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1.
Pediatr Nephrol ; 39(6): 1783-1789, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38197956

RESUMO

BACKGROUND: The prevalence of Müllerian anomalies (MA) among patients with congenital solitary functioning kidney (SFK) is not well defined. A delay in diagnosis of obstructive MA can increase the risk of poor clinical outcomes. This study describes the prevalence of MA in patients with congenital SFK. METHODS: A retrospective review was performed of patients within the Nationwide Children's Hospital system with ICD9 or ICD10 diagnostic codes for congenital SFK defined as either unilateral renal agenesis (URA) or multicystic dysplastic kidney (MCDK) and confirmed by chart review. Patients with complex urogenital pathology were excluded. Renal anomaly, MA, reason for and type of pelvic evaluation, and age of diagnosis of anomalies were evaluated. RESULTS: Congenital SFK occurred in 431 girls due to URA (209) or MCDK (222). Pelvic evaluation, most commonly by ultrasound for evaluation of abdominal pain or dysmenorrhea, occurred in 115 patients leading to MA diagnosis in 60 instances. Among 221 patients ages 10 years and older, 104 underwent pelvic evaluation and 52 were diagnosed with an MA of which 20 were obstructive. Isolated uterine or combined uterine and vaginal anomalies were the most common MA. MA were five-fold more common in patients with URA compared to MCDK. In 75% of patients, the SFK was diagnosed prior to the MA. CONCLUSIONS: The prevalence of MA in patients with congenital SFK was 24% among those age 10 years or older, and 38% were obstructive. This justifies routine screening pelvic ultrasound in girls with congenital SFK to improve early diagnosis.


Assuntos
Nefropatias , Rim Displásico Multicístico , Rim Único , Sistema Urinário , Criança , Feminino , Humanos , Rim Único/epidemiologia , Rim/anormalidades , Nefropatias/diagnóstico , Estudos Retrospectivos
2.
BMC Nephrol ; 23(1): 70, 2022 02 21.
Artigo em Inglês | MEDLINE | ID: mdl-35189836

RESUMO

BACKGROUND: Given the importance of the function of the remnant kidney in children with unilateral renal agenesis and the significance of timely diagnosis and treatment of reflux nephropathy to prevent further damage to the remaining kidney, we aimed to determine the prevalence of reflux nephropathy in this subgroup of pediatric patients. METHODS: In general, 274 children referred to pediatric nephrologists in different parts of Iran were evaluated, of whom 199 had solitary kidney and were included in this cross-sectional study. The reasons for referral included urinary tract infection (UTI), abnormal renal ultrasonography, being symptomatic, and incidental screening. Demographic characteristics, including age and gender were recorded. History of UTI and presence of vesicoureteral reflux (VUR) were evaluated. RESULTS: Of the 274 children evaluated in this study with the mean age (SD) of 4.71 (4.24) years, 199 (72.6%) had solitary kidney. Among these, 118 (59.3%) were male and 81 (60.7%) were female, 21.1% had a history of UTI, and VUR was present in 23.1%. The most common cause of referral was abnormal renal ultrasonography (40.2%), followed by incidental screening (21.1%), being symptomatic (14.1%), and UTI (5.5%). In 116 children (58.3%), the right kidneys and in 83 (41.7%) the left kidneys were absent. Besides, 14.6% of the participants had consanguineous parents and 3% had a family history of solitary kidney. Upon DMSA scan, the single kidney was scarred in 13.1%, of which only 7.5% were associated with VUR. In addition, proteinuria and hematuria were observed in 6.5% and 1.5% of children, respectively. CONCLUSIONS: The prevalence of reflux nephropathy was 7.5% in children with solitary kidney with a male predominance. Given the relatively high prevalence of reflux nephropathy in these children, screening for VUR in the remnant kidney appears to be essential in this population.


