Malformaciones renales
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Revisión selectiva sobre malformaciones renales, formas de presentación, orientación diagnóstica y tratamiento
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Malformaciones renales
- 6. Outcome of isolated antenatal hydronephrosis: a systematic review and meta-analysis Grignon 1986 SFU 1993 Fee008d
- 7. Antenatal Hydronephrosis as a Predictor of Postnatal Outcome: A Meta-analysis
- 10. Current management of infants with fetal renal pelvis dilation
- 11. Outcome of isolated antenatal hydronephrosis: a prospective cohort study Uropatía 18% 40% 88% Cirugía 0 5% 56%
- 13. Survival probabilities by CAKUT category,
Editor's Notes
- Purpose of review— Antenatally detected renal abnormalities are frequently encountered. Recommended postnatal evaluation of these infants has evolved to minimize invasive testing while maximizing detection of significant abnormalities. Recent findings— There is a low rate of detectable renal abnormalities in infants with a normal postnatal sonogram at 4–6 weeks of age. Routine prophylactic antibiotics are not indicated in infants with isolated antenatal hydronephrosis. Infants with a multicystic dysplastic kidney and a normal contralateral kidney on renal ultrasound do not require further evaluation. Parents of these children should be counseled on symptoms of urinary tract infections to allow prompt diagnosis. Summary— All infants with abnormalities on antenatal sonogram should undergo postnatal evaluation with a sonogram after birth and at 4–6 weeks of age. Further evaluation can be safely limited when the postnatal sonogram is normal at 6 weeks of age. Antenatal renal abnormalities There is a variety of renal abnormalities that may be detected on antenatal sonogram. The most common renal abnormality is hydronephrosis (also known as renal pelvic dilatation) with an incidence estimated between 0.5 and 1%. Two grading systems exist for fetal hydronephrosis – the anterior posterior diameter (APD) and the Society for Fetal Urology (SFU) grade. The majority of studies use the APD, but the normal values for APD are not universally agreed upon. Many studies use an APD greater than or equal to 5 mm at any gestational age as abnormal, necessitating postnatal evaluation.
- Introducción Varios trastornos que se presentan durante la vida fetal o en el período neonatal son malformaciones congénitas o parte de un trastorno hereditario. Las malformaciones suelen ser esporádicas, a menudo con una patogenia mal definida; las lesiones heredadas, por el contrario, con frecuencia tienen patrones claros de herencia autosómica dominante como recesivo y en muchos casos, el locus del gen anormal y la proteína anormal asociada han sido identificados
- Among a recent large cohort of patients for whom prenatal diagnosis was available, the early mortality due to ARPKD was slightly more than 25%, primarily as a result of respiratory failure and sepsis. A total of 41% of patients required mechanical ventilation after birth, and almost 12% of the survivors developed chronic lung disease. Longer term, 42% developed chronic renal insufficiency, and more than 25% of patients manifested slowed or delayed growth in infancy and early childhood, related to poor renal and pulmonary function. Importantly, the median age of the patients was 5.4 years, demonstrating that survival at least through childhood is possible with this disease. Hyponatremia occurred in a large percentage of affected patients, particularly during the neonatal period. Severe hypertension, the most common comorbidity, developed in about 65% of patients, and in almost all those who had hyponatremia. The ultimate result is severe hypertension but with low plasma renin concentrations. Only about 15% of patients in the modern cohort developed periportal fibrosis and portal hypertension, Low-set ears, Posteriorly rotated ears, Hypertelorism, Prominent epicanthal folds, Flattened nasal bridge, Micrognathia, Short neck, Redundant skin, Pulmonary hypoplasia, Orthopedic complications, Vertebral anomalies, Talipes equinovarus, Bowing of legs, Limb contractures, Wide, broad hands, Hip dislocation, Abnormal genitalia
- Congenital anomalies of the kidney and urinary tract (CAKUT) are detected frequently in up to 1% of newborns, and nowadays diagnosis is often established before birth by fetal ultrasound. Bilateral renal disease with oligohydramnios, however, indicates significant global fetal renal dysfunction and is a risk factor for the development of pulmonary hypoplasia
- Our objective was to review the current literature to determine whether the degree of antenatal hydronephrosis and related antenatal ultrasound findings are associated with postnatal outcome. The risk of any postnatal pathology per degree of antenatal hydronephrosis was 11.9% for mild, 45.1% for moderate, and 88.3% for severe. There was a significant increase in risk per increasing degree of hydronephrosis. The risk of vesicoureteral reflux was similar for all degrees of antenatal hydronephrosis. Moderate and severe antenatal hydronephrosis have a significant risk of postnatal pathology, indicating that comprehensive postnatal diagnostic management should be performed.
- We determined the risk of any pathology (UPJ, VUR, posterior urethral valves, ureteral obstruction, and other) for each degree of ANH. The overall risk of any pathology was 11.9% (95% confidence interval [CI]: 4.5–28.0) for mild ANH, 45.1% (95% CI: 25.3–66.6) for moderate ANH, and 88.3% (95% CI: 53.7–98.0) for severe ANH. The risk of postnatal pathology rose significantly with increasing degree of ANH ( P .001).
