Aerococcus urinae - significance of detection in the paediatric urinary tract: a case series.

Aerococcus urinae (A. urinae) is primarily recognized as a common pathogen in the geriatric population, causing urinary tract infection (UTI), sepsis, and endocarditis, predominantly in female patients. In the paediatric population, only a few case reports exist suggesting A. urinae causes malodorous urine in otherwise healthy boys. In this study, we investigated the spectrum of clinical and laboratory presentations of A. urinae detection in children. A retrospective, single-centre, case series including all patients with the detection of A. urinae during a 7-year study period. Patients with detection of A. urinae only in non-urogenital skin swabs were excluded. A total of 40 samples from 33 patients were identified of which 20 patients were included in the final analysis. The median (IQR) age was 6.8 (2.9-9.5) years; 18 (90%) patients were boys. Four patients were diagnosed with a UTI, six had malodorous urine without UTI, three were diagnosed with balanitis and seven showed A. urinae colonization in the urine culture. Urogenital disorders were present in 12 patients. Additional pathogens were detected in 13 patients. Recurrence of detection during our study period was observed in four (20%) patients.  Conclusion: Beyond malodorous urine, A. urinae detection is associated with more severe presentations including UTI in the paediatric population. Pre-existing urogenital disorders were frequent, and therefore, a nephro-urological investigation should be considered in all cases of A. urinae detection in the paediatric population. What is Known: • Aerococcus urinae (A. urinae) is known to be a common pathogen in the geriatric population, causing urinary tract infection (UTI), sepsis, and endocarditis, predominantly in female patients. • In the paediatric population, A. urinae is mainly described as a low-grade pathogen. Some case reports describe A. urinae as the cause of extraordinary malodorous urine in otherwise healthy boys. What is New: • Beyond malodorous urine, A. urinae detection is associated with more severe presentations including UTI in the paediatric population. • A. urinae was mainly detected in boys with pre-existing urogenital disorders; therefore, a nephro-urological investigation should be considered in cases of A. urinae detection in the paediatric population.

Diagnostic accuracy of an interdisciplinary tertiary center evaluation in children referred for suspected congenital anomalies of the kidney and urinary tract on fetal ultrasound - a retrospective outcome analysis.

BACKGROUND: Fetal ultrasound organ screening has become a standard of care in most high-income countries. This has resulted in increased detection of congenital abnormalities, which may lead to major uncertainty and anxiety in expectant parents, even though many of them are of minor relevance. In order to optimize prenatal counselling, we introduced an interdisciplinary approach for all pregnant women referred to our center by private obstetricians for a co-assessment of suspected relevant fetal abnormalities of the kidney or urinary tract, involving both experienced prenatal ultrasound specialists and a pediatric nephrologist or urologist. METHODS: In a retrospective analysis, we evaluated reports of intrauterine evaluation and postnatal follow-up in order to assess accuracy of explicit intrauterine diagnoses and outcome of hydronephroses according to their severity in this setting. RESULTS: A total of 175 fetuses were examined between 2012 and 2019 and followed postnatally at our Pediatric Nephrology or Urology Department. There was a high concordance (85.9%) between explicit intrauterine and final diagnoses. Resolution rate of hydronephrosis was higher in patients with intrauterine low-grade than high-grade hydronephrosis (61.8% versus 11.9%). An etiological diagnosis was found in 62.5%, 52.0%, and 11.1% of patients with intrauterine bilateral high-grade, unilateral high-grade, and unilateral high-grade with contralateral low-grade hydronephrosis, respectively, but in none of the patients with intrauterine low-grade hydronephrosis. CONCLUSIONS: The results of our study demonstrate that, through interdisciplinary teamwork, intrauterine assessment of the fetal kidneys and urinary tract is highly accurate and allows a good discrimination between relevant and transient/physiological hydronephroses. A higher resolution version of the Graphical abstract is available as Supplementary information.

Early Urinary Biomarkers in Pediatric Autosomal Dominant Polycystic Kidney Disease (ADPKD): No Evidence in the Interest of Urinary Neutrophil Gelatinase-Associated Lipocalin (uNGAL).

