RESUMO
Polycystic kidney disease (PKD) is the most prevalent inherited kidney disease. The disease is usually asymptomatic until adulthood. End-stage renal disease occurs generally after the age of 55 years, with a large inter-individual variability. Renal cyst formation begins early in life, and animal models have shown that treatments able to prevent the cyst growth slow down the renal function decline. A treatment by tolvaptan is currently used in adults to decelerate PKD progression. Until now there is no consensus about the appropriate time to screen for PKD in children. However, these scientific progresses raise the interest of determining early (i.e. pediatric) predictive markers of renal function decline.
La polykystose rénale autosomique dominante (PRAD) est la maladie rénale génétique la plus fréquente. Le développement insidieusement progressif des kystes rénaux fait que la PRAD est, le plus souvent, asymptomatique jusqu'à l'âge adulte, mais la croissance kystique survient très précocement. L'insuffisance rénale terminale survient, généralement, après l'âge de 55 ans, avec, cependant, une grande variabilité interindividuelle. Les modèles animaux montrent que les traitements ralentissant la croissance du volume rénal freinent parallèlement le déclin de la fonction rénale. Par ailleurs, un traitement récemment utilisé chez l'adulte (le tolvaptan) permet de ralentir la progression de la PRAD. A ce jour, il n'y a pas de consensus sur l'âge de dépistage de la PRAD chez les enfants. Toutefois, les récentes avancées scientifiques suggèrent l'intérêt de déterminer des marqueurs prédictifs précoces, y compris pédiatriques, du déclin de la fonction rénale.
Assuntos
Falência Renal Crônica , Rim Policístico Autossômico Dominante , Adulto , Animais , Criança , Progressão da Doença , Humanos , Rim , Rim Policístico Autossômico Dominante/diagnóstico , Rim Policístico Autossômico Dominante/tratamento farmacológico , TolvaptanRESUMO
Compression of the left renal vein between the aorta and the superior mesenteric artery causes a physiological condition, the so-called nutcracker phenomenon, but it can sometimes lead to left venous hypertension, or "nutcracker syndrome". Classical manifestations of which are an association of left flank pain, unilateral proteinuria and unilateral hematuria, without renal impairment. We report an atypical association of nutcracker syndrome with IgM nephropathy.
Assuntos
Dor Abdominal/diagnóstico , Doenças Vasculares Periféricas/diagnóstico , Veias Renais/patologia , Aorta Abdominal/patologia , Criança , Constrição Patológica/diagnóstico , Feminino , Seguimentos , Humanos , Hipertensão Renal/diagnóstico , Artérias Mesentéricas/patologia , RecidivaRESUMO
Retrocaval ureter is an uncommon abnormality of the inferior vena cava, which is rarely detected in the child due to its non-specific symptomatology. Despite a varying state of severity, chronic ureteral obstruction generally leads to the deterioration of renal function during adulthood. Treatment depends on the symptomatology in question, and surgical management should be as conservative as possible.