Assuntos
Rim Único/epidemiologia , Refluxo Vesicoureteral/epidemiologia , Pré-Escolar , Comorbidade , Estudos Transversais , Diagnóstico Precoce , Feminino , Humanos , Irã (Geográfico)/epidemiologia , Masculino , Prevalência , Rim Único/diagnóstico por imagem , Ultrassonografia , Refluxo Vesicoureteral/diagnóstico por imagem
4.
Pediatr Nephrol ; 36(11): 3673-3680, 2021 11.
Artigo em Inglês | MEDLINE | ID: mdl-33954810

RESUMO

BACKGROUND: Multicystic dysplastic kidney (MCDK) disease and unilateral renal agenesis (URA) are well-known causes of a solitary functioning kidney (SFK) and are associated with long-term kidney injury. The aims of this study were to characterize the natural history of SFK at our center, define the risk factors associated with chronic kidney injury, and identify distinguishing features between URA and MCDK that predict outcome. METHODS: This was a retrospective cohort study of 230 SFK patients. We compared MCDK (n=160) and URA (n=70) according to clinical features at diagnosis and kidney outcomes over follow-up. Univariate and multivariate binary regression analysis was used to determine independent risk factors for chronic kidney injury, defined as the composite outcome of hypertension, proteinuria, or chronic kidney disease (eGFR <60 mL/min/1.73m2). RESULTS: URA had a higher prevalence of comorbid genetic syndromes (15 vs. 6%, p=0.04), non-renal anomalies (39 vs. 11%, p<0.001), and congenital anomalies of the kidney and urinary tract (CAKUT) (51 vs. 26%, p<0.001) than MCDK. Over follow-up, URA experienced more hypertension (19 vs. 3%, p=0.002), proteinuria (12 vs. 3%, p=0.03), and the composite outcome (19 vs. 6%, p=0.003) than MCDK. Independent risk factors for chronic kidney injury included CAKUT (OR 5.01, p=0.002) and URA (OR 2.71, p=0.04). CONCLUSIONS: In our population, URA was more likely to have associated syndromes or anomalies, and to have worse outcomes over time than MCDK. URA diagnosis was an independent risk factor for chronic kidney injury. Our results will be used to develop a standardized clinical pathway for SFK management. A higher resolution version of the Graphical abstract is available as Supplementary information.


Assuntos
Anormalidades Congênitas , Nefropatias/congênito , Rim/anormalidades , Rim Displásico Multicístico , Rim Único , Anormalidades Congênitas/epidemiologia , Seguimentos , Humanos , Nefropatias/epidemiologia , Rim Displásico Multicístico/epidemiologia , Estudos Retrospectivos , Fatores de Risco , Rim Único/epidemiologia
5.
Pediatr Nephrol ; 36(9): 2847-2855, 2021 09.
Artigo em Inglês | MEDLINE | ID: mdl-33723672

RESUMO

BACKGROUND: Children born with unilateral renal agenesis (URA) are thought to have a risk of developing hypertension, proteinuria, and progressive chronic kidney disease (CKD). The present study aimed to evaluate the long-term prognosis and clinical characteristics of children with URA. METHODS: The study included 171 patients aged < 18 years diagnosed as URA who were followed-up for ≥ 1 year and 121 healthy controls matched for age, gender, and BMI. RESULTS: Median age at diagnosis was 2 years (IQR: 1 month-16 years) and the incidence of URA in males (65.4%) was higher than in females. Among the patients, 21 (12.2%) had other urinary system anomalies. It was noted that 2.3% of the patients had proteinuria, 15.2% had hyperfiltration, and 2.9% had CKD. Hypertension based on ambulatory blood pressure monitoring (ABPM) was diagnosed in 18 (10.5%) of the patients, of whom 10 had masked hypertension. Diastolic blood pressure in the URA patients was significantly higher than in the healthy controls. The incidence of hypertension and CKD was significantly higher in the patients with other urinary system anomalies. CONCLUSIONS: Patients with a single functional kidney should be periodically evaluated throughout their lifetime for urine protein, blood pressure, and kidney functions. The most remarkable finding of this study is the importance of the use of ABPM for evaluating blood pressure in pediatric URA patients, especially for the detection of masked hypertension and the non-dipper phenomenon, which cannot be achieved with office blood pressure measurement.


