The impact of surgical intervention on renal function in cystinuria / O impacto da intervenção cirúrgica sobre a função renal na cistinúria

ABSTRACT Introduction: Cystinuria is an autosomal recessive disorder due to intestinal and renal transport defects in cystine and dibasic amino acids, which result in recurrent urolithiasis and surgical interventions. This study aimed to assess the impact of surgical interventions on renal function by analyzing estimated glomerular filtration rates. Methods: Thirteen pediatric patients with cystinuria, who were followed-up in a single tertiary institution between 2004 and 2016, were included in the study. Medical records were reviewed to collect data on clinical presentation of patients, urine parameters, stone formation, medical treatment, surgical intervention, stone recurrence after surgical procedure, stone analysis, ultrasonography, 99m-technetium dimercaptosuccinic acid (99mTc-DMSA) radionuclide imaging results, and follow-up time. Creatinine clearances estimated by modified Schwartz (eGFR) formula before and after surgery were used to assess renal function and compared statistically. Results: Nine patients (69.2%) had renal scarring which were detected with 99mTc-DMSA radionuclide imaging. In ten patients (76.9%), open surgical intervention for stones were needed during follow-up. Significant difference was not detected between eGFR before and after surgical intervention (mean 92 versus 106, p = 0.36). Nine of the patients (69.2%) were stone free in the last ultrasonographic examination. Relapses of stone after surgery were seen in 66.6% of patients who underwent surgical intervention. Conclusions: Surgical interventions for urinary stones are commonly required in patients with cystinuria. Renal scarring is a prevalent finding in cystinuric patients. Surgical interventions have no negative impact on eGFR in patients with cystinuria according to the present study.

RESUMO Introdução: A cistinúria é um distúrbio autossômico recessivo causado por defeitos de transporte intestinal e renal da cistina e aminoácidos dibásicos que resultam em urolitíase recorrente e necessidade de intervenção cirúrgica. O presente estudo teve por objetivo avaliar o impacto das intervenções cirúrgicas sobre a função renal por meio da análise da taxa de filtração glomerular estimada. Métodos: Treze pacientes pediátricos com cistinúria acompanhados em uma instituição terciária entre 2004 e 2016 foram incluídos no estudo. Os prontuários médicos foram analisados e utilizados como fonte de dados sobre a apresentação clínica dos pacientes, parâmetros urinários, formação de cálculos, tratamento clínico, intervenção cirúrgica, recidiva de cálculos após procedimento cirúrgico, análise de cálculos, ultrassonografia, resultados de imagens com ácido dimercaptossuccínico marcado com tecnécio metaestável (99mTc-DMSA) e tempo de seguimento. A depuração de creatinina estimada pela fórmula modificada de Schwartz (TFGe) antes e após a cirurgia foi utilizada para avaliar e comparar estatisticamente os níveis de função renal. Resultados: Nove pacientes (69,2%) apresentaram cicatrizes renais detectadas por exame de imagem com 99mTc-DMSA. Dez pacientes (76,9%) necessitaram intervenção cirúrgica aberta por cálculo renal durante o seguimento. Não foram detectadas diferenças significativas entre os valores de TFGe anteriores e posteriores à intervenção cirúrgica (média de 92 vs. 106, p = 0,36). Nove pacientes (69,2%) não apresentaram cálculos no último exame ultrassonográfico. Recidivas de cálculos renais após cirurgia foram observadas em 66,6% dos pacientes submetidos a cirurgia. Conclusões: Intervenções cirúrgicas relativas a cálculos renais são frequentemente necessárias em pacientes com cistinúria. Cicatrizes renais são um achado prevalente em pacientes com cistinúria. De acordo com o presente estudo, cirurgia não afeta negativamente a TFGe de pacientes com cistinúria.

