Bourneville-Pringle disease for kidney transplantation: a single-center experience.

The Bourneville-Pringle disease is an autosomal-dominant disease affecting the kidneys in about 60%, causing end-stage renal disease in about 10% of the cases. Among more than 2800 renal transplant recipients during the last 33 years, we had two patients with this original disease. A third patient who underwent bilateral nephrectomy is currently awaiting a graft. The first patient was diagnosed at the age of 20 years after a few episodes of retroperitoneal bleeding. At the age of 26 years her left kidney was removed after a rupture; it measured 7500 g, and the histology described angiomyolipomatosis. A year later she underwent a cadaveric kidney transplantation. Subsequently her right kidney was removed due to bleeding. She is currently 5 years posttransplant with stable kidney function and good health. Our second patient was nephrectomized at the age of 35 years and 38 years because of angiomyolipomatosis. She underwent a cadaveric kidney transplantation 7 years later. After 5 years of excellent kidney function and a year after her arteriovenous fistula was ligated her upperarm had to be amputated because of uncontrollable bleeding. After another 6 months, she displayed rapid progression of a jejunal tumor and during operation received 54 U of blood transfusion but died at the age of 49 years with a well-functioning graft. Our third patient consecutively underwent two nephrectomies because of angiomyolipomatosis of her kidneys at the ages of 25 and 28 years. She has two children with the same disease. In addition she carries Leyden mutation, which has caused deep venous thromboses and pulmonary emboli. She is currently on our waiting list for kidney transplantation. The Bourneville-Pringle disease is a rare indication for kidney transplantation; the prognosis of the patient is dependent on the original disease.

Mixed lymphocyte cultures (MLC) and acute pancreatitis after renal transplantation.

MLC was performed preoperatively in 35 out of 57 candidates for renal transplantation. Three patients developed acute pancreatitis in the early postoperative stage, 2 of them died. In these three cases MLC revealed complete non-reactivity of the recipient lymphocytes, compared with the donor as well as with the positive control lymphocytes. None of the other patients showed this phenomenon and none of them developed acute pancreatitis. It is suggested that the recipient's immune responsiveness may be involved in the aetiology of the production of acute pancreatitis after renal transplantation.

Herpes zoster and acute rejection crisis of renal homograft.

The case of a patient developing acute rejection crisis 8 months after transplantation in the prodromal stage of a herpes zoster infection is reported. The joint therapeutic measures resulted in suppression of rejection and control of the infection. Complications did not occur. The case suggests a definite association between the viral infection and acute homograft rejection. The case report is followed by a critical review of the pertinent literature.

[Availability of livers from cadaver donors in Hungary]. / Cadaver donorból származó máj elérhetösége Magyarországon.

The availability of cadaver livers for transplantation have been studied based on data recorded prospectively by authors. Between January 1, 1995 and August 31, 1995, 98 cadaver kidney donors were accepted in Hungary. Using well established selection criteria 52 of them could have been suitable for liver donation. Steatosis or cirrhosis of the donor livers have been considered most frequent causes of unsuitability for transplantation. Based on these data 70-75 cadaver livers could be transplanted yearly in Hungary that's enough to start a transplant program. The treatment of acute hepatic failure with transplantation seems to be uncertain because of unequal time and blood group distribution of donors. There are just few available cadaver livers for children therefore transplantation of reduced sized liver is needed.

[Haemodialysed, hemiparetic patient operated on for aortic aneurysm followed by successful renal transplantation]. / Vascularis encephalopathiában szenvedó krónikus hemodializált betegen végzett aortaaneurysma-mútétet követó vesetranszplantáció.

