Riñón único funcionante y su seguimiento en Pediatría

Contexto: el riñón único funcionante (RUF) es una entidad que puede ser de tipo congénito y es causada por una agenesia renal unilateral o un riñón displásico multiquístico, o adquirido de forma secundaria a una nefrectomía unilateral, condicionando al paciente a un seguimiento estricto rutinario, así como en la implementación de algunas pautas de prevención en los pacientes pediátricos. Objetivo: analizar la importancia del seguimiento en los pacientes pediátricos con RUF. Metodologí:a se hace una revisión de la literatura mostrando desde la embriología, fisiología, etiología, aspectos clínicos y estudios diagnósticos en pacientes con RUF para así, posteriormente, ofrecer unas pautas de seguimiento y manejo en pacientes pediátricos. Resultados: los pacientes con RUF pueden presentar hiperfiltración glomerular compensatoria, seguida de lesión glomerular con hipertensión, albuminuria y reducción de la tasa de filtración glomerular, sin embargo, para prevenir el daño renal se deben realizar seguimientos clínico, paraclínico e imagenológico cuidadosos de todo paciente con RUF, según la condición lo amerite. Conclusiones: se hace necesaria la unificación de pautas de seguimiento en los pacientes pediátricos con RUF y hacer énfasis en aquellos factores de riesgo que predisponen a compromiso renal.

Background: The Solitary functioning kidney (SFK) is an entity that can be congenital type caused by unilateral renal agenesis or multicystic dysplastic kidney, or acquired secondary to unilateral nephrectomy conditioning to a strict routine follow-up, as well as the implementation of some prevention guidelines in pediatric patients. Purpose: To analyze the importance of follow-up in pediatric patients with a single functioning kidney. Methodology: A review of the literature is made showing embryology, physiology, etiology, clinical aspects and diagnostic studies in patients with SFK in order to subsequently offer guidelines for follow-up and management in pediatric patients. Results: Patients with SFK may present compensatory glomerular hyperfiltration, followed by glomerular injury with hypertension, albuminuria and reduced glomerular filtration rate, however, to prevent renal damage a careful clinical, paraclinical and imaging follow-up of every patient with SFK should be performed as the condition warrants. Conclusions: It is necessary to unify follow-up guidelines in pediatric patients with SFK and to emphasize those risk factors that predispose to renal compromise.

Solitary renal duplication with misaligned malrotation of upper and lower moieties complicated by complete staghorn stones in the lower moiety: a case report / 中华泌尿外科杂志

Solitary kidney, renal duplication and malrotation are rare congenital renal malformations in urology department, and probably contributed to some complications such as obstruction, hydronephrosis, infection, stones. In this case report, we firstly presented a male patient with rarely multiple renal malformations, including solitary kidney, renal duplication, misaligned malrotation of upper and lower moieties, and accompanied by complete staghorn stones and hydronephrosis, who was treated with open pyelolithotomy under general anesthesia. After the operation, obstruction, hydronephrosis, and infection were relieved.

Herlyn-Werner-Wunderlich syndrome: Case report / Síndrome de Herlyn-Werner-Wunderlich. Reporte de caso

Abstract Introduction: Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital malformation of the urogenital tract characterized by the triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis, which is usually diagnosed after menarche. It is treated by resecting the vaginal septum and draining the obstructed hemivagina and has a favorable postoperative prognosis. Case presentation: This is the case of 22-year-old woman with a 9-year history of dysmenorrhea, pelvic pain, and irregular menstrual cycles that started after she had her first menstruation, who visited the gynecology service of a secondary care hospital in Sogamoso, Boyacá (Colombia). The patient reported having visited multiple specialists and being treated with vitamin E, metformin, and contraceptives, without experiencing an improvement of symptoms and signs. After being assessed, and taking into account ultrasound and MRI findings, she was diagnosed with HWW syndrome. She underwent a colpotomy plus resection of right paracervical mass of approximately 60x60mm and vaginal septum, achieving complete resolution of symptoms. Finally, 10 months after the surgery, she reported being pregnant without complications. Conclusion: HWW syndrome is a rare malformation that should be considered as a differential diagnosis in women of any age with paramesonephric duct anomalies, pelvic pain, dysmenorrhea, and presence of masses in the genital tract, since its early diagnosis and timely treatment considerably improve the quality of life of these patients by reducing the severity of symptoms, decreasing the incidence of complications, and improving obstetric prognosis.