Assuntos
Rim Único , Adolescente , Pressão Sanguínea , Monitorização Ambulatorial da Pressão Arterial , Criança , Feminino , Humanos , Hipertensão/epidemiologia , Masculino , Hipertensão Mascarada , Proteinúria/diagnóstico , Proteinúria/epidemiologia , Proteinúria/etiologia , Insuficiência Renal Crônica/diagnóstico , Insuficiência Renal Crônica/epidemiologia , Estudos Retrospectivos , Rim Único/complicações , Rim Único/diagnóstico , Rim Único/epidemiologia , Anormalidades Urogenitais
6.
Pediatr Nephrol ; 36(9): 2837-2845, 2021 09.
Artigo em Inglês | MEDLINE | ID: mdl-33619660

RESUMO

BACKGROUND: We investigated the risk of kidney injury among adolescents with and without a congenital single functioning kidney (SFK). METHODS: This retrospective study is based on a medical evaluation database of 17-year-old Israeli conscripts, born during 1989-1999. Those with congenital SFK diagnosis, verified by a pediatric nephrologist's review of the original military medical committee classifications, were compared to the rest of the cohort. Kidney injury (KI) was defined as proteinuria, high blood pressure (BP), or estimated glomerular filtration rate (eGFR) < 90 ml/min/1.73 m2 prior to army recruitment. Risk factors for KI were examined using logistic regression. RESULTS: Of 979,630 screened candidates, 353 were diagnosed with SFK. The yearly incidence of SFK gradually increased in the first years of the study, reaching a plateau in 1995 (5.5 ± 1.2/10,000 births/year). The male to female ratio was 2.7:1. Concomitant genital malformations were documented in 5.5% of those with SFK. KI was more prevalent in the SFK than the control group (42.2% vs. 23.5%, p < 0.001). All three components of KI were more common in the SFK than the control group: high BP (31.7% vs. 23.1%, p < 0.001), proteinuria (18.2% vs. 0.4%, p < 0.001), and eGFR <90 ml/min/1.73m2 (12.0% vs 0.1%, p < 0.001). Multivariate analysis of the SFK group revealed associations of higher mean BMI, male sex, and smaller ultrasonographic kidney length with KI. CONCLUSIONS: This large population-based study documents a significant risk for KI among adolescents with SFK. Obesity represents a major modifiable risk factor for KI, implicating the need for closer follow-up in this group during childhood.


Assuntos
Rim Único , Adolescente , Feminino , Taxa de Filtração Glomerular , Humanos , Hipertensão , Rim , Masculino , Prognóstico , Proteinúria/epidemiologia , Estudos Retrospectivos , Rim Único/epidemiologia
7.
Rev. cuba. med ; 60(supl.1): e2505, 2021. graf
Artigo em Espanhol | CUMED, LILACS | ID: biblio-1408966

RESUMO

Introducción: Los quistes enterogénicos son malformaciones producidas durante el período de diferenciación y desarrollo embriológico del intestino primitivo. Objetivo: Presentar una malformación digestiva infrecuente en la edad adulta. Caso clínico: Paciente de 58 años que acude por dolor abdominal y aumento de volumen en hipogastrio. Al examen físico se palpa masa en hipogastrio de 15 cm de diámetro. La tomografía axial computarizada simple y contrastada mostró una masa tumoral en la excavación pélvica hipodensa con densidad de 18 UH, pared gruesa, de 2 cm, que realza con el contraste y agenesia renal derecha. El estudio anatomo-patológico informa quiste enterogénico. Discusión: La clínica de las duplicaciones intestinales es inespecífica, el dolor abdominal recurrente y la presencia de masa abdominal, son frecuentes. El diagnóstico preoperatorio de las duplicaciones intestinales es infrecuente, depende de la disponibilidad de estudios de imagen y la sospecha clínica. Es infrecuente la asociación de quiste enterogénico y agenesia renal. Conclusión: Se presenta una paciente masculino con una masa pélvica de etiología dudosa y agenesia renal derecha, en el que se diagnosticó un quiste enterogénico. La asociación de quiste enterogénico y agenesia renal es una malformación rara en el adulto(AU)


Introduction: Enterogenic cysts are malformations produced during the period of differentiation and embryological development of the primitive intestine. Objective: To report an rare digestive malformation in adulthood. Clinical case report: A 58-year-old patient came for abdominal pain and increased volume in the hypogastrium. On physical examination, a 15-cm diameter mass was palpated in the hypogastrium. Simple and contrast computerized axial tomography showed a tumor mass in the hypodense pelvic excavation with a density of 18 HU, a thick wall of 2 cm, which was enhanced with contrast and right renal agenesis. The anatomo-pathological study reports an enterogenic cyst. Discussion: The symptoms of intestinal duplications are nonspecific, recurrent abdominal pain and the presence of an abdominal mass are frequent. The preoperative diagnosis of intestinal duplications is occasional, it depends on the availability of imaging studies and clinical suspicion. The association of enterogenic cyst and renal agenesis is uncommon. Conclusion: A male patient is reported, with a pelvic mass of doubtful etiology and right renal agenesis, in whom an enterogenic cyst was diagnosed. The association of enterogenic cyst and renal agenesis is a rare malformation in adults(AU)