The authors present the case of a 58 years old male patient. According to his preceding record in 1991 he suffered speech disturbance and left-side hemiparesis due to multiplex vascular lesions. A fresh cerebral ictus caused a right side hemiparesis mainly in the upper limb in 1993 and sensomotororic aphasia has also been developed. In 1995 the patient was begun acute haemodialysis treatment due to his gravis uremic state, then his dialysis was continued 3 times 4 hours weekly. In 1997 the then 56 years old inveterate right-sided hemiparetic patient, treated with chronic haemodialysis requested to be put on the transplantation waiting list. The first thing that had to be done in case of this high-risk patient was the resection of the abdominal aortic aneurysm (38 mm x 67 mm x 115 mm in size) noticed at the ultrasound examination which was carried out in January 1997. The continuity of the vessel was secured by graft-interposition where the arteria mesenterica was also implanted. Following the successful operation, the patient was qualified for the transplantation list and in the February of 1998 a successful kidney-transplantation was carried out. Following the temporary, post-transplantation difficulties (post-operative 5th day acute rejection well-reacting to 3 steroid-shots; the two re-operations due to partial necrosis in the uretero-ureteralis anastomosis and successfully overcoming the Pseudomonas aeruginosa uroinfection) the patient is currently doing well and has no complaint.

[Qualitative and quantitative detection of hepatitis C virus RNA by PCR technique. Monitoring of viral copies after liver transplantation]. / Hepatitis C-vírus (HCV)-RNS kvalitatív és kvantitatív kimutatása PCR technikával. A HCV-RNS-kópiaszám monitorozása májtranszplantációt követóen.

The authors demonstrate the HCV nucleic acid amplification method is not wide-spread in Hungary yet. The HCV-RNA is usually detectable 2-4 weeks after infection independently the immunostate of the patients. The authors help to select the adequate measurement(s) in logical order when HCV infection is suspected. The benefit of the PCR method is emphasized. Monitoring of the HCV-RNA titer of the liver transplanted patients promotes to establish the fluctuation of HCV-RNA copies and the effectivity of therapy following transplantation. The detection of HCV-RNA by PCR method is a proof of an acute or chronic infection and rules out past infection. The quantitative PCR measurement is useful for determination of indication and control of efficacy of antiviral therapy.

Results and complications after living related kidney transplantation in Hungary: 30 years' experience.

The number of patients suffering from kidney disorders is increasing the need for kidney transplantation. Kidneys originating from living donors (LD) show substantially better results than those originating from cadaveric donors (CD). We performed 3000 kidney transplantations between November 1973 and December 2007, including 154 from LD (5.13%). The early kidney function as measured by the delta creatinine clearance was significantly better among the LD group (P < .001). There was no significant difference in the immunologic data between the LD and the CD groups (P = .047). Four years after transplantation the glomerular filtration rate (GFR) and the serum creatinine level treated to be better among the LD group with tacrolimus versus cyclosporine immunosuppression (P = .089). In the LD group, the acute rejection rate was lower with tacrolimus- versus cyclosporine based immunosuppression (P = .014).

Disseminated Kaposi sarcoma in immunosuppressed patients.

UNLABELLED: Tumour incidence was examined in kidney-transplanted patients receiving immunosuppressive therapy. Eight hundred and fifty immunosuppressed patients (mean age: 34.5 years; mean follow-up time: 67 months; men/women = 3/2), were followed up. Two cases of disseminated visceral kaposi sarcoma (K.S.) are reported in detail. RESULTS: long-term immunosuppression significantly raises the risk of tumour development (30/850); one must reckon with the appearance of visceral K.S. (2/850), which is exceptionally rare in the general population. CONCLUSION: the classical lower extremity cutaneous manifestation is fairly benign, it appears later and responds to radiotherapy well. The visceral form appears early (in 3-6 months), it is aggressive, progressing quickly. Only early diagnosis followed by immediate reduction or discontinuation of immunosuppression, helps successful oncological treatment.

Preventive OKT3 treatment with cyclosporine (Sandimmun) for second kidney transplantation.

