Mycophenolate mofetil versus Rapamycin in Han: SPRD rats with Polycystic Kidney Disease

Objective: We aimed to explore the effect of Mycophenolate mofetil (MMF) on loss of renal function and cyst progression compared to rapamycin in Han: SPRD rats. We also sought to assess whether the effect of combination therapy of MMF plus rapamycin was better than that of monotherapy. Methods: Sixty heterozygous (Cy/+) and littermate control (+/+) male Han: SPRD rats were weaned at 4 weeks of age, then divided into four groups randomly to receive different treatments by intragastric administration for 2 months: vehicle-treated group as control, MMF-treated group (20mg/kg/day), rapamycin-treated group (2mg/kg/day), and MMF+Rapa- treated group (MMF 20mg/kg/day plus Rapamycin 2mg/kg/day). Resulls: After 2 months of treatment, rapamycin caused a 22 percent decrease in body weight in comparison to the control group, whereas MMF had no significant effect on weight gain. The steady increase of BUN in Cy/+ rats was reduced by 15 percent in MMF-treated Cy/+ rats. However, rapamycin and combination therapy reduced BUN by 42 percent and 43 percent, respectively. CCr was 0.93±0.11ml/min in vehicle-treated Cy/+ rats, 1.67±0.23 ml/min in MMF-treated Cy/+ rats (P<0.05), 1.72±0.44 ml/min and 1.83±0.21 ml/min in rapamycin- and MMF+Rapa-treated Cy/+ rats, respectively (.P<0.01). Cyst volume density was 57.1 percent in vehicle-treated Cy/+ rats, 45.2 percent in MMF-treated Cy/+ rats (P<0.05), 32.9 percent and 37.7 percent in rapamycin- and MMF+Rapa-treated Cy/+ rats, respectively (P<0.01). MMF markedly ameliorated interstitial inflammation and fibrosis. Rapamycin showed a similar effect on interstitial inflammation and fibrosis, but to a lesser degree. Conclusion : MMF is more tolerable than rapamycin and can retard deterioration of renal function in Han: SPRD rats, though its effect is weaker than that of rapamycin. Combination therapy does not exert more favorable effect than monotherapy.

Clinical Characteristics of Renal Transplant Recipients that Underwent Urologic Surgery for de novo Disease Before and After Transplantation

The pre-transplantation goal of the urologist is the optimization of urinary tract condition. Therefore, urologic surgery may be needed before or after renal transplantation. We analyzed the results of urologic surgery performed because of de novo urologic diseases. Between January 1986 and January 2001, 281 patients underwent renal transplantation, and 23 urologic surgical procedures were performed on 21 transplant recipients before or after renal transplantation because of de novo urologic diseases. By review the major reasons for urologic surgery in recipients were polycystic kidney diseases, vesicoureteral reflux, and dysfunctional voiding disorders. Nineteen surgical corrective procedures were done average 2.9 months before transplantation. The mortality rate was 10.5%. Four patients underwent urologic surgery at an average 57.5 months after transplantation. We highlight the fact that patients with uremia are vulnerable to surgical complications, and conclude that more intensive longterm urologic follow-ups should be conducted on recipients.

Multicystic nephroma--report of two cases.

Multicystic Nephroma (MCN) is an uncommon renal pathology, characterized by the presence of usually unilateral circumscribed focal lesion consisting of multiple thin walled cysts. The etiology and pathogenesis of multicystic nephroma is not clear, and it is considered as a neoplastic lesion by many authors. To the best of our knowledge it has not yet been reported from India. We report first two pediatric cases of MCN from India. Computerized Tomography (CT) scan in both the cases revealed a unilateral cystic lesion in the lower pole of kidney. Keeping in mind the age, clinical presentation and radiological appearance, a possibility of Wilm 's tumour with cystic change could not be ruled out preoperatively and both children underwent nephrectomy. Since MCN has a benign behaviour it must be differentiated from focal cystic neoplastic lesions, including Cystic Partially Differentiated Nephroblastoma (CPDN), which has a low but distinct capability for local recurrence, and from Wilm's tumour with cystic change. Segmental form of unilateral renal dysplasia also needs to be considered in the clinical and radiological differential diagnoses.

Benign multilocular cystic nephroma.

This article reports a case of cystic nephroma to bring awareness about the benign nature of this condition. The patient presented with a painless abdominal mass. Computed tomography showed a homogeneous, multicystic tumor of the superolateral portion of the left kidney with thin septa without solid parts. Histology confirmed the diagnosis of cystic nephroma.

