Myopathological features in MELAS syndrome without significant changes in muscle biopsy pathology

Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes （MELAS） are common types of mitochondrial encephalomyopathy. The involved muscular pathology is characterized by typical changes of mitochondrial abnormalities. Gene screening has been the gold diagnostic standard for MELAS diagnosis. This study presents three primary MELAS patients, with an age of onset from 13 to 18 years, including one patient with seizure, and two with headache and vomiting. All patients had a family history of disease, with maternal inheritance. Cerebral magnetic resonance imaging revealed abnormally high signals in T2-weighted images： temporal lobe in three cases, occipital lobe in two cases, and parietal lobe in one case. Migrating stroke-like lesions were confirmed in one patient. Muscle biopsy revealed several strongly succinate dehydrogenase-reactive vessels scattered in muscle sections of three patients, but ragged-red fibers and cytochrome c oxidase-negative/dense （COX-/＋） fibers were not observed. Mitochondrial DNA A3243G mutation was identified in all three cases. MELAS syndrome has obvious clinical heterogeneity, and muscle weakness was not prominent in some of the cases. Muscle pathological changes did not accompany ragged-red fibers or COX-/＋ fibers, but succinate dehydrogenase- reactive vessels are important for MELAS diagnosis.

Prolapse gastropathy syndrome may be a predictor of pathologic acid reflux

AIM： To assess the occurrence of gastric acid reflux into the esophagus in endoscopically confirmed prolapse gastropathy syndrome （PGS）. METHODS： Using ambulatory esophageal pH measurement （BRAVOTM wireless esophageal pH monitoring system）, twenty-six patients with PGS were compared with twenty-one patients with erosive esophagitis （EE） as controls. We assessed several reflux parameters, including the percentage of total time at pH 〈 4, and the DeMeester score. RESULTS： There were no statistical differences between the PGS group and the EE group as to mean age, sex ratio and pH recording time. The EE group showed more severe reflux than the PGS group, as evaluated in terms of the longest duration of reflux, the number of reflux episodes, the number of reflux episodes lasting 〉 5 min, the total time with pH 〈 4 during acid reflux episodes, and the DeMeester score, but none of these parameters showed statistically significant difference. Although 53.8% （14/26） of the PGS group and 76.2% （16/22） of the EE group demonstrated pathologic acid reflux （DeMeester score 〉 14.72）, there was no statistically significant difference between the two groups in the incidence of pathologic acid reflux （P = 0.11）. CONCLUSION： There was no statistically significant difference in pathologic acid reflux between the PGS and EE group. These data suggest that endoscopically diagnosed PGS might be a predictor of pathologic acid reflux.

Mystery behind labial and oral melanotic macules: Clinical, dermoscopic and pathological aspects of Laugier-Hunziker syndrome

Labial and oral melanotic macules are commonly encountered in a broad range of conditions ranging from physiologic pigmentation to a sign of an underlying life-threatening disease. Although Laugier-Hunziker syndrome(LHS) shares some features of labial and oral pigmentation with a variety of conditions, it is a benign and acquired condition, frequently associated with longitudinal melanonychia. Herein, the demographic, clinical, dermoscopic, and pathological aspects of LHS were reviewed comprehensively. The important differential diagnoses of mucocutaneous and nail pigmentation are provided. An accurate diagnosis is crucial to design a reasonable medical strategy, including management options, malignant transformation surveillance, and psychological support. It is important that clinicians conduct long-term follow-up and surveillance due to the potential risks of malignant transformation and local severe complications in some conditions.

Posner-Schlossman syndrome induced laser in situ keratomileusis keratectasia–a case report

Iam Dr. Xiu Wang, from Tianjin Medical University Eye Hospital, Tianjin, China. I write to present one case report of Posner-Schlossman syndrome （PSS） induced laser in situ keratomileusis （LASIK） keratectasia.

