ABSTRACT
Kidney transplant is the best therapeutic option for patients with end-stage kidney disease. However, kidney transplant is not exempt from postoperative complications. One of the most frequent urological complications is lymphocele, which can appearin up to 20% of patients. Lymphocele most often appears during the first month after surgery. However, its appearance after the first yearis completely infrequent. Here, we report a case of a giant idiopathic lymphocele 18 years after kidney transplant and its resolution with lymphatic embolization.The patient, a 34-year-old man who received a deceased-donor kidney transplant in 2002, had presented with no complications until the lymphocele was diagnosed. The lymphocele presented as a voluminous organ-compressing mass. A percutaneous drainage was placed, and 3600 cm3 of lymphatic fluidwere drained.Afterthat, 800 cm3 continued to leak every day. An intranodal lymphography and lymphatic embolization with Lipiodol Ultra-Fluide (Guerbet Australia) were performed, owing to the high amount of leakage. At 50 days after embolization, an ultrasonograph showed no fluid collections, so the percutaneous catheter was removed. In most patients, the treatment ofthe lymphocele after kidney transplant is frequently conservative. However,for patients whose situation cannot be resolved spontaneously, there are few therapeutic choices. As described here, intranodal lymphatic embolization is a mini-invasive option, with a success rate of up to 80%, and should be offered as the first approach.
Subject(s)
Kidney Transplantation , Lymphocele , Adult , Drainage/adverse effects , Ethiodized Oil , Humans , Kidney Transplantation/adverse effects , Lymphocele/diagnostic imaging , Lymphocele/etiology , Lymphocele/therapy , Male , Postoperative Complications/etiology , Retrospective Studies , Treatment OutcomeABSTRACT
BACKGROUND: Fabry disease is an X-linked lysosomal storage disorder caused by α-galactosidase enzyme deficiency. We present clinical, biochemical, and histologic findings in children with classical phenotypic presentation of Fabry disease. METHODS: A retrospective analysis was performed using charts from 14 children with confirmed diagnosis. Clinical parameters were evaluated. Globotriaosylsphingosine -lysoGb3- detection in plasma, podocyturia, and kidney biopsy were carried out in all cases. RESULTS: All patients except one demonstrated at least one symptom of Fabry disease. LysoGb3 levels were above the normal range in all patients. Podocyturia was documented in all patients. Kidney biopsy revealed glomerular, interstitial, vascular, and tubular changes on light microscopy in nearly all patients. Electron microscopy showed podocyte inclusions in all patients. CONCLUSIONS: No difference in symptomatology was discernible between boys and girls. Podocyturia was detectable in children serving as a possible early marker of kidney injury. LysoGb3 was elevated in all cases, emphasizing the importance for diagnosis especially in female patients with normal αGal A activity. A possible association between lysoGb3 and symptom severity and histological involvement in kidney biopsy should be assessed in prospective studies with enough statistical power to determine if lysoGb3 can be used to predict nephropathy in children with Fabry disease.
