Patrizia Tulissi

Preemptive Liver–Kidney Transplantation in Von Gierke Disease: A Case Report

Type 1a glycogen storage disease (GSD 1a), or von Gierke disease, is a rare, autosomal-recessive disease caused by a deficiency of glucose-6-phosphatase, which leads to glycogen accumulation in the liver, kidney, and intestinal mucosa. Clinical manifestations include hypoglycemia, growth retardation, hepatomegaly, lactic acidemia, hyperlipidemia, and hyperuricemia. Long-term complications include renal disease, gout, osteoporosis, pulmonary hypertension, short stature, and hepatocellular adenomas, which may undergo malignant transformation. Herein we have described the management and the clinical course of a GSD1a patient who underwent simultaneous preemptive liver- kidney transplantation (SPLKT), which solved the liver and renal disease. We confirmed the rapid normalization of glucose metabolism, and correction of hyperlipemia after liver transplantation. In our opinion uremic patients with GSD 1a with or without adenomas must be considered for SPLKT. To our knowledge this is the fifth case of SPLKT and the first preemptive one to be described in the literature.

De novo gastrointestinal tumours after renal transplantation: Role of CMV and EBV viruses

Abstract:  The development of new and more effective immunosuppressive agents has provided long‐term survival for transplant recipients, thereby increasing the risk of de novo malignancy in chronic immunocompromised hosts. While de novo post‐transplant lymphoproliferative diseases and skin cancer has been shown to have an increased incidence in long‐term surviving solid organ transplant recipients, the association with gastrointestinal (GI) cancer is controversial. Over 12 yr, 20 patients (5%) out of 395 renal transplant recipients developed 23 de novo tumours; 11 skin cancer and 12 non‐skin cancer. Four patients (1%) developed de novo tumours of the GI tract (three colon, and one gastric cancer). Immediately after tumours diagnosis, immunosuppressive therapy was reduced; all patients were shifted from cyclosporine to Rapamicine within 30 d. The tumour was surgically resected with curative intent in three cases, while one patient had only palliative surgery because of metastatic disease. The post‐operative courses was uneventful. All patients maintained normal graft function. However, three out of four patients (75%) died of progression of the neoplasm, within a median time from the diagnosis of 12 months. Further, we investigated a possible correlations between de novo GI cancer and HCV, HBV status, infections, cytomegalovirus (CMV) and Epstein–Barr virus (EBV) reactivation, episodes of rejection, and blood transfusions. All cases with GI de novo cancers reported in this paper developed CMV and EBV reactivation within three months after transplantation. Thereafter we suggest a closer follow‐up for de novo GI cancer in renal transplants with early CMV and EBV reactivation in order to avoid delayed diagnosis.

Detection of transplant renal artery stenosis with contrast-enhanced ultrasound

Transplant renal artery stenosis (TRAS) is a vascular complication occurring during the first 2 years after kidney transplantation, with an incidence and a prevalence ranging from 1% to 23%, and from 1.5% to 4%, respectively. Detection of TRAS is the key, since most stenoses may progress to renal graft loss, however it may be difficult to detect due to its nonspecific clinical manifestations. Although Doppler ultrasound has become a primary imaging technique, digital subtraction angiography (DSA) remains the gold standard for diagnosing TRAS. We present a case of delayed graft function following kidney transplantation complicated by a lateral by-pass with prosthesis upstream and downstream of renal anastomosis, TRAS criteria were unclear using Doppler ultrasound, contrast-enhanced computed tomography-scan, and DSA. Only contrast-enhanced ultrasound (CE-US), observing a delayed and pulsating contest impregnation of renal parenchyma, supported the hypothesis of TRAS that was confirmed by the measurement of trans-anastomosis pressure gradient during DSA.

Ureterovesical Anastomosis and Urinary Infections after Kidney Transplantation: Two Techniques Comparison

Objective: Urinary infections developing after kidney transplantation may depend on the type of ureterovesical anastomosis performed. Patients and Methods: A randomized prospective study was performed on 56 patients, from October 2004 to March 2006, receiving kidney transplants from cadaveric donors to compare 2 types of ureterovesical anastomosis. We considered the number and types of urinary infections, the duration of their treatment, and their complete/ partial resolution during the first year after transplantation. Twenty-eight patients (group A) underwent ureterovesical anastomosis according to the Lich-Gregoir technique, the other 28 patients (group B) using the Knechtle method. The 2 groups were comparable in terms of donor and recipient characteristics. Results: The mean duration of the period of antibiotic treatment was 17 ± 11 days in group A and 15 ± 7 days in group B (p = 0.63), while the intravenous antibiotic treatment lasted a mean 11 ± 6 days in group A and 10 ± 3 days in group B (p = 0.54). The antibiotic treatment completely resolved the urinary infection in all cases treated. No grafts were lost due to complications of urinary infections. Conclusion: Our data revealed no statistically significant differences between the 2 types of ureterovesical anastomosis considered in terms of the prevalence of infections or graft survival during the first year of follow-up.