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Id: biblio-1056325
Autor: Catalina, Sánchez Basto; Katherine, Puerto Niño Angie; Nicolas, Fernandez; Mariangel, Castillo; Zilac, Espitaleta Vergara; Gómez, Ana María Quintero; Jaime, Pérez Niño.
Título: The natural history of solitary post-nephrectomy kidney in a pediatric population
Fonte: Int. braz. j. urol;45(6):1227-1237, Nov.-Dec. 2019. tab, graf.
Idioma: en.
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.
Descritores: 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
Limites: Humanos
Masculino
Feminino
Recém-Nascido
Lactente
Pré-Escolar
Criança
Adolescente
Responsável: BR1.1 - BIREME


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Id: biblio-1154453
Autor: Çakici, Mehmet Çaglar; Keser, Ferhat; Atis, Ramazan Gokhan; Yildirim, Asif.
Título: Mini-percutaneous nephrolithotomy in a child with multiple urogenital anomalies and a solitary duplex kidney
Fonte: Int. braz. j. urol;47(2):464-467, Mar.-Apr. 2021.
Idioma: en.
Resumo: ABSTRACT Purpose: To report a case of successful removal of right staghorn renal calculi in a 3-year-old girl with Arnold-Chiari malformation and multiple urogenital anomalies. Case report: A 3-year-old female child with the diagnosis of Arnold-Chiari type 2 malformation was referred to our clinic due to presence of 9 kidney stones with a total volume of 10743mm3. The total of the longest diameters of all stones was calculated as 11.4cm. The patient had a urogenital septum, bifid bladder, and duplicated collecting system on the right side. An 18F Amplatz sheath was placed and mini-percutaneous nephrolithotomy was performed successfully by laser and pneumatic lithotripter. Any residual urinary tract stones or urinary tract infection were not detected during the 6th-month follow-up. Conclusion: Urolithiasis requires a thorough understanding of the underlying causes, as well as an effective and minimally invasive treatment. It is important for urologists to understand the complexity of the optimal stone management in pediatric patients in order to maximize treatment efficacy and minimize morbidity. We conclude that it is essential to treat urolithiasis in a single session in children with urogenital anomalies and accompanying congenital anomalies who have past surgical history.
Descritores: Nefrostomia Percutânea
Cálculos Renais/cirurgia
Cálculos Renais/diagnóstico por imagem
Rim Único
Nefrolitotomia Percutânea
Nefropatias
-Estudos Retrospectivos
Resultado do Tratamento
Rim/cirurgia
Rim/diagnóstico por imagem
Limites: Humanos
Criança
Responsável: BR1.1 - BIREME


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Id: biblio-1099930
Autor: Enríquez, Yessenia; Salguero, Nancy; Trujillo, Feddy.
Título: Agenesia renal unilateral a propósito de un caso que se presentó en el Hospital Provincial General Latacunga Servicio de Pediatría / Unilateral renal agenesis regarding a case that was presented at the General Provincial Hospital Latacunga Pediatric Service
Fonte: VozAndes;18(1):18-23, 2007.
Idioma: es.
Resumo: Las malformaciones congénitas inherentes a las estructuras renales y conductos ureterales están asociados con defectos en la formación del brote ureteral o divertículo metanéfrico. La agenesia renal puede ser unilateral (izquierda o derecha), con una frecuencia de 1: 500 o bilateral del 0.3: 1000. La agenesia renal congénita unilateral se caracteriza por no presentar sintomatología mientras que la bilateral es incompatible con la vida. la agenesia renal unilateral generalmente se descubre durante el ultrasonido prenatal de rutina, durante el estudio de pacientes con otras anomalías, o que presentan problemas del tracto urinario recurrentes. Se analiza un caso de dos hermanos con la misma malformación. Uno de ellos tubo una operación para ano imperforado a los dos días de edad. se enfatiza la importancia de una valuación completa de cada uno de los pacientes pediátricos.