Xanthogranulomatous Pyelonephritis due to calculi: report of 63 cases and review of literature

To examine the relationship between clinical history and results of renal investigations in patients with xanthogranulomatous pyelonephritis. A retrospective review was conducted on 63 cases presenting with histopathological diagnosis of xanthogranulomatous pyelonephritis between 1995 to 2002, at the department of Urology, Bolan Medical College and Sandeman Provincial Teaching Hospital Quetta. There were 26 males and 37 females having an average follow up of two years. Positive findings on examination and investigations at presentation were fever and flank pain in 59 [93.6%] patients and pyuria in 34 [53.9%] patients. All the patients had renal and ureteric calculi with no or severe reduction in the function of the affected kidney on DTPA scan. Hypertrophy of the contra lateral kidney was seen in 56[88.8%] patients. Xanthogranulomatous pyelonephritis is a common entity in this part of the world. Late referral leads to loss of the kidney. Pre-operative diagnosis of the condition is desirable and if surgery is mandatory then all infected tissues have to be removed

Absceso perinefrítico, revisión de casos. Servicio de Urología HEALF 1992- 2003 / Abscess perinefrítico, revision of cases. Service of Urología HEALF 1992 - 2003

,l presente estudio es una revisión de 34 casos de pacientes con abscesos perinefríticos, manejados por el servicio de urología del Hospital Escuela Antonio Lenin Fonseca, entre enero de 1992 y diciembre del 2003. Los resultados del estudio se discuten con relación a los hallazgos de revisiones de casos expuestos en la literatura internacional. El objetivo general del estudio es: Describir las características generales de:Epidemiología, cuadro clínico, agentes etiológicos, diagnostico, tratamiento y evolución de los pacientes con absceso perinefrítico, manejados por el servicio de urología del Hospital Escuela Antonio Lenin Fonseca entre 1992 y el 2003. La edad promedio de los pacientes estudiados fue de 45.9 años, oscilando entre 28 y 73 años. La relación entre hombres y mujeres es de 1:1.2. difiriendo significativamente de la mayoría de los reportes que es de 3:1. (7, 9,11 y 13) La litiasis urinaria y la diabetes mielitus figuran como los principales factores que favorecen la aparición del absceso perinefrítico con 72 porciento, asociadas a obstrucción urinaria que representó el 62.5 porciento de los pacientes. Los signos y síntomas, fueron de tipo general e inespecíficos, dificultando él diagnostico clínico y retrasando el tratamiento. El diagnostico bacteriológico del absceso perinefrítico es infrecuente, al 43.7 porciento de los pacientes no se les practico ningún tipo de estudios bacteriológicos. Las bacterias aisladas tanto de urocultivos, como de cultivos del pus fueron: Klepsiela, E. Coli y Proteus

Study on the complication of urinary stones in Thuy Van commune, Huong Thuy district, Thua Thien Hue province

In the study of 43 cases of urinary stones in aldults in Thuy Van commune, Huong Thuy district, Thua Thien Hue province showed that: Hydronephrosis was 27.3%, level III examined by renal ultrasound was the highest rate of 23.3%, three cases of urinary stones had the complication of Hydronephrosis III grade. Urinary tract infection with nitrite and white blood cell (+) in urine was 18.6%. Chronic renal failure was 16.3% with the most was level I (9.3%). Hematuria was 18.6% and red blood cell in urine was the highest (25.6%).

La nueva y vieja tecnologia en el tratamiento de la litiasis urinaria / Old and new tecnology in treatment of urinary lithiasis

Se presenta una revisión del manejo de la urolitiasis a la luz de las diferentes tecnologías disponibles. El autor expresa su aproximación y experiencia personal en el manejo del paciente con litiasis, enfatizando en la disponibilidad de las diversas tecnologías, especialmente en ciudades intermedias y hace una invitación a los colegas al trabajo en equipo, optimizando los conocimientos individuales para beneficio del paciente

Unenhanced ultrafast computerized tomography for the evaluation of patients with acute flank pain.