Resumen Introducción. El síndrome de Herlyn-Werner-Wunderlich (SHWW) es una malformación congénita rara del tracto urogenital que se caracteriza por la triada útero didelfo, hemivagina obstruida y anomalía renal ipsilateral, y que suele diagnosticarse después de la menarquia. Su tratamiento consiste en resección del tabique vaginal y drenaje de la hemivagina obstruida, con un buen pronóstico postquirúrgico. Presentación del caso. Mujer de 22 años con un cuadro clínico de 9 años de evolución que inició cuando tuvo su primera menstruación, consistente en dismenorrea, dolor pélvico y ciclos menstruales irregulares, quien asistió al servicio de ginecología de un hospital de segundo nivel en Sogamoso, Boyacá (Colombia). La paciente reportó haber visitado múltiples especialistas y recibido tratamiento con vitamina E, metformina y anticonceptivos, sin mejoría de los síntomas y signos. Luego de ser valorada, y teniendo en cuenta los hallazgos en ecografía y resonancia magnética, fue diagnosticada con SHWW, por lo que se le realizó colpotomía más resección de masa paracervical derecha de aproximadamente 60x60mm y de tabique vaginal, lográndose la resolución completa de los síntomas. Finalmente, 10 meses después de la cirugía, la joven refirió encontrarse en estado de embarazo, sin presentar complicaciones. Conclusión. El SHWW es una malformación poco común que debe considerarse como diagnóstico diferencial en mujeres de cualquier edad con anomalías de los conductos paramesonéfricos, dolor pélvico, dismenorrea y masa en el tracto genital, pues su diagnóstico temprano y un tratamiento oportuno mejoran considerablemente la calidad de vida de estas pacientes al reducir la severidad de los síntomas, disminuir la incidencia de complicaciones y mejorar el pronóstico obstétrico.

Síndrome de Herlyn-Werner-Wunderlich: reporte de caso y revisión de la literatura / Herlyn-Werner-Wunderlich syndromne: case review and report of the literatura

RESUMEN Objetivo: Presentar un reporte de caso de síndrome de Herlyn Werner Wunderlich (SHWW) y hacer una revisión de la literatura para determinar los hallazgos clínicos e imagenológicos en estas pacientes. Materiales y métodos: Se presenta el caso de una mujer de 16 años que consultó, en un hospital de las fuerzas militares en Bogotá, por dolor pélvico recurrente, su diagnostico final fue SHWW. Se realizó una búsqueda sistemática de la literatura en las diferentes bases de datos, revisiones sistemáticas, cohortes, series de casos y reportes de casos en pacientes con diagnóstico de SHWW en cualquier edad, se obtuvo información sobre las características de presentación clínica, y las tecnologías diagnósticas más frecuentemente utilizadas. Se hace resumen narrativo de los hallazgos. Resultados: Se incluyeron 77 publicaciones, un total de 676 pacientes. Los síntomas más frecuentes fueron dismenorrea (63,9 %), seguido de dolor pélvico (35,2%). Las tecnologías diagnósticas más utilizadas fueron el ultrasonido pélvico en un 92,1% y la resonancia magnética nuclear en un 74,6% de los casos. La histeroscopia y laparoscopia son poco utilizados en el diagnóstico. Conclusión: El SHWW es una entidad poco frecuente, debe hacer parte del estudio complementario de la agenesia renal del paciente pediátrico y del diagnóstico diferencial de la dismenorrea primaria en pacientes en la adolescencia. Se requiere evaluar con estudios de cohorte más grandes la utilidad de la histeroscopia en estas pacientes.

ABSTRACT Objective: To report a case of Herlyn-Werner-Wunderlich syndrome (HWWS) and conduct a review of the literature to determine clinical and imaging findings in these patients. Material and methods: A 16-year-old female patient who presented to a military hospital in Bogota complaining of recurrent pelvic pain, receiving a final diagnosis of HWWS. A systematic search was conducted in the different databases of systematic reviews, cohort studies, case series and case reports of patients of any age diagnosed with HWWS. Clinical presentation characteristics and the most frequent diagnostic technologies used were obtained. A narrative summary of the findings is presented. Results: Overall, 77 publications with a total of 676 patients were included. The most frequent symptom was dysmenorrhea (63.9%), followed by pelvic pain (35.2%). The most frequently used diagnostic technologies were pelvic ultrasound in 92.1% of cases and nuclear magnetic resonance in 74.6%. Hysteroscopy and laparoscopy are seldom used for diagnosing this condition. Conclusion: HWWS is an infrequent disease condition. It must be part of the work-up for renal agenesis in pediatric patients and of the differential diagnosis in primary dysmenorrhea in adolescence. The role of hysteroscopy in this condition must be further assessed in larger cohort studies.

