Delayed renal dysfunction after total body irradiation in pediatric malignancies

The purpose of this study was to retrospectively evaluate the incidence of delayed renal dysfunction after total body irradiation (TBI) in long-term survivors of TBI/hematopoietic stem cell transplantation (HSCT). Between 1989 and 2006, 24 pediatric patients underwent TBI as part of the conditioning regimen for HSCT at Chiba University Hospital. Nine patients who survived for more than 5 years were enrolled in this study. No patient had any evidence of renal dysfunction prior to the transplant according to their baseline creatinine levels. The median age at the time of diagnosis was 6 years old (range: 1–17 years old). The follow-up period ranged from 79–170 months (median: 140 months). Renal dysfunction was assessed using the estimated glomerular filtration rate (eGFR). The TBI dose ranged from 8–12 Gy delivered in 3–6 fractions over 2–3 d. The patients were treated with linear accelerators in the supine position, and the radiation was delivered to isocentric right–left and left–right fields via the extended distance technique. The kidneys and the liver were not shielded except in one patient with a left adrenal neuroblastoma. No patient required hemodialysis. The eGFR of four patients (44.4%) progressively decreased. The remaining patients did not demonstrate any eGFR deterioration. Only one patient developed hypertension. By evaluating the changes in eGFR, renal dysfunction among long-term survivors of TBI/HSCT could be detected. Our results suggested that the TBI schedule of 12 Gy in 6 fractions over three consecutive days affects renal function.     

Structure-Based Design of Substituted Piperidines as a New Class of Highly Efficacious Oral Direct Renin Inhibitors

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Hepatic artery reconstruction preserving the pancreaticoduodenal arcade in pediatric liver transplantation with celiac axis compression syndrome: Report of a case

CACS is rare, although it has been reported to be a potential risk factor for hepatic artery thrombosis following LT. We herein present the case of a 14‐yr‐old male with stenosis of the origin of the celiac trunk. Preoperative CT and color ultrasonography showed narrowing of the proximal celiac artery. The patient underwent DDLT with standard arterial reconstruction without dividing the gastroduodenal artery. His postoperative course was uneventful, with an excellent hepatic artery flow on Doppler ultrasonography. Applying a meticulous preoperative evaluation and the appropriate surgical technique is crucial in patients with CACS.     

Central pontine myelinolysis following pediatric living donor liver transplantation: A case report and review of literature

CPM is one of the most serious neurological complications that can occur after OLT and is characterized by symmetrical demyelinization in the basis pontis. The etiology of CPM remains unclear, although the rapid correction of the serum sodium and CNI concentrations may be associated with the development of CPM. With recent advances in MRI technology, early diagnosis of CPM has become possible. Here, we present the case of a five‐yr‐old female who developed CNI‐associated CPM after undergoing LDLT. A decreased level of consciousness and dysphasia was noted one wk after LDLT, and MRI revealed findings compatible with a diagnosis of CPM. The patient fully recovered from the neurological deficits related to CPM following the switch from the CNI to sirolimus. We propose MRI to be promptly considered for patients with abnormal neurological findings, together with the substitution of CNI with an mTOR inhibitor as a management regimen for CNI‐related CPM.     

Efficacy of preoperative portal vein embolization prior to major hepatectomy for patients with impaired liver function: A retrospective study

Hepatocellular carcinoma (HCC) is often associated with chronic liver disease, such as hepatitis or cirrhosis, and this association may limit the use of surgery as a therapy, and if surgery is pursued, may give rise to postoperative hepatic failure. We evaluated the outcome in patients with HCC given preoperative portal vein embolization (PVE) before they underwent major hepatectomy. After PVE, portal pressure increased significantly. Two weeks after PVE, both the volume of the non-embolized lobe and the 15-min indocyamine green retention rate (ICG R15) were significantly increased. The prognostic score, calculated on the basis of age, ICG R15, and the resection rate, was significantly decreased. The operative mortality rate was significantly lower in patients who underwent PVE before surgery than in patients who did not receive PVE. The cumulative survival rate of the PVE patients, even those with cirrhosis of the liver, was significantly higher. Prior PVE appears to allow more extensive major hepatectomy and to lessen the risk of this invasive surgery. However, patients in whom the portal pressure immediately after PVE was more than 30cm H₂O and/or whose prognostic score exceeded 50 points developed postoperative hepatic failure. These features should be kept in mind when it is decided whether surgery is indicated. Nevertheless, preoperative PVE appears to be a beneficial procedure for patients undergoing major hepatectomy, particularly those with chronic liver disease.     