Congenital malformations inherent in the renal structures and ureteral ducts are associated with defects in the formation of the ureteral bud or metanephric diverticulum. Renal agenesis can be unilateral (left or right), with a frequency of 1: 500 or bilateral of 0.3: 1000. Unilateral congenital renal agenesis is characterized by no symptoms, while bilateral is incompatible with life. Unilateral renal agenesis is usually discovered during routine prenatal ultrasound, during the study of patients with other abnormalities, or who have recurrent urinary tract problems. A case of two brothers with the same malformation is analyzed. One of them had an operation for an imperforate anus at two days of age. The importance of a complete evaluation of each of the pediatric patients is emphasized.
Descritores: Anus Imperfurado
Anormalidades Congênitas
Rim Único
-Saúde do Homem
Limites: Humanos
Masculino
Recém-Nascido
Tipo de Publ: Relatos de Casos
Responsável: EC147.1 - Biblioteca


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Id: biblio-1139850
Autor: Paz-Montañez, John Jamer; Gaitán-Guzmán, Luisa Fernanda; Acosta-Aragón, María Amparo.
Título: Síndrome de OHVIRA, a propósito de un caso / A case of OHVIRA syndrome
Fonte: Univ. salud;22(3):288-291, set.-dic. 2020. graf.
Idioma: es.
Resumo: Resumen Introducción: El síndrome de obstrucción hemivaginal y anomalía renal ipsilateral (OHVIRA, por sus siglas en inglés) o también llamado síndrome de Herlyn-Werner-Wunderlich es una rara malformación mulleriana que consiste en la presencia de útero didelfo, obstrucción hemivaginal y anomalía renal ipsilateral. La incidencia de malformaciones mullerianas son del 2 al 3%, y la menos común es el síndrome de OHVIRA. Objetivo: Descripción de un caso excepcional de alteraciones mullerianas poco conocido. Presentación del caso: Adolescente de 14 años con historia de dolor pélvico, dismenorrea e infecciones urinarias a repetición. Se estudió con gammagrafía renal que reveló agenesia renal derecha y resonancia magnética pélvica que mostró útero didelfo con hematocolpos derechos. Se confirmaron los hallazgos por laparoscopia diagnóstica. Conclusiones: Este síndrome es poco conocido. Debe sospecharse en casos de agenesia renal, dolor pélvico crónico y alteraciones menstruales en pacientes postpuberes. El abordaje es multidisciplinario.

Abstract Introduction: The obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome, also known as the Herlyn-Werner-Wunderlich syndrome, is a rare müllerian anomaly characterized by didelphic uterus, hemivaginal obstruction, and ipsilateral renal anomaly. The incidence of müllerian malformations is 2% - 3%, OHVIRA being the least common of these cases. Objective: To describe an exceptional case of a müllerian disorder. Case presentation: A 14-year-old adolescent with a history of pelvic pain, dysmenorrhea, and recurrent urinary infections. Renal gammagraphy analysis revealed right renal agenesis, and pelvic magnetic resonance imaging showed a didelphic uterus with right hematocolpos. These findings were confirmed by diagnostic laparoscopy. Conclusions: This syndrome is not well known. Renal anegesis, chronic pelvic pain, and menstrual abnormalities in post-pubertal patients must be presumed as indicators of OHVIRA, whose treatment requires a multidisciplinary approach.
Descritores: Hemorragia Uterina
-Anormalidades Urogenitais
Rim Único
Tipo de Publ: Relatos de Casos
Responsável: CO146.1 - CESUN - Centro de Estudios en Salud


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Id: biblio-1004481
Autor: Vallejo-Urrego, Michael A; Parra-Morales, Alejandra M; González, Adriana.
Título: HDR syndrome in a Colombian woman with a genital tract malformation: first case report in Latin America / Síndrome HDR en una mujer colombiana con una malformación genitourinaria: primer reporte de caso en Latinoamérica
Fonte: Rev. salud pública;20(5):637-640, oct.-nov. 2018. graf.
Idioma: en.
Resumo: ABSTRACT Objectives Hypoparathyroidism, sensorineural deafness and renal disease (HDR) syndrome, also known as Barakat syndrome, is an autosomal dominant transmission hereditary disease with a wide range of penetrance and expressivity. Haploinsufficiency of the GATA3 two finger zinc transcription factor is believed to be its cause. This is the first time this orphan disease is reported in Latin America, so the publishing of this report is expected to raise awareness on these types of syndrome, that are usually underdiagnosed in our region, which in turn causes an increase in the years lost to disability (YLDs) rates, as well as higher costs to be assumed by public health systems. Methods A 36-year-old Colombian woman diagnosed with parathyroid gland agenesis was referred from the Endocrinology Service to the Outpatient Service. According to her medical record, in the past she had developed hypocalcaemia, left renal agenesis, hypoparathyroidism, bicornate uterus and sensorineural hearing loss. Through a genetic analysis a pathological mutation on the short arm of the GATA 3 gen (c.404dupC, p Ala136 GlyfsTER 167) was confirmed, which led to a HDR syndrome diagnosis. Discussion This case proves that there is a possibility that mutations described in other continents may be developed by individuals from our region. Regardless of ethnicity, Barakat syndrome should be considered as a possible diagnosis in patients presenting the typical triad that has been described for this condition, since there could be underdiagnosis of this disease in Latin-America due to the lack of knowledge on this condition in said region, and that genetic counseling in these patients is of great importance for the implications of the syndrome in future generations.(AU)