Posner-Schlossman syndrome:a 10-year review of clinical experience

Background:To describe our 10-year experience with Posner-Schlossman syndrome(PSS)in terms of morbidity and prognosis.Methods:We investigated 72 cases(74 eyes;both eyes of 2 cases)of PSS between July 2005 and July 2015.Data were collected on intraocular pressure(IOP),keratic precipitates(KP),anterior chamber,pupil,optic nerve disc and visual field,as well as general characteristics.Each case was examined every 3 to 7 days during attacks and every 2 to 6 months between attacks.Results:The mean age of the 72 patients(41 men)was 40±6 years(range,16-64 years).The mean disease duration was 4.3 years(range,1-7 years)and mean follow-up 4.9 years(range,1-8 years).Medical treatment was efficacious for 62 patients(62 eyes,83.78%).Ten patients exhibited progressive cupping and visual field loss(12 eyes,16.22%),all had normal IOP and no recurrence of PSS after trabeculectomy with anti-metabolite treatment.Conclusions:Although the prognosis of most PSS is usually benign,the IOP and optic nerve disc must be monitored.Visual-field damage can occur with long-term disease and frequent attacks.

Inflammation in irritable bowel syndrome:Myth or newtreatment target?

Low-grade intestinal inflammation plays a key role in the pathophysiology of irritable bowel syndrome(IBS), and this role is likely to be multifactorial. The aim of this review was to summarize the evidence on the spectrum of mucosal inflammation in IBS, highlighting the relationship of this inflammation to the pathophysiology of IBS and its connection to clinical practice. We carried out a bibliographic search in Medline and the Cochrane Library for the period of January 1966 to December 2014, focusing on publications describing an interaction between inflammation and IBS. Several evidences demonstrate microscopic and molecular abnormalities in IBS patients. Understanding the mechanisms underlying low-grade inflammation in IBS may help to design clinical trials to test the efficacy and safety of drugs that target this pathophysiologic mechanism.

Adrenal myelolipoma within myxoid cortical adenoma associated with Conn's syndrome

The coexistence of myelolipoma within adrenal cortical adenoma is extremely rare,for both tumors present usually as separate entities.There are only 16 such cases reported worldwide.To the best of our knowledge,the case we reported here is the first one of myxoid adrenal cortical adenoma associated with myelolipoma reported.A 32-year-old Chinese woman with 4-year history of hypertension was presented in our study.Computed tomography(CT)of the abdomen showed a large heterogene- ously-enhancing mass(4.5 cm in diameter)in the left suprarenal region.Clinical history and laboratory results suggest a metabolic disorder as Conn's syndrome.The patient underwent a left adrenalectomy,and a histopathological study confirmed the mass to be a myxoid adrenal cortical adenoma containing myelolipoma.The patient was postoperatively well and discharged uneventfully.In the present case report,we also discuss the etiology of simultaneous myelolipoma and adrenal adenoma associated with Conn's syndrome,and the methods of the diagnosis and differential diagnosis.

Expression of bcl-2 oncogene in gastric precancerous lesions and its correlation with syndromes in traditional Chinese medicine

AIM: To observe the protein and mRNA expression of bcl-2 oncogene in gastric precancerous lesions (GPL) and to analyze its correlation with syndromes in traditional Chinese medicine (TCM). METHODS: Sixty-seven patients with GPL confirmed by gastroscopy and pathology were studied,including 39 cases of moderate gastric mucosal dysplasia,19 cases of severe gastric mucosa dysplasia, 9 cases of incomplete colon metaplasia. In syndrome differentiation of TCM,17 cases belonged to the syndrome of qi and yin deficiency of the spleen and stomach complicated by qi stagnation, 21 cases belonged to the syndrome of qi and yin deficiency of the spleen and stomach complicated by stomach heat, 29 cases belonged to the syndrome of qi and yin deficiency of the spleen and stomach complicated by blood stasis. Protein and mRNA expression of bcl-2 oncogene were detected by labeled streptavidin biotin (LSAB) immunohist-ochemistry and in situ hybridization respectively. RESULTS: Abnormal expression of protein and mRNA on bcl-2 oncogene was found in GPL,which increased gradually with the course of lesions. In moderate and severe gastric mucosal dysplasia and incomplete colon metaplasia,there was no difference in the expression of bcl-2 oncogene (P>0.05).In different accompanying syndromes, the expression of protein and mRNA on bcl-2 oncogene increased gradually in the following order: deficiency of both qi and yin of the spleen and stomach accompanying qi stagnation→stomach heat→blood stasis. In GPL, compared with accompanying blood stasis, there was an obvious difference in the expression of bcl-2 oncogene between the syndrome of qi and yin deficiency of the spleen and stomach and accompanying stomach heat, so did accompanying qi stagnation (the level of protein: X2=8.45, P<0.05;the level of mRNA: X2=7.35, P<0.05). CONCLUSION: Apoptosis-associated bcl-2 oncogene is abnormally expressed in GPL,which correlates with different accompanying syndromes in TCM.