A total of 793 kidney transplantations (KTx) were performed from November 1073 to March 1993. Two hundred and forty-two patients were treated with conventional immunosuppression (azathioprine + prednisolone) and all the others with cyclosporine (Sandimmun) and prednisolone (SIM + PRED). The survival of the second graft was less good in both therapeutic groups than that of the first ones, so we have started to use preventive immunotherapy with OKT3 (CILAG) in combination with SIM (both before operation) and PRED. We compared 32 SIM-PRED patients with 20 OKT3 + SIM + PRED patients. All underwent a second KTx. The two groups were found to be comparable and homogeneous with regard to 14 of 18 parameters analysed statistically. Statistically significant differences were found between the two groups as regards the frequency of acute rejection within 30 days (46.69% vs 20%), the delta creatinine value on the 1st and 2nd postoperative days (-4.3: -8 vs -8.6: -19.7%), patient survival after 4 years (78.2 vs 100%), and graft survival after 1 and 4 years (-58.9: -42.8 vs -83.5: -83.5%), with better results in the OKT3 group. We conclude that the preventive use of OKT3 simultaneously with SIM + PRED for the second KTx is the method of choice to prevent rejection and improve survival. This treatment results in patient and graft survival following the second KTx being as good as after the first KTx with SIM + PRED.

Benefits of cell saver during the operation of gastric haemorrhage following repeated pancreatitis.

Authors report on a case of repeated pancreatitis causing several characteristic complications and concluded to splenic vein thrombosis, with an enlarged perisplenic vein network. The source of gastrointestinal bleeding is uncommon: originates from the submucous vein of the stomach. Cell saver was used under splenectomy because of great blood loss, and due to hyperimmunization occurred as consequence of massive transfusion. The benefit of cell saver was clearly evident from both point of view.

Malignant tumours after renal transplantation.

The incidence of malignant tumours in 570 patients with kidney transplants was examined. It was found to be 20-30 times higher than in the average (normal) population. In accordance with the literature, mainly skin cancers were observed, but at variance with these data, the number of lymphoreticular malignancies was small. On the basis of their study, the authors emphasize the oncogenetic effect of immunosuppression. By comparing the conventional (AZA+PRED) and Cyclosporine (CYA+PRED) treatments, they point out that this risk should be taken into consideration even in Cyclosporine therapy which has otherwise a much more favourable effect.

Our preliminary results in application of Stoppa technique for recurrent groin hernia.

In 1994 we have started the Stoppa technique which was only used for the recurrent groin hernia repair, when Mersilene mesh was implanted in preperitoneal position from a lower midline incision. From March, 1994 to March, 1996 seventeen Stoppa procedures were performed in our department (14 men, 3 women, mean age of 66 years). Type of hernias: 13 unilaterale, 3 bilaterale recurrent groin hernias and 1 primary bilaterale groin hernia combined with a lower midline incisional hernia. Operations were performed by 7 surgeons. The mean time of the surgery was 85 min. (35-165). The mean postoperative stay in the hospital was 9 days (7-19). The Stoppa technique could be performed in all patients. Serious early postoperative complications didn't occur. The two late recurrences were observed amongst the first five patients. All implanted mesh were well tolerated by the patients. Author believe, this procedure has some advantages: the site of the incision is located in a new place from where the dissection is easier than in the previous incision, the stich fistulas from the previous operation can be avoided, furthermore this method could be applicated for combined hernias located in the lower part of abdomen and it is cheaper than the laparoscopic hernioplasty.

Echography of renal transplant patients.

Grey-scale echography has been found useful in the detection of rejection, dilatation of the renal cavity system and fluid collections around the renal transplant in 55 patients. Serial ultrasound examinations are essential for the diagnosis of acute rejection as demonstrated in 13 of 19 cases. Authors recommend the extension of ultrasonic examination to the upper abdominal organs and pelvis of renal transplant patients even in symptom-free cases, since in this way it is possible for early detection of occult abdominal diseases, which may jeopardize the success of surgery.