RESUMEN Objetivos El síndrome de hipoparatiroidismo, sordera neurosensorial y displasia renal (HDR) también llamado síndrome de Barakat, es una enfermedad hereditaria de transmisión autosómica dominante con amplia penetrancia y expresividad genética. El síndrome es causado por la haploinsuficiencia del factor de transcripción de dedos de Zinc GATA3. Esta es la primera vez que esta enfermedad huérfana es reportada en latinoamerica, y buscamos generar consciencia de la presencia de estas enfermedades, las cuales usualmente son infradiagnósticadas en nuestro medio y llevan a un aumento de años perdidos por discapacidad y costos para el sistema de salud pública. Métodos Una mujer colombiana de 36 años ingresó a consulta externa de genética referida por el servicio de endocrinología por una agenesia de paratiroides. La paciente tenía antecedentes de hipocalcemia, agenesia renal izquierda, hipoparatiroidismo, sordera neurosensorial y útero bicorneo. Se realizó un análisis genético que confirmo una mutación patológica en el brazo corto del gen GATA3 (c.404dupC, p Ala136 GlyfsTER 167) diagnóstica del síndrome de Barakat. Discusión Este caso demuestra la posibilidad de existencia de mutaciones descritas en otros continentes en nuestra población. Sin importar la etnia, el síndrome de Barakat debe ser estudiado en pacientes que presenten la triada típica, ya que podría existir un infra diagnóstico de la enfermedad secundario al desconocimiento de la misma en Latinoamérica y teniendo en cuenta la importancia que tiene la consejería genética en estos pacientes por las implicaciones de la enfermedad en futuras generaciones.(AU)
Descritores: Doenças do Colo do Útero/fisiopatologia
Dedos de Zinco
Fator de Transcrição GATA3/análise
Hipoparatireoidismo/genética
-Colômbia
Surdez
Rim Único
Hipocalcemia
Limites: Humanos
Feminino
Adulto
Tipo de Publ: Relatos de Casos
Responsável: BR1.1 - BIREME


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Id: lil-747051
Autor: Santos, E. R; Rosa, N. S; Barni, B. S; Oliveira, M. P; Camargo, V. M. F; Contesini, E. A.
Título: Agenesia renal unilateral e criptorquidismo ipsilateral em um felino: relato de caso / Unilateral renal agenesis and ipsilateral cryptorchidism in a cat: case report
Fonte: Arq. bras. med. vet. zootec;67(2):400-404, Mar-Apr/2015. ilus.
Idioma: pt.
Resumo: A agenesia renal é uma afecção congênita rara na espécie felina, frequentemente associada a uma malformação reprodutiva. O presente trabalho relata o caso de um felino com agenesia renal unilateral associada a criptorquidismo ipsilateral, com ênfase no diagnóstico, tratamento e acompanhamento. O paciente foi conduzido ao Hospital de Clínicas Veterinárias da Universidade Federal do Rio Grande do Sul para avaliação de criptorquidismo. A agenesia renal foi um achado durante a ecografia abdominal do felino. Durante a laparotomia, foi confirmada a ausência do rim e ureter direito, hipertrofia do rim esquerdo e presença de um testículo ectópico. O paciente teve alta após a recuperação anestésica e se mantém clinicamente estável, transcorridos seis meses da cirurgia. A agenesia renal unilateral é uma condição compatível com a vida, contanto que o rim existente apresente funcionamento aceitável. Assim, sugere-se que a possibilidade de rim único em felinos criptorquidas deve ser investigada sempre que possível, tendo em vista a alta correlação entre essas malformações, e objetivando um acompanhamento da função renal do paciente ao longo da vida.(AU)

Renal agenesis is a rare disorder in feline species, commonly associated with reproductive malformation. This study aims to report the case of a cat with unilateral renal agenesis combined with ipsilateral cryptorchidism, emphasizing the diagnosis, treatment and patient follow up. The patient was taken to the Veterinary Hospital of the Federal University of Rio Grande do Sul to evaluate the cryptorchidism. The renal agenesis was an incidental finding during the abdominal ultrasound. At laparotomy, the absence of the right kidney and ureter was confirmed, hypertrophy of the left kidney and the presence of an ectopic testicle were found. The patient was discharged after recovering from anesthesia and remains clinically stable six months after surgery. The unilateral renal agenesis is a life compatible condition as long as the existing kidney has an acceptable performance. Therefore, it is suggested that the chance of a single kidney in cats whit cryptorchidism should be investigated, given the correlation between these malformations, and aiming to monitor renal function throughout the life of the patient.(AU)
Descritores: Anormalidades Congênitas/veterinária
Criptorquidismo/veterinária
Rim Único/veterinária
Limites: Animais
Gatos
Tipo de Publ: Relatos de Casos
Responsável: BR68.1 - Biblioteca Virginie Buff D'Ápice


