Standardization of the management of rheumatoid purpura nephropathy in the West of France. What are the repercussions on the renal sequelae?

Introduction: Rheumatoid purpura is the most common vasculitis in children, and its renal involvement determines the prognosis. To date, no national protocol exists for its management. A protocol was drafted for the French Grand Ouest inter-region in 2011 in order to standardize practices.

Rare cause of life-threatening hypercalcemia in an infant: a case report.

Background: 25-Hydroxyvitamin D 24-hydroxylase (CYP24A1) deficiency is a rare cause of autosomal recessive infantile hypercalcemia due to vitamine D hypersensitivity.

Case Presentation: We report the case of a 2-year-old boy who presented with severe hypercalcemia-hypercalciuria and a bilateral nephrocalcinosis. Laboratory investigations detected a collapsed parathormone and a highly elevated 1α,25-dihydroxycholecalciferol along with an increased phosphate excretion (hypophosphatemia and hyperphosphaturia).

Results from a nationwide retrospective cohort measure the impact of C3 and soluble C5b-9 levels on kidney outcomes in C3 glomerulopathy.

C3 glomerulopathy (C3G) is a rare complement-mediated disease. Specific treatments are not yet available and factors predictive of kidney survival such as age, kidney function and proteinuria are not specific to C3G. The prognostic value of biomarkers of complement activation, which are pathognomonic of the diseases, remains unknown.

Cardiac involvement in pediatric hemolytic uremic syndrome.

Background: Cardiac involvement is a known but rare complication of pediatric hemolytic uremic syndrome (HUS). We conducted a nationwide observational, retrospective case-control study describing factors associated with the occurrence of myocarditis among HUS patients.

Methods: Cases were defined as hospitalized children affected by any form of HUS with co-existent myocarditis in 8 French Pediatric Intensive Care Units (PICU) between January 2007 and December 2018.

Clinical course and cost assessment of infants with a first episode of acute bronchiolitis presenting to the emergency department: Data from the GUERANDE clinical trial.

Introduction: Bronchiolitis is the leading cause of hospitalization for infants but its economic burden is not well documented. Our objective was to describe the clinical evolution and to assess the 1-month cost of a first episode of acute bronchiolitis presenting to the emergency department (ED).

Methods: Our study was an epidemiologic analysis and a cost study of the cohort drawn from the clinical trial GUERANDE, conducted in 24 French pediatric EDs.

A rare association between group A Streptococcus purpura fulminans and diffuse alveolar hemorrhage in a young girl: A case report.

We report the case of a 7-year-old girl with septic shock and coagulopathy associated with purpura fulminans (PF) and diffuse alveolar hemorrhage (DAH) due to group A Streptococcus (GAS) infection identified with 16S ribosomal RNA analysis performed on the skin biopsy. GAS infection with PF associated with DAH is rare in healthy young children but pediatricians should be aware of this condition because of the poor prognosis. The initial treatment for circulatory failure and severe disseminated intravascular coagulation as well as the prompt initiation of antibiotic treatment may be crucial for the outcomes of S.

Anti-Factor B Antibodies and Acute Postinfectious GN in Children.

Background: The pathophysiology of the leading cause of pediatric acute nephritis, acute postinfectious GN, including mechanisms of the pathognomonic transient complement activation, remains uncertain. It shares clinicopathologic features with C3 glomerulopathy, a complement-mediated glomerulopathy that, unlike acute postinfectious GN, has a poor prognosis.

Methods: This retrospective study investigated mechanisms of complement activation in 34 children with acute postinfectious GN and low C3 level at onset.

Evaluation of an oral analgesia protocol for upper-limb fracture reduction in the paediatric emergency department: Prospective study of 101 patients.

Background: Upper limb injuries are common in children. When required, closed fracture reduction can be performed in the emergency department without general anaesthesia but causes pain. The primary objective of this study was to assess an oral analgesia protocol for fracture reduction without general anaesthesia.

Trampoline injuries compared with other child activities.

Introduction: Trampolines are responsible for specific injuries. We examined the severity of these injuries in children compared with those occurring in other activities. Our primary goal was to compare the injury severity between trampolining and other activities.

Urine collection methods used for non-toilet-trained children in pediatric emergency departments in France: A medical practice analysis.

Introduction: Since April 2015, the French Society of Pediatrics has encouraged suprapubic aspiration (SA) and urethral catheterization (UC) for urine collection in non-toilet-trained children suspected of having urinary tract infections (UTIs) and has tried to reduce the use of urine bag (UB).

Objective: To analyze the medical practices concerning urine collection methods used for non-toilet-trained children in pediatric emergency departments in France.

Methods: We conducted a descriptive medical practice study in October 2017.

Prevalence of Novel Mutations in Antenatal Bartter Syndrome.

Background And Objectives: Mutations in the gene, located on the X chromosome, have been recently detected in males with a transient form of antenatal Bartter syndrome or with idiopathic polyhydramnios. The aim of this study is to analyze the proportion of the population with mutations in this gene in a French cohort of patients with antenatal Bartter syndrome.

Design, Setting, Participants, & Measurements: The French cohort of patients with antenatal Bartter syndrome encompasses 171 families.

