Acute fulminant ulcerative colitis with toxic megacolon

An 11-year-old boy with acute fulminant ulcerative colitis (UC) is presented. He had systemic deterioration with frequent diarrhea and lethargy. Acute fulminant UC associated with toxic megacolon was diagnosed by rectal endoscopy and biopsied specimen. He was treated with intensive intravenous administration of prednisolone and total parenteral nutrition. He recovered completely without any surgical intervention.     

Treatment of Acute Cerebral Artery Occlusion Using the Penumbra System: Our Early Experience

Intravenous recombinant tissue-type plasminogen activator (rt-PA) therapy is highly recommended to patients who are diagnosed with ischemic stroke within 4.5 hours after the onset while mechanical clot retrieval can be attempted in patients who are not indicated for or cannot effectively receive intravenous rt-PA therapy. In this article, we report early treatment outcomes and discuss the usefulness of mechanical clot retrieval using the Penumbra system (Penumbra Inc., Alameda, California, USA), especially in terms of technical cautions during the procedure and adaptability to elderly and high National Institutes of Health Stroke Scale (NIHSS) patients. We included 7 patients with thromboembolic occlusion. Pre-treatment NIHSS score ranged from 11 to 36 (mean: 24.9). All patients achieved good recanalization [thrombolysis in cerebral infarction (TICI) grade 2a or greater] without complications. The NIHSS score at 30 days after the treatment ranged between 0 and 28 (mean: 12.4), and improved more than 10 points in 4 of the 7 patients (57.1%). To obtain good recanalization without complications, selection of suitable reperfusion catheter and careful manipulation of separator prefiguring the occluded distal vessels are essential. The improved NIHSS score at 30 days after the treatment may have led to favorable results, such as an increased participation in available rehabilitation programs and the alleviation of the burden of care. Our findings suggest that the Penumbra system might be effective for treatment in elderly patients or patients with high NIHSS score wherein rt-PA therapy is inadvisable or ineffective in ischemic stroke secondary to large vessel occlusion. Recanalization can improve their quality of life on condition that the procedure is performed successfully without serious complications.     

CASE REPORT: Primary hepatic lymphoma associated with chronic liver disease

We report on a case of primary hepatic lymphoma that developed in a patient with chronic hepatitis C. Given that Japan is an area endemic for both hepatitis B and C viruses, we reviewed 51 Japanese cases of primary hepatic lymphoma, addressing the question as to whether the Japanese cases have unique characteristics and whether there is a causal relationship to the presence of chronic liver disease. Primary hepatic lymphoma most commonly affected middle-aged males. Presenting symptoms and physical findings were non-specific. Aminotransferases tended to stay in the low range compared with marked increases in lactate dehydrogenase. Sixteen patients (31%) had chronic liver disease, eight had liver cirrhosis and eight had chronic hepatitis, suggesting that there is a possible aetiological link between chronic liver disease and primary hepatic lymphoma.     

Vasopressin plus oxygen vs vasopressin alone in cirrhotic patients with portal-hypertensive gastropathy: Effects on gastric mucosal haemodynamics and oxygenation

The effects of vasopressin plus oxygen and vasopressin alone on gastric mucosal perfusion and oxygenation were studied using reflectance spectrophotometry and laser Doppler velocimetry in 23 cirrhotic patients with portal-hypertensive gastropathy. The measurements were performed under basal conditions and after double-blinded administration of placebo (n= 7), vasopressin (0.3 U/min; n= 8) or vasopressin (0.3 U/min) plus nasal oxygen (4 L/min; n= 8). No significant effects on gastric mucosal haemodynamics and oxygenation were observed after placebo. In contrast, vasopressin and vasopressin plus oxygen induced a similar reduction in haemoglobin content (-26 ± 2 and -21 ± 4%, respectively P < 0.01) and laser Doppler signal (-23 ± 2 and -22 ± 2%, respectively, P < 0.01). Although each treatment induced a significant reduction in oxygen saturation (-21 ± 2 and -7 ± 1%, respectively P < 0.01), the effect was less pronounced in patients receiving the combination than in those receiving vasopressin alone (P < 0.01). These data suggest that vasopressin and vasopressin plus oxygen reduce gastric mucosal hyperaemia and that the oxygen supplement partially protects against gastric mucosal hypoxia during vasopressin infusion in cirrhotic patients with portal-hypertensive gastropathy.     

Gene therapy for metachromatic leukodystrophy

Metachromatic leukodystrophy (MLD) is an inherited metabolic disease which is characterized by a deficiency of arylsulfatase A (ASA). This deficiency causes progressive accumulation of cerebroside sulfate in oligodendrocytes (OL) in the brain, resulting in dysmyelination. Approaches being developed by the authors to treating MLD are based on direct delivery of ASA genes into the brain. In the present report, it has been shown that the recombinant adenovirus (Adex 1SRLacZ) was able to transduce the OL very efficiently. Moreover, primary fibroblasts from MLD patients were exposed to recombinant adenovirus expressing the ASA gene (Adex1SRASA) and the cells expressed the transgene. The influence of overexpression of ASA on the activity of other sulfatases was also tested in fibroblasts from patients with MLD using a retrovirus vector (MFG-ASA). It was demonstrated that the overexpression of ASA reduces the activity of various sulfatases by a small amount but does not induce an accumulation of glycosaminoglycan. These results indicate that the influence of ASA overexpression on other sulfatases is different from that of the N-acetygalactosamine-4-sulfatase overexpression in a previous report. It was concluded that the correction of ASA deficiency by a recombinant adenovirus that potentially could be used to transfer the gene to the brain, and gene therapy for MLD based on gene transfer of the ASA gene to mutant cells will be feasible because the overexpression of ASA in cells does not lead to profound deficiency of other sulfatases or result in a new phenotype.     

