Heart Transplantation in a Patient Without a Heart: A Case Report

We report a heart transplantation in a patient with no heart. A 60-year-old man suffered from severe infective endocarditis, and due to extensive involvement of the myocardium, only minimal myocardium was left after debridement of the necrotic myocardium and aortic annulus. We finally excised the entire heart to eradicate the infection source and employed 2 extracorporeal membrane oxygenations for full life support. The infection was controlled with strong antibiotics. The patient underwent successful heart transplantation 16 days following the excision and the patient fully recovered without any complications.     

Successful Combined Heart and Kidney Transplantation in Patient With Fabry's Disease: A Case Report

Fabry’s disease is a X-linked hereditary disease that causes the accumulation of glycosphingolipids in tissues and organs, including the kidneys and heart. This can result in both chronic kidney disease and cardiac dysfunction, including arrhythmias and heart failure. We describe a case of a 62-year-old male with Fabry’s disease undergoing successful combined heart and kidney transplantation for chronic renal failure and low-output systolic heart failure. The patient has normal cardiac function and normal renal function 7 years after transplantation, while being maintained on enzyme replacement therapy with recombinant human alpha-galactosidase A. Fabry’s disease is not a contraindication for organ transplantation, even in patients presenting with both renal failure and heart failure.     

Kidney Transplantation After Hematopoietic Cell Transplantation in Plasma Cell Dyscrasias: Case Reports

The plasma cell dyscrasias (PCDs) include a number of entities such as multiple myeloma, primary amyloidosis, and monoclonal immunoglobulin deposition disease. Hematopoietic cell transplant (HCT) is the only cure for a variety of hematologic and oncologic diseases. Clinically significant renal impairment is a common feature in plasma cell myeloma, affecting 20% to 55% of patients at initial diagnosis; 2% to 3% of patients present with failure sufficiently severe to require hemodialysis.This circumstance is associated with a high early mortality. The necessity for immunosuppression after HCT could complicate its management and may precipitate the development of complications. In some patients an effective alternative could be kidney transplant (KT); however, the presence of 2 transplants will require optimal adjustment of immunosuppression and management of complications.At present, there are few published cases of KT after HCT, and the experience of managing 2 transplants is limited. We would like to describe our experience with 4 patients who had a PCD and initially received HCT and received subsequent KT.In our experience the progress and outcome of KT after HCT were optimal. We would like to address that a higher incidence of cytopenia associated with the combination of immunosuppression (lenalidomide, tacrolimus, mycophenolate, etc.) and other drugs (ie, valganciclovir) should be considered together with an increased risk of opportunistic infections and PCD relapse.     

Long-term Survival in a Kidney Transplantation Patient With Progressive Multifocal Leukoencephalopathy: A Case Report

Post–kidney transplantation progressive multifocal leukoencephalopathy (PML) is a rare disease on which there are very few published reports on record. PML is a demyelinating disease caused by a destructive infection of the oligodendrocytes by the JC polyomavirus. No effective therapeutic protocol has been established other than measures to revive the immune function by reducing or discontinuing the administration of immunosuppressive agents. Most cases are progressive and show a poor prognosis. We herein report a case in which renal function has been maintained for 2 years following the onset of PML, which was initially diagnosed 3 years after kidney transplantation.     

Successful Treatment of Cutaneous Blastomycosis in a Renal Transplant Patient With BK Virus Infection: A Case Report

This is the first case report from Turkey to describe a renal transplant complicated by subcutaneous blastomycosis and BK infection. The cutaneous lesions were successfully treated with amphotericin B and fluconazole. The presence of BK infection led to graft failure. Infections with uncommonly seen organisms should be kept in mind due to the impaired T-cell immunity in transplantation.     

Liver Transplantation Following Life-threatening Abdominal Trauma: A Case Series of 5 Patients at a Single Institution

Managing traumatic liver injury (TLI) is always challenging and demands precise clinical judgment. Currently, treatment of TLI in most circumstances is non-operative; however, surgical therapy might be required for severe TLI, particularly those that result in extensive blood loss. In the current institutional study carried out from June 1995 to April 2017, we describe our experience with 5 patients who received an orthotopic liver transplant for severe TLI. One patient passed away postoperatively from cerebral edema; 1 patient died of renal failure 4 years after the liver transplantation, and 3 patients are still alive. Based on our experience, we conclude that in patients with TLI, especially those with uncontrollable bleeding or those who develop liver failure, liver transplantation should be taken into consideration.     

