2型糖尿病合并糖原性肝病1例报告

糖原性肝病(glycogenic hepatopathy,GH),又称莫里阿克综合征,是由肝脏糖原沉积导致的肝脏功能及组织学变化,主要表现为转氨酶升高、肝肿大,常见于血糖控制较差的1型糖尿病患者,偶见于2型糖尿病患者[1]。现对1例2型糖尿病合并GH进行报道。

1型糖尿病合并糖原性肝病一例

患者,女,22岁,因“口渴、多饮、多尿2年,加重1周”于2013年10月7日收入我院。2年前患者无明显诱因出现口渴、多饮、多尿,于当地医院多次检查空腹血糖8~12 mmol/L,诊断为2型糖尿病,予二甲双胍片0.5 g/次、每日3次口服及饮食控制治疗,症状缓解。1年前上述症状再次加重,调整治疗方案为二甲双胍片0.5 g/次、每日3次口服,诺和锐三餐前12 U-12 U-12 U皮下注射,监测空腹血糖5~6 mmol/L。

1型糖尿病并发糖原性肝病1例

报道1例1型糖尿病并发糖原性肝病患者的诊疗经过。患者为46岁女性,以"烦渴、多饮、体重减轻4个月,伴腹痛2 d余"为主诉入院,通过临床资料分析及肝脏穿刺活检,其符合1型糖尿病并发糖原性肝病的特点。入院后积极补液、降糖治疗后酮症酸中毒及高渗高血糖状态纠正,糖原累积较前好转。患者于46岁被确诊为糖原性肝病,与此病常见诊断年龄及病程不同。该文结合患者诊疗过程探讨糖原性肝病临床特征、诊治要点,以期提高临床对该疾病的认识。

糖原贮积型心肌病的诊治现状及进展

糖原贮积型心肌病是一类由糖原代谢障碍引起糖原在心肌细胞内过度累积所致的心肌病,其中最常见的类型包括糖原贮积病Ⅱ型(庞贝病)、Danon病及PRKAG2心脏综合征,是儿童肥厚型心肌病的重要病因。糖原贮积型心肌病的主要表现包括心肌肥厚、心律失常、心力衰竭等,甚至可引起早期死亡。早期识别和诊断,并做出精准的干预,有望改善心肌病的症状,提高患者的生存质量。因此,提高临床医师对糖原贮积性心肌病的认识具有重要意义。

1型糖尿病并发糖原性肝病一例及文献复习

糖原性肝病(glycogenic hepatopathy,GH)又称莫里阿克综合征(Mauriac syndrome),是糖尿病患者血糖控制欠佳时一种罕见的并发症。该病由肝细胞中糖原的过度累积导致,糖原沉积可引起肝脏肿大、转氨酶升高,腹痛是其常见的临床表现。该疾病通过良好的血糖控制可以完全逆转,大多数预后良好。本文报道1例以肝酶急剧升高、腹痛、伴轻度肝纤维化为主要表现的1型糖尿病合并糖原性肝病的病例。

糖原累积性肌病的临床和病理学特点

目的：探讨糖原累积性肌病（ MGSD）患者的临床及病理特点。方法采用开放式肌肉组织活检术及肌肉酶组织化学染色方法观察29例MGSD患者的病理特点，并收集患者的一般资料、临床症状及体征、血清肌酶及EMG等临床资料进行归纳和总结。结果本组MGSD检出率为1．88％（29／1540）。29例MGSD患者中男19例，女10例。起病年龄1～67．5岁，中位数为13岁。病程3个月～41年，中位数为7年。主要的首发症状为肢体无力（65．52％）、不耐受疲劳（24．14％）和活性耐力差伴反复呼吸困难（3．45％），主要临床表现为肢体无力（96．55％）、颈肌无力（37．93％）和呼吸肌无力（13．79％）等。27例患者行肌酸激酶（CK）检查，中位数为1266．00 U／L，其中CK正常者2例（7．41％），CK升高者25例（92．59％），且以轻-中度升高为主。29例患者EMG检查均有异常，其中86．20％的患者EMG表现为肌源性损害或肌源性损害合并肌强直电位。 HE染色29例患者均出现特征性的空泡样变性坏死肌纤维，空泡大小不一、形态多样，且20例空泡样变纤维中出现嗜碱性颗粒。 PAS染色阳性。结论 MGSD患者发病年龄及病程波动范围大，患者均以进行性肢体无力为主要表现，部分患者有颈部肌肉及呼吸肌受累。肌肉酶组织化学染色有明显肌纤维空泡样坏死变性，有助于明确MGSD的诊断。