Bladder smooth muscle cell phenotypic changes and implication of expression of contractile proteins (especially caldesmon) in rats after partial outlet obstruction

BACKGROUND: The purpose of the present study was to investigate morphological changes in bladder smooth muscle of rats with partial outlet obstruction. We investigated smooth muscle cell phenotypic changes and implication of synthetic phenotype in contractility decrease and bladder compliance after bladder outlet obstruction. METHODS: Partial bladder outlet obstruction was introduced in female rats. Bladder were removed at 1, 3, 6, 10 and 20 weeks after the obstruction. Temporal pattern of changes in bladder mass, light microscopic pathogenesis and phenotypic expression of the bladder smooth muscle cells in the electron micrographs were investigated. Expression of contractile protein was also investigated by the immunoblotting method. RESULTS: Marked increase in bladder mass with marked thickening of smooth muscle layer was observed at 1 week after obstruction. The ratio of myocytes exhibiting contractile and synthetic phenotypes was almost constant until 6 weeks after the obstruction, but thereafter, synthetic phenotypes gradually increased and the ratio (synthetic/contractile phenotype) was 1.5-fold at 20 weeks after the obstruction. Caldesmon was most markedly expressed after the obstruction among contractile proteins examined by the immunoblotting method. CONCLUSION: Phenotypic changes were confirmed in bladder smooth muscle, and the decrease of the ratio of contractile phenotype was observed after long-term obstruction of the bladder outlet. Among the contractile proteins in the bladder smooth muscle cell, caldesmon was considered a reliable marker for predicting the pathogenetic conditions of the bladder.     

Primary carcinoid tumor of the testis with teratoma metastatic to the para-aortic lymph node

A primary testicular carcinoid tumor with teratoma metastasized to the para-aortic lymph node. After inguinal orchiectomy, serum and urinary levels of 5-hydroxyindoleacetic acid (5-HIAA) were found to be elevated. Chemotherapy consisting of cisplatin, etoposide and bleomycin was not effective on the metastatic lesions. Retroperitoneal lymphadenectomy normalized the levels of 5-HIAA. The patient is alive without recurrence 25 months after the retroperitoneal lymphadenectomy.     

Reversibility of organic anion-induced cholestasis: Association with compensatory hypersecretion of biliary phospholipid and protein in the dog

Abstract The effect of a concomitant infusion of organic anions, structurally related phthaleins, on bile flow was studied in anaesthetized dogs. A combination of rose bengal and sulfobromophthalein was found to uniquely and synergistically produce an acute, reversible form of intrahepatic cholestasis (< 10% of control level). This phenomenon was not observed with the administration of those individual organic anions at concentrations previously associated with the induction of intrahepatic cholestasis. The infusion of either a micelle forming bile salt, sodium taurocholate, or a non-micelle forming bile salt, sodium dehydrocholate, rapidly reversed the intrahepatic cholestasis (within 20 min after bile salt infusion). During the choleretic phase immediately following the bile salt infusion, a transient but marked hypersecretion, a disproportionately increased output in relation to that of bile acids, of biliary phospholipid (176% of control level by taurocholate and 138% of control level by dehydrocholate), and an even more striking amount of biliary protein hypersecretion were observed (392% of control level by taurocholate and 357% of control leverl by dehydrocholate). Although the significance of these new post-cholestatic observations requires clarification, it is suggested that the intrahepatic cholestasis induced by organic anions reflects a reversible defect in the mechanism(s) involved in transcellular transport.     

13C-phenylalanine breath test correlates with liver fibrosis in postoperative biliary atresia

BACKGROUND: Values derived from the (13)C-phenylalanine breath test (PBT) may serve as an index for liver fibrosis and clinically predictive readings for liver diseases in adults. In the present study the PBT was conducted in postoperative biliary atresia (BA) children to evaluate phenylalanine metabolism in the liver, and the results based on biochemical data, especially the index on liver fibrosis, were compared with PBT findings. METHODS: Hepatofunctional evaluations were conducted in 10 postoperative BA children with moderate (group B; n = 4) and severe (group A; n = 6) liver dysfunction, and the PBT results were compared with those of 13 normal healthy children (group C). Subjects were orally given single-bolus (13)C-phenylalanine at 3.5 mg/kg (maximum dosing: 100 mg) in the morning. Time-related exhaled gas was periodically collected until 120 min after dosing. The (13)CO(2) levels were monitored with gas chromatography-mass spectrometry before and after administration, and the (13)C excretion rate, (13)C cumulative excretion and time of maximum (13)C excretion rate were monitored accordingly. RESULTS: Total bile acid, hyaluronic acid, type IV collagen 7S, total bilirubin or albumin and the PBT findings were significantly correlated. The PBT findings in group A were significantly lower those of group B, indicating that phenylalanine metabolism was markedly attenuated in the former. CONCLUSION: The PBT values correlated well with liver fibrosis in postoperative BA children. Because PBT is a non-invasive approach, results from this method may serve as a useful and reliable index for post-surgical monitoring of children operated on for liver fibrosis.