OBJECTIVE: To determine the value of unenhanced ultrafast computerized tomography (CT) in the diagnosis of acute flank pain in 43 patients evaluated for suspected stone disease. MATERIAL AND METHOD: Noncontrasted ultrafast CT was performed in 43 consecutive patients seen in the emergency department to evaluate acute flank pain. All CT studies were reviewed for the presence of ureteral and renal calculi, perinephric and periureteral stranding, presence and degree of pelvicalicectasis or other radiological findings. If necessary, an excretory urogram was performed to confirm the presence or absence of urinary stone. Patients were followed to determine clinical outcome including the need for urological intervention. RESULTS: Of the 28 patients determined to have stones 16 (57.14%) had spontaneous stone passage, 7 (25%) had improved symptoms without documented stone passage and 4 (14.29%) required surgical intervention. In 6 of 14 patients (42.86%) with negative CT readings for stone disease a diagnosis was established by other intra-abdominal findings. In 7 patients (50%) no clinical diagnosis could be established, and 1 scan in a patient with a ureteral calculus was interpreted as falsely negative. These findings yielded a sensitivity of 96.63 per cent, Specificity 92.85 per cent and overall accuracy 95.24 per cent for diagnosing ureteral stones. CONCLUSIONS: Unenhanced ultrafast CT is an accurate, safe and rapid imaging modality for the detection of urinary tract calculi and obstruction. The majority of patients required no further imaging to determine the need for urological intervention. Ultrafast CT could be used as the standard method to evaluate patients with acute flank pain.

Hiperparatiroidismo primario, aislado y de carácter familiar: caso clínico / Familial and isolated primary hyperparathyroidism: report of an affected family

Familial hyperparathyroidism can be a part of a type 1 or 2 multiple endocrine neoplasia syndrome, can be associated to mandibular fibromas or can appear as an isolated disease. We report a family with 11 members affected by a primary hyperparathyroidism, all with a history of kidney stones and without evidences of other endocrine tumors. Not knowing the familial history of the disease, only one adenoma was resected in four cases and in all, the disease recidivated. Two were operated again, performing a total parathyroidectomy and heterologous autotransplantation of parathyroid tissue in the forearm. The presentation form of primary hyperparathyroidism in this family, is similar to other reported cases. It is more aggressive, is diagnosed at a lower age, has a higher incidence of recurrence and multiglandular involvement than the sporadic disease

Hematuria en niños: análisis de la casuística en un centro de referencia nacional, Valencia, Venezuela / Haematuria in children: analysis of the etiology and characteristics in a national reference center, Valencia, Venezuela

Hematuria es una manifestación frecuentemente encontrada en la práctica clínica pediátrica. El objetivo central del presente trabajo es reportar las características clínico-epidemiológicas de 362 niños con hematuria atendidos durante el periodo junio 1998 a mayo 1999 en nuestra institución; esta cifra correspondió al 1,1 por ciento de todas las consultas y admisiones pediátricas y al 8.4 por ciento de las correspondientes a nefrología pediátrica para el período estudiado. El promedio de edad fue de 7,7 ñ 6,1 años, rango 0-17 años, 56 por ciento varones y 44 por ciento hembras. 62 por ciento presentaba hematuria microscópica y 38 por ciento macro y microscópica. La etiología de la hematuria para el grupo total fue: hipercalciuria idiopática y otras alteraciones metabólicas 23,5 por ciento, nefritis aguda 19,3 por ciento. infección documentada de vía urinarias 19 por ciento, urolitiasis 16 por ciento, malformaciones congénitas del tracto urinario 8,3 por ciento "hematuria primaria" 4,4 por ciento síndrome nefrótico 2,2 por ciento, hipoxia neonatal 1,6 por ciento, traumatismos del tracto urinario 1,4 por ciento, nefropatía por IgA 1,4 por ciento y otras 2,9 por ciento. El grupo etario más afectado fue el de los preescolares (34,3 por ciento), seguido por los escolares (27,3 por ciento), luego lactantes, preadolescentes y adolescentes y recién nacidos. Se especifican las causas de hematuria para cada grupo etario y las probables causas que expliquen la frecuencia de determinadas patologías en nuestra área geográfica. La presente casuística, eminentemente descriptiva, muestra la frecuencia, etiología y otras características de la hematuria en clínica nefrológica pediátrica en un centro de referencia y abre la posibilidad de estudios comparativos