Orthotopic Kidney Transplantation in an Elderly Patient With Various Severe Comorbid Conditions: A Case Report

In recent years, the frequency of high-risk kidney transplantations has increased. We report a case in which a 72-year-old man with various severe comorbidities (prostate cancer, diabetes mellitus, complete atrioventricular block, coronary artery stenosis, severe stenosis of the popliteal arteries, and severe calcification of the iliac arteries) who received an orthotopic kidney transplantation. To prevent the occurrence of acute limb ischemia due to the steal phenomenon (caused by the kidney graft), we decided that a heterotopic kidney transplantation involving the iliac arteries was not an appropriate option. Therefore, as an alternative, left native nephrectomy was performed followed by an orthotopic kidney transplantation to the native renal artery and renal vein through a left subcostal incision. Postoperative ureteral stenosis occurred, and so stent exchange was required every 6 months. Despite the ureteral complication, the patient's serum creatinine level was 1.5 mg/dL at 2 years after the procedure.     

Living Donor Kidney Transplantation in a Patient With Epidermolysis Bullosa: A Case Report

Severe recessive dystrophic epidermolysis bullosa is a very rare inherited disease with excessive blisters forming starting at birth. Surgical intervention in this population creates a challenge: preventing formation of new lesions while managing previously scarred tissues.We present a case of a 27-year-old patient with end-stage renal disease caused by rapidly progressive IgA nephropathy. Living donor kidney transplantation was performed under local, spinal and epidural anesthesia.Living kidney transplantation in epidermolysis bullosa patients with end-stage renal disease should not be a contraindication for transplantation and should be considered as a viable and feasible option after careful preparation.     

Living Kidney Donor With Small Lymphocytic Lymphoma at the Time of Donation: A Case Report

Living kidney donor guidelines recommend that donors in whom a malignancy is diagnosed should be excluded. Although preoperative screening for malignancies was performed, we experienced a case of living donor with small lymphocytic lymphoma (SLL) at the time of donation. A 53-year-old woman was referred to our hospital for a kidney donation to her son. She had no past medical history of malignancy. We screened the patient using medical examinations, but there was no obvious presence of malignancy. Although preoperative computed tomography showed a small lymph node swelling at the left renal hilum, we diagnosed it as an insignificant lymph node. When a laparoscopic donor nephrectomy was performed, however, we recognized the small lymph node during the surgery and performed a lymphadenectomy. Postoperatively, pathologic examination showed that the small node was lymphocytic lymphoma, known as a low malignant potential disease. Currently, there is no presence of malignancy transmission with the recipient. To the best of our knowledge, this is the first case report of living kidney donor with SLL. Although SLL is considered a low-grade malignancy, it is crucial to follow it carefully in both the donor and the recipient.     

Recurrent Membranous Nephropathy After Kidney Transplantation Associated With Phospholipase A2 Receptor and Successfully Treated With Rituximab: A Case Report

Primary membranous nephropathy (MN) is an organ-specific autoimmune disease mainly caused by autoantibodies acting against the podocyte antigen M-type phospholipase A2 receptor 1 (PLA2R). Herein we present the clinical and histologic findings, including PLA2R staining, of early recurrent MN after kidney transplantation that was successfully treated with rituximab.A 60-year-old Japanese man had end-stage renal failure due to steroid-resistant primary MN and underwent ABO-incompatible living donor kidney transplantation. At 1 month after transplantation, a protocol biopsy revealed positive granular staining of IgG, C4d, and PLA2R on glomerular capillaries (GCs) without any abnormalities on light microscopy (LM). Although the patient had low-level proteinuria, recurrent MN was suspected based on the positive PLA2R staining; he was treated with an angiotensin receptor blocker and a single dose of 200 mg rituximab. However, proteinuria gradually increased to 877 mg/d. At 21 months after transplantation, a graft biopsy revealed spikes along the outer aspects of GC on LM, with stronger staining for PLA2R than that at 1 month after transplantation. A single dose of 500 mg rituximab was added, which effectively reduced proteinuria, and clinical remission continued until 3 years after transplantation. The latest graft biopsy showed reduced staining of PLA2R. The disease activity and therapeutic effect were well-reflected in the intensity of PLA2R staining.An approach intending an early diagnosis by protocol biopsy using PLA2R immunostaining is made and early treatment with rituximab will help reduce the risk of kidney graft loss due to recurrent primary MN.     

Clearance of Hepatitis C Virus Prior to Lung Transplantation: A Case Report

Hepatitis C virus (HCV) continues to be considered a relative contraindication to lung transplantation due to concerns of progression of liver disease with the introduction of immunosuppression. Since the recent introduction of effective antiviral therapy for HCV, new approaches in the management of the HCV-positive recipient are being utilized in liver transplantation to clear HCV pre- and post-transplant. Herein, we report use of ledipasvir/sofosbuvir for HCV clearance prior to lung transplantation in a patient with usual interstitial pneumonia. Listing for transplant was delayed until completion of HCV treatment, and he subsequently required extracorporeal membrane oxygenation as a bridge to transplantation due to progressive hypoxia. With antiviral cure rates exceeding 90%, HCV should no longer be considered a relative contraindication to lung transplant, and timing of antiviral treatment should consider the progressive nature of the recipient's lung disease.     