Cambios fisiológicos y morfológicos tempranos en el riñón remanente de donantes vivos / Early physiological and morphological changes in the remnant kidney of living donors

RESUMEN Con el objetivo de describir los cambios funcionales y morfológicos tempranos en el riñón remanente de donantes vivos, se realizó un estudio retrospectivo en el Hospital Cayetano Heredia, en el que se incluyeron 55 individuos. De las historias clínicas, se obtuvieron los datos clínicos y demográficos, así como la depuración de creatinina, la proteinuria, la presión arterial y las dimensiones renales a los 1, 2, 3, 6 y 12 meses después de la donación del riñón. La edad media fue de 40,88 (±9,84) años; el 80% eran mujeres y el índice de masa corporal medio fue de 25,68 (±3,5) kg/m2. Se utilizaron modelos lineales y cuadráticos para estudiar las variables fisiológicas y morfológicas. Durante el tiempo de seguimiento, la tasa de filtración glomerular, la proteinuria, la presión arterial diastólica y la longitud de los riñones mostraron cambios significativos (p < 0,05).

ABSTRACT In order to describe the early functional and morphological changes in the remnant kidney of living donors, a retrospective study was carried out at the Cayetano Heredia Hospital. Data from 55 individuals was included. Clinical and demographic data were obtained from the clinical records, as well as data for creatinine clearance, proteinuria, blood pressure and renal dimensions at 1, 2, 3, 6 and 12 months after kidney donation. The mean age was 40.88 (±9.84) years; 80% were women and the mean body mass index was 25.68 (±3.5) kg/m2. Linear and quadratic models were used to study physiological and morphological variables. During the follow-up time, glomerular filtration rate, proteinuria, diastolic blood pressure, and kidney length showed significant changes (p < 0.05).

Síndrome de OHVIRA, a propósito de un caso / A case of OHVIRA syndrome

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.

Rare case: enucleation of multiple fibroids after intraoperative vaginal sonographic detection of the solitary pelvic kidney by patient with Mayer-Rokitansky-Kuster-Hauser syndrome

Mayer-Rokitansky-Kuster-Hauser syndrome (MRKH) is a congenital anomaly of the genital tract that occurs in about 1 in 4000 women. MRKH syndrome can be associated with renal, skeletal, heart and hearing abnormalities. The frequency of renal/urinary tract abnormalities is 33%. Only a few cases of fibroid development in MRKH syndrome have been described in the literature. The diagnosis and surgery of a fibroid in MRKH syndrome may be complicated in associated kidney abnormality by an atypical kidney position, as in this case: pelvic kidney on one side and renal agenesia on the contralateral side. Authors present the case of a 47-year-old female patient with a known MRKH syndrome and a pelvic kidney on the right side who had presented with an unclear tumour in the right lower abdomen. A completed CT scan revealed the tumour directly next to the pelvic kidney. A malignancy could not be excluded with certainty, so that a laparoscopy in laparotomy readiness was indicated and performed. During surgery, two rudimentary uterine horns were found; on the right side retroperitoneally, below the uterine horn, the tumour was located and directly below it there was a soft tissue alteration, probably the kidney. For safety, a vaginal sonography was performed in between, to clearly identify the only kidney and to avoid damaging it. The tumour could be extirpated laparoscopically without kidney injury. The two uterine horns were removed simultaneously. Histologically the fibroid could be confirmed. In addition, three other fibroids (one on the left side and two on the right side) were detected. Due to the high probability of a simultaneous kidney abnormality in the MRKH syndrome, authors suggest an accurate kidney diagnosis preoperatively. If necessary, in the case of a pelvic kidney and/or renal agenesia, as in this case, an additional intraoperative kidney check should be performed.