Long-Term Outcomes of Japanese Type 2 Diabetic Patients With Biopsy-Proven Diabetic Nephropathy

OBJECTIVE

We evaluated the structural-functional relationships and the prognostic factors for renal events, cardiovascular events, and all-cause mortality in type 2 diabetic patients with biopsy-proven diabetic nephropathy.

RESEARCH DESIGN AND METHODS

Japanese type 2 diabetic patients with biopsy-proven diabetic nephropathy (n = 260) were enrolled. Patients were stratified by albuminuria (proteinuria) and estimated glomerular filtration rate (eGFR) at the time of renal biopsy. The outcomes were the first occurrence of renal events (requirement of dialysis or a 50% decline in eGFR from baseline), cardiovascular events (cardiovascular death, nonfatal myocardial infarction, coronary interventions, or nonfatal stroke), and all-cause mortality.

RESULTS

The factors associated with albuminuria (proteinuria) regardless of eGFR were hematuria, diabetic retinopathy, low hemoglobin, and glomerular lesions. The factors associated with low eGFR regardless of albuminuria (proteinuria) were age and diffuse, nodular, tubulointerstitial, and vascular lesions. The glomerular, tubulointerstitial, and vascular lesions in patients with normoalbuminuria (normal proteinuria) and low eGFR were more advanced compared to those in patients with normoalbuminuria (normal proteinuria) and maintained eGFR. In addition, compared to patients with micro-/macroalbuminuria (mild/severe proteinuria) and low eGFR, their tubulointerstitial and vascular lesions were similar or more advanced in contrast to glomerular lesions. The mean follow-up period was 8.1 years. There were 118 renal events, 62 cardiovascular events, and 45 deaths. The pathological determinants were glomerular lesions, interstitial fibrosis and tubular atrophy (IFTA), and arteriosclerosis for renal events, arteriosclerosis for cardiovascular events, and IFTA for all-cause mortality. The major clinical determinant for renal events and all-cause mortality was macroalbuminuria (severe proteinuria).

CONCLUSIONS

Our study suggests that the characteristic pathological lesions as well as macroalbuminuria (severe proteinuria) were closely related to the long-term outcomes of biopsy-proven diabetic nephropathy in type 2 diabetes.Diabetic nephropathy occurs in 20–40% of patients with diabetes (1). The prevalence of diabetic nephropathy is increasing in proportion to the increase in prevalence of diabetes, and it has been predicted to continue to increase in future (2). Diabetes is a risk factor of cardiovascular disease and death, and diabetic nephropathy further increases these risks (3). In addition, diabetic nephropathy is the leading cause of end-stage renal disease requiring dialysis or transplantation in developed countries (4–6).In recent years, many clinical studies have suggested strict glycemic control and blood pressure management by use of appropriate medication to suppress the onset and progression of diabetic nephropathy. Thus, it is important to identify patients at risk in the early stages to improve prognosis in patients with diabetic nephropathy (1). Albuminuria and glomerular filtration rate (GFR) are recommended for use as clinical markers of diabetic nephropathy (1,7–9). On the other hand, selection of pathological markers is complicated because a variety of renal lesions can be found in diabetic nephropathy in addition to factors such as obesity, hypertension, dyslipidemia, and aging, which are frequently complicated in type 2 diabetes, causing a wide variety of pathological changes (10).We previously reported on the clinical factors related to the development and progression of renal lesions in diabetic nephropathy by the evaluation of serial renal biopsies or autopsy (11). In this report, we demonstrated a significant relationship between the progression of diabetic glomerulosclerosis and clinical factors such as the control of blood glucose, type of diabetes, age at onset, type of treatment, and degree of obesity.After this study, we conducted a long-term retrospective study to evaluate the structural-functional relationships and the predictive impacts of clinicopathological parameters for renal events, cardiovascular events, and all-cause mortality among Japanese patients with biopsy-proven diabetic nephropathy in type 2 diabetes.