Background: Autosomal Dominant Polycystic Kidney Disease (ADPKD) is increasingly diagnosed during childhood by the presence of renal cysts in patients with a positive familial history. No curative treatment is available and early detection and diagnosis confronts pediatricians with the lack of early markers to decide whether to introduce renal-protective agents and prevent the progression of renal failure. Neutrophil Gelatinase-Associated Lipocalin (NGAL) is a tubular protein that has been recently proposed as an early biomarker of renal impairment in the ADPKD adult population. Methods: Urinary NGAL (uNGAL) levels were measured in 15 ADPKD children and compared with 15 age and gender matched controls using parametric, non-parametric, and Bayesian statistics. We also tested the association of uNGAL levels with markers of disease progression, such as proteinuria, albuminuria, blood pressure, and Total Kidney Volume (TKV) using correlation analysis. TKV was calculated by ultrasound, using the ellipsoid method. Results: No difference in mean uNGAL levels was observed between groups (ADPKD: 26.36 ng/ml; Controls: 27.24 ng/ml; P = 0.96). Moreover, no correlation was found between uNGAL and proteinuria (P = 0.51), albuminuria (P = 0.69), TKV (P = 0.68), or mean arterial pressure (P = 0.90). By contrast, TKV was positively correlated with proteinuria (P = 0.04), albuminuria (P = 0.001), and mean arterial pressure (P = 0.03). Conclusion: uNGAL did not confirm its superiority as a marker of disease progression in a pediatric ADPKD population. In the contrary, TKV appears to be an easy measurable variable and may be promising as a surrogate marker to follow ADPKD progression in children.

[Vitamin D deficiency and rickets : screening and treatment, practical aspects for clinicians]. / Carence en vitamine D et rachitisme : dépistage et traitement, aspects pratiques pour le clinicien.

Vitamin D deficiency is increasing in Switzerland. If cases of rickets are scarce, pediatricians are often dealing with patients presenting vitamin D deficiency. The increase in vitamin D deficiency is certainly due to modification of life habits in recent decades. Clinical presentation varies according to age and severity of deficit. Treatments differ based on the level of vitamin D deficiency and symptoms. Vitamin D deficiency rickets is the most common cause of rickets and is predominantly seen in patients with risks factors They are other types of rickets like pseudo-vitamin D deficiency and hypophosphatemic rickets that the clinician needs to recognize. In which situation should the clinician suspect vitamin D deficiency or rickets ? Different types of rickets and practical aspects of treatment are reviewed in this article.

Le déficit en vitamine D est en augmentation en Suisse. Si les cas de rachitisme restent peu fréquents, les pédiatres sont souvent confrontés à un déficit en vitamine D. Cette augmentation est en lien avec les changements des habitudes de vie sur les dernières décennies. Le déficit en vitamine D se présente sous plusieurs formes et le traitement varie en fonction du degré du déficit et des symptômes. Le rachitisme carentiel est la forme la plus fréquente de rachitisme et concerne principalement les populations avec des facteurs de risque. Le diagnostic différentiel comprend le rachitisme pseudo-carentiel et le rachitisme hypophosphatémique. Quand et chez qui le praticien doit-il suspecter un déficit en vitamine D ou un rachitisme ? Le diagnostic différentiel et les aspects pratiques du traitement sont présentés dans cet article.

Can school health check-ups serve as screening tool for growth anomalies and obesity in children?

OBJECTIVE: To evaluate the prevalence of growth disorders and obesity in schoolchildren and determine whether school health check-ups are effective in their screening. Subjects-methods: Analysis of anonymized growth and body mass index (BMI) data from 2887 children attending the 3rd grade from 2008 to 2009 after selection of 75 elementary schools in Paris. RESULTS: Linear growth velocity was abnormal in 198 children. Height and weight were above the French reference values (+ 0.9 ± 1.2 SD and + 1 ± 1.7 SD, respectively). BMI was higher, compared to reference values (+ 0.4 ± 1.4 SD). At their last check-up, 20.9% of children had a BMI > + 2 SD. CONCLUSIONS: School health check-ups constitute a good screening tool for growth and obesity. However, further work is needed to determine the most effective modality. The reference values currently used in France are no longer suitable and new reference charts need to be established. The high prevalence of obesity in schoolchildren remains a public health challenge.

Natural history and management of congenital hypothyroidism with in situ thyroid gland.

BACKGROUND/OBJECTIVE: Normally sited glands account for increasing congenital hypothyroidism (CH). Mechanisms often remain unknown. To report the incidence of CH with in situ thyroid gland (ISTG) and describe the natural history of the disease without known etiology. METHOD: Clinical, biochemical and imaging data at diagnosis were retrospectively analyzed in 285 children positively screened for CH in Ile-de-France between 2005 and 2008. If treatment was discontinued, management of hormonal substitution and follow-up of biochemical thyroid function was performed. RESULTS: 93 full-term CH neonates displayed ISTG (40.6%), including 50 with unexplained mechanism. Follow-up data were available in 32 of them. Therapy was withdrawn from 20 children at a median age of 23.5 months (6-66), among whom 18 remained still untreated over a median duration of 15.3 months (4.4-29.6). In 11 children, levothyroxine (L-T4) dosage was increased over time to maintain biochemical euthyroidism. No statistical differences in initial TSH or FT4 levels, iodine status or birth weight were found between children with transient and permanent hypothyroidism. CONCLUSION: Withdrawal of L-T4 substitution was feasible in 56.2% of full-term children with CH with ISTG but unexplained mechanism, emphasizing the need for systematic therapy withdrawal. However, further studies are warranted to standardize withdrawal protocol.