized nutrition, complication prophylaxis, and delayed renal replacement therapy), which led to similarly good outcomes and low complication rates. This questions the indications for early preemptive bilateral nephrectomy and dialysis given the mortality and morbidity rates in dialysis in infants and their life-long management with possible repeated transplantations. Two large series provide the most recent evidences supporting the conservative management: firstly, at least 55% children with CNS are not spontaneously in ESKD at the age of 2 years; secondly, albumin tapering/discontinuation and hospital discharge are possible before nephrectomy; and lastly, CNS complication rates are similar in case of preemptive nephrectomies or conservative care. Until now, no clear genotype-phenotype correlation has been identified to guide clinical management. Taken together, these data support the safety of conservative care until ESKD in a subset of patients with CNS.

IgA nephropathy (IgAN) is one the most common primary glomerulonephritis in children and adolescents worldwide, with 20% of children developing end-stage kidney disease (ESKD) within 20 years of diagnosis. There is a need for treatment guidelines, especially for steroids in children with primary IgAN, since the STOP-IgA trial casts doubts on the use of steroids in adults with intermediate risk. Pediatricians are prone to prescribe steroids in addition to renin–angiotensin system blockade (RASB) when proteinuria is > 0.5 g/l, eGFR deteriorates

Anti-complement factor H (CFH) autoantibody (Ab)-associated atypical hemolytic uremic syndrome (aHUS) has a poor prognosis, but no consensus exists on its treatment.We report the follow-up of four children with anti-CFH Ab (8,000 to32,000 arbitrary units)-associated aHUS after plasma exchanges (PEs), prednisone, and cyclophosphamide pulse therapy with the evolution of anti-CFH Ab titers and kidney function.Patient 1 received PEs + prednisone + cyclophosphamide pulses after two relapses following PEs and then PEs + rituximab. The other three patients were treated with PEs + prednisone + cyclophosphamide pulses as a first-line therapy. In our four patients, the induction protocol combining PEs + prednisone + cyclophosphamide pulses led to a rapid and sustained remission up to 6 years, 4 years and 4 months without any maintenance therapy. Kidney function was normal and anti-CFH Ab titer decreased, but remained detectable during remission without any clinical or biological signs of relapse.We demonstrate the long-term efficiency and safety of cyclophosphamide pulses combined with PEs and prednisone in anti-CFH Ab-associated aHUS leading to a prolonged decrease in anti-CFH Ab titers and prevention of relapses without the need for maintenance therapy.

We report the case of a 4-year-old boy, diagnosed with atypical hemolytic uremic syndrome (HUS) due to a hybrid factor H. He progressed to end-stage renal failure despite plasmatherapy and underwent bilateral nephrectomy because of uncontrolled hypertension. Three days after, he had partial complex seizures with normal blood pressure, normal blood count and normal magnetic resonance imaging (MRI), which recurred 1 month later. Eight months later, he had a third episode of seizures, with hemoglobin of 10 g/dl without schizocytes, low haptoglobin of 0.18 g/l, and moderate thrombocytopenia (platelets 98 × 10(9)/l). He remained hypertensive and deeply confused for 2 days. The third MRI showed bilateral symmetrical hyperintensities of the cerebral pedunculas, caudate nuclei, putamens, thalami, hippocampi, and insulae suggesting thrombotic microangiopathy secondary to a relapse of HUS rather than reversible posterior leukoencephalopathy syndrome (RPLS), usually occipital and asymmetrical. Plasmatherapy led to a complete neurological recovery within 2 days although hypertension had remained uncontrolled. The fourth MRI 10 weeks after, on maintenance plasmatherapy, was normal and clinical examination remained normal, except for high blood pressure. In conclusion, brain MRI allows differentiating thrombotic microangiopathy lesions from RPLS in atypical HUS, which is crucial since lesions may be reversible with plasmatherapy.