- Antenatal renal abnormalities There is a variety of renal abnormalities that may be detected on antenatal sonogram. The most common renal abnormality is hydronephrosis (also known as renal pelvic dilatation) with an incidence estimated between 0.5 and 1%. Two grading systems exist for fetal hydronephrosis – the anterior posterior diameter (APD) and the Society for Fetal Urology (SFU) grade. The majority of studies use the APD, but the normal values for APD are not universally agreed upon. The likelihood of significant renal abnormality correlates with the severity of APD dilatation. A meta-analysis of 17 studies reported the risk of renal abnormality for three classifications of antenatal hydronephrosis: mild, moderate, and severe. Mild hydronephrosis (APD ≤7 mm in the second or ≤9 mm in the third trimester) had an 11.9% risk of postnatal abnormality. The risk of postnatal abnormality increased to 45.1% in the moderate hydronephrosis group (APD 7–10 mm in the second or 9–15 mm in the third trimester), and the risk further increased to 88.3% in the severe group (APD ≥10 mm in the second or ≥15 mm in the third trimester). The probability of ureteropelvic junction (UPJ) obstruction increased with increasing APD, and there was no association of vesicoureteral reflux (VUR) with APD measurement. Lee RS, Cendron M, Kinnamon DD, Nguyen HT. Antenatal hydronephrosis as a predictor of postnatal outcome: a meta-analysis. Pediatrics 2006;118:586–593. [PubMed: 16882811] Therefore, routine VCUG is not indicated in infants with normal postnatal ultrasounds after birth and again at 4–6 weeks of age. A `normal‘ ultrasound includes no hydronephrosis, no ureteral dilatation, normal-sized kidneys, no renal cortical abnormalities, and no bladder abnormalities.
- Postnatal evaluation The postnatal evaluation of an infant with antenatally detected renal abnormalities should always begin with a physical examination. A palpable abdominal mass may be detected in an infant with a multicystic dysplastic kidney (MCDK), UPJ obstruction, or autosomal recessive polycystic kidney disease (ARPKD). Absent abdominal wall musculature with bilateral undescended testicles suggests a diagnosis of prune belly syndrome (PBS). Infants with a palpable bladder may have posterior urethral valves (PUV) or urethral atresia or stricture. Features of Potter sequence secondary to low amniotic fluid volume may be present. Infants with severe bilateral hydronephrosis or severe unilateral hydronephrosis in a single functioning kidney warrant immediate postnatal evaluation with a renal sonogram and voiding cystourethrogram (VCUG). Infants with persistent hydronephrosis on postnatal ultrasound (APD >7 mm) should undergo evaluation with a VCUG. Fluoroscopic VCUG is recommended as it provides visualization of the anatomy of the bladder and urinary system, allowing the diagnosis of PUV, ureteroceles, and other anatomic abnormalities that would be missed on a nuclear VCUG. If the VCUG is normal and the ultrasound shows a pelvic dilatation >10 mm, diuretic renography should be done to assess for UPJ or ureterovesical (UVJ) obstruction
- Diagnostic tools used by the responders for morphological and functional evaluation of infants with antenatal pelvic dilatation (DMSA dimercaptosuccinic acid, MAG3 mercaptoacetyl-triglycine) All responders perform renal ultrasound examinations after birth. Postnatal renal pelvis dilatation was considered abnormal if the anteroposterior diameter was >11 + 1.9 mm by the pediatric urologists and >9+2.9 mm by the pediatric nephrologists (P=0.003). Pediatric urologists were more likely than nephrologists to recommend routine voiding cystourethrography [41% versus 20% (P=0.04)]. Mercaptoacetyl-triglycine renography was the most routinely used tool to achieve functional evaluation during follow-up among the responders. Pediatric urologists were more likely to recommend surgical treatment in dilated kidneys with initial function <40% In conclusion, pediatric urologists had significantly higher thresholds for the detection of prenatal and neonatal renal pelvis dilatation. They also more frequently recommended routine voiding cystourethrography and surgical therapy of dilated kidneys with low function than pediatric nephrologists. La variabilidad en la actitud probablemente se deba a la ausencia de guías basadas en ensayos controlados prospectivos
- The purpose of this study was to report the outcome of infants with antenatal hydronephrosis. Between May 1999 and June 2006, all patients diagnosed with isolated fetal renal pelvic dilatation (RPD) were prospectively followed. The events of interest were: presence of uropathy, need for surgical intervention, RPD resolution, urinary tract infection (UTI), and hypertension. RPD was classified as mild (5–9.9 mm), moderate (10–14.9 mm) or severe (≥15 mm). Resultados. Un total de 192 pacientes fueron incluidos en el análisis; 114 fueron asignados al grupo de hallazgos no significativos (59,4%) y 78 al grupo de uropatía significativa (40,6%). De los 89 pacientes con hidronefrosis leve, 16 (18%) presentaron uropatía; siete (7,8%) presentaron infección urinaria durante el seguimiento. La función renal, presión arterial se encontraban en rango normal durante el último control De los 43 lactantes con hidronefrosis severa, 24 (56%) necesitaron cirugía, mientras solo tres (5%) de 60 con hidronefrosis moderada; en contraste ninguno de los 89 con hidronefrosis leve necesitaron procedimientos quirúrgicos Los hallazgos sugieren que, en contraste con hidronefrosis moderada/severa, los lactantes con hidronefrosis leve no requieren procedimientos diagnósticos invasivos pero necesitan vigilancia clínica para infección urinaria e hidronefrosis progresiva
- In this paper, we report longitudinal data on a cohort of 312 children referred for different renal and urinary tract anomalies who were followed until the age of 30 years. The basic criteria for enrollment included the presence of CAKUT characterized by a defect of the number or size of at least one kidney, excluding cases with isolated ureteric anomalies such as vesicoureteral reflux and duplicated collecting systems.
- Durante un período de 10 años, una anomalía renal fue sospechada en 125 fetos, incidencia de 5/1000. Nacimientos vivos 107 (0,856). Muerte fetal en 14 embarazos asociados con oligoamnios y vejiga fetal no visible. Nueve fetos mostraron hiperecogenicidad parenquimatosa aislada