组织化学和免疫组织化学技术对脂质沉积性肌病和糖原沉积性肌病的鉴别

目的脂质沉积性肌病和糖原沉积性肌病是罕见的肌肉疾病,其常规病理形态十分相似。本文探讨这两种疾病组织化学和免疫组化反应,寻找其鉴别诊断的形态差异。方法采用三磷酸腺苷(ATP)酶、还原性尼克酰胺腺嘌呤二核苷酸(NADH-Tr)、苏丹Ⅲ、PAS组织化学染色和抗肌萎缩蛋白(Dystrophin)免疫组化染色,分析2例脂质沉积性肌病和2例糖原沉积性肌病的组织形态特征和差别。结果脂质沉积性肌病和糖原沉积性肌病在常规HE染色均表现为肌纤维内出现大量的空泡,但前者空泡大小较一致,且边界清楚,后者空泡大小差异大,边界不清。ATP酶组化染色显示脂质沉积性肌病出现空泡变性的肌纤维均为Ⅰ型肌纤维,而糖原沉积性肌病出现空泡变性的肌纤维两型均有,以Ⅰ型严重。脂肪染色和糖原染色可作为这两种肌病的确诊依据。Dystrophin免疫组化染色显示脂质沉积性肌病的肌纤维强阳性,而在糖原沉积性肌病的反应为不连续弱阳性。结论组化和免疫组化检测可用于脂质沉积性肌病和糖原沉积性肌病的鉴别诊断。

糖原性肝病肝穿刺病理诊断一例

本文报道1例T1DM伴肝脏肿大患者转氨酶升高,经肝脏穿刺诊断为糖原性肝病,经严格控制血糖5个月后复查转氨酶降至正常。

自拟止糖泻合剂治小儿迁延性、慢性糖源性腹泻46例

用中药止糖泻合剂治疗小儿迁廷性、慢性糖源性腹泻患儿46例，并与西医治疗的30例作对比观察，结果治疗组治愈率、有效率均明显优于对照组（P＜0．01）。

Effects of Different Storage Temperature on Nutritional Quality of Potato Cultivar Kexin No.1

Objective] This study almed to investigate the effects of different storage temperature on nutritional quality of potato cuItivar Kexin No.1 and expIore the opti-mal storage temperature and time for Kexin No.1 with different uses. [Method] After storage at different temperature （1, 4, 10 and 16 ℃）, changes in dry matter, starch, reducing sugar and protein contents, amyIase activity and germination rate of potato cuItivar Kexin No.1 were observed and analyzed. [Result] Reducing sugar content of Kexin No.1 stored at 1 and 4 ℃ increased significantIy compared with that of Kexin No.1 stored at 10 and 16 ℃. Kexin No.1 stored at 1 ℃ exhibited no chiI ing injury. After storage at 16 ℃, Kexin No.1 began to germinate at 30 d, and the Iength and number of germinated buds increased significantIy with the extension of storage time, dispIaying a significant reduction in moisture content. Kexin No.1 potato couId be stored at 1 and 4 ℃ for more than 120 d without any changes in various indi-cators. Therefore, 1-4 ℃ was the optimal storage temperature for potato cuItivar Kexin No.1. [Conclusion] This study provided theoretical basis and technical support for the optimization of post-harvest storage conditions of potatoes.

