Antibiotic Prophylaxis in Infants with Grade III, IV, or V Vesicoureteral Reflux.

Background: The efficacy of continuous antibiotic prophylaxis in preventing urinary tract infection (UTI) in infants with grade III, IV, or V vesicoureteral reflux is controversial.

Methods: In this investigator-initiated, randomized, open-label trial performed in 39 European centers, we randomly assigned infants 1 to 5 months of age with grade III, IV, or V vesicoureteral reflux and no previous UTIs to receive continuous antibiotic prophylaxis (prophylaxis group) or no treatment (untreated group) for 24 months. The primary outcome was the occurrence of the first UTI during the trial period.

[Renal infarction in an adolescent carrier of the sickle cell trait].

We report the case of a 15-year-old teenager, carrier of the sickle cell trait (haemoglobin AS), who presented a renal infarction. Besides, the patient also presented a renal ectopia. It is tempting to link these two particularities and the ischemic attack.

Three years pilot of spinal muscular atrophy newborn screening turned into official program in Southern Belgium.

Three new therapies for spinal muscular atrophy (SMA) have been approved by the United States Food and Drug Administration and the European Medicines Agency since 2016. Although these new therapies improve the quality of life of patients who are symptomatic at first treatment, administration before the onset of symptoms is significantly more effective. As a consequence, newborn screening programs have been initiated in several countries.

Corrigendum: Consensus Recommendations for the Diagnosis and Management of X-Linked Hypophosphatemia in Belgium.

[This corrects the article DOI: 10.3389/fendo.2021.

Consensus Recommendations for the Diagnosis and Management of X-Linked Hypophosphatemia in Belgium.

X-linked hypophosphatemia (XLH) is the most common genetic form of hypophosphatemic rickets and osteomalacia. In this disease, mutations in the gene lead to elevated levels of the hormone fibroblast growth factor 23 (FGF23), resulting in renal phosphate wasting and impaired skeletal and dental mineralization. Recently, international guidelines for the diagnosis and treatment of this condition have been published.

[A case of severe congenital nephrotic syndrome secondary to NPHS1 mutation].

The congenital nephrotic syndrome is a rare and severe pathology, and its management represents a real challenge for pediatric nephrologists. We report the case of a congenital nephrotic syndrome secondary to a homozygous mutation of the NPHS1. The young patient has a severe clinical course, and benefits of a management by anti-proteinuric treatment and a unilateral nephrectomy.

Vitamin D-Resistant Rickets and Cinacalcet-One More Favorable Experience.

Hereditary vitamin D-resistant rickets (HVDRR) is an autosomal recessive disorder characterized by early onset of severe rickets, with a complete triad of clinical, biochemical and skeletal abnormalities. Homozygous or heterozygous mutations in the vitamin D receptor () gene leading to complete or partial target organ resistance to the action of 1α, 25-dihydroxyvitamin D3 (the active form of vitamin D) are responsible for HVDRR. Theoretically the therapeutic goal is to overcome this tissue resistance, and to normalize calcium and phosphate homeostasis.

Malignancy-Induced Hypercalcemia-Diagnostic Challenges.

Hypercalcemia in children is a rare metabolic finding. The clinical picture is usually non-specific, and the etiology includes several entities (metabolic, nutritional, drug-induced, inflammatory, cancer-associated, or genetic) depending on the age at presentation, but severe hypercalcemia is associated mainly with malignancy in childhood and sepsis in neonates. Severe parathyroid hormone (PTH)-suppressed hypercalcemia is challenging and requires multidisciplinary diagnostic and therapeutic approaches to (i) confirm or rule out a malignant cause, (ii) treat it and its potentially dangerous complications.

[How I explore and treat a neonatal renal vein thrombosis: a case report].

Neonatal renal vein thrombosis is a rare condition. The present case is rather unfrequent and particularly educative since it shows the complete diagnostic triad including hematuria, flank mass and thrombocytopenia. The diagnosis relies on the demonstration, by Doppler ultrasound, of an obstructed renal venous bed.

Neonatal progeroid variant of Marfan syndrome with congenital lipodystrophy results from mutations at the 3' end of FBN1 gene.

We report a 16-year-old girl with neonatal progeroid features and congenital lipodystrophy who was considered at birth as a possible variant of Wiedemann-Rautenstrauch syndrome. The emergence of additional clinical signs (marfanoid habitus, severe myopia and dilatation of the aortic bulb) lead to consider the diagnosis of the progeroid variant of Marfan syndrome. A de novo donor splice-site mutation (c.

[Empirical antibiotic therapy for urinary tract infection in children. Analysis of three hundred patients admitted to a general hospital].

For more than 25 years, treatment of acute pyelonephritis in children has been the subject of debates. Recent publications (including of Cochrane database review) let think that oral antibiotics could be as safe as intravenous treatement at least regarding the major concerns of urinary infection: renal scaring, time to defervescence and sterilization of urine at 72 hours. To investigate if such a protocol could be applied in our country, we first determine the rate of bacterial resistance to oral antibiotics commercially available in Belgium.