Many children with steroid-dependent nephrotic syndrome (NS) have significant sequelae despite steroid-sparing therapies. Levamisole may reduce short-term relapse frequency (RF) with minimal side effects. Little data exist, however, as to its long-term effect. To assess both short- and long-term efficacy in NS, RF and cumulative annual steroid burden were quantified in ten consecutive children with steroid-dependent NS treated with levamisole. Data were analyzed for three time periods: 1 year prior to levamisole therapy (Pre-Lev), during 1 year of levamisole therapy (During-Lev), and the year after cessation of all levamisole therapy (Off-Lev). Median RF fell from 6.0 (4.0-9.0) relapses/patient per year Pre-Lev to 0.0 (0.0-4.0) During-Lev (p = 0.002) with 6/10 patients having no relapse and 0.5 (0.0-8.0) Off-Lev (p = 0.01) with 5/10 patients without relapse. Concurrently, cumulative annual steroid burden fell from 6,067 (1,660-8,691) mg/m(2) per year Pre-Lev to 2,920 (782-5,271) During-Lev (p = 0.002) and 716 (0-3,637) Off-Lev (p = 0.002). In 4/5 hypertensive children, blood pressure normalized During-Lev. Somatic indices also improved: height Z scores, which fell from 0.8 (-2.4 to 3.6) at diagnosis to -0.6 (-2.7 to 0.4) Pre-Lev (p = 0.004), remained stable at -0.6 (-3.0 to 0.6) after 1 year of therapy and -0.5 (-2.6 to 0.2) Off-Lev. Height velocity improved from 3.0 (0.3-6.0) cm/year Pre-Lev to 3.7 (0.0-8.0) cm/year During-Lev and 5.4 (0.0-9.1) Off-Lev. We conclude that levamisole is an effective short- and long-term steroid-sparing agent in pediatric NS.

Recent data suggest that the histologic finding of focal and segmental glomerulosclerosis (FSGS) is increasing among children. There are, however, limited longitudinal pediatric data on prevalence, demographics, and steroid responsiveness in FSGS. We identified 201 consecutive nephrotic children diagnosed between 1977 and 2002 with 2 years follow-up; 51% had undergone renal biopsy due to steroid sequelae or resistance; 48 children with FSGS were diagnosed. Compared with non-FSGS children, FSGS children were older at diagnosis (6.9 years vs 4.4 years, P

The use of prenatal ultrasonography has resulted in increased numbers of fetuses being diagnosed with autosomal dominant polycystic kidney disease (ADPKD), but the long-term prognosis is still not well-known. Between 1981 and 2006 we followed 26 consecutive children with enlarged hyperechoic kidneys detected between the 12th week of pregnancy and the first day of life (Day 1) as well as one affected parent. Three other fetuses were excluded following the termination of the pregnancy. The mother was the transmitting parent in 16 of the 26 children (ns, p = 0.1). Clinical features that presented during follow-up were oligoamnios (5/26), neonatal pneumothorax (3/26), pyelonephritis (5/26), gross hematuria (2/26), hypertension (5/26), proteinuria (2/26) and chronic renal insufficiency (CRI) (2/26). At the last follow-up (mean duration of follow-up: 76 months; range: 0.5–262 months), 19 children (mean age: 5.5 years) were asymptomatic, five (mean age: 8.5 years) had hypertension, two (mean age: 9.7 years) had proteinuria and two (mean age: 19 years) had CRI. Children presenting enlarged kidneys postnatally tended to have more clinical manifestations than their counterparts who did not. Of 25 siblings of the patients, seven had renal cysts; these were detected during childhood in five siblings and in utero in two siblings. In conclusion, prognosis is favourable in most children with prenatal ADPKD, at least during childhood. The sex of the transmitting parent is not a risk factor of prenatal ADPKD. A high proportion of siblings develop early renal cysts. Abnormalities visualized by ultrasonography appear to be associated to more clinical manifestations.

Audrey Pawtowski and Olivia Boyer were omitted from the list of authors. The correct list is given above. The authors apologize for this error.