Successful Treatment of Fibrosing Cholestatic Hepatitis With Daclatasvir and Asunaprevir After Liver Transplantation: A Case Report

Background

Fibrosing cholestatic hepatitis (FCH) is an aggressive form of hepatitis C virus (HCV) recurrence after liver transplantation (LT). Most FCH cases are fatal, occurring as a secondary disease following rapidly progressive liver dysfunction and graft failure. We report a case of early-onset FCH after LT that was successfully treated using daclatasvir and asunaprevir.

Severe Late-Onset Acute Cellular Rejection in a Pediatric Patient With Isolated Small Intestinal Transplant Rescued With Aggressive Immunosuppressive Approach: A Case Report

Small intestinal transplantation is performed for patients with intestinal failure who failed other surgical and medical treatment. It carries notable risks, including, but not limited to, acute and chronic cellular rejection and graft malfunction. Late severe acute intestinal allograft rejection is associated with increased risk of morbidity and mortality and, in the majority of cases, ends with total enterectomy. It usually results from subtherapeutic immunosuppression or nonadherence to medical treatment. We present the case of a 20-year-old patient who underwent isolated small bowel transplant for total intestinal Hirschsprung disease at age 7. Due to medication nonadherence, she developed severe late-onset acute cellular rejection manifested by high, bloody ostomy output and weight loss. Ileoscopy showed complete loss of normal intestinal anatomic landmarks and ulcerated mucosa. Graft biopsies showed ulceration and granulation tissue with severe architectural distortion consistent with severe intestinal graft rejection. She initially received intravenous corticosteroids and increased tacrolimus dose without significant improvement. Her immunosuppression was escalated to include infliximab and finally antithymocyte globulin. Graft enterectomy was considered repeatedly; however, clinical improvement was noted eventually with evidence of histologic improvement and salvage of the graft. The aggressive antirejection treatment was complicated by development of post-transplant lymphoproliferative disorder that resolved with reducing immunosuppression. Her graft function is currently maintained on tacrolimus, oral prednisone, and a periodic infliximab infusion. We conclude that a prompt and aggressive immunosuppressive approach significantly increases the chance of rescuing small bowel transplant rejection.     

Short-term Use of Ventricular Assist Device in a Patient With Restrictive Cardiomyopathy: A Case Report

The CentriMag (Levitronix LLC, Waltham, MA, USA) ventricular assist device is a centrifugal pump designed for short-term support in patients with cardiogenic shock. In patients with restrictive physiology, there is some concern about the use of these devices due a very small ventricular cavity. We review the evolution of a 32-year-old woman with restrictive cardiomyopathy, moderate pulmonary hypertension, and severe biventricular systo-diastolic dysfunction in whom a biventricular Centrimag device was implanted due to a progressive clinical worsening.     

Budd-Chiari Syndrome Associated With Hypereosinophilic Syndrome Treated by Deceased-Donor Liver Transplantation: A Case Report

IntroductionBudd-Chiari syndrome (BCS) associated with hypereosinophilic syndrome (HES) is very rare, and only a few reports have described its treatment. Furthermore, no report to date has described the performance of liver transplantation for the treatment of BCS associated with HES. We herein describe a 54-year-old man who underwent deceased-donor liver transplantation (DDLT) for treatment of BCS associated with HES.CaseA 54-year-old man was found to have an increased eosinophil count during a medical check-up. After exclusion of hematopoietic neoplastic diseases and secondary eosinophilia, idiopathic hypereosinophilia was diagnosed. Oral prednisolone was administered to the patient, and his eosinophil count immediately decreased to a normal level. He had an uneventful course without complications for 11 months but then presented with bloating and malaise. Imaging studies including ultrasonography, enhanced computed tomography, and angiography revealed BCS associated with HES. Transjugular intrahepatic portosystemic shunt failed because of complete obstruction of the hepatic veins. Therefore, the patient was introduced to our hospital for liver transplantation. DDLT was performed with venovenous bypass 1 month after the patient was placed on the DDLT waiting list. The explanted hepatic veins were completely occluded and organized. The patient’s eosinophil count was maintained at a normal level with prednisolone treatment after DDLT.ConclusionsLiver transplantation can be a treatment option for BCS associated with HES if neoplastic diseases and secondary eosinophilia have been excluded. Life-long oral steroid therapy is required to control HES even after liver transplantation.     

