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.

Principles of renal transplantation in the dog: A review.

The practically relevant body of knowledge available on renal transplantation in the dog is reviewed. A review of the history of renal transplantation is followed by a detailed description of the immunological fundamentals of transplantation, with especial regard to the structures directing rejection, the significance of D-LA-typing and the MLR test serving for their determination, and the possible methods of nonspecific and specific immunosuppression. The most important criteria of recipient and donor selection are presented, together with the main aspects and medicinal protocol of prednisolone-azathioprine and prednisolone-cyclosporin A immunosuppression within the framework of preparation for the surgical intervention. Among the surgical aspects of renal transplantation, first the recommended anaesthetic techniques are outlined, then the three stages of the surgical procedure, i.e. donor nephrectomy, renal perfusion and renal grafting, are described in detail. The last chapter of the article presents the physical, laboratory and instrumental diagnostic methods of rejection diagnosis, with special regard to their practical importance and different roles played in signalling the rejection.

Clinical renal allograft transplantation in a Dalmatian dog: case report.

A case of successful renal allograft transplantation performed in a two-year-old female Dalmatian dog suffering from end-stage chronic renal failure is reported. A one-year-old male German shepherd with severely injured spinal cord was used as kidney donor. Simultaneous kidney allograft transplantation combined with hypothermic initial perfusion as graft conservation was done, placing the donor kidney into the right iliac fossa of the recipient. The immunosuppression protocol consisted of prednisolone and azathioprine. Regular physical, laboratory, ultrasonographic and scintigraphic examinations were used for assessing both the morphology and the function of the allograft. After a two-week period of hospitalisation the patient was discharged in a remarkably improving condition. The recipient died on postoperative day 45 of respiratory insufficiency resulting from secondary pneumonia and pulmonary oedema. Neither macroscopic nor microscopic abnormalities of the allograft were revealed by necropsy.

Experimental vascular graft for liver transplantation.

Hepatic artery thrombosis is a major cause of graft failure in liver transplantation. Use of donor interponates are common, but results are controversial because of necrosis or thrombosis after rejection. Reperfusion injury, hypoxia and free radical production determinate the survival. The aim of the study was to create an 'ideal' arterial interponate. Autologous, tubular graft lined with mesothelial cells, prepared from the posterior rectus fascia sheath, was used for iliac artery replacement in eight mongrel dogs for six months under immunosuppression. Patency rate was followed by Doppler ultrasound. Eight grafts remained patent and another two are patent after one year. The patency rate was good (median Doppler flow: 370 cm/sec) and there was no necrosis, thrombosis or aneurysmatic formation. The grafts showed viable morphology with neoangiogenesis, appearance of elastin, smooth muscle and endothelial cells. Electron microscopy showed intact mitochondrial structures without signs of hypoxia. Tissue oxygenation was good in all cases with normal (< 30 ng/ml) myeloperoxidase production. In conclusion, this autologous graft presents good long-term patency rate. Viability, arterialisation and low thrombogenicity are prognostic factors indicating usability of the graft in the clinical practice without the risk of rejection. Further investigations such as cell cultures and standardisation are necessary.

Experimental autologous substitute vascular graft for transplantation surgery.

Vascular complications in liver transplantation are a major cause of graft failure and mortality. The aim of the study was to create autologous vascular graft without risk of rejection. Posterior rectus fascia sheath lined with peritoneum was used for iliac artery replacement in seven mongrel dogs. The patency was followed by palpation and Doppler ultrasound. The grafts were removed after one month. Five grafts remained patent. The Doppler showed good, relatively increased flow (median flow rate: 383 cm/sec) after one month in all of the cases. Slight increase in diameter was present in all cases. By microscopy the five patent grafts showed viable morphology, fibroblasts, smooth muscle cells and thin fibrin layer in the wall. The grafts were lined partially with a neoendothelial monolayer and a thin fibrin layer. In conclusion, this graft presents an acceptable patency rate and low thrombogenicity, and could be useful in transplantation. Further investigations are needed to study the effect of immunosuppression and rejection on long-term morphology and patency of the grafts.

[Pathologic evaluation of orthotopic liver transplantation in Hungary]. / A hazai májtranszplantációk értékelése patológiai szempontból.

A total of 81 orthotopic liver transplantations were performed on 74 patients between January 1995 and December 1999 at the Department of Transplantation and Surgery of the Semmelweis University in Budapest. Indication for transplantation was liver cirrhosis in 57 cases, 10 patients were transplanted due to fulminant liver failure, while 7 patients underwent transplantation because of liver metastasis of different semimalignant tumours. During the above period, retrospective studies on 205 pre- and posttransplantation liver biopsies, 74 explanted livers, 7 explanted liver grafts and 22 autopsy cases were performed at the First Institute of Pathology and Experimental Cancer Research of the Semmelweis University in Budapest. A number of 116 protocol biopsies (dates as zero time, 7th day, 6th month and 12th month) and 73 non-protocol biopsies (taken due to liver allograft dysfunction) were analysed. Different gradings of acute rejection--characterised by trias of portal inflammation, venous endothelitis and bile duct damage--were detected in 62 cases. Chronic rejection occurred in 7 patients, with 4 cases of vanishing bile duct syndrome and one of the case of foam cell arteriopathy, add to 2 cases of chronic rejection characterized by undetermined bile duct damage. The present study includes the evaluation of 22 autopsy cases according to liver transplantation in Hungary, with the finding that liver allograft insufficiency was the main cause of mortality. Authors conclude that pathomorphological analysis has an important role in relation to liver transplantation.