Long-term analysis of safety and efficacy of standard percutaneous nephrolithotomy in patients with solitary kidneys / 北京大学学报(医学版)

OBJECTIVE@#To evaluate the long-term efficacy and safety of ultrasound-guided percutaneous nephrolithotomy (PCNL) in the treatment of patients with solitary kidney stones.@*METHODS@#The clinical data of 22 patients with solitary kidney stones treated with PCNL in Peking University People's Hospital from September 2008 to June 2014, with the follow-up data of more than 5 years were analyzed retrospectively. Perioperative indicators, postoperative stone free rate (SFR) and incidence of complications were recorded. Ultrasonography was used to evaluate the long-term stones recurrence rate. Serum creatinine and estimated glomerular filtration rate (eGFR) were used to assess the long-term renal function.@*RESULTS@#In this group of 22 patients, the average age was (50.3±11.8) years, with 10 cases of anatomic solitary kidneys, 12 functional solitary kidneys, and the median stone diameter was 1.65 (1.1-3.9) cm. All the patients had multiple stones, including 7 cases of staghorn stones. The median pre-operative serum creatinine was 104.5 (60.0-460.0) μmol/L, and the mean eGFR was (60.3±29.4) mL/min, showing no statistically significant difference compared with that before surgery. The mean operative time was (88.2±42.0) min, and there were 11 cases of single-channel and double-channel PCNL. The median serum creatinine on the first day after surgery was 102.0 (63.0-364.0) μmol/L, and the mean eGFR was (58.0±25.1) mL/min. The mean postoperative hospital stay was (8.7±5.2) days. In this group, 5 patients (22.7%) presented short-term complications, among which 4 patients presented postoperative infection and massive hemorrhage at the same time, which improved after conservative treatment, and 1 patient presented pleural injury and improved after closed thoracic drainage. Two patients (9.1%) developed long-term complications, and ureteral stricture occurred 3 months after operation, which improved after balloon dilatation. The median follow-up time was 6.2 (4.7-11.1) years. The median serum creatinine at the last follow-up was 104.0 (72.4-377.0) μmol/L, and the mean eGFR was (60.1±23.7) mL/min, showing no statistically significant difference compared with that before surgery. Renal function decreased in 6 patients (27.3%). Initial and final SFR were 72.7% and 100%, respectively. During the 6.2-year follow-up, 9 patients (40.9%) experienced recurrence of kidney stone. After stone recurrence, 13 lithotomy surgeries were performed, and the SFR by the latest follow-up was 63.6%.@*CONCLUSION@#This study had the longest follow-up time for patients with solitary kidney stones after PCNL reported at home and abroad. Ultrasound-guided standard PCNL was safe and effective in the treatment of solitary kidney stones. Long-term follow-up results showed that the recurrence rate of kidney stones was still high, but the long-term renal function was stable after operation, and some patients showed mild renal function decline.

The natural history of solitary post-nephrectomy kidney in a pediatric population

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.

Fibrodisplasia osificante progresiva plus por una variante patogénica del gen ACVR1: caso clínico / Fibrodysplasia ossificans progressiva: report of one case

Fibrodysplasia ossificans progressiva (FOP) or myositis ossificans, is a genetic disease, with a prevalence of 1 in 2.000.000. It is caused by pathogenic variants in ACVR1 gene and characterized by soft tissue heterotopic ossification, starting in the second decade of life. It is associated to early mortality caused by respiratory complications. It evolves in flare-ups, triggered by soft tissue injuries; therapy is symptomatic, using analgesia, steroids and diphosphonates. We report a 12-year-old female with left renal agenesis, hallux valgus and intellectual disability, presenting with a six months history of thoracic kyphosis, tender nodules in the thorax, and rigidity of right elbow and left knee. Clinical examination revealed dysmorphic facial features. A magnetic resonance showed heterotopic ossification nodules, which was confirmed with spinal radiography. These findings prompted the diagnosis of FOP. Pain treatment was started, and prednisone was used during flare-ups. The ACVR1 gene was analyzed and a pathogenic variant, p. Arg206His, was found, confirming the diagnosis of FOP.