[Renal tumors in children. A single center study of 31 cases].

In order to illustrate epidemiologic features and survival rate, 31 Wilm's tumours treated in our institution have been retrospectively studied. The mean age at diagnosis in our series was surprisingly higher than usually described: 25% of the patients were older than 8 years. Moreover, a mesoblastic nephroma congenital kidney tumour--appeared in a 10 month old girl.

[Respiratory syncytial immunoprophylaxis with palivizumab].

Respiratory syncytial virus (RSV) is a serious pathogen causing significant morbidity, especially in premature infants and infants with chronic lung disease or significant congenital heart disease. There is no specific treatment for RSV infection and the therapy is essentially supportive. Therefore, prophylaxis is the best strategy against RSV disease.

[Management of acute bronchiolitis in newborns].

Acute bronchiolitis is a common condition of viral origin with attention of treating hypoxia and maintaining hydration. Medications are often ineffective, although widely used in our countries. If the spontaneous cure is the rule, the persistence of a respiratory symptomatology (cough or wheezing) during several weeks is not exceptional.

[Vaccination against Rotavirus: an old challenge, an ongoing reality].

Rotavirus are the leading cause of diarrhea and diarrhea related death among infants and young children. Every year rotavirus is associated with over half a million of deathss, mainly in developping countries. Development of a safe vaccine is nowaday the only way to control the disease.

[The hemolytic uremic syndrome: prime reason for acute renal failure in children].

The Hemolytic Uremic Syndrome (HUS) is the prime cause for acute renal failure in children. The HUS is a combination of hemolytic anemia, thombopenia and acute nephropathy: all signs of a thrombotic microangiopathy. Onset occurs generally in infancy and is often associated with severe bloody diarrhea.

The safety and efficacy of nitric oxide therapy in premature infants.

Objectives: To assess the safety-efficacy balance of low-dose inhaled nitric oxide (iNO) in hypoxemic premature infants because no sustained beneficial effect has been demonstrated clearly and there are concerns about side effects.

Study Design: Eight hundred and sixty infants <32 weeks were randomized at birth to receive 5 ppm iNO or placebo when they presented with hypoxemic respiratory failure (HRF) defined by a requirement for mechanical ventilation, fraction of inspired oxygen (FIO 2 ) >40%, and arterio-alveolar ratio in oxygen (aAO 2 ) <0.22.

Bony syngnathia, vertebral segmentation defect, coloboma, microcephaly and mental retardation: confirmation of Dobrow syndrome and review of syndromal syngnathias.

Congenital bony fusion of the maxilla and mandible is a rare condition. Two classifications were previously proposed dealing exclusively with craniofacial malformations. Most of the reported cases to date represent either aglossia-adactylia or hemifacial microsomia syndromes.

The EPIBEL study: outcomes to discharge from hospital for extremely preterm infants in Belgium.

Objective: To determine mortality and morbidity at discharge from the hospital of a large population-based cohort of infants who were born at
Methods: Perinatal data were collected on extremely preterm infants who were alive at the onset of labor and born between January 1, 1999, and December 31, 2000, in all 19 Belgian perinatal centers.

Results: A total of 525 infants were recorded.

The flow-pressure plot: a new look on the patient-ventilator interaction in neonatal care.

Most modern neonatal ventilators have now a built-in flow sensor and, as a spin-off of their mechanical action, provide some information about lung function characteristics as compliance and resistance after computation of the flow and pressure signals. Additionally, respiratory graphics as volume-pressure and flow-volume plots can be displayed. In clinical practice, however, they are rarely used to refine the ventilator setting.

Heart rate response profiles to tilting in healthy and unhealthy neonates.

Background: Clinical observations suggest the immaturity of the neonate's ability to respond appropriately to cardio-respiratory challenges and tilt test could help in detecting autonomic dysfunction in neonates.

Material/methods: Heart rate (HR) responses following a 45 degrees head-up tilt during sleep were measured within the first week of adjusted age in 7 healthy full-term neonates, 7 healthy preterm infants and 5 neonates with abstinence syndrome.

Results: The tilt and the return to the horizontal position generally provoked a sustained or transient tachycardia, or a flat response in healthy full-term neonates and in preterm neonates, but a sustained or transient bradycardia in neonates with abstinence syndrome.

Fronto-otopalatodigital osteodysplasia: clinical evidence for a single entity encompassing Melnick-Needles syndrome, otopalatodigital syndrome types 1 and 2, and frontometaphyseal dysplasia.

Otopalatodigital syndrome type 2 is an X-linked disorder with minimal expression in carrier females and comprises typical facial anomalies and a generalized bone dysplasia with osteodysplastic changes, brachydactyly, and impaired survival. Recently several other severe malformations were reported in the condition. Melnick-Needles syndrome is an X-linked dominant disorder.