Successful Treatment for BK Virus Nephropathy by Leflunomide in a Kidney Transplant Patient: A Case Report

IntroductionThe immunosuppressant agents in kidney transplantation (KT) may lead to various complications such as opportunistic infections and malignancies. BK virus associated nephropathy is a significant complication following KT, and it can result in graft failure. BK virus causes tubulointerstitial nephritis, ureter stenosis, and even graft failure in KT recipients with impaired immune system. We described a 63-year-old woman, who was a hepatitis C carrier and on dialysis for 22 years before KT, who received cadaveric-donor KT 2 years previously. She reported decreasing urine output and general weakness. The serum creatinine level was slightly increased from 2.94 to 4.38 mg/dL.MethodsImmunosuppressant medications including prednisolone, everolimus, cyclosporin, and mycophenolate sodium were continued as maintenance therapy post KT. Kidney biopsy was performed due to deterioration of graft function.ResultsThe kidney biopsy showed consistent results with early-stage polyomavirus nephropathy, characterized by focal viral cytopathic changes with positive immunohistochemical signals and mesangial proliferative glomerulonephritis, immune-complex-mediated (Fig 1 and Fig 2). Negative C4d staining at peritubular capillary was reported. The dosage of mycophenolate sodium was tapered from 720 to 360 mg daily and that of everolimus increased from 0.5 to 1.0 mg daily due to BK viral infection with BK nephropathy. The serum creatinine level was 2.75 mg/dL after treatment.ConclusionEarly detection of BK nephropathy and decreasing immunosuppressant agents are the mainstay of treatment. Substituting leflunomide for mycophenolate sodium and increasing dosage of everolimus has been proposed to solve BK nephropathy. We presented that the use of leflunomide in such situation is in a timely manner.     

Early Focal Segmental Glomerulosclerosis as a Cause of Delayed Graft Function With Combined Acute Antibody Rejection: A Case Report

Focal segmental glomerulosclerosis (FSGS) is the most common form of post-transplant glomerulonephritis. We describe a case where a biopsy proved that early recurrence of FSGS on postoperative day 1 was the cause of delayed graft function. A 39-year-old man, on hemodialysis for 15 years due to polycystic kidney disease, received a cadaveric renal transplantation. On postoperative day 1, his hourly urine output decreased from 700–800 mL to 50 mL. The graft biopsy showed a mild acute kidney injury confusing nephrotic syndrome. On postoperative day 45, his creatinine level increased to 3.02 mg/dL with severe proteinuria. A kidney biopsy showed focal segmental glomerulosclerosis. On postoperative day 120, his creatinine level elevated again, concomitant with proteinuria. A kidney biopsy showed FSGS with antibody-mediated rejection. After plasmapheresis, his creatinine level decreased to 1.3 mg/dL with mild proteinuria. Once active in the allograft, de novo FSGS is a potentially aggressive process. In this case, it could be managed because of an accurate diagnosis and appropriate treatment.     

Therapeutic Problems and Pregnancy in a Patient With Infantile Nephropathic Cystinosis: A Case Report

Background

Cystinosis is a rare genetic disorder characterized by the abnormal accumulation of cystine in the lysosomes of various tissues and organs leading to their dysfunction. The most common type is the infantile nephropathic cystinosis which without treatment leads to renal failure and before the introduction of cysteamine was the cause of death before puberty.

Aortobisiliac Bypass Using a Venous Homograft Concomitant With Kidney Transplantation in a Patient With Severe Bilateral Iliac Occlusive Disease: A Case Report

Aortoiliac occlusive disease (AOD) is a great threat for kidney transplantation (KT). Here we report the case of an aortoiliac bypass, performed simultaneously with renal transplantation using venous grafts obtained from the deceased donor. The recipient was a 68-year-old woman with significant stenosis of the aortoiliac axis. We performed an aortobisiliac bypass using donor’s femoral veins because presence of methicillin-resistant Staphylococcus aureus was detected on donor hemoculture and contraindicated a prosthetic implant on the recipient. KT was then carried out using standard technique. Operative time amounted to 330 minutes and cold ischemia time of the renal graft was 900 minutes. Delayed graft function was observed until postoperative day 12, but the patient showed a good urine output and a serum creatinine of 2.1 mg/dL at discharge. AOD is not an absolute contraindication to renal transplantation, and simultaneous surgical repair of aortoiliac lesions with KT seems feasible. The patient’s return to function after initial delayed graft function suggests that such interventions may allow transplantation to be offered to those patients who otherwise may be excluded for severe vascular comorbidities. Homologous vascular grafts are an excellent choice because prosthetic vascular replacement during immunosuppression must be avoided as long as possible, especially in patients with coexisting infective risk.