Pre-emptive renal transplantation in children

BACKGROUND: Renal transplantation is a definitive therapeutic modality in end-stage renal disease (ESRD). Most ESRD patients in Japan experience dialysis prior to renal transplantation. The present study was undertaken to examine the usefulness of pre-emptive renal transplantation (PET). METHODS: Between 1987 and 1998, 255 renal transplantations were carried out by the authors. Among those consecutive cases, 10 were cases of PET. In nine pediatric cases, demographics, graft and patient survival, height growth and benefits from successful transplantation were studied and compared with age-matched dialyzed transplantation controls. RESULTS: All transplantation was living-related. There was a disparity of causes of ESRD between the two groups. In PET, acquired renal deterioration due to a congenital lower urinary tract disorder was the major cause. Graft and patient prognosis was favorable in both groups. Growth retardation in PET patients under 15 years of age was significantly less apparent at the time of transplantation and after 3 years compared to the control. The benefits from transplantation were different in the two groups. Most PET patients felt an improvement of their physical condition; however, all of the control patients felt that the major boon was the freedom from the restriction of the daily diet and time for dialysis. CONCLUSION: In pediatric renal transplantation, short-term preceding dialysis does not have a detrimental effect, but PET could benefit ESRD patients by maintaining their quality of life. Moreover, PET minimizes the production of renal dwarfism in prepubertal children. Thus, PET should be taken into consideration in the choice of renal replacement therapy.     

Increased levels of serum matrix metalloproteinase-3 in haemodialysis patients with dialysis-related amyloidosis

Background: It is recognized that matrix metalloproteinase‐3 (MMP‐3) is abundantly expressed in active rheumatoid synovium, and that serum level of MMP‐3 is a useful marker for diagnosis of rheumatoid arthritis and for evaluation of prognosis in joint destruction. Little is known about serum MMP‐3 levels in haemodialysis (HD) patients, and thus, the association between serum MMP‐3 and dialysis‐related amyloidosis (DRA) has yet to be elucidated. Methods: Serum levels of MMP‐3 were measured by enzyme immunoassay in 150 HD patients, 90 without DRA and 60 with DRA, before HD. Simple regression analysis was performed to investigate the relationship between serum level of MMP‐3 and clinical parameters, including age, HD duration, C‐reactive protein and β2 microglobulin (BMG). Results: Serum levels of MMP‐3 were significantly higher in HD patients with DRA than in HD patients without DRA (258.2 ± 118.1 vs 201.5 ± 98.4 pg/mL, P = 0.0017), and both levels were significantly higher than those of healthy subjects (45.6 ± 13.4 pg/mL, P < 0.0001). Serum MMP‐3 levels significantly correlated with serum levels of BMG (r = 0.197, P = 0.0164) and HD duration (r = 0.168, P = 0.0427). Moreover, serum MMP‐3 levels significantly correlated with serum BMG levels in HD patients without DRA (r = 0.341, P = 0.0012), but not in HD patients with DRA. Conclusion: Our results suggest that matrix metalloproteinase activity increases in HD patients, which may be associated with BMG and DRA.     

Expression of Carbohydrate Antigens in Human Esophageal Squamous Cell Carcinoma: Prognostic Application and Its Diagnostic Implications

Background Tumor markers whose antigenic determinants have been demonstrated to consist of carbohydrates are probably one of the most extensive tools that have been used in routine cancer diagnosis. In this study, the relevance of carbohydrate antigen expression profile was examined in esophageal squamous cell carcinoma together with prognosis in 130 patients. Methods The expression of carbohydrate antigens was estimated immunohistochemically by anti–sialyl Lewis a (sialyl Le^a) and anti–sialyl Lewis x (sialyl Le^x) monoclonal antibodies, and correlation between their staining and clinicopathological status was examined. In addition, the correlation of both carbohydrate antigens expression was evaluated with microvessel density (MVD). Results Expressions of sialyl Lewis antigens and MVD were associated with several clinicopathological features that reflect the tumor aggressiveness in esophageal cancer. The 5-year survival rate of patients was found to be associated with expression of sialyl Le^a and sialyl Le^x antigens and with MVD; thus, all of them were revealed to be independent prognostic factors. Conclusions Combination of these factors offered a better prediction of prognosis of esophageal squamous cell carcinoma. Further, carbohydrate antigens represent a promising target for therapeutic approaches against the disease.     

