Changes in patterns of left ventricular hypertrophy after aortic valve replacement for aortic stenosis and regurgitation with St. Jude Medical cardiac valves

In this study, we analyzed the extent and pattern of regression of left ventricular (LV) hypertrophy after aortic valve replacement in patients with aortic stenosis (AS) and compared the results with those of another group of patients with aortic regurgitation (AR). Seventy patients who underwent isolated aortic valve replacement were divided into 2 groups. Group 1 was comprised of 29 patients who underwent aortic valve replacement for aortic stenosis, and Group 2 of 41 patients who underwent aortic valve replacement for aortic regurgitation. A third group of 10 healthy subjects served as a healthy control group. Echocardiographic studies were done before the operation and 5 years postoperatively. At follow-up, a significant reduction in the left ventricular mass was found in both groups, but it remained significantly greater than in the healthy control group. The ratio of LV wall thickness to radius (th/r) in Group 1 decreased significantly, and at follow-up it was within the normal value. In Group 2, the th/r ratio increased, and at follow-up it was within the normal value. After aortic valve replacement, the wall thickness remained significantly greater than normal for patients with AS, and the chamber radius remained significantly greater than normal for patients with AR. For these reasons, LV hypertrophy still existed in both groups at postoperative follow-up. The actuarial survival rate was 85.3% at 16 years for Group 1 and 83.4% at 18 years for Group 2. There was no significant difference in the long-term survival rates between the 2 groups. Actuarial freedom from valve-related events was 91.9% at 16 years for Group 1 and 82% at 18 years for Group 2. There was no significant difference in the valve-related event free curves between groups. After 5 years of follow-up, th/r reached normal for both groups, indicating remodeling of the LV geometry after aortic valve replacement.     

Comparing quality of life following liver transplantation for Laennec's versus non-Laennec's patients

The overall success of orthotopic liver transplantation (OLTX) includes not only survival, but quality of life (QOL) as well. We studied one controversial group of OLTX recipients, patients transplanted for alcoholic liver disease (Laennec's), to determine if their post-OLTX QOL was similar to that of patients transplanted for non-alcoholic liver disease (non-Laennec's). Over a 10-yr period, patients undergoing OLTX at our institution were asked to complete a QOL questionnaire addressing a wide range of topics from demographics and employment to symptom distress/frequency, activities of daily living, and effect of loss of health on daily life. Twenty-four Laennec's and 100 non-Laennec's OLTX recipients completed the questionnaire at both their 2- and 5-yr follow-up visits at our institution. Both groups were well-matched in age, race, and patient location status at the time of OLTX. No significant differences could be detected between Laennec's and non-Laennec's scores regarding overall QOL, including one's ability to function, health perception, and self-perception at 2 and 5 years post-OLTX, and between 2 and 5 years post-OLTX. Although not between groups, a significant difference was noted regarding patients' satisfaction with life, with less satisfaction reported at the 5-yr versus the 2-yr time point post-OLTX. Rates of current/recent employment between both groups were also similar at 2 years post-OLTX, and again at 5 years post-OLTX. We conclude that overall QOL and employment levels appear similar between patients transplanted for alcoholic and non-alcoholic liver disease. This similarity appears to extend to 5 years post-OLTX.     

Concordant xenotransplantation--non-vascularized pancreatic islets are more difficult to regraft than the vascularized heart