Analysis of incidence and risk factors of renal insufficiency in solitary kidney patients / 中华肾脏病杂志

Objective@#To investigate the incidence of renal insufficiency in solitary kidney patients and analyze the risk factors.@*Methods@#Patients with solitary kidney who were admitted to the Second Hospital of Lanzhou University from January 2012 to January 2019 were retrospectively selected as subjects. According to estimated glomerular filtration rate (eGFR) level, the patients were divided into two groups: eGFR<60 ml·min-1·(1.73 m2)-1 group and eGFR≥60 ml·min-1·(1.73 m2)-1 group. The data of the general information, laboratory examinations and kidney size were collected, and the differences of the above indicators between the two groups were compared. Logistic regression model was used to analyze the related factors of renal function decline.@*Results@#(1) A total of 323 solitary kidney patients with age of (53.8±15.8) years and median duration of 10.0 years were enrolled in the study, including 203 males (62.8%). There were 150 cases (46.4%) with hypertension, 136 cases(42.1%) with proteinuria, and 134 cases (41.5%) with renal insufficiency, even 29 cases(9.0%) had developed into end-stage renal disease. (2) Compared with those in eGFR≥60 ml·min-1·(1.73 m2)-1group, patients in eGFR<60 ml·min-1·(1.73 m2)-1 group had higher age, mean arterial pressure, serum creatinine, serum uric acid, fasting blood glucose, and higher proportion of hypertension and proteinuria, but had lower proportion of congenital solitary kidney, hemoglobin, plasma albumin and residual kidney diameter. The differences of above indicators were statistically significant (all P<0.05). (3) Logistic regression analysis showed that increasing age (every ten years, OR=1.752, 95% CI 1.455-2.109, P<0.001), anemia (OR=2.327, 95% CI 1.356-3.994, P=0.002), hyperuricemia (OR=5.097, 95% CI 2.873-9.042, P<0.001) and high urine protein level (every 1+, OR=1.515, 95% CI 1.197-1.919, P=0.001) were independent risk factors for renal dysfunction in solitary kidney patients.@*Conclusions@#The incidence of renal insufficiency in solitary kidney patients is 41.5%. Patients with solitary kidney may perform varying degrees of kidney damage, such as hypertension, proteinuria and eGFR decline. Increasing age, anemia, hyperuricemia and high urine protein level are independent risk factors for renal insufficiency in solitary kidney patients.

Analysis of incidence and risk factors of renal insufficiency in solitary kidney patients / 中华肾脏病杂志

Objective To investigate the incidence of renal insufficiency in solitary kidney patients and analyze the risk factors. Methods Patients with solitary kidney who were admitted to the Second Hospital of Lanzhou University from January 2012 to January 2019 were retrospectively selected as subjects. According to estimated glomerular filtration rate (eGFR) level, the patients were divided into two groups: eGFR<60 ml·min-1·(1.73 m2)-1 group and eGFR≥60 ml·min-1·(1.73 m2)-1 group. The data of the general information, laboratory examinations and kidney size were collected, and the differences of the above indicators between the two groups were compared. Logistic regression model was used to analyze the related factors of renal function decline. Results (1) A total of 323 solitary kidney patients with age of (53.8 ± 15.8) years and median duration of 10.0 years were enrolled in the study, including 203 males (62.8％). There were 150 cases (46.4％) with hypertension, 136 cases(42.1％) with proteinuria, and 134 cases (41.5％) with renal insufficiency, even 29 cases(9.0％) had developed into end-stage renal disease. (2) Compared with those in eGFR≥60 ml·min-1·(1.73 m2)-1group, patients in eGFR<60 ml·min-1·(1.73 m2)-1 group had higher age, mean arterial pressure, serum creatinine, serum uric acid, fasting blood glucose, and higher proportion of hypertension and proteinuria, but had lower proportion of congenital solitary kidney, hemoglobin, plasma albumin and residual kidney diameter. The differences of above indicators were statistically significant ( all P<0.05). (3) Logistic regression analysis showed that increasing age (every ten years, OR=1.752, 95％CI 1.455-2.109, P<0.001), anemia (OR=2.327, 95％CI 1.356-3.994, P=0.002), hyperuricemia (OR=5.097, 95％CI 2.873-9.042, P<0.001) and high urine protein level (every 1+, OR=1.515, 95％CI 1.197-1.919, P=0.001) were independent risk factors for renal dysfunction in solitary kidney patients. Conclusions The incidence of renal insufficiency in solitary kidney patients is 41.5％. Patients with solitary kidney may perform varying degrees of kidney damage, such as hypertension, proteinuria and eGFR decline. Increasing age, anemia, hyperuricemia and high urine protein level are independent risk factors for renal insufficiency in solitary kidney patients.