Assuntos
Humanos , Feminino , Cistos Ovarianos/diagnóstico por imagem , Tomografia Computadorizada de Emissão de Fóton Único/métodos , Rim Único/epidemiologia
8.
Int Braz J Urol ; 45(6): 1227-1237, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31808412

RESUMO

INTRODUCTION: Children with a solitary post-nephrectomy kidney (SNK) are at potential risk of developing kidney disease later in life. In response to the global decline in the number of nephrons, adaptive mechanisms lead to renal injury. The aim of this study was to determine the prevalence and time of onset of high blood pressure (HBP), proteinuria, glomerular filtration rate (GFR) disruption and renal tubular acidosis (RTA) in children with SNK. MATERIALS AND METHODS: After obtaining the approval from our institution's ethics committee, we reviewed the medical records of patients under 18 years of age who underwent unilateral nephrectomy between January 2005 and December 2015 in three university hospitals. RESULTS: We identifi ed 43 patients, 35 (81.4%) cases of unilateral nephrectomy (UNP) were due to a non-oncologic pathology and Wilm's tumor was identified in 8 (18.6%) cases. In patients with non-oncologic disease, 9.3% developed de novo hypertension, with an average time of onset of 7.1 years, 25% developed proteinuria de novo, with an average time of onset of 2.2 years. For GFR, 21.8% presented deterioration of the GFR in an average time of 3.4 years. Ten (43.5%) patients developed some type of de novo renal injury after UNP. Patients with oncolo-gic disease developed the conditions slowly and none of them developed proteinuria. CONCLUSIONS: Taking into account the high rate of long term postoperative renal injury, it can be considered that nephrectomy does not prevent this disease. The follow-up of children with SNK requires a multidisciplinary approach and long-term surveillance to detect renal injury.


Assuntos
Nefrectomia/efeitos adversos , Rim Único/epidemiologia , Rim Único/fisiopatologia , Acidose Tubular Renal/epidemiologia , Acidose Tubular Renal/fisiopatologia , Adolescente , Idade de Início , Criança , Pré-Escolar , Colômbia/epidemiologia , Feminino , Seguimentos , Taxa de Filtração Glomerular/fisiologia , Humanos , Hipertensão/epidemiologia , Hipertensão/fisiopatologia , Lactente , Recém-Nascido , Masculino , Período Pós-Operatório , Prevalência , Prognóstico , Proteinúria/epidemiologia , Proteinúria/fisiopatologia , Insuficiência Renal Crônica/epidemiologia , Insuficiência Renal Crônica/fisiopatologia , Estudos Retrospectivos , Fatores de Risco
9.
Int. braz. j. urol ; 45(6): 1227-1237, Nov.-Dec. 2019. tab, graf
Artigo em Inglês | LILACS | ID: biblio-1056325

RESUMO

ABSTRACT Introduction: Children with a solitary post-nephrectomy kidney (SNK) are at potential risk of developing kidney disease later in life. In response to the global decline in the number of nephrons, adaptive mechanisms lead to renal injury. The aim of this study was to determine the prevalence and time of onset of high blood pressure (HBP), proteinuria, glomerular filtration rate (GFR) disruption and renal tubular acidosis (RTA) in children with SNK. Materials and methods: After obtaining the approval from our institution's ethics committee, we reviewed the medical records of patients under 18 years of age who underwent unilateral nephrectomy between January 2005 and December 2015 in three university hospitals. Results: We identified 43 patients, 35 (81.4%) cases of unilateral nephrectomy (UNP) were due to a non-oncologic pathology and Wilm's tumor was identified in 8 (18.6%) cases. In patients with non-oncologic disease, 9.3% developed de novo hypertension, with an average time of onset of 7.1 years, 25% developed proteinuria de novo, with an average time of onset of 2.2 years. For GFR, 21.8% presented deterioration of the GFR in an average time of 3.4 years. Ten (43.5%) patients developed some type of de novo renal injury after UNP. Patients with oncologic disease developed the conditions slowly and none of them developed proteinuria. Conclusions: Taking into account the high rate of long term postoperative renal injury, it can be considered that nephrectomy does not prevent this disease. The follow-up of children with SNK requires a multidisciplinary approach and long-term surveillance to detect renal injury.