CYP24A1 Mutations in a Cohort of Hypercalcemic Patients: Evidence for a Recessive Trait.

Context: Loss-of-function mutations of CYP24A1 (which encodes the 25-OH-D3-24-hydroxylase) have recently been reported to cause hypercalcemia.

Objectives: The aims of this study were: 1) to evaluate the frequency of CYP24A1 mutations in patients with medical history of hypercalcemia; 2) to show the clinical utility of a simultaneous assay of serum 25-hydroxyvitamin D3 (25-OH-D3) and 24,25-dihydroxyvitamin D3 (24,25-[OH]2D3) by liquid chromatography tandem mass spectrometry (LC-MS/MS); and 3) to investigate biochemical parameters in heterozygous gene carriers with CYP24A1 mutations.

Patients And Methods: We screened for CYP24A1 mutations in 72 patients with serum calcium levels > 2.

Kidney function and influence of sunlight exposure in patients with impaired 24-hydroxylation of vitamin D due to CYP24A1 mutations.

Loss-of-function mutations of CYP24A1, the enzyme that converts the major circulating and active forms of vitamin D to inactive metabolites, recently have been implicated in idiopathic infantile hypercalcemia. Patients with biallelic mutations in CYP24A1 present with severe hypercalcemia and nephrocalcinosis in infancy or hypercalciuria, kidney stones, and nephrocalcinosis in adulthood. We describe a cohort of 7 patients (2 adults, 5 children) presenting with severe hypercalcemia who had homozygous or compound heterozygous mutations in CYP24A1.

Early protocol biopsies in pediatric renal transplantation: interest for the adaptation of immunosuppression.

GPB are often performed in PRT to detect subclinical acute rejection or IF/TA. Reducing immunosuppression side effects without increasing rejection is a major concern in PRT. We report the results of GPB in children transplanted with a steroid-sparing protocol adapted to immunological risk.

Failure of bortezomib to cure acute antibody-mediated rejection in a non-compliant renal transplant patient.

Bortezomib has appeared recently as a potential active treatment for acute AMR for few years. We reported a patient who received two courses of bortezomib for the treatment of an acute AMR associated with de novo HLA DSA that occurred 18 months after renal transplantation because of non-compliance. Graft biopsy revealed features of acute humoral rejection with plasmocyte infiltration and C4d staining.

[Haemophilus meningitis in properly vaccinated children: report of three cases].

The 1993 introduction in France of the vaccine against the serotype b of Haemophilus influenzae (Hib) resulted in a fast reduction of invasive infections caused by this species. However, despite the introduction of a booster dose, cases of Hib meningitis can still be observed, even if they are exceptional. We report here on 3 cases of Hib meningitis observed at Rennes University Hospital, which occurred during the winter seasons between 2007 and 2010, in properly vaccinated infants and children aged 9, 14, and 29 months.

[Results of autopsy examination of the knee cartilage of 120 patients dying in the hospital. II. The femoro-tibial joint].

The authors have studied the autopsy results of both tibio-femoral joints in 120 patients: 57 women and 63 men, 112 of whom were over the age of 50. The condylar and tibial cartilages were classified into 5 categories: no lesion (0); slight fissure (I); severe fissure (II); slight deep ulceration (III); large ulceration (in more than 25 p. cent of the cartilage surface) exposing the sub-chondral bone (IV).

Solitary plasmacytoma of the spine. Long-term clinical course.

The data for 19 patients with solitary plasmacytoma of the spine were reviewed with regard to clinical course and prognosis (median follow-up, 96 months). Eight patients presented with spinal cord compression. A monoclonal immunoglobulin was initially detected in seven of 15 evaluable patients.

[Lupus disease with antinuclear antibodies of the nucleolar type. Apropos of a series of 13 cases].

An exclusive or predominant nucleolar location of antinuclear antibodies is rare in the course of lupus disease: less than 1 p. cent of our patients. Thirteen cases of lupus disease with exclusive or predominant antinucleolar antibodies are analyzed: clinically, the only difference from other lupus diseases is the absence of pleurisy (p less than 0.

Anti-mitochondrial type 5 antibodies and anti-cardiolipin antibodies in systemic lupus erythematosus and auto-immune diseases.

Twenty sera from patients with systemic lupus erythematosus (SLE) and high titre of IgG anti-cardiolipin antibodies (ACA) were studied in order to evaluate the prevalence of anti-mitochondrial type 5 antibodies (AMA 5). None of these sera were found to be AMA 5 positive but five of 18 were positive for VDRL. Twenty sera from patients with AMA 5 were studied in order to evaluate the prevalence of ACA: only six of 20 were positive for ACA.

Profile and cross-reactivities of antiphospholipid antibodies in systemic lupus erythematosus and syphilis.

Antiphospholipid antibodies have been determined in two groups of 48 sera from patients with systemic lupus erythematosus (SLE) and syphilis. Using an ELISA, IgG anticardiolipin (CL), antiphosphatidyl serine (PS) and antiphosphatidyl ethanolamine (PEA), antibodies have been detected with the same frequency in both groups of patients. Titres of antiphosphatidyl serine (PS) (p less than 0.