Physiological Responses of Yixing Lily Leaf and Bulb to Drought Stress

[Objective]To explore an effective method for evaluating drought resistance of Yixing lily and provide a reference for selecting drought-resistant Yixing lily varieties. [Method]Through artificial simulation of drought stress environment,the changes of physiological indexes,such as content of proline,malonaldehyde,and reducing sugar in Yixing lily leaf and bulb were observed,when soil water content changed. [Result]The response to drought stress of the Yixing lily leaf was greater than that of the bulb,and the content of the proline and malonaldehyde was higher than that of the reducing sugar. [Conclusion]The leaves as an appropriate material for evaluation and analysis on drought resistance can better reflect the physiological responses of Yixing lily to drought stress. And the content of the malonaldehyde and that of proline are preferable indexes.

Changes and Correlations of Duroc Muscle pH,Glycogen,Lactic Acid and TBA under Different Storage Condition

The changes and correlations of muscle pH, glycogen, lactic acid and in- tramuscular fat oxidation in Duroc pigs 10 d after their slaughter, and the effects of different storage temperature and time on Duroc muscle pH value, water loss rate, glycogen, lactic acid and 2-thiobarbituric acid （TBA） were studied. The results showed that during the 10 h after the slaughter, the pH value was decreased rapid- ly, the lactic acid content was increased significantly, while the glycogen and TBA contents were remained stable. At the storage temperature of 4 ℃, storage time showed no significant effects on Duroc muscle pH value and glycogen, lactic acid and TBA contents. At the storage temperature of -20 ℃, storage temperature had significant effects on pH value, while no significant effects on other indicators. The correlation analysis demonstrated that during the 10 h after the slaughter, the TBA content was negatively related to glycogen content （P〈0.05）, but positively related to lactic content （P〈0.05）; the pH value was negatively related to lactic acid content （P〈0.05）. At the storage temperature of 4 ℃, the TBA content was negatively relat- ed to water loss rate （P〈0.01） and lactic acid content （P〈0.05）; the water loss rate was positively related to pH value （P〈0.01） and lactic acid content （P〈0.05）. At the storage temperature of -20 ℃, the TBA content was negatively related to pH value （P〈0.01） and positively related to water loss rate （P〈0.05）; the water loss rate was negatively related to pH value （P〈0.01） and lactic acid content （P〈0.05）.

Dynamical changing patterns of glycogen and enzyme histochemical activities in rat liver graft undergoing warm ischemia injury

AIM:To investigate the changing patterns of glycogen and enzyme histochemical activities in rat liver graft under a different warm ischemia time (WIT) and to predict the tolerant time limitation of the liver graft to warm ischemia injury. METHODS: The rats were randomized into five groups, WIT was 0,15,30,45,60 min, respectively, and histochemical staining of liver graft specimens was observed. The recovery changes of glycogen and enzyme histochemistry activities were measured respectively 6 and 24 h following liver graft implantation. RESULTS: The activities of succinic dehydrogenase, cytochrome oxidase, apyrase (Mg++-ATPase) and content of glycogen were decreased gradually after different WIT in a time-dependent manner. The changes were significant when WIT was over 30 min. CONCLUSION: Hepatic injury is reversible within 30 min of warm ischemia injury. Glycogen and enzyme histochemistry activities of liver grafts and their recovery potency after reperfusion may serve as criteria to evaluate the quality of liver grafts.

Glycogen synthase kinase-3β positively regulates the proliferation of human ovarian cancer cells

Although glycogen synthase kinase-3 （GSK-3） might act as a tumor suppressor since its inhibition is expected to mimic the activation of Wnt-signaling pathway, GSK-3β may contribute to NF-κB activation in cancer cells leading to increased cancer cell proliferation and survival. Here we report that GSK-3β activity was involved in the proliferation of human ovarian cancer cell both in vitro and in vivo. Inhibition of GSK-3 activity by pharmacological inhibitors suppressed proliferation of the ovarian cancer cells. Overexpressing constitutively active form of GSK-3β induced entry into the S phase, increased cyclin D1 expression and facilitated the proliferation of ovarian cancer cells. Furthermore, GSK-3 inhibition prevented the formation of the tumor in nude mice generated by the inoculation of human ovarian cancer cells. Our findings thus suggest that GSK-3β activity is important for the proliferation of ovarian cancer cells, implicating this kinase as a potential therapeutic target in ovarian cancer.