Safety and efficacy of flexible ureteroscopy lithotripsy for the treatment of renal stone in solitary kidney patients / 中华泌尿外科杂志

Objective To evaluate the safety and efficacy of flexible ureteroscopy lithotripsy (FURL) for the treatment of renal stone in solitary kidney patients.Methods The clinical data of 46 patients of solitary kidney,who were treated with FURL from March 2015 to May 2018 in our hospital,were analyzed retrospectively.There were 34 males and 12 females,aged (48.6 ± 9.6) years.Maximum diameter of stone was (15.3 ±4.8) mm,and 29 cases in left kidney and 17 cases in right.34 cases were non-renal calcaneal calculi,12 cases were subrenal calyceal stones.There were 3 cases of congenital solitary kidney,31 cases of functional solitary kidney (contralateral kidney GFR ＜ 10 ml/min) and 12 cases of acquired solitary kidney (7 cases of renal calculi,4 cases of tumor,1 case of tuberculosis).The mean reoperative serum creatinine was (116.38 ± 25.77)μmol/L.All patients were treated with general anesthesia,lithotomy,soft ureteroscopy combined with holmium laser lithotripsy,and assisted lithotripsy.All operations were performed by the same surgeon.The data of operation time,hospital stay,blood loss,renal function before and after operation,postoperative complications and stone clearance rate were recorded.Results In this study,46 cases of the operation were successfully completed.The mean operation time was (58.6 ±16.4) min,the average hospitalization time was (5.6 ± 1.4) days.The mean hemoglobin was decreased (1.4 ± 0.9) g/L.The mean operative time was (58.6 ± 16.4) min.The average postoperative hospitalization time was (5.6 ± 1.4) days.The Postoperative hematuria occurred in 32 cases,low back pain in 3 cases and fever in I case.Stone-free reached in 39 of 46 patients,the stone-free rate(SFR)of primary operation was 84.8％ (39/46).There were 7 cases of residual calculi,five patients were treated with secondary FURL,2 patients were required conservative treatment.The SFR was 95.7％ (44/46) after the second stage operation.The mean serum creatinine was (112.29 ± 20.62) μ mol/L on the first day after operation,which was not different statistically with that before operation (P =0.177).The mean serum creatinine was (81.54 ± 10.75) μmol/L one month after operation,which was significantly lower than preoperative and 1 day postoperative (P ＜ 0.05).Conclusions FURL could be a safe and effective treatment for renal stone in solitary kidney patients.It has a definite stone-free effect,low incidence of complications.

Functional and oncologic outcomes after nephron-sparing surgery in a solitary kidney: 10 years of experience

ABSTRACT Objectives: To evaluate functional and oncologic outcomes of partial nephrectomy (PN) in patients with a solitary kidney. Materials and Methods: A retrospective analysis of patients with a solitary kidney undergoing nephron-sparing surgery between March 2003 and March 2013 was performed. GFR was recorded before the procedure and 3 months after surgery, thus establishing a change (cGFR). Several variables that may influence cGFR were analyzed. Complications are herein described, namely bleeding, fistula, acute renal failure and end-stage renal disease (ESRD). Local recurrence and margin status are also described. Survival rates were calculated using the Kaplan Meier method (2 patients with metastasis at the time of surgery were excluded from the analysis). Results: Forty-five patients were available for analysis. Median follow-up was 27.56 months (r 3-96). Mean cGFR was-7.12mL/min (SD 2.1). Variables significantly related with lower GFR after surgery were loss of renal mass (p=0.01)) and male gender (p=0.03). Four patients (8.8%) experienced hemorrhage. Nine patients (20%) developed a urinary fistula. Only one patient with bleeding required open surgery. Two patients (4.4%) needed transient dialysis. Three patients (6.6%) developed ESRD. Four patients (8.8%) had positive surgical margins (PSMs) and four patients (88%) had local recurrence (2 of these had PSMs). Five patients (11.1%) died during follow-up. Four patients (8.8%) died because of renal cancer. Estimated 2-year overall survival, disease-free survival and cancer specific survival rates were 88.4% (CI 95% 70.5-96); 87.7% (CI 95% 68.1-96) and 92.4% (CI 95% 75-98), respectively. Conclusion: Loss of renal mass and male gender were associated with lower postoperative GFR. Our outcomes were comparable with those in the World literature.