Mucosal necrosis of the small intestine in myopathy,encephalopathy,lactic acidosis,and stroke-like episodes syndrome

This report presents a case of massive mucosal necrosis of the small intestine in a patient with mitochondrial myopathy,encephalopathy,lactic acidosis,and stroke-like episodes(MELAS),which particularly affects the brain,nervous system and muscles.A 45-year-old Japanese female,with an established diagnosis of MELAS,presented with vomiting.Computed tomography showed portomesenteric venous gas and pneumatosis intestinalis.She underwent a resection of the small intestine.A microscopic study showed necrosis of the mucosa and vacuolar degeneration of smooth muscle cells in the arterial wall.Immunohistochemistry showed anti-mitochondrial antibody to be highly expressed in the crypts adjacent the necrotic mucosa.The microscopic and immunohistochemical findings suggested the presence of a large number of abnormal mitochondria in MELAS to be closely linked to mucosal necrosis of the small intestine.

Fatal Acute Compartment Syndrome Following a Falling Injury： A Case Report

Acute compartment syndrome （ACS） usually occurs after an injury to a limb or other adjacent organ, when there is insufficient blood supply to muscles and nerves due to swelling and increased pressure within a body compartment. Classically, there are six symptoms and signs associated with ACS, all of which have an initial letter ＇P＇： pain, paresthesia, pallor, paralysis, pulselessness and poik- ilothermia The complexities of the clinical diag- nosis and management of ACS remain a challeng- ing oroblem for orthooedic surgeons.

Sudden Death due to Diffuse Alveolar Hemorrhage Induced by Pul- monary Renal Syndrome： Two Case Reports

Patients developing hemoptysis or pulmonary infiltrates together with glomerulonephritis, particu- larly the rapidly progressive cases, are considered to be pulmonary renal syndrome （PRS）. The occur- rence of PRS, an etiologically rare heterogeneous group of diseases, constitutes a medical emergency associated with a high risk of fatal outcome.

A novel mouse model of central cord syndrome

Patients with potential spinal stenosis are susceptible to central cord syndrome induced by blunt trauma.Suitable animal models are helpful for studying the pathogenesis and treatment of such injuries.In this study,we established a mouse model of acute blunt traumatic spinal cord injury by compressing the C6 spinal cord with 5 and 10 g/mm~2 compression weights to simulate cervical central cord syndrome.Behavioral testing confirmed that this model exhibited the characteristics of central cord syndrome because motor function in the front paws was impaired,whereas basic motor and sensory functions of the lower extremities were retained.Hematoxylin-eosin staining showed that the diseased region of the spinal cord in this mouse model was restricted to the gray matter of the central cord,whereas the white matter was rarely affected.Magnetic resonance imaging showed a hypointense signal in the lesion after mild and severe injury.In addition,immunofluorescence staining showed that the degree of nerve tract injury in the spinal cord white matter was mild,and that there was a chronic inflammation reaction.These findings suggest that this mouse model of central cord syndrome can be used as a model for preclinical research,and that gray matter is most vulnerable to injury in central cord syndrome,leading to impaired motor function.