Transitional cell carcinoma of the ureter and struvite calculi

Context: The association of primary carcinoma of the ureter and lithiasis is extremely rare. We report a rare case of a primary carcinoma of the ureter with corariform calculus. Case Report: 60-year-old phaeodermal female, reported a history of right-side nephritic colic, hyperthermia and pyuria during the past 20 years andhad received treatment for urinary infections a number of times. The first clinical presentation was related to lithiasis and the tumor had not been shown up by excretory urography, cystoscopy or ultrasonography. Two months after the calculus had been eliminated, the patient began to have serious symptoms and a grade III transitional cell carcinoma of the ureter was discovered. Total nephroureterectomy and M.V.A.C. (Metrotrexate + Vinblastina + Doxo Rubicina + Cisplatina) chemotherapy were tried unsuccessfully. In this report we emphasize the diagnostic difficulty caused by the concomitant presence of the two pathologies. In our opinion, the rapid evolution in this case is directly related to the high grade of the tumor.

Haematuria as the presenting symptom: experience from a tertiary referral centre in Sri Lanka.

OBJECTIVES: To examine the causes of macroscopic (gross) haematuria in patients where it is the main presenting symptom and to evaluate the role of cystoscopy in their investigation. DESIGN: Prospective audit. SETTING: One urology unit at the National Hospital of Sri Lanka, Colombo. PATIENTS: 174 consecutive new patients with macroscopic haematuria, where haematuria was the presenting symptom. OUTCOME MEASURES: Causes of macroscopic haematuria in the under 40 years and over 40 age groups; role of cystoscopy. RESULTS: Of the 156 (130 male and 26 female) patients evaluated for macroscopic haematuria 67 were under the age of 40 years and 89 over 40. The causes of macroscopic haematuria in the under 40s in descending order of frequency are urinary stone disease, idiopathic and inflamed urothelium. In the over 40 group bladder cancer was the commonest cause, affecting 31.5% of patients, followed by stone disease. CONCLUSIONS: Macroscopic haematuria in a patient over the age of 40 years could be due to bladder cancer and merits cystourethroscopy. In younger patients, urolithiasis is the predominant cause.

Rotura espontánea de cáliz renal. Presentación de dos casos / Spontaneous rupture of renal calix. Report of two cases and literatura review

Se presentan dos casos de rotura de cáliz renal, trastorno cuya causa no se conoce, en dos varones jóvenes, sin antecedentes de trastornos urinarios. Una rotura ocurrió en el lado izquierdo, y en el otro paciente se produjo en el derecho. Se trataron de manera conservadora, y los resultados fueron excelentes

Papel del hueso en la fisiopatologia de la hipercalciuria idiopatica: efecto del aminobisfosfonato alendronato / Role of the bone in the physiopathology of idiopathic hypercalciuria: effect of amino-bisphosphonate alendronate