Safety and efficacy of flexible ureteroscopy and holmium laser lithotripsy for the treatment of renal stones in patients with a solitary kidney / 国际外科学杂志

Objective To investigate the safety and efficacy of flexible ureteroscopy and holmium laser lithotripsy for the treatment of renal stones in patients with a solitary kidney.Methods Between May 2014 and June 2016,Clinical records of 17 cases of stones in a solitary kidney who underwent flexible ureteroscopy and holmium laser lithotripsy were reviewed.10 of these cases were male and the others female;mean age of these cases was 45.8 (32-67) years.9 were on the left side while 8 were on the right.6 cases were with functional solitary kidney (with the GFR of the contralateral kidney less than 10ml/min),while 11 others were with anatomical solitary kidney (6 due to tumor,2 due to tuberculosis,3 being congenital).All cases underwent BUS,KUB as well as CTU to confirm diagnosis before surgery,with 8 diagnosed with renal pelvis calculi,5 were with upper or middle calyx calculi,and 4 were with subrenal calyx calculi.The mean ± SD size of the stones was 1.2 ± 0.3 cm.Clinical m anifestations were asymptomatic in 10cases,backache in 5 cases and fever in 2 cases.All patients underwent exams of CBC,urinalysis,liver and kidney function,coagulation function,ECG,chest X-ray as well as cleaning midstream urine cultivation after admission.Results Mean ± SD operation time among all cases was (74.2 ± 23.6) min,the amount of bleeding during surgery was 5-15 (mean:8) ml,duration of hospitalization was 3-12 (mean:4.5)days,ureteral stents are kept for an average time of 4 weeks after surgery.3 patients developed fever after operation,1 developed urosepsis;all these patients recovered after active anti-infection treatment.No post-operative complications such as fever,renal colic,hematuresis,ureteral perforation,active bleeding as well as sepsis were found among the other patients.The preoperative and postoperative serum creatinine had statistical difference (P ＜0.05).14 patients were proved to be stone-free by KUB or CTU.The stone-free rate after a single operation was 82.3％ (14/17).Residual stone were found in the 3 patients,who subsequently underwent a second flexible ureteroscopy and holmium laser lithotripsy.The stone-free rate after a second operation was 94.1％.Conclusions Flexible ureteroscopy and holmium laser lithotripsy is a safe and effective method for the treatment of nephrolithiasis in patients with a solitary kidney with a lower procedure-related injury rate,smaller amount of bleeding,a lower complication rate and a shorter time required to recover after surgery.

Functional and oncologic outcomes after partial nephrectomy in anatomical solitary kidney / 中华泌尿外科杂志

Objective To investigate the effect of partial nephrectomy of anatomical solitary kidney on tumor control and renal function preservation.Methods 10 patients with anatomical solitary kidney underwent partial nephrectomy from December 2007 to May 2016,including 6 males and 4 females ranged from 38 to 71 years old with an average of 57 years old.The PADUA score ranged from 6-10,with an average of 7.6.The tumor diameter ranged from 1.0 to 7.4 cm,with an average of 3.2cm.Results 7 cases underwent laparoscopic surgery.3 cases underwent open surgery.Mean operation time was 168min (ranged 103-217min).Mean blood loss was 135ml (ranged 20-400ml).The renal arteries were blocked in all patients.The ischemia time ranged from 9 to 33min,with an average of 20.6min.The mean estimated glomerular filtration rate at one week after surgery [40.7ml/(main · 1.73 m2)] decreased 44.7％ compared with pre-operation [73.6 ml/(min · 1.73 m2)].Acute kidney injury developed in 2 patients.One required temporary dialysis.The other improved after conservative treatment.There were no other complications such as urinary fistula and hemorrhage.The pathology analysis confirmed that 2 cases were angiomyolipoma,one case was benign cyst,6 cases were renal clear cell carcinoma,and one case was multilocular cystic renal cell carcinoma.4 cases were stage pT1a,one case was stage pT1b,one case was stage pT2a,and one case was stage pT4.Positive margin was found in one case.The average follow-up period was 28.2 months(ranged 3-58months).Only one patient need long-term dialysis 20 months after surgery at the latest follow up.Another patient had local recurrence and ipsilateral adrenal metastasis.Conclusions Satisfactory effect of tumor control and renal function preservation can be achieved by partial nephrectomy in solitary kidney,which prevent or delay the occurrence of hemodialysis effectively.

