Long-term follow-up of renal transplantation in children: A 20-year experience at one kidney center in Japan

Background

We reviewed the results of renal transplantation in children who has received transplants between October 1973 and January 1984 at our center.

Methods

Twenty-six children, 4 to 15 years of age, underwent renal transplantation from livingrelated donors and were treated using conventional immunosuppressive drugs.

Results

Patient survival rates were 96% at 5 years and 88% at 10 years. Graft survival rates were 69% at 5 years and 45% at 10 years. Four patients died. The main cause of graft loss was chronic rejection. One patient lost the graft due to recurrence of the original disease. Henoch-Schönlein purpura nephritis. The graft was functioning at the end of follow-up in 7 patients, and their serum creatinine levels were all below 1.6 mg/dL at the last follow-up. Noncompliance with medication was confirmed in 4 patients. There was no uncontrollable hypertension observed in any case. Children younger than 5 years showed catch-up growth, but overal growth in these children with renal transplants without growth hormone therapy was not good.

Conclusion

The long-term prognosis of children who underwent living-related renal transplantation using conventional immunosuppressive drugs was favorable, compared to that of adults.     

Loss of BAP1 protein expression in the first metastatic site predicts prognosis in patients with clear cell renal cell carcinoma

Objectives

To investigate the intratumoral heterogeneity of BAP1 and PBRM1 expression at the primary site and metastatic sites and to evaluate whether BAP1 and PBRM1 expression in metastatic sites of clear cell renal cell carcinoma (ccRCC) has prognostic value.

Pulmonary cryptococcosis combined with pulmonary tuberculosis]

We report a rare combination of pulmonary cryptococcosis and pulmonary tuberculosis in a diabetic patient. A 63-year-old man was admitted to our hospital in January 2002 for evaluation of an abnormal chest radiograph. In 1999, the abnormality had first been detected by mass screening radiography. In 2000, an abnormality in a chest radiograph was again detected in a mass screening, and he visited another hospital. A chest CT scan revealed a cavitating lesion and several nodules in the left lower lobe. Fiberoptic bronchoscopy was performed, but was not diagnostic. The patient was referred to our hospital. He had a history of diabetes mellitus starting in 1984. The chest CT scan revealed solid nodules in the left lower lobe and several micronodules in both upper lobes. Video-assisted thoracoscopic surgery was performed and specimens were obtained from the left S8 and left S1 + 2. Histologically, cryptococci were detected in the resected left S8. In addition, mycobacterium tuberculosis was cultured from the resected left S1 + 2. A diagnosis of combined pulmonary cryptococcal and tuberculous infections was made and treatment with itraconazole, isoniazid sodium methansulfonate, rifampicin, and ethambutol hydrochloride was given.     

Time-course changes in clinical features of early-onset Japanese type 1 and type 2 diabetes: TWMU hospital-based study

Using a database of patients with type 1 (n=1675) and type 2 (n=2259) diabetes diagnosed before the age of 30 years at the Diabetes Center, Tokyo Women's Medical University (TWMU), in which such Japanese patients have been registered at the time of first visit since the 1960s, we performed a hospital-based study over the last 40 years to clarify time-course changes in clinical features of type 1 and type 2 diabetes diagnosed before the age of 30 years. Type 2 diabetes had a male dominancy, while there has been a female dominancy in patients with type 1 diabetes as in previous reports of Japanese childhood-onset type 1 diabetes. Such dominances had been continued over the last 40 years. The number of patients with type 2 diabetes and with a past history of obesity increased with time. The age at which type 2 diabetes was diagnosed was suggested to have been getting lower with time, whereas that of type 1 diabetes has been higher with time. There was no marked difference in family history of diabetes in the first-degree relatives of patients with type 2 diabetes, regardless of the presence or absence of a past history of obesity. More female patients with type 2 diabetes diagnosed before the age of 15 years had mothers with type 2 diabetes compared to corresponding male patients.     

Intraductal carcinoma in a surgically resected pancreas with chronic pancreatitis

A 59-yr-old Japanese male presented with epigastralgia. Endoscopic retrograde cholangiopancreatography (ERCP) revealed narrowing of the inferior common bile duct and protein plugs in the main pancreatic duct. He was diagnosed as suffering from chronic pancreatitis with suspicion of a pancreatic head tumor, and a pancreatoduodenectomy was performed. Histologically, a diffuse chronic pancreatitis was evident in the resected pancreas. Although no tumors were seen in the head portion of the pancreas around the inferior common bile duct, an intraductal carcinoma was found in the second branch of Santorini’s duct. Precancerous alteration of the duct epithelium, presenting papillary hyperplasia, and atypical hyperplasia were observed in areas continuous with the intraductal carcinoma. Immunohistochemically, carcinoembronic antigen (CEA) was specifically expressed in atypical hyperplasia and intraductal carcinoma, but not in papillary hyperplasia.     