Quantitative ultrastucture analysis of neuroendocrine cells of gastric mucosa on normal and pathological conditions

AIMS To study the quantitative ultrastucture of neu- roendocrine cells of gastric mucosa on normal anc pathological conditions including the duodenal ulcer (DU) and Zollinger-Ellison syndrome (ZES). METHODS The neuroendocrine cells of the gastric mucosa of eight normal subjects,six patients with DU and five patients with ZES were quantitatively investi- gated with electro microscope and ultrastructure image analyzer. RESULTS The volume density of neuroendocrine cells in DU was 1.3% and 0.8% (vs 1.6% and 0.9%,P>0.05) in gastric antrum and corpus respectively. In antrum,G cells was of 65% (P< 0.05),D cells decreased in cell density (3% vs 9.5%) and in number of cell per unit area (P<0.01). In corpus,the cell density of ECL cells increased (49% vs 30%,P<0.05);D cells and EC cells decreasec (2% P<0.01 and 4% P<0.05,respectively),and the number of D cell per unit area markedly decreased. In ZES,D cells in corpus decreased in cell density (4% vs 22%,P<0.01) and P cells also decreased (11% vs 24%,P<0.05). The density of ECL cells increased (65% vs 30%,P<0.01). CONCLUSIONS In DU and ZES,both the number and type of NE cells present some changes. Incresed gastrin in DU and ZES patients may be caused by the decrease of D cells and somatostatin secretion.

Study on the Relationship between Blood Stasis Syndrome and Clinical Pathology in 227 Patients with Primary Glomerular Disease

To investigate the relationship between the severity of Chinese medicine(CM) bloodstasis syndrome(BSS) with clinical features and renal lesion indexes of the primary glomerular disease. Methods:An epidemiological survey was conducted to collect the data of 227 patients diagnosed as chronic primary glomerular diseases,and their severity of BSS were scored three days before renal biopsies were performed.The following clinical indexes were analyzed:age,course of glomerular diseases,24-h urine protein ration(Up...

Potential of Renal Pathology on Refining Syndrome Typing of Chinese Medicine in IgA Nephropathy

Objective： To investigate the potential of renal pathological index as a differential diagnosis factor for Chinese medicine （CM） syndromes typing in IgA nephropathy （IgAN）. Methods： A total of 1,016 patients with IgAN was recruited from November 2001 to November 2004. All the signs and symptoms including picture of the tongue and pulse tracings were collected. All patients were typed according to the CM syndrome typing scheme for chronic primary glomerulopathy. The severity of glomerulus and tubulointerstitial lesions （mild, moderate- severe） were evaluated using lee＇s grading system and the Katafuchi score system. Results： The syndrome types transform in turn by deficiency of both the Spleen （Pi） and Lung （Fei） qi, deficiency of both qi and yin, deficiency of Liver （Gan） and Kidney （Shen） yin and deficiency of Spleen-Kidney （Shen） yang, with the aggravation of pathogenetic condition and that the manifestation of deficiency of qi clinically showed proliferative lesion of glomerular mesangium, while the glomerular sclerosis pathologically showed the manifestation of yin deficiency. Conclusion： Renal pathological findings may be a candidate of objective factors to refine CM syndrome typing process.