We have previously demonstrated that it is possible to perform retransplantation of a xenogeneic heart (mouse-to-rat) using cyclosporine A as monotherapy, provided that the first heart is transplanted under a short course of deoxyspergualin (DSG). If DSG is omitted, the first heart is rejected within four days and the second heart succumbs to hyperacute rejection within minutes. A mouse heart as first graft does not protect a consecutive pancreatic islet graft, although the heart continues to function after rejection of the cellular graft. One explanation for this discrepancy may be the fact that cellular grafts, as pancreatic islets, lack an endothelial lining. We have, therefore, further investigated possible differences between vascularized and non-vascularized xenografts regarding their capacity to induce unresponsiveness. The use of pancreatic islets as primary graft neither accelerated nor decelerated the speed of rejection of the vascularized heart used as secondary graft. Furthermore, hemagglutinating and cytotoxic antibody titres responded in the same manner as in naive rats transplanted with a mouse heart. Retransplantation with pancreatic islets also resulted in complete rejection of both the primary and secondary grafts. Thus, the lack of unresponsiveness cannot simply be explained by differences, between the pancreatic and cardiac tissues, in antigen expression. In addition, intraperitoneal transplantation of mouse heart cells as primary graft resulted in rejection of a secondary cardiac graft after three days. However, it cannot be totally excluded that the time of antigen exposure had an impact on these results. In conclusion, our previous and present studies suggest that the presence of an intact vascular bed, both in the first and second graft, is necessary to create a state of unresponsiveness. Because the pancreatic islets lack an endothelial lining, they do not benefit from an unresponsiveness of the immune system. Neither are they able to induce such an unresponsiveness.     

Partial venous thrombosis of the pancreatic allografts after simultaneous pancreas-kidney transplantation

Despite new advances in transplantation, complete venous thrombosis (VT) of the pancreas after simultaneous pancreas kidney (SPK) transplantation usually results in graft loss. Data are limited regarding the outcome and treatment of partial VT of the pancreas allograft. From July 1994 to December 1999, 126 patients with IDDM/end-stage renal disease underwent SPK with systemic bladder drainage at the University of Miami. We retrospectively reviewed our experience regarding the outcome and treatment options of partial VT of the pancreas allografts. From July 1994 to April 1997, partial VT was not seen in the first 66 SPK patients induced with anti-CD3 rnAb and oral or intravenous (i.v.) tacrolimus (TAC) in the operating room. From May 1997 to June 1999, 14 (29%) out of 48 patients had VT. These cases were identified following the i.v. use of TAC with anti-IL-2R antibody-induction therapy (7/15) or without (7/33). Partial thrombosis of the splenic vein (PTSV) was documented in 10 patients, 2 had complete thrombosis of the splenic vein (CTSV), 1 had partial thrombosis of the superior mesenteric vein (PTSMV), and 1 patient had PTSV and PTSMV. These were identified incidentally during routine color Doppler ultrasonography (CDU). None of these SPK recipients demonstrates a change in clinical parameters. The first 8 patients were systemically heparinized, followed by oral anticoagulation, except 1 patient with CTSV. He progressed to complete thrombosis of the pancreas allograft and was treated with percutaneous thrombectomy and urokinase infusion, followed by heparinization and oral anticoagulation. One patient required exploration for bleeding. In an attempt to reduce the morbidity of heparinization, we treated the next 6 patients with PTSV with aspirin followed by serial CDU. All 14 patients had preservation of the endocrine and exocrine pancreatic functions. CDU showed resolution with recanalization of the thrombosed vein(s). From July 1999 to December 1999, 12 SPK recipients were administered TAC orally with or without induction therapy with anti-IL-2R antibody. So far, in this group, VT has not been identified. In summary, a total of 14 out of 126 patients (11%) had isolated VT with a mean follow-up of 36.4 months. Based on our experience, we suggest that extensive VT after pancreas transplantation, including splenic and superior mesenteric VT, be treated with heparin and subsequent oral anticoagulation for 3 months. For more limited, partial splenic VT, aspirin may be sufficient. Follow-up CDU is critical for a successful outcome. The i.v. use of TAC appears to be a risk factor for the increased incidence of VT. Currently, using IL-2rmAb as induction, TAC is started orally on postoperative days 3 or 4 and aspirin on postoperative day 2.     

药品行政保护品种介绍:抗组胺药(一)(待续)

地氯雷他定片剂通用名:地氯雷他定(desloratadine).商品名:Aerius片剂.化学名:8-氯-6,11-二氢-11-(4-哌啶亚基)-5H-苯[5,6]-环庚[1,2-b]吡啶.结构式见图1.     