Impact on mortality and incidence of end-stage renal disease of education and treatment at a diabetes center among patients with type 1 diabetes: comparison of two subgroups in the Japanese DERI cohort

The aim of this study was to compare mortality and incidence of end-stage renal disease (ESRD) in patients with type 1 (insulin-dependent) diabetes who had attended a diabetes center with those who had not. The cohort consisted of a total of 1430 patients diagnosed with Type 1 diabetes at 18 years or younger, and between 1965 and 1979. This population-based cohort in Japan was subdivided into two groups: patients who had visited a large diabetes center in Tokyo (n=162) and those who had not (n=1212). Mortality and incidence of ESRD were compared between the two subgroups as of January 1, 1990. Crude mortality was 1.95 per 1000 person-years (95% CI: 0.49-5.06) for those who had visited the center and 6.05 (4.86-7.41) for those who had not. A multivariate Cox proportional hazard model showed that the patients who had visited the center were three times less likely to die (hazard ratio: 0.31, 95% CI: 0.10-0.98) than those who had not. Crude incidence of ESRD was 1.32 (0.22-4.09) and 5.86 (4.65-7.26) for those who had visited the center and for those who had not, respectively. After adjusting for covariates, the patients who had visited the center were five times less likely to develop ESRD (hazard ratio: 0.19, 0.05-0.78) than those who had not. Education and treatment of type 1 diabetes with an integrated management system under specialists and a multidisciplinary team appears to be associated with a better prognosis.     

Role of Albumin and High-Density Lipoprotein as Endotoxin-Binding Proteins in Rats with Acute and Chronic Alcohol Loading

In the present study, the role of albumin and high-density lipoprotein (HDL) as endotoxin (Et)-binding proteins in chronically alcohol-fed rats was studied. In acute ethanol-loaded rats, the Et clearance in the blood was slightly prolonged, and the amount of albumin and HDL-bound Et in the blood was markedly increased. In chronic ethanol-loaded rats, the Et clearance was significantly faster than that in the control, and HDL-bound Et was increased. In the chronic ethanol-fed rats with an additional 5 g/kg body weight of ethanol load, the Et clearance was much prolonged, and blood tumor necrosis factor and ALT was elevated, when HDL-bound Et was not further increased. Et-binding capacity of total proteins, albumin, and HDL in the hepatocyte culture medium were increased when the Kupffer cells were preincubated in the medium containing ethanol, and the resultant culture supernatant was added to the hepatocyte culture system. In the culture experiment in the chronic ethanol-loaded rats, such increases were not observed. These results suggest that the increase in Et-binding capacity of HDL and albumin may serve as a protective mechanism against Et in chronic ethanol-loaded rats. An addition of high-dose ethanol to these rats may lead to impaired Et binding and inactivation, which may finally result in increased endotoxicity.     

Development of pure red cell aplasia by transmission and persistent infection of parvovirus B19 through a kidney allograft

We report a case of pure red cell aplasia (PRCA) caused by parvovirus B19 (PVB19) infection, which was transmitted through a kidney allograft. The patient underwent a living‐donor kidney transplant from his wife at the age of 60. Despite successful engraftment with a normal creatinine level, he developed severe anemia that required frequent blood transfusions 2 months after transplantation. Renal anemia was unlikely as his serum erythropoietin level was extremely high. A bone marrow aspiration test demonstrated the existence of large proerythroblasts. Although anti‐PVB19 IgM antibody levels were not increased, polymerase chain reaction (PCR) detected PVB19 DNA in his serum. Thus, he was diagnosed as having PRCA induced by PVB19 infection. PCR analysis of total DNA isolated from 0‐hour biopsy sections showed the existence of PVB19 DNA. Furthermore, PVB19 proteins was detected on renal tubules of 0‐hour allograft by immunoperoxidase staining. Thus, transmission of PVB19 through the allograft was confirmed. A single course of intravenous immunoglobulin (IVIG) therapy resulted in substantial improvement; however, the effect was limited, and severe anemia relapsed after 5‐6 months. Several courses of IVIG with adjustment of immunosuppressive drugs resulted in long‐term remission. Our case demonstrates that donor‐transmitted PVB19 infection should be suspected in kidney transplant recipients who develop refractory anemia during the early post‐operative phase.     

Transition of children with membranoproliferative glomerulonephritis to adolescence and adulthood

Background Many chronic renal diseases in children, including membranoproliferative glomerulonephritis (MPGN), often continue into adulthood, and these patients require continuing management. Despite the importance of the topic, there has been limited discussion about the problems of transition in children with continuing renal disease. We report our experience in patients with MPGN, as they matured from childhood to adolescence and adulthood, so-called “carry-over” cases. Methods The clinical course of diffuse MPGN in 27 children was retrospectively reviewed. Patients were over 18 years old at the end of follow-up. Results The mean follow-up period was 12.6 years; 20 children (74%) were identified by school urinary screening. The clinical course was favorable, and none of the patients progressed to end-stage renal failure during follow-up. However, eight patients (30%) continued to demonstrate proteinuria; two patients were nephrotic. Four patients were non-compliant and discontinued medication by themselves. Three patients were still on low dose of alternate-day (ALD) prednisolone. Twenty patients finished the treatment and were followed for an average of 4.6 years. Only one demonstrated trace amounts of proteinuria 1 year after discontinuing ALD prednisolone. Conclusions MPGN often continues during maturation from childhood to adulthood, and patients are usually referred to adult nephrologists. Good communication between pediatric and adult nephrologists is important. In addition, more in depth explanation and reeducation about their disease and its management are helpful when these patients reach adolescence. These measures will improve their care and help to assure compliance with their medication regimen.