代谢综合征对肾透明细胞癌分级分期及围手术期相关指标的影响

目的探讨代谢综合征(metabolic syndrome,MS)对肾透明细胞癌(clear cell renal cell carcinoma,ccRCC)的病理分级、分期及围手术期相关指标的影响。方法选取2020年1月至2022年12月在苏州大学附属第一医院接受手术治疗的226例ccRCC患者的临床资料进行回顾性分析,根据是否合并MS分为MS组(n=67)与非MS组(n=159)。比较两组患者的病理分级、分期以及围手术期相关指标,主要包括美国麻醉医师协会分级(American society of anesthesiologists,ASA)评分、术前准备时间、手术时间、肾动脉阻断时间、术中出血量、术中输血率,术后肠道功能恢复、拔出导尿管时间、拔出腹膜后引流管时间、术后发热情况、术后住院时间等。结果高血压、糖尿病、高血脂与ccRCC病理分级、分期相关,差异均有统计学意义(P<0.05)。多因素logistic回归分析结果显示,高血压、糖尿病、高血脂是ccRCC分级的独立危险因素(P<0.05),高血脂是ccRCC分期的独立危险因素(P<0.05)。在腹腔镜下肾部分切除术(laparoscopic partial nephrectomy,LPN)与腹腔镜下肾根治切除术(laparoscopic radial nephrectomy,LRN)围手术期,MS组术前准备、手术、拔出腹膜后引流管、术后住院时间均长于非MS组,ASA分级≥Ⅲ级比例与术后发热比例高于非MS组,术中出血量大于非MS组,差异均有统计学意义(P<0.05)。结论MS与ccRCC病理分级、分期及围手术期相关指标均有相关性。因此,在ccRCC诊疗过程中,应重视MS,加强围手术期管理,帮助患者取得良好预后。

培本清利通络方治疗慢性肾脏病3~5期伴CKD-MBD脾肾两虚兼湿瘀证的临床研究

目的:观察基于吴门医派“络病理论”创立的培本清利通络方联合骨化三醇胶丸治疗慢性肾脏病(CKD)3~5期伴CKD-矿物质及骨代谢紊乱(CKD-MBD)脾肾两虚兼湿瘀证患者的效果。方法:选择2022年7月—2023年6月于南京中医药大学附属苏州市中医医院就诊的脾肾两虚兼湿瘀证CKD 3~5期合并有CKD-MBD的患者60例,随机分为治疗组和对照组,每组30例。对照组予控制血压、控制血糖、改善贫血等基础治疗,同时口服骨化三醇胶丸;治疗组在对照组治疗基础上加服培本清利通络方,两组疗程均为12周。比较两组患者治疗前后肾功能[血肌酐(Scr)、血尿素氮(BUN)、尿酸(UA)]、矿物质及骨代谢[钙(Ca)、磷(P)、全段甲状旁腺激素(iPTH)、碱性磷酸酶(ALP)]、中医症候积分、生活质量评分,并评估临床疗效;治疗前后检测两组血常规、肝功能、血钾,以评估用药安全性。结果:两组治疗后中医症候积分均较治疗前降低,且治疗组低于对照组(P<0.05);治疗组总有效率为93.33%,明显高于对照组的73.33%(P<0.05);治疗后,治疗组BUN、UA均明显低于治疗前,且Scr、BUN均明显低于对照组,差异均有统计学意义(P<0.05);治疗后,治疗组P、iPTH均明显低于治疗前,Ca明显高于治疗前,且治疗组Ca高于对照组,iPTH低于对照组,差异均有统计学意义(P<0.05);治疗后,治疗组生活质量评分较治疗前明显下降,且治疗组低于对照组(P<0.05);两组治疗后安全性指标比较,差异均无统计学意义(P>0.05)。结论:培本清利通络方可改善CKD 3~5期合并CKD-MBD脾肾两虚兼湿瘀证患者Ca、P、iPTH指标,延缓肾功能减退,减轻患者腰脊酸痛、皮肤瘙痒、倦怠乏力等症状,并可提高患者生活质量。

疏肝理气活血汤联合常规西药治疗慢性萎缩性胃炎临床研究

目的:观察疏肝理气活血汤联合常规西药治疗肝胃不和型慢性萎缩性胃炎(CAG)的临床疗效。方法:选择肝胃不和型CAG患者80例,按照随机数字表法分为观察组和对照组各40例。对照组给予枸橼酸莫沙必利片和瑞巴派特片治疗,观察组在对照组基础上加用疏肝理气活血汤治疗。评价2组临床疗效,比较2组治疗前后中医证候积分及病理检查评分,统计不良反应发生率。结果:观察组总有效率为85.00%,高于对照组70.00%(P<0.05)。治疗后,2组中医证候积分较治疗前下降(P<0.05),且观察组中医证候积分低于对照组(P<0.05)。治疗后,2组胃黏膜萎缩、肠上皮化生、异型增生评分较治疗前下降(P<0.05);且观察组胃黏膜萎缩、肠上皮化生、异型增生评分低于对照组(P<0.05)。观察组不良反应发生率低于对照组(P<0.05)。结论:疏肝理气活血汤联合常规西药治疗CAG可改善患者的临床症状,对于缓解或逆转胃黏膜萎缩、肠上皮化生和异型增生具有一定的治疗效果,且不良反应较少。