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).

Central opiate receptor blockade by naloxone impairs thalamic and hypothalamic autoregulation in the cat.

Experiments were carried out in order to determine the effects of centrally induced opiate receptor blockade on the autoregulation of the regional cerebral blood flow (rCBF, measured by the H2-gas clearance technique) of the anesthetized, ventilated cats. General opiate receptor blockade was induced by intracerebroventricularly (i.c.v) injected naloxone. Changes of teh lower limit of hypothalamic, thalamic and white matter blood flow autoregulation were studied by reducing the systemic mean arterial pressure (MAP) in a stepwise manner to 80 mmHg, 60 mmHg and 70 mmHg by hemorrhage. Centrally administered naloxone (10 microg/10 microl/kg, i.c.v) resulted in an abolishment of the autoregulatory mechanisms in the hypothalamic and thalamic regions but caused no such changes in the white matter. These observations suggest that 1.) intracerebral opiate receptors and/or central opioid peptiderg mechanisms play a significant role in the maintenance of the thalamic and hypothalamic blood flow autoregulation during hemorrhagic hypotension and 2.) the involvement of the endogenous opioid peptide system in rCBF autoregulatory mechanisms is regionally different.

Experimental investigation of preservation injury in animal kidneys after reperfusion with Euro-Collins.

The authors evaluated the pathomorphologic alterations of removed and reperfused dog kidneys by means of light and electronmicroscopic examination. In each sample the following reversible signs were found: Hypereosinophilia (HE), Hydropic dystrophy (HD), Nuclear polymorphism (NP), Epithelial desquamation (ED), Brush border lesion (BBL), Single cell necrosis (SCN), Total tubular epithel necrosis (TTEN), Interstitial edema (IE), Perivascular edema (PE). The irreversible signs were: Basement membrane rupture (BMR), Cellular infiltration (CI), Glomerular mesangial matrix expansion (GME) and vascular lesions (VL). The most severe and mostly irreversible alterations occur in the 54-72 hours after harvesting. The authors emphasize the significance of basement membrane rupture, because the impossibility of tubular epithelial regeneration, the cellular infiltration due to its fibrogenic effect, glomerular lesion because it makes decrease the glomerular filtration rate, proceeding juxtaglomerular cell proliferation and hypertension through renin-angiotensin mechanism and vascular lesions causing renovascular hypertension and tubulopathy. The authors believe that reperfusion injury is very important factor in kidney allograft survival. Its mechanism is similar to the normal necrosis pathway, but the timing is delayed. Further investigations are needed to understand what specific alterations may occurred under blood circulation in the host to reveal more exact cause of primary graft failure after transplantation.

Endogenous opioid mechanisms in hypothalamic blood flow autoregulation during haemorrhagic hypotension and angiotensin-induced acute hypertension in cats.

The influence of naloxone-induced general opiate receptor blockade on hypothalamic blood flow autoregulation was investigated in anaesthetized, artificially ventilated, temperature controlled cats. In order to study the changes of the hypothalamic blood flow (H2-gas clearance technique) at the lower limit of autoregulation systemic arterial pressure was reduced in a stepwise manner to 100, 80, 60 and 40 mmHg, by haemorrhage. Autoregulatory mechanisms of the hypothalamic vessels remained effective and hypothalamic blood flow showed no significant reduction until the arterial pressure was reduced to 60 mmHg in the vehicle-treated control cats. General opiate receptor blockade by 1 mg kg-1 mL-1 i.v. injected naloxone resulted in a significant reduction of the autoregulatory capacity of the hypothalamic vessels: the blood flow followed passively the arterial pressure fall already from 100 mmHg mean arterial pressure. The effect of opiate receptor blockade on the upper limit of the autoregulation was studied during acute arterial hypertension, induced by angiotensin-II infusion (25 micrograms 0.1 mL-1 min-1 i.v.). Hypothalamic blood flow remained remarkably steady following angiotensin-II infusion in the saline-treated control animals. Naloxone pretreatment (1 mg kg-1 mL-1 i.v.), however, induced a significant downward shift of the upper limit of the autoregulation, and hypothalamic blood flow started to increase in the 125-145 mmHg arterial pressure range. The narrowing of the autoregulatory range following general opiate receptor blockade suggests an important role of endogenous opioid peptides in hypothalamic blood flow autoregulatory mechanisms both in hypotensive and in hypertensive conditions.

Evaluation of urinary enzymes in renal allograft damages by histology examination.

Acute allograft rejection (ARE) is one of the most current problem in kidney transplantation. Urinary enzymes (glutathione-S-transferase /GST/. dipeptidil-dipeptidase /DPP/) are frequently used as prognostic factors of ARE. The authors compared the results of light microscopic study (by Banff scheme), and the GST, and DPP secretion in acute rejection. The correlation between the laboratory, and histology findings wasn't significant. our results suggest that both GST, and DPP are very sensitive, but less specific indicators in ARE, since their activity increases in many other damages as well.