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Id: biblio-975915
Autor: Radhakrishna, Veerbhadra; Govindarajan, Krishna Kumar; Sambandan, Kumaravel; Jindal, Bibekanand; Naredi, BikashKumar.
Título: Solitary functioning kidney in children: clinical implications / Rim solitário em funcionamento em crianças: implicações clínicas
Fonte: J. bras. nefrol;40(3):261-265, July-Sept. 2018. tab.
Idioma: en.
Resumo: ABSTRACT Introduction: Children with solitary functioning kidney (SFK) are prone to develop long term problems, which are not well represented in the literature. The extent to which the presence of associated congenital anomalies of kidney and urinary tract (CAKUT) further de-stabilize renal function is to be addressed. Objective: This study was conducted to evaluate the etiology, presentation, presence of CAKUT, and renal damage in children with SFK. Methods: All children with SFK who presented to the department of pediatric surgery from March 2014 to May 2016 were included in the study. Children with malignancy were excluded from the study. Results: Of the 20 patients with SFK, 14 (70%) had primary SFK (8 with agenesis and 6 with multicystic dysplastic kidney), 6 (30%) belonged to secondary SFK group, among them 3 had pelviureteric junction obstruction, 2 had posterior urethral valves and 1 had vesicoureteric reflux. Eight (40%) had associated CAKUT, 4 (20%) were asymptomatic while 8 (40%) had UTI and 6 (30%) had hypertension. Ten (50%) patients had reduced glomerular filtration rate (GFR) suggesting compromised renal function. Conclusion: Children with SFK have high morbidity especially when associated with ipsilateral CAKUT. Long-term periodical follow up is essential in these patients to improve clinical outcome.

RESUMO Introdução: Crianças com rim solitário funcional (RSF) tendem a desenvolver problemas de longo prazo, que não são bem representados na literatura. Devemos abordar o quanto a presença de malformações congênitas do rim e do trato urinário (CAKUT) desestabiliza ainda mais a função renal. Objetivo: Este estudo foi realizado para avaliar a etiologia, apresentação, presença de CAKUT e dano renal em crianças com RSF. Métodos: Todas as crianças com RSF que se apresentaram no departamento de cirurgia pediátrica de março de 2014 a maio de 2016 foram incluídas no estudo. Crianças com doença maligna foram excluídas do estudo. Resultados: dos 20 pacientes com RSF, 14 (70%) tinham RSF primário (8 com agenesia e 6 com rim displásico multicístico), 6 (30%) pertenciam ao grupo RSF secundário, dentre eles 3 tinham obstrução da junção pelveuretérica, 2 tinham valvas uretrais posteriores e 1 refluxo vesico-uretral. Oito (40%) tinham ACRTU associado, 4 (20%) estavam assintomáticos, enquanto 8 (40%) tinham ITU e 6 (30%) tinham hipertensão. Dez (50%) pacientes apresentaram redução da taxa de filtração glomerular (TFG), sugerindo comprometimento da função renal. Conclusão: Crianças com RSF apresentam alta morbidade, especialmente quando associadas à CAKUT ipsilateral. O acompanhamento periódico a longo prazo é essencial nesses pacientes para melhorar o resultado clínico.
Descritores: Anormalidades Urogenitais/diagnóstico
Anormalidades Urogenitais/etiologia
Refluxo Vesicoureteral/diagnóstico
Refluxo Vesicoureteral/etiologia
Rim Único/complicações
-Estudos Prospectivos
Limites: Humanos
Masculino
Feminino
Recém-Nascido
Lactente
Responsável: BR1.1 - BIREME


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Id: biblio-1009233
Autor: Cabrera, Asanty; Ascanio, Careli.
Título: Agenesia renal izquierda en paciente con quiste renal / Neumonía a focos múltiples / Left renal agenesis in a patient with renal cyst giant right. Pneumonia with multiple foci
Fonte: Med. interna (Caracas);33(1):[19], 2017. ilus.
Idioma: es.
Descritores: Pneumonia/fisiopatologia
Rim Único/fisiopatologia
Neoplasias Renais
-Diagnóstico por Imagem
Medicina Interna
Limites: Humanos
Masculino
Adulto
Pessoa de Meia-Idade
Tipo de Publ: Relatos de Casos
Responsável: VE1.1 - Biblioteca Humberto Garcia Arocha