Assuntos
Humanos , Masculino , Feminino , Recém-Nascido , Lactente , Pré-Escolar , Criança , Adolescente , Rim Único/fisiopatologia , Rim Único/epidemiologia , Nefrectomia/efeitos adversos , Período Pós-Operatório , Prognóstico , Proteinúria/fisiopatologia , Proteinúria/epidemiologia , Acidose Tubular Renal/fisiopatologia , Acidose Tubular Renal/epidemiologia , Prevalência , Estudos Retrospectivos , Fatores de Risco , Seguimentos , Colômbia/epidemiologia , Idade de Início , Insuficiência Renal Crônica/fisiopatologia , Insuficiência Renal Crônica/epidemiologia , Taxa de Filtração Glomerular/fisiologia , Hipertensão/fisiopatologia , Hipertensão/epidemiologia
10.
Eur J Epidemiol ; 34(9): 879-888, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31025238

RESUMO

The renal outcome of solitary kidney remains controversial. We examined the longitudinal association of congenital or acquired solitary kidney with the development of chronic kidney disease (CKD). A cohort study was performed involving 271,171 Korean men and women free of CKD at baseline who underwent a health screening program and who were followed annually or biennially for an average of 5.4 years. Solitary kidney was determined based on ultrasonographic findings. CKD was defined as an estimated glomerular filtration rate of < 60 ml/min/1.73 m2 and/or the presence of proteinuria in two or more consecutive visits. During 1,472,519.6 person-years of follow-up, 2989 participants developed CKD (incidence rate: 2.0 per 1000 person-years). After adjustment for potential confounders, the aHR (95% CIs) for incident CKD comparing solitary kidney to the control was 3.26 (1.63-6.54). In analyses of cause-specific solitary kidney, aHR (95% CIs) for CKD comparing unilateral nephrectomy and congenital solitary kidney to the control were 6.18 (2.31-16.49) and 2.22 (0.83-5.92), respectively. The association between solitary kidney and CKD was stronger in men. Having a solitary kidney was independently associated with an increased risk of CKD development. Therefore, preventive strategies for reducing the risk of CKD are required in individuals with a solitary kidney.


Assuntos
Taxa de Filtração Glomerular , Rim/fisiopatologia , Nefrectomia/estatística & dados numéricos , Insuficiência Renal Crônica/epidemiologia , Insuficiência Renal Crônica/fisiopatologia , Rim Único/diagnóstico por imagem , Adulto , Estudos de Coortes , Feminino , Humanos , Incidência , Estimativa de Kaplan-Meier , Rim/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Proteinúria/diagnóstico , Proteinúria/epidemiologia , Proteinúria/fisiopatologia , Insuficiência Renal Crônica/diagnóstico , República da Coreia/epidemiologia , Fatores de Risco , Rim Único/epidemiologia , Fatores de Tempo , Ultrassonografia
11.
Clin Exp Nephrol ; 23(6): 792-798, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30734167

RESUMO

BACKGROUND: We aimed to investigate the clinical characteristics of Chinese patients with unilateral renal agenesis. METHODS: We enrolled patients with unilateral renal agenesis diagnosed radiologically at the Department of Nephrology from January 2008 to January 2016. Patients with a solitary kidney due to nephrectomy or renal atrophy due to secondary factors were excluded. Clinical data were recorded and analyzed. RESULTS: In this study, 118 Chinese patients with unilateral renal agenesis were recruited, and the gender ratio (male/female) was 1.11:1. A total of 14 (11.9%) patients had additional abnormalities, 15 (12.7%) had a family history, and 30 (25.4%) presented with renal insufficiency. Kidney length, serum creatinine level and estimated glomerular filtration rate were significantly different between patients with and without family history (P < 0.05, respectively). Gender showed a significant difference between patients with and without other abnormalities. Kidney length and the incidence of proteinuria, hematuria, hypertension, and hyperuricemia were significantly different between patients with and without renal insufficiency. Logistic regression analysis revealed that family history was associated with severe renal failure (OR = 7.11, 95% CI 1.52-33.25). CONCLUSION: Renal insufficiency is common in patients with unilateral renal agenesis. Patients with renal insufficiency have shorter kidney lengths and a higher incidence of proteinuria, hypertension, hematuria, and hyperuricemia. Family history is considered a risk factor for severe renal failure.