A rare case of renal hydatidosis in a child with congenital solitary kidney

Hydatid cyst of a solitary congenital kidney is a rare entity because of the small percentage of cases with renal hydatidosis and the reduced number of cases with this renal anomaly. We report a case presenting this extremely rare combination and having a favorable outcome. The diagnosis was confirmed based on an association of imagistic techniques and positive serology. The case was managed using a minimal invasive surgical technique (PAIR) that reduced the operative risks. Additionally, an antihelminthic agent (albendazole) was administered. To our knowledge, this is the first case with such comorbidity and treated through percutaneous approach.

Solitary kidney with renal artery aneurysm : one case report with literature review / 中华泌尿外科杂志

Objective To summarize the treatment experience of the solitary kidney with renal artery aneurysm.Methods Clinical data of 1 solitary renal artery aneurysm patient treated in our hospital in May.2012 was retrospectively analyzed.The 48 yrs man with a history of hypertension for 10 years,but responded to medical treatment poorly.The highest blood pressure was up to 180/100 mmHg (1 mmHg =0.133 kPa).Preoperative check found that the serum creatinine was 64 μmol/L.Color Doppler ultrasound showed no detection of left kidney,the renal artery enlarged to the range of 21 mm× 14 mm.CT scan showed that the right renal artery locally enlarged,suggesting artery aneurysm.Renal computerized tomography angiography showed that left renal agenesis,right renal with artery aneurysm close to the renal hilum with size of 17.1 mm× 19.1 mm,was located on the distal renal artery bifurcation,involving 2 artery branches.The diagnosis was right solitary kidney with renal artery aneurysm.Renal aneurysm clip occlusion was performed under general anesthesia,blood pressure was controlled to 70/40 mmHg during the procedure.After exposing the renal artery,the renal artery was visible with a diameter of 25 mm and with a basement width about 19 mm,which located in the main renal artery and overrode the branches of the two renal arteries.After the clamp of the artery aneurysm from two sides to center and the middle part of it was overlapped,the artery aneurysm reduced obviously,and clipped the aneurysmal wall with noose suture.When loosen the clamp of main renal artery,the artery aneurysm was not enlarged,blood pressure up to 120/80 mmHg.Results The operation was successful,operation time was 75 min,intraoperative blocking time was 15 min,intraoperative blood loss was 50 ml,and there was no intraoperative and postoperative complication.Postoperatively immediate and the first postoperative day serum creatinine were 95 μmol/L and 150 μmol/L.2 week after surgery the index decreased to 74 μmol/L.After operation the blood pressure was control successful,and kept at 130/80 mmHg.Conclusions Solitary kidney with renal artery aneurysm is rare.Renal aneurysm clip occlusion is a safe,effective and feasible treatment option,especially for the patients with solitary kidney.

Clinical characteristics and prognosis of congenital solitary kidney combined with renal damage in children / 中华实用儿科临床杂志

Objective To analyze the clinical characteristics and the prognosis of congenital solitary kidney in 9 children and to find the causes of renal impairment and factors associated with the prognosis.Methods The clinical data of 9 children with congenital solitary kidney in Beijing Children's Hospital Affiliated to Capital Medical University were analyzed,retrospectively.Data of long-term prognosis were collected through follow-up study.Results Five girls and 4 boys were involved in the analysis,ranging from 3.9 to 16.0 years old.The mean inpatient age was 9.9 yearsold.The patients showed different symptoms in renal impairment of solitary kidney:9 patients with proteinuria,and 3 of them had manifestation of asymptomatic proteinuria,in the early stage,3 with abnormalities of renal functions,3 diagnosed as nephrotic syndrome,and 2 with purpuric nephropathy.In the ultrasound examination of renal morphology,4 cases had normal echo image,5 cases had enhanced echo image and 2 patients had developmental malformation of the solitary kidney.The patients received follow-up visits within 1.5 to 7.0 years.The disease deteriorated into chronic kidney disease (stage 4-5) in 3 patients,1 patient underwent dialysis treatment,1 patient developed consistent and progressive proteinuria;whereas proteinuria disappeared in 4 patients and renal functions remained normal.Conclusions The renal impairment of congenital solitary kidney is mainly manifested as proteinuria,without symptoms in the early stage.In some patients,renal impairment can deteriorate into abnormalities of renal function or renal failure.The early ultrasound screening of the urinary system is recommended.Long-term follow-up should be conducted in children of solitary kidney to find early renal impairment and take appropriate treatment for them,thus to delay the occurrence and deterioration of renal impairment.