[Case report of an infant hyperkalemia: Suggestion of a hospital procedure for the milk pretreatment with sodium polystyrene sulfonate resin]. / Exemple d'un cas d'hyperkaliémie du nourrisson : proposition d'un circuit pour le prétraitement du lait infantile par résine de polystyrène sulfonate de sodium à l'hôpital.

Sodium polystyrene sulfonate (SPS) is used to reduce intestinal potassium absorption in hyperkalemia during conservative management of chronic renal failure in infants. Milk can be pretreated by SPS to reduce the risk of enteropathy associated with oral or rectal administration. We report the case of an infant for whom this pre-treatment has been implemented. The objective of this work was to define the hospital procedure for the pre-treatment of milk by the SPS. This pre-treatment involves both a drug and infant milk. Each product has its own regulation and their processes do not normally cross each other. The roles of each contributor were therefore defined: prescription of pre-treated milk (dose of SPS and volume of milk) by the physician, dispensing of SPS by the pharmacist, delivery of milk by the milk kitchen staff, pre-treatment by a nurse and administration by a nursing auxiliary. The preparation of the bottles is as follows: placing approximately 1g of SPS per 100mL of milk in contact, stirring, resting in the refrigerator for one hour, taking the supernatant to be administered. In the reported case, serum potassium levels were reduced from 5.57mmol/L before treatment to 4.53mmol/L after treatment, in line with the 20% decrease found in the literature. This method of administration is beneficial in terms of tolerance and acceptability. The preparation is simple and allows going back home under treatment.

Long-term renal outcome in infants with congenital lower urinary tract obstruction.

INTRODUCTION: Congenital Lower Urinary Tract Obstructions (LUTO) is a heterogeneous group of diseases in which urine elimination is obstructed at the level of bladder neck or urethra. The aim of the study is to evaluate the long-term renal outcome of patients suffering of congenital LUTO. PATIENTS AND METHOD: We retrospectively reviewed patients with congenital LUTO. All patients had at least 1 year follow-up. Data on surgery, renal imaging and Schwartz estimate creatinine clearance were collected. Incidence of Chronic Renal Disease (CRD) is presented with Kaplan-Meier method. RESULTS: 40 patients were included, 23 patients with Posterior Urethral Valve (PUVs) and 17 patients with other aetiologies: anterior urethral valve (2), urethral atresia (2), urethral stenosis (2), cloacal malformations (2), obstructive ureterocele (1), bladder trigone malformation (1) and neonatal bladder-sphincter dysfunction without neurological abnormalities (7). Incidence of CRD at age 10 years was 37% in congenital LUTO, 42% in PUVs and 30% in other aetiologies, and was significantly higher in PUVs (P=0.032). Renal prognosis was significantly worsened by discover of retentional bladder wall changes in initial cystoscopy, and by loss of parenchymal differentiation or cortical microcysts in first ultrasonography. The use of urinary diversion was significantly higher in LUTO of other aetiologies. CONCLUSION: A high incidence of CRD is observed in patients with congenital LUTO, significantly higher in patients with PUV. LUTO of other aetiologies require step by step surgical management and higher use of urinary diversion. Precise initial evaluation in cystoscopy and ultrasonography is required and participate to evaluate future renal outcome. LEVEL OF INCIDENCE: 4.

[Treatments of steroid-dependent nephrotic syndrome in children]. / Traitements du syndrome néphrotique cortico-dépendant de l'enfant.

Primary nephrotic syndrome (NS) is the most common glomerular disease in children. It is characterized by massive proteinuria and hypoalbuminemia. It typically has a sudden onset and more than 70% of patients will experience at least one relapse. An immunological origin has long been postulated, although the precise molecular mechanisms underlying the disease remain debated. Steroids are the first-line therapy with cumulative dose and duration of initial treatment varying among countries. Steroid-sparing agents may be indicated in case of steroid-dependency or frequent relapses. However, no consensus exists regarding the different treatment options. These treatments are mostly suspensive and therefore, need to be prolonged for several months. Levamisole, an antihelminthic drug, also has an immunomodulatory function, and alone or in combination with steroids, it can decrease cumulative steroid dose and relapses. It is usually well tolerated, and its principal side effects are cytopenia and elevated liver enzymes. Mycophenolate mofetil is an immunosuppressive agent whose reported side effects are cytopenia and diarrhea. Calcineurin inhibitors (cyclosporine or tacrolimus) have long been used in steroid-dependent patients. Their major side effects are hirsutism, gum hypertrophy, and nephrotoxicity, leading to interstitial kidney fibrosis and chronic kidney disease. Cyclophosphamide is an efficient treatment but its gonadal toxicity is a major drawback to its use. More recent drugs such as rituximab are very effective but require hospitalization for the infusion and induce an increased risk of opportunistic infection, prolonged neutropenia, and anaphylaxis. In this review, we present the available treatments, their indications, and the side effects.

[Idiopathic nephrotic syndrome]. / Aspects cliniques du syndrome néphrotique idiopathique de l'enfant.

Nephrotic syndrome (NS) is defined by massive proteinuria and hypoalbuminemia, with resulting hyperlipidemia and edema. The most common cause of NS in children is idiopathic nephrotic syndrome (INS), also called nephrosis. Its annual incidence has been estimated to 1-4 per 100,000 children and varies with age, race, and geography. Many agents or conditions have been reported to be associated with INS such as infectious diseases, drugs, allergy, vaccinations, and malignancies. The disease may occur during the 1st year of life, but it usually starts between the ages of 2 and 7 years. INS is characterized by a sudden onset, edema being the major presenting symptom, but may rarely be discovered during a routine urine analysis. The disease may also be revealed by a complication such as hypovolemia, infection (pneumonia and peritonitis due to Streptococcus pneumoniae), deep-vein or arterial thromboses, and pulmonary embolism. Renal biopsy is usually not indicated in a child aged 1-10 years with typical symptoms and a complete remission with corticosteroids. Conversely, it is indicated in children under 1 year in case of macroscopic hematuria, hypertension, low C3 levels, persistent renal failure, or steroid resistance. Steroid therapy is applied in all children whatever the histopathology. Initial prednisone therapy in France consists of 60mg/m2 administered daily for 4 weeks (maximum dose, 60mg/day), followed by alternate-day prednisone with tapering doses. Eight-five to 90 % patients are steroid-responsive and may relapse, but the majority still responds to steroids over the subsequent courses. Only 1-3 % of patients who are initially steroid-sensitive subsequently become steroid-resistant. Children with primary or secondary steroid-resistance are at risk of end-stage kidney disease. Symptomatic treatment includes salt restriction, fluid restriction when natremia is less than 125 meq/L, reduction of saturated fat and carbohydrates, calcium and vitamin D supplements, anticoagulation, and vaccination. Albumin infusions are only indicated in case of complications. Diuretics should be restricted to cases of severe edema, after hypovolemia has been corrected.