Estudios previos de nuestro laboratorio han demonstrado una disminución del contenido mineral del hueso y una correlación entre la disminución del contenido mineral y la producción de distintas citoquinas que intervienen en el proceso de resorción ósea. Al mismo tiempo, observamos que el tratamiento a corto plazo con alendronato produce una disminución del calcio urinario en pacientes con hipercalciuria idiopática. En el presente estudio analizamos los efectos del alendronato a largo plazo (10 mg/día por un año) sobre el calcio y la hidroxiprolina urinaria y el contenido mineral óseo en 18 hipercalciúricos y 8 normocalciúricos con litiasis urinaria. Las características clínicas, así como la distribución por edades y sexo fue similar en ambos grupos. En calcio urinario disminuyó significativamente al final del primer mes y continuó bajo posteriormente (277 + 28, antes vs. 202 + 26 mg/g creatinina, después de 12 meses con alendronato, p<0.01). La hidroxiprolina urinaria disminuyó significativamente durante el estudio (125,5 + 32.1 vs. 39.66 + 17.5 mg/g creatinina, p<0.05). El calcio sérico, la filtración glomerular y el sodio urinario no se modificaron durante el estudio. La densidad mineral ósea en columna lumbar, determinada por densitometría por rayos X, se incremento significativamente el primer año de 1.162 + 0.231 a 1.197 + 0.248 g/cm2 (p<0.01). No se observaron cambios en la densidad mineral del cuello de fémur. Estos cambios se asociaron a una disminución en la transcripción del mRNA para IL-1 alpha, determinados por la reacción en cadena de polimerase (PCR), en células mononucleares no estimuladas. Los sujetos normocalciúricos no demostraron cambios significativos en la excreción urinaria de calcio. En resumen, los cambios observados en el calcio urinario y otros parámetros metabólicos óseos sugieren un papel importante del hueso en la fisiopatología de la hipercalciuria idiopática.

The usage of Andrographis paniculata following Extracorporeal Shock Wave Lithotripsy (ESWL).

One hundred consecutive cases with renal stones less than 3 cm in size and normal renal function underwent ESWL from January to March 1994. Out of these, 50 were given Andrographis paniculata tablets (250 mg), 4 tablests tid, 25 were given cotrimoxazole 2 tablets bid and 25 received norfloxacin 200 mg bid, started immediately after ESWL and continued for 5 days. All tolerated the treatment well and none had complications. At one month follow-up, pre- and post-ESWL pyuria, hematuria and proteinuria among the Andrographis paniculata group were 84, 58, 72, 40, 52, 22 per cent; the cotrimoxazole group 88, 64, 84, 64, 56, 44 per cent and the norfloxacin group 92, 56, 72, 40 per cent and 56, 28 per cent respectively. The results showed that post ESWL pyuria and hematuria in patients receiving Andrographis peniculata were reduced to 0.69 and 0.55 time of pre ESWL value. We think that this herbal medicine is beneficial in the treatment of post ESWL urinary tract infection. Besides the herbal drug given to eighteen previously sulfa sensitized patients resulted in no allergic reaction.

Is hypocitraturia associated with phosphaturia-a potential cause of calcium urolithiasis in first-time stone formers

The serum and 24 hour urinary excretion levels of various lithogenic and inhibitory substances were assessed in 24 male patients with calcium stone and no previous history of urolithiasis and in 19 age-matched controls. Two groups did not differ significantly [P<0.01] except in the excretions of sodium, citric acid [being higher in normals] and inorganic phosphate [being higher in patients]. Fifty percent patients had hyperphosphaturia, 29.2% hypocitraturia, 20.8% hyperoxaluria and 16.7% hypercalciuria. The present data suggests that hypocitraturia in association with phosphaturia might be one of the main risk factors responsible for calcium urolithiasis in this area

Analgesia y sedación endovenosa para litotripsia extracorpórea / Endovenous analgesia and sedation for extracorporeal lithotripsy

Los autores analizan el procedimiento anestésico empleado en 100 procedimientos consecutivos de litotripsia extracorpórea utlizando el Sonolith 3000. Se aplicó un protocolo prospectivo de sedación y analgesia con Midazolam y Fentanila, en 92 pacientes, 61 hombres y 31 mujeres, con una edad promedio de 41 años. Se obtuvo analgesia satisfactoria en el 94 por ciento de los casos, siendo el tiempo necesario para obtener analgesia de 10 minutos. El período de recuperación postanestésica fue de 50 minutos en promedio. Las complicaciones fueron mínimas. La técnica de sedación y analgesia con Midazolam y Fentanila es simple de realizar, con rápido inicio de acción, exenta de complicaciones significativas y con una rápida recuperación