Assuntos
Rim Único/epidemiologia , Adolescente , Adulto , Idoso , China/epidemiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Insuficiência Renal/etiologia , Rim Único/complicações , Adulto Jovem
12.
Med. clín (Ed. impr.) ; 152(1): 19-21, ene. 2019. tab
Artigo em Espanhol | IBECS | ID: ibc-181669

RESUMO

Introducción: La diabetes tipo MODY engloba un grupo heterogéneo de formas monogénicas de diabetes de baja prevalencia. Pacientes y métodos: Presentamos un caso clínico con clínica cardinal, diabetes e insuficiencia renal sin acidosis, con antecedentes familiares de diabetes y padre monorreno. Resultados: Dado el aumento de obesidad y antecedentes familiares de diabetes en la población general, su sospecha diagnóstica no resulta fácil. Distinguir diabetes tipo MODY de DM1 y DM2 es crucial ya que el tratamiento óptimo y el riesgo de complicaciones varía con el defecto genético subyacente. Discusión: Un adecuado diagnóstico precisa de una historia clínica orientada y detallada, permitiendo una identificación más temprana de los miembros en riesgo de la familia y adecuar el tratamiento, ya que muchos de estos pacientes pueden ser tratados con éxito en monoterapia retirando la insulinoterapia innecesaria


Introduction: MODY diabetes encompasses heterogeneous group of monogenic forms of diabetes with low prevalence. It is not easily diagnosed because of the increase in obesity and family history of diabetes in the general population. Patients and methods: We present a clinical case with cardinal symptoms, diabetes,renal insufficiency with no acidosis and with a family history of diabetes and renal agenesis. Results: Distinguishing MODY diabetes from DM1 and DM2 is very important to ensure optimal treatment, and because the risk of complications depends on each genetic defect. A proper diagnosis needs a detailed medical history. Discussion: An earlier identification of family members at risk and a correct and individualised treatment could be possible. Many of these patients can be managed successfully in monotherapy without insulin therapy


Assuntos
Humanos , Masculino , Adulto , Rim Único/epidemiologia , Diabetes Mellitus/etiologia , Obesidade/epidemiologia , Insulina Glargina/administração & dosagem , Rim Único/complicações , Diabetes Mellitus/genética , Obesidade/complicações , Índice Glicêmico , Diagnóstico Diferencial
13.
Medicine (Baltimore) ; 97(32): e11819, 2018 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-30095653

RESUMO

To evaluate the prognostic significance of factors frequently associated with a reduction in renal mass, such as prematurity, low birth weight, and congenital anomalies of kidney and urinary tract (CAKUT), in patients with solitary functioning kidney (SFK) and investigate signs of early renal injury due to glomerular hyperfiltration damage or dysplasia in the remaining kidney.Retrospective observational study of congenital SFK diagnosed and followed at a tertiary care hospital over a period of 10 years in which 32,900 newborns underwent routine neonatal abdominal ultrasound screening. We analyzed age at diagnosis, sex, gestational age, anthropometric measurements at birth and prenatal and neonatal ultrasound findings, in addition to follow-up data corresponding to imaging findings (ultrasound, micturating cystourethrography, dimercaptosuccinic acid renal, and scintigraphy), ipsilateral CAKUT, compensatory hypertrophy, and renal injury in the form of albuminuria, blood pressure, and estimated glomerular filtration rate (eGFR).In total, 128 congenital SFK cases were detected (1 per 257 live births). Of these, 117 (91.4%) were diagnosed by neonatal ultrasound screening and 44.5% of these had been previously identified by prenatal ultrasound. Neonatal ultrasound had a specificity of 100% and a sensitivity of 92.1%. Forty-five patients (35.2%) had ipsilateral CAKUT and the most common type was urinary collecting system anomalies (75.5%). Over a median follow-up of 6.3 years (1-10 years), compensatory renal hypertrophy was observed in 81 patients (63.7%), most of whom had ipsilateral CAKUT (76.1% vs 56.6% of patients without ipsilateral CAKUT). Albuminuria and hypertension were observed in 3.12% and 5% of patients, respectively, and both were associated with ipsilateral CAKUT (P < .05). In addition, 75% of albuminuria cases (P = .031), 83.3% of hypertension cases (P = .004), and 100% of decreased eGFRcases (P = .031) were significantly associated with CAKUT (renal parenchymal anomaly category), being the strongest predictor of GFR the presence or absence of CAKUT.Neonatal ultrasound screening is useful for the early diagnosis of SFK. The presence of ipsilateral CAKUT should be evaluated in all patients with SFK as congenital anomalies of the renal parenchyma are associated with a poorer prognosis. Because morbidity from CAKUTs may not develop until adulthood, patients should be closely followed throughout life.