Endoscopic pancreatic duct drainage and stenting for acute pancreatitis and pancreatic cyst and abscess

Background/Purpose Endoscopic drainage of pancreatic pseudocysts using transpapillary and transmural approaches has been reported. In this study, endoscopic nasopancreatic drainage (ENPD) and pancreatic stenting were performed in patients with pseudocyst and abscess associated with acute pancreatitis, and the usefulness and problems of the procedures were investigated. Methods After endoscopic retrograde pancreatography was done, ENPD and/or pancreatic stenting were performed in 13 patients with pancreatitis and pseudocyst or abscess that communicated with the main pancreatic duct. Results ENPD was performed in seven patients, and was effective in all five patients with cysts: the cysts disappeared or shrank. However, the condition in the two patients with abscess was unchanged, and percutaneous drainage was performed. Stenting was carried out in six patients, and the cyst disappeared or pancreatitis was improved in all six. The stent was removed from two patients, but no recurrence has been noted so far. Conclusions ENPD and stenting are effective therapeutic choices for acute and chronic pancreatitis and pseudocysts, and they are superior to percutaneous drainage to avoid pancreatic fistula, but they may not be effective for pancreatic abscess. Selection of therapeutic methods corresponding to individual cases is important.     

Radiofrequency (RF)-Assisted Hepatectomy May Induce Severe Postoperative Liver Damage

Background Radiofrequency (RF) ablation for the treatment of the section line prior to liver resection has been proposed as a way to reduce blood loss during hepatectomy. Our group compared hepatectomy with and without RF ablation to determine whether this technique actually reduces blood loss during liver resection and whether it affects the perioperative outcome. Method Of 151 patients who underwent a hepatectomy between January 2002 and October 2005 at the Division of Gastrointestinal Surgery in the Department of Surgery of Saitama Medical University, 48 who had a partial hepatectomy or resection of a portion of liver smaller than a single Couinaud segment were included in the study. Twenty patients who had RF-assisted hepatectomy [RF (+) group] and 28 patients who had hepatectomy without ablation [RF (-) group] were studied to compare the rates of intraoperative blood loss and the effects of RF ablation on the perioperative outcome. Results Intraoperative blood loss was significantly reduced in the RF (+) group. In contrast, the alanine aminotransferase activity in the RF (+) group was significantly elevated immediately after the operation. There was no significant difference in the incidence of postoperative complications between the groups, although bile leakage did occur in three RF (+) patients. Conclusions Our results demonstrate that the RF ablation technique can be a useful way to reduce surgical blood loss. In view of its association with severe postoperative liver damage, the technique must be applied with caution. The danger may be especially relevant to patients with chronic liver disease and decreased liver reserve.     

Ectopic cystic thymoma associated with Raynaud's phenomenon.

Mediastinal cystic tumors are well-marginated round lesions that comprise 12% to 18% of all mediastinal masses. These lesions include a variety of diseases with overlapping radiologic appearances and variable prognoses. Pathological examinations are almost always required for differential diagnosis. We encountered a case of anterior mediastinal tumor discovered in the process of investigation of Raynaud's phenomenon. Taking into account the tumor location, a pericardial cyst was initially suspected. However, the tumor was surgically resected and histopathological examinations demonstrated thymus-like tissue in the cyst walls. Raynaud's phenomenon greatly improved after surgery. These findings suggested that cystic thymoma originated from ectopic thymic tissue and is accompanied by paraneoplastic syndrome.     

Outcomes of liver transplantation for Alagille syndrome after Kasai portoenterostomy: Alagille Syndrome with agenesis of extrahepatic bile ducts at porta hepatis

BackgroundAlagille syndrome (ALGS) is an autosomal dominant disorder, characterized by a paucity of intrahepatic bile ducts, resulting in significant cholestasis, and peculiar extrahepatic features. Some ALGS patients show a considerable overlap with biliary atresia (BA), and they can undergo Kasai procedure. The purpose of this study is to show the manifestations of BA overlapped ALGS cases in our institution, and to compare the outcomes of ALGS patients following liver transplantation (LT) between those who previously underwent Kasai surgery (ALGS-Kasai group) and those who did not (ALGS-non-Kasai group).MethodsMedical records of ALGS patients who underwent LT in Kyoto University Hospital, Japan from January 1992 to March 2018 were analyzed. ALGS diagnosis was determined according to physical, radiologic, and histopathological findings.ResultsThirty-one patients were ascertained (ALGS-Kasai: 4 males and 5 females vs. ALGS-non-Kasai: 14 males and 8 females, p = 0.43). Of 31 ALGS patients, 96.8% of children had pulmonary artery stenosis, 54.8% showed facial features, 29% revealed skeletal anomalies and 9.7% demonstrated ocular anomalies. The age at LT was significantly younger in ALGS-Kasai than ALGS-non-Kasai group (1.47 [interquartile range (IQR), 0.75–1.92] vs. 5.1 [IQR, 1.4–9.29] years; p = 0.038). Overall patient survival did not significantly differ between ALGS-Kasai (88.9%) and ALGS-non-Kasai patients (86.4%) (p = 0.84). Furthermore, the 1-year, 5-year, and 10-year patient survival rates for ALGS-Kasai group were 100%, 88.9%, and 88.9%, respectively, whereas those for ALGS-non-Kasai group were 90.9%, 90.9%, and 86.4%, respectively, with p-values of 0.36, 0.90, and 0.84, respectively.ConclusionsBA overlapped ALGS cases had neonatal progressive cholestasis which prompted Kasai procedure, and early liver dysfunction after Kasai led to performing LT. The ALGS-Kasai patients undergo LT at earlier ages than the ALGS-non-Kasai patients, however, overall patients' survival rates are similar between groups. Overall ALGS patients' survival rate after LT is considered high.Levels of EvidenceLevel III; Case–control study or Retrospective comparative study.     