Association of C(-106)T Polymorphism in Aldose Reductase Gene with Diabetic Retinopathy in Chinese Patients with Type 2 Diabetes Mellitus

Objective To identify the possible association between C（-106）T polymorphism of the aldose reductase （ALR） gene and diabetic retinopathy （DR） in a cohort of Chinese patients with type 2 diabetes mellitus （T2DM）. Methods From November 2009 to September 2010, patients with T2DM were recruited and assigned to DR group or diabetic without retinopathy （DWR） group according to the duration of diabetes and the grading of 7-field fundus color photographs of both eyes. Genotypes of the C（-106）T polymorphism （rs759853） in ALR gene were analyzed using the MassARRAY genotyping system and an association study was performed. Results A total of 268 T2DM patients （129 in the DR group and 139 in the DWR group） were included in this study. No statistically significant differences were observed between the 2 groups in the age of diabetes onset （P=0.10） and gender （P=0.78）. The success rate of genotyping for the study subjects was 99.6% （267/268）, with one case of failure in the DR group. The frequencies of the T allele in the C（-106）T polymorphism were 16.0% （41/256） in the DR group and 19.4% （54/278） in the DWR group （P=0.36）. There was no signit^cant difference in the C（-106）T genotypes between the 2 groups （P=0.40）. Compared with the wild-type genotype, odds ratio （OR） for the risk of DR was 0.7 （95% CI, 0.38-1.3） for the heterozygous CT genotype and 0.76 （95% CI, 0.18-3.25） for the homozygous TT genotype. The risk of DR was positively associated with microalbuminuria （OR=4.61; 95% CI, 2.34-9.05） and insulin therapy （OR=3.43; 95% CI, 1.94-6.09）. Conclusions Microalbuminuria and insulin therapy are associated with the risk of DR in Chinese patients with T2DM. C（-106）T polymorphism of the ALR gene may not be significantly associated with DR in Chinese patients with T2DM.

Glycogenotic hepatocellular carcinoma with glycogen-ground-glass hepatocytes: A heuristically highly relevant phenotype

Glycogenotic hepatocellular carcinoma (HCC) with glycogen-ground-glass hepatocytes has recently been described as an allegedly "novel variant" of HCC, but neither the historical background nor the heuristic relevance of this observation were put in perspective. In the present contribution, the most important findings in animal models and human beings related to the emergence and further evolution of excessively glycogen storing (glycogenotic) hepatocytes with and without ground glass features during neoplastic development have been summarized. Glycogenotic HCCs with glycogen-ground-glass hepatocytes represent highly differentiated neoplasms which contain subpopulations of cells phenotypically resembling those of certain types of preneoplastic hepatic foci and benign hepatocellular neoplasms. It is questionable whether the occurrence of glycogen-ground-glass hepatocytes in a glycogenotic HCC justifies its classification as a specific entity. The typical appearance of ground-glass hepatocytes is due to a hypertrophy of the smooth endoplasmic reticulum, which is usually associated with an excessive storage of glycogen and frequently also with an expression of the hepatitis B surface antigen. Sequential studies in animal models and observations in humans indicate that glycogen-ground-glass hepatocytes are a facultative, integral part of a characteristic cellular sequence commencing with focal hepatic glycogenosis potentially progressing to benign and malignant neoplasms. During this process highly differentiated glycogenotic cells including ground-glass hepatocytes are gradually transformed via various intermediate stages into poorly differentiated glycogen-poor, basophilic (ribosome-rich) cancer cells. Histochemical, microbiochemical, and molecular biochemical studies on focal hepatic glycogenosis and advanced preneoplastic and neoplastic lesions in tissue sections and laser-dissected specimens in rat and mouse models have provided compelling evidence for an early insulinomimetic effect of oncogenic agents, which is followed by a fundamental metabolic switch from gluconeogenesis towards the pentose-phosphate pathway and the Warburg type of glycolysis during progression from preneoplastic hepatic glycogenosis to the highly proliferative malignant phenotype.