Assuntos
Insuficiência Renal Crônica/epidemiologia , Rim Único/diagnóstico , Rim Único/epidemiologia , Albuminúria/epidemiologia , Pesos e Medidas Corporais , Feminino , Idade Gestacional , Taxa de Filtração Glomerular , Humanos , Hipertensão/epidemiologia , Recém-Nascido , Masculino , Triagem Neonatal/métodos , Estudos Retrospectivos , Fatores de Risco , Sensibilidade e Especificidade , Fatores Sexuais , Rim Único/diagnóstico por imagem
14.
J Pediatr Urol ; 14(2): 144-149, 2018 04.
Artigo em Inglês | MEDLINE | ID: mdl-29459133

RESUMO

BACKGROUND: Mullerian anomalies have a known association with renal agenesis yet, to date, there are no formal recommendations for screening women with certain renal anomalies for associated genital tract disorders. OBJECTIVE: The objective of this study is to review current data regarding the association between renal and Mullerian anomalies, and propose screening recommendations. STUDY DESIGN: A comprehensive review of the literature was performed to identify relevant articles using the keywords "unilateral renal agenesis," "renal anomalies," and "Mullerian anomalies." RESULTS: Over 30% of patients with unilateral renal agenesis have an associated Mullerian anomaly. However, diagnosis is frequently delayed in this population until after menarche when complications of retrograde menstruation with obstructive anomalies lead to significant problems including endometriosis, pelvic inflammatory disease, and infertility. No clear guidelines exist for communication among the antenatal sonographer, the obstetrician, the parents, and the child's pediatrician, which creates a barrier to effective screening and follow-up. Further, no current guidelines exist for screening women with certain renal anomalies for Mullerian anomalies. DISCUSSION: The complications of Mullerian anomalies are easily preventable if identified early. We propose new guidelines for education and screening for Mullerian anomalies in patients with unilateral renal agenesis (URA) and multicystic dysplastic kidney (MCDK) to guide providers, patients, and parents on proper identification and management (Table). CONCLUSIONS: Screening young women with URA and MCDK for Mullerian anomalies has the potential to prevent long-term complications from untreated obstructive malformations. Identification of unilateral renal agenesis on antenatal ultrasound must be clearly articulated with parents and the child's pediatrician so that proper screening can be performed before menarche. Pelvic sonography is a low-cost, high-yield screening tool to identify these anomalies.


Assuntos
Anormalidades Congênitas/diagnóstico , Nefropatias/congênito , Nefropatias/prevenção & controle , Rim/anormalidades , Ductos Paramesonéfricos/anormalidades , Triagem Neonatal/métodos , Guias de Prática Clínica como Assunto , Rim Único/diagnóstico , Anormalidades Congênitas/epidemiologia , Diagnóstico Precoce , Feminino , Humanos , Recém-Nascido , Nefropatias/diagnóstico , Nefropatias/epidemiologia , Masculino , Ductos Paramesonéfricos/embriologia , Medição de Risco , Rim Único/epidemiologia
15.
Nefrologia (Engl Ed) ; 38(1): 73-78, 2018.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-28869042