Spectrum of cystic variants of Wilm's tumour: cystic nephroma (multilocular cyst) and cystic partially differentiated nephroma--a report of four cases.

Two cases of cystic nephroma (multilocular cyst of the kidney), and one case each of cystic partially differentiated nephroblastoma (CPDN) and rhabdomyomatous Wilms' tumour are described. All were male and in the pediatric age group. Grossly tumours were unilateral, unicentric and multiloculated. The need for proper designation of these lesions is highlighted because of difference in the treatment and prognosis of these tumours.

Displasia renal multiquística: presentación de tres casos / Three cases of multicystic dysplastic kidney disease

Se informan tres casos de displasia renal multiquística en fetos y neonatos autopsiados en el Instituto Anatomopatológico de Caracas, Venezuela, durante el período comprendido entre 1994 y 1995. Para cada caso se consignó la información gineco-obstétrica disponible de la madre, más no así, los antecedentes familiares de patología renal. Dos de los casos fueron diagnosticados como displasias renales multiquísticas bilaterales; el tercer correspondió a una displasia renal multiquística asociada a una agenesia del riñon y uréter contralaterales. Se comparan los resultados obtenidos con aquellos hallazgos descritos en la literatura mundial. Se discuten además, investigaciones recientes sobre la importancia diagnóstica y pronóstica de la ultrasonografía prenatal, la progresión o no de las lesiones renales, y su patogenia multifactorial, con especial énfasis en los factores genéticos asociados.

Meckel-Gruber syndrome: a lethal combination of abnormalities

We herein describe two cases of Meckel-Gruber Syndrome identified in stilborn infants. Both had all three elements of the classical triad, namely, occipital encephalocele, renal cystic dysplasia and post-axial polydactyly. In addition, many of the other well-known accompanying abnormalities were present. Awareness of this entity in this region is important because of its high risk of recurrence in subsequent pregnancies

Diagnóstico prenatal del síndrome de Meckel-Gruber / Prenatal diagnostic of Meckel-Gruber syndrome

Se diagnosticaron por ecosonografía dos casos con el síndrome de Meckel-Gruber, caracterizados por microcefalia con encefalocele occipital, riñones poliquísticos y polidactilia. Se comenta su frecuencia, asociación con otras malformaciones. Se discute el valor de la alfa-fetoproteína en el suero y el líquido amniótico. Enfatizamos la necesidad del consejo genético y de la valoración ecográfica, debido a la racurrencia de un 25% de este síndrome

Displasia renal multicística total bilateral: apresentaçäo de 1 caso / Total bilateral multicystic renal dysplasia: report of a case

Apresenta-se um caso de Displasia Multicística Total Bilateral tecendo consideraçöes sobre os aspectos macro e microscópicos desta entidade. Säo discutidas as lesöes císticas renais ocorrendo no grupo pediátrico

Rins policísticos e displasia renal na infância: classificaçäo e patogenia / Polycystic kidneys and renal dysplasia in infancy: classification and pathogenesis

O presente trabalho compreende estudo anatomoclínico retrospectivo de 19 casos de crianças portadoras de rins policísticos e displasia renal (18 autópsias e uma biópsia); foram revistos os dados clínicos e a presença de malformaçöes e analisados os achados macro e microscópicos dos rins (classificaçäo de Potter). Os casos foram subdivididos segundo a distribuiçäo e freqüência das malformaçöes observadas, salientando-se a presença de dois grandes distintos sob o ponto de vista morfológico e patogenético: Grupo 1 (oito crianças), apresentando rins policísticos e displásicos (Tipos II e III) associados a malformaçöes sistêmicas múltiplas, ambos provavelmente conseqüentes a um mesmo agente agressor, atuante em diferentes fases da embriogênese e Grupo 2 (sete crianças), apresentando rins policísticos e displásicos (Tipo IV) associados a malformaçöes do trato urinário inferior. Postula-se neste grupo a açäo patogenética da obstruçäo e refluxo vesicoureteral na gênese da lesöes renais

Rim policístico em natimorto: apresentaçäo de um caso / Policystic kidney in stillbirth: report of a case

Apresenta-se um caso de rim policístico em natimorto e discutem-se duas classificaçöes anátomo-patológicas em razäo da doença policística infantil e doença policística do adulto se apresentarem com alteraçöes anatômicas e funcionais variadas, recebendo denominaçöes diferentes, imprecisas e confusas