Reversible posterior leukoencephalopathy in a patient with Wegener granulomatosis

A 14-year-old girl with rapidly progressive glomerulonephritis was transferred to our hospital because of acute renal failure. A diagnosis of Wegener granulomatosis was made according to the symptom triad of a renal biopsy demonstrating crescentic glomerulonephritis, severe sinusitis, and serological findings of raised proteinase 3 anti-neutrophil cytoplasmic antibody level. In spite of combination therapy with methylprednisolone, cyclophosphamide, and plasma exchange, her renal function gradually deteriorated. Thereafter, she suffered a severe headache and generalized seizures. Brain computed tomography (CT) scan revealed bilateral low-density areas in the parieto-occipital lobes. Magnetic resonance imaging (MRI) disclosed a high-intensity area on T2-weighted images and a low-signal intensity area on T1-weighted images in the same lesion. Follow-up brain CT scan 3 weeks and MRI 2 months after the first studies showed complete resolution of the abnormal lesions, which indicated reversible posterior leukoencephalopathy syndrome. In addition to renal failure, hypertension, and cyclophoshamide, the primary disease may have played a role in the development of this uncommon syndrome in our patient.     

Increasing myocardial<Superscript>123</Superscript>I-BMIPP uptake in non-ischemic area in a patient with acute myocardial ischemia

The subject was a 65-year-old woman with chest pain. An electrocardiogram revealed T-wave-inversion in leads III, aVF, V1-V5. 99mTc-tetrofosmin myocardial SPECT showed mildly reduced uptake in the anteroseptal wall and the apex. These findings suggested acute myocardial ischemia. Coronary angiography did not show any stenotic lesions, but diffuse coronary ectasia was noted in three vessels. Coronary flow velocity was remarkably reduced on coronary angiography. Epicardial coronary spasm was not provoked by ergonovine loading test. Left ventriculography showed diffuse hypokinesis. 123I-BMIPP myocardial SPECT showed mildly reduced uptake in the anteroseptal wall and the apex on the early images. But 4-hour delayed images showed an increase of 8% in myocardial 123I-BMIPP uptake. We treated this patient with ticlopidine and nicorandil. After drug therapy her symptoms and left ventriculography improved. 123I-BMIPP myocardial SPECT findings on the early images improved, whereas delayed images showed a decrease of 28% in myocardial 123I-BMIPP uptake after two weeks and 36% after four weeks. These dynamic changes in 123I-BMIPP findings might be a reflection of myocardial fatty acid metabolism in patients with acute myocardial ischemia. Delayed 123I-BMIPP myocardial SPECT images are useful for the assessment of fatty acid metabolism.     

The role of smooth muscle cell differentiation in the mechanism of obliteration of processus vaginalis

Background/Purpose

Development of indirect inguinal hernia and hydrocele in childhood is readily explained by the persistence of smooth muscle component around the processus vaginalis (PV) after the descent of the testis into the scrotum. The aim of this study was to investigate the expression of smooth muscle myosin heavy chain (SM MHC) isoforms as the markers of smooth muscle cell (SMC) differentiation in childhood inguinal hernia and hydrocele and in age-matched controls.

Methods

The authors analyzed sacs from patients with inguinal hernia (male, 10; female, 10) and hydrocele (n = 10) immunohistochemically using monoclonal antibodies against α-smooth muscle actin, SM1, SM2 and SMemb. Peritoneal samples (male, 5; female, 5) obtained from age-matched patients served as controls. Immunostaining was evaluated with semiquantitative scoring and χ² test.

Results

The expression pattern of SM MHC isoforms did not differ among sacs obtained from female inguinal hernia when compared with that of controls. However, strong expression of SMemb within the sac walls of male inguinal hernia and SM1 in hydrocele groups were observed.

Conclusions

Our results indicate that SMC differentiation may play an important role in the obliteration of processus vaginalis in male inguinal hernia and hydrocele after the descent of the testis.