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Id: biblio-999828
Autor: Devezas, Vítor; Canão, Pedro Amoroso; Barbosa, Laura Elisabete.
Título: A rare case of abdominal pain
Fonte: Autops. Case Rep;9(2):e2019088, Abr.-Jun. 2019. ilus.
Idioma: en.
Descritores: Dor Abdominal/diagnóstico
Erros de Diagnóstico/prevenção & controle
Nefropatias/diagnóstico
-Autopsia
Anormalidades Congênitas/diagnóstico
Proteínas Secretadas pela Vesícula Seminal
Rim Único
Limites: Humanos
Masculino
Adulto
Tipo de Publ: Relatos de Casos
Responsável: BR26.7 - Serviço de Biblioteca e Documentação Científica


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Id: biblio-995824
Autor: Egas, María de Fátima; Naranjo, Alfredo.
Título: Incidencia de ectasia pielocalicial neonatal en el Hospital Metropolitano de Quito, 2014 a 2018: continuación de un estudio / Pyelocalyceal incidence of neonatal ectasia in Hospital Metropolitano of Quito, from 2014 to 2018: the next part of the study
Fonte: Metro cienc;26(2):66-71, Diciembre 2018.
Idioma: es.
Resumo: Objetivo: determinar la incidencia de ectasia pielocalicial e hidronefrosis en el Hospital Metropolitano de Quito durante el período 2014-2018. Métodos: se realizó un estudio analítico retrospectivo de incidencia de ectasia pielocalicial en el Hospital Metropolitano de Quito. Se presentaron 7 casos de neonatos diagnosticados de esta entidad durante un período de 4 años 5 meses, desde 2014 a 2018 (incidencia: 0,18%) en el Hospital. Edad: 25 meses promedio; 5 de sexo masculino y 2 de sexo femenino; 57% diagnosticados durante el período prenatal y 43% en el periodo neonatal. Resultados: de los neonatos diagnosticados por ecografía prenatal, en 100% se ratificó el diagnóstico ecográfico posterior al nacimiento. En este estudio se encontró que 85,7% padecía ectasia I-II, 0% ectasia III-IV y en 14,28% no se pudo identificar el tamaño de la dilatación. El 71,42% presentó asociación con hidronefrosis, 14,28% con doble sistema pielocalicial, 42,85% megauréter, 14,28% agenesia renal. Localización: 57,14% izquierdo, 28,57% derecho y 14,28% bilateral. De los 7 pacientes, 14% acudió al Hospital por fiebre, 57% para control diagnóstico prenatal, 14% por control posnatal y 14% por hematuria. Conclusiones: la ectasia pielocalicial es una entidad de importancia que debe ser diagnosticada durante la etapa prenatal y confirmada en la etapa neonatal para lograr su correcto y oportuno manejo.

Objective: Determine the incidence of pyelocalyceal ectasia at Hospital Metropolitano of Quito from 2014 to 2018. Methods: A retrospective analytical study of pyelocalyceal ectasia incidence was performed at the Metropolitan Hospital of Quito. There were 7 patients with this disease from 2014 to 2018 (incidence of 0,18%) at the Hospital. The average age was 25 months. Of this, 5 were male and 2 female, 57% were diagnosed during prenatally and 43% of neonatal form. Results: From the group of patients that were diagnosed by prenatal ultrasound, 100% the diagnosis was confirmed by ultrasound performed within a few days of birth. In this study it was found that 85,7% had I-II ectasia grade, 0% ectasia III-IV, and 14,28% could not be identified due to lack of information about the size of expansion. Of the cases, 71,42% was associated with hydronephrosis, 14,28% with double pyelocalicial system, 42,85% megaureter, 14,28% renal agenesis. 57% affected the left side, 28% the right side and 14% afected bilaterally. From the 7 patients, 14% went to hospital due to fever, 57% for prenatal diagnosis, 14% for postnatal control, and 14% for hematuria. Conclusions: The pyelocalyceal ectasia is an important entity that must be diagnosed during the prenatal stage and confirmed in newborns for its correct management.
Descritores: Ultrassonografia Pré-Natal
Rim Único
Hidronefrose
-Recém-Nascido
Incidência
Limites: Humanos
Recém-Nascido
Tipo de Publ: Artigo Clássico
Responsável: EC110.1 - Biblioteca



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