前列腺素E1脂微球载体制剂治疗肝硬化大量腹水42例疗效观察

目的观察前列腺素E1脂微球载体制剂(Lipo-PGE1)治疗肝硬化大量腹水的疗效.方法选择肝硬化大量腹水84例,分为两组,治疗组42例,用Lipo-PGE1联合综合护肝+利尿剂治疗;对照组42例采用综合护肝+利尿剂治疗.结果治疗组对肝硬化大量腹水治疗显效率和总有效率显著高于对照组(P＜0.001).Lipo-PGE1治疗前后的丙氨酸转氨酶(ALT)、天门冬酸氨基转移酶(AST),总胆红素(SB)、肌酐(Cr)等指标均在疗程结束时得到显著改善(P＜0.01).结论 Lipo-PGE1有一定退腹水作用,且具有保护肝细胞、促进肝细胞再生的作用.     

INVITED REVIEW GASTROINTESTINAL MANIFESTATIONS IN PEDIATRIC AIDS

The pathologic changes in the gastrointestinal tract of children with AIDS are variable, clinically significant, and reflect multisystemic disease processes. Inflammation, changes in the lymphoid tissue, miscellaneous lesions, and tumors are documented in 58 patients in addition to cases reported in the literature. Cytomegalovirus infection of the gastrointestinal tract, associated with ulcerations, hemorrhage, perforation, and intestinal obstruction, carries a high morbidity and mortality, whereas the remaining infections are not life threatening. Special stains and electron micrographic examination are important to identify correctlycertain microorganisms such asmycobacterium avium intracellulare, cryptosporidia, and microsporidia. Lymphoproliferative changes of the gastrointestinal tract, a component of the generalized lymphoproliferative process, need to be characterized by tumor markers and cytogenetic studies. Within the miscellaneous lesions, AIDS associated arteriopathy can be complicated by intestinal ulceration and perforation. Both lymphomas and smooth muscle tumor in children with AIDS are related to Epstein-Barr virus infection. The smooth muscle tumors are frequently malignant and multiple.     

PATHOLOGY OF LONG-CHAIN 3-HYDROXYACYL-COA DEHYDROGENASE DEFICIENCY CAUSED BY THE G1528C MUTATION

Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency is a recently discovered disorder affecting the mitochondrial-oxidation of fatty acids. There have been few reports of the pathologic findings in-oxidation defects. We examined pathologic specimens from 16 patients with this disorder (11 patients were homozygous for the common mutation G1528C, 5 patients were siblings with a similar clinical presentation). Autopsies were performed on all 15 patients who died, and liver biopsy specimens were available from 8 patients. Hepatomegaly and steatosis of the liver, found in every patient, were often combined with fibrosis or cirrhosis. Cardiomegaly and accumulation of fat in the myocardium, renal tubules, and skeletal muscle were found in many patients. A detailed neuropathologic examination was performed on six patients, and brain specimens obtained at autopsy were examined in four others. In general, neuropathologic findings were mild and unspecific, but vacuolization was detected in the deep gray matter and in the cerebellum and brain stem nuclei of five patients. In one patient the vacuolization was prominent; in the other four it was milder and more focal. The vacuoles seemed to be either in the neuropil or associated with swollen hydropic cells. The uniform pattern of histopathologic changes facilitates the diagnostics in this severe disorder, allowing opportunities for therapy and prenatal diagnosis.     

A new case of malonyl coenzyme A decarboxylase deficiency presenting with cardiomyopathy

A new case of mitochondrial malonyl coenzyme A decarboxylase deficiency is described. The patient presented with an initial episode of metabolic acidosis, seizures, hypoglycemia, and cardiac failure at 2 months of age which slowly resolved. Subsequent evaluations at 4 years of age for developmental delay revealed a prominent elevation of malonic acid in urine. Malonyl carnitine was also elevated. The activity of malonyl CoA decarboxylase in cultured fibroblasts was 7% of normal. Conclusion Malonyl CoA decarboxylase deficiency may result in inhibition of fatty acid oxidation, which may account for the cardiomyopathy. Received: 12 April 1996 / Accepted: 24 September 1996     

Pseudoinfarction Pattern in an Infant

A 4-week-old female infant presented with congestive heart failure, moderate mitral regurgitation, and an electrocardiographic pattern of anterolateral myocardial infarction. Angiography revealed normal coronary arteries and moderate mitral regurgitation. A single-catheter electrophysiology study confirmed the presence of an accessory atrioventricular conduction pathway.