RESUMO

BACKGROUND AND OBJECTIVES: Hyperuricemia plays a major role in the development and progression of chronic kidney disease (CKD). Many large observational studies have indicated that increased serum uric acid level predicts the development and progression of CKD in some population, however this hypothesis has not been yet studied in patients with reduced renal mass. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Retrospective study with a cohort of 324 patients with reduced renal mass from an outpatient basis, followed during 60 (36-98) months. Demographics variables, cardiovascular factors, concomitant medications, albuminuria and uric acid levels were recorded yearly. The primary endpoint was the annual fall of estimated glomerular filtration rate (eGFR) by MDRD-4. The sample was divided into three successive groups (A1: patients with fall of eGFR lower than median, A2: greater than median, B: without fall of eGFR). Factors associated and predictors of kidney function decline were analyzed. RESULTS: One hundred and seventy out of 324 patients suffered a fall of eGFR (group A), (median of fall -1.6ml/min/1.73m2/year (-3.0, -0.7)). Male gender, albuminuria>100mg/day and higher pulse pressure were associated to progression in our cohort (group A). Hyperuricemia was more frequent among patients with higher kidney disease progression (group A2) (33% vs 49%, p=0.04) when comparing to lower progression (group A1). Adjusted Cox regression models showed that hyperuricemia, pulse pressure and albuminuria were independent predictors of kidney disease progression (HR 1.67 (1.06-2.63), p=0.023; 1.02 (1.01-1.03), p=0.001 and HR: 2.14 (1.26-3.64), p=0.005, respectively). Kidney disease progression was higher in patients with unilateral renal atrophy or agenesis than nephrectomy (log rank: 7.433, p=0.006). CONCLUSIONS: Hyperuricemia is independently associated with kidney disease progression in patients with reduce functioning renal mass.


Assuntos
Hiperuricemia/etiologia , Insuficiência Renal Crônica/sangue , Idoso , Atrofia , Comorbidade , Diabetes Mellitus/epidemiologia , Progressão da Doença , Dislipidemias/epidemiologia , Feminino , Seguimentos , Taxa de Filtração Glomerular , Humanos , Hipertensão/epidemiologia , Hiperuricemia/epidemiologia , Rim/anormalidades , Rim/patologia , Masculino , Pessoa de Meia-Idade , Nefrectomia , Tamanho do Órgão , Complicações Pós-Operatórias/sangue , Insuficiência Renal Crônica/epidemiologia , Insuficiência Renal Crônica/patologia , Insuficiência Renal Crônica/fisiopatologia , Estudos Retrospectivos , Rim Único/sangue , Rim Único/epidemiologia
16.
Urolithiasis ; 46(3): 291-296, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-28555349

RESUMO

The objective of this study was to determine risk factors of hospital admission for treatment of complications after extracorporeal shock wave lithotripsy (SWL). The electronic files and images of all patients who underwent SWL for treatment of renal stones between January 2011 and December 2015 were retrospectively reviewed. All patients underwent SWL with the same electromagnetic lithotripter (Dornier Lithotripot S). The data of those who needed hospital admission for treatment of complications within 30 days after SWL were compared with patients who did not require hospital admission. Compared data included patients' demographics (age, gender, BMI, ASA score, and pre-SWL stenting), renal characters (side, hydronephrosis, and solitary kidney), and stone characters (site, length, density, and previous treatment). Univariate and multivariate statistical analyses were used to identify risk factors. The study included 1179 patients. Complications that required hospital admission were observed in 108 patients (9.2%). They included obstructing steinstrasse in 91 (7.7%), peri-renal hematoma in 3 (0.25%), and fever (>38.0 °C) in 14 (1.2%). Independent risk factors on multivariate analysis were solitary kidney (OR 2.855, P = 0.017), pre-SWL stenting (RR 2.03, P = 0.044), ASA II (OR 1.965, P = 0.007), hydronephrosis (RR 1.639, P = 0.024), and stone length (RR 1.083, P < 0.001). Patients with medical co-morbidities, pre-SWL ureteral stents, large stones and those with obstructed and/or solitary renal unit are more liable to post-SWL complications that need hospital admission. The probability of hospital admission has to be explained to patients with these risk factors.


Assuntos
Hospitalização/estatística & dados numéricos , Cálculos Renais/cirurgia , Litotripsia/efeitos adversos , Complicações Pós-Operatórias/epidemiologia , Adulto , Feminino , Hematoma/epidemiologia , Hematoma/etiologia , Humanos , Hidronefrose/epidemiologia , Hidronefrose/etiologia , Cálculos Renais/complicações , Litotripsia/instrumentação , Litotripsia/métodos , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/terapia , Estudos Retrospectivos , Fatores de Risco , Rim Único/epidemiologia , Rim Único/cirurgia , Resultado do Tratamento
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