^(18)F-fluorodeoxyglucose PET/CT findings of a solitary primary hepatic lymphoma:A case report

Primary hepatic lymphoma is extremely rare,and only a few cases have been described on positron emission tomography(PET) or PET/computed tomography(PET/CT) imaging in the English literature.We report a case of a 55-year-old woman who presented with low-grade fever and weight loss of three months.On CT scanning,a mass was identified which appeared to be a hypoattenuating lesion,on ultrasonographic imaging,the mass was hypoechoic,therefore,liver abscess or hepatic metastasis from a gastrointestinal primary was initially suspected.Tumor markers such as alpha-fetoprotein,carcinoembryonic antigen and carbohydrate antigen 19-9 were within normal limits.PET/CT demonstrated a large abnormal ring-like hypermetabolic focus in the right liver lobe.The lesion was resected and the histo-pathological findings were consistent with lymphoma.The patient was discharged two weeks after surgery and did not receive any further treatment.After 25 mo follow-up,she is in good health.18F-fluorodeoxyglucose PET/CT is useful in confirming the diagnosis of primary hepatic lymphoma by demonstrating no other foci with high uptake in other parts of the body.

Adenoviral-mediated localized CTLA-4Ig gene expression induces long-term allograft pancreas survival and donor-specific immune tolerance in rats

T cell activation following alloantigen recognition plays a critical role in the development of the rejection in all solid organ, tissue and cell transplantation. A recombinant molecule, cytotoxic T lymphocyte antigen 4 antibody （CTLA-4Ig）, is known to induce to T-cell into ＂anergy＂ by blocking the costimulatory B7-CD28 interaction. Either systemic or localized administration of CTLA-Ig has been shown to prolong allograft survival and induce donor-specific tolerance in some transplant models. In this study, we characterized the expression and immunosuppressive effectiveness of adenoviral-mediated CTLA-4Ig gene transfer. We demonstrated transduction of the allografts with AdCTLA-4Ig resulted in localized expression, permanent graft survival and stable donor-specific tolerance. In addition, by performing simultaneous dual-organ through a local expression of CTLA-4Ig via adenoviral-mediated transplantation, we targeted on immunosuppression gene transfer into pancreatic allografts.

Effect of Acupuncture on Plasmic Levels of Insulin,Glucagon and Hypercoagulability in NIDDM Complicated by Acute Cerebral Infarction

Twenty-one cases of acute cerebral infarction secondary to NIDDM were treated with acupuncture and conventional therapy, and compared with 16 cases treated with conventional therapy alone. The results showed that acupuncture was more effective in reducing insulin and glucagon levels (P

High doses of α-galactosylceramide potentiate experimental autoimmune encephalomyelitis by directly enhancing Th17 response

α-Galactosylceramide （u-GC） is widely known to activate invariant natural killer T （iNKT） cells to suppress my- elin antigen-specific Thl responses, protecting susceptible mice against experimental antoimmune encephalomyelitis 0EAE）. Here, we demonstrate an unexpected finding that high doses of α-GC exacerbated, rather than ameliorated, EAE. Similar results were observed when MOG35.ss-specific T cells treated with high-dose α-GC were transferred into naive syngeneic recipient mice. Further study showed that high doses of a-GC directly enhance the Thl7 and Thl re- sponse by activation of CD4＋CD44＋ memory T cells through phosphorylation of STAT3 and activation of NF-kB. Un- like the activation of iNKT cells by low doses of a-GC, high doses of a-GC directly interacted with CDld expressed on T ceils and activated Thl7 and Thl cells. Furthermore, antigen-presenting cells （APCs） predominantly express CDldl, whereas the majority of CD4~ T cells express CDld2. Knockdown of CDldl or CDld2 gene expression by RNAi interfered with the activation of iNKT or Thl7/Thl cells, respectively. Therefore, α-GC treatment could im- prove or worsen EAE by engaging either APCs or Thl7/Thl cells depending on the dose used.