原发性胆汁性胆管炎不同证型与肝组织病理相关性研究

目的探讨原发性胆汁性胆管炎(PBC)不同证型与肝组织病理的相关性。方法将88例PBC患者进行辨证分型、实验室检查、肝组织活检,统计合并疾病情况,常规判定PBC患者炎症评分和肝纤维化分期。结果PBC患者的中医证型分布由高到低依次为肝肾阴虚证、肝郁脾虚证、湿热蕴结证、瘀血阻络证。统计结果显示,肝郁脾虚证、湿热蕴结证熊去氧胆酸(UDCA)应答率高于瘀血阻络证(P<0.05),肝肾阴虚证、瘀血阻络证肝硬化发生率高于肝郁脾虚证(P<0.05);瘀血阻络证血清碱性磷酸酶(ALP)、总胆红素(TBiL)、直接胆红素(DBiL)、总胆汁酸(TBA)水平高于肝郁脾虚证(P<0.05),肝肾阴虚证TBA水平高于肝郁脾虚证(P<0.05),湿热蕴结证血清总胆固醇(TC)水平高于肝郁脾虚证(P<0.05)。4种证型在合并高脂血症、干燥综合征(SS)上比较差异有统计学意义(P<0.05),其中湿热蕴结证PBC患者高脂血症发生率高于肝郁脾虚证(P<0.05),肝肾阴虚证PBC患者SS发生率高于湿热蕴结证(P<0.05)。4种证型在肝组织炎症分级及纤维化分级分布上有显著差异(P<0.01)。其中,肝肾阴虚证、瘀血阻络证的纤维化分级显著高于肝郁脾虚证(P<0.01)。结论PBC肝肾阴虚证、瘀血阻络证可能与肝纤维化的程度相关。

基于溶藻弧菌感染的虾苗细菌性玻化症(BVS)及其防控中药筛选和药效学评价

为了筛选可用于防治虾苗细菌性玻化症(bacterial vitrified syndrome,BVS)的中药复方,本研究首先通过牛津杯法和二倍稀释法进行体外抑菌实验,从50种中药中筛选出对BVS致病菌溶藻弧菌(Vibrio alginolyticus)和副溶血弧菌(V.parahaemolyticus)具有良好抑菌效果的中药。然后,以不同浓度的溶藻弧菌浸浴感染虾苗,建立BVS病理模型;再通过体内药效学实验从死亡率、组织病理学和超微组织病理学角度比较不同中药复方对于患BVS虾苗的防治效果。体外抑菌实验结果显示,五味子(Schisandrae chinensis fructus)、马鞭草(Verbenae herba)、乌梅(Mume fructus)等中药对溶藻弧菌的抑菌圈直径可达12 mm以上;诃子(Chebulae fructus)、五味子、牡丹皮(Moutan cortex)等中药对副溶血弧菌的抑菌圈直径可达13mm以上。五倍子(Gallachinensis)、诃子、五味子、丁香(Caryophylliflos)对溶藻弧菌和副溶血弧菌的最小抑菌浓度(MIC)均≤12.5mg/mL,最小杀菌浓度(MBC)均≤50 mg/mL。结合香附(Cyperi rhizoma)、栀子(Gardeniae fructus)2味中药组成3种中药复方,处方1、处方2和处方3;以5×10^(4) CFU/mL的溶藻弧菌感染虾苗成功建立BVS病理模型,在此基础上采用药物治疗患病虾苗7d,各组死亡率由低至高依次为空白对照组、处方1组、20%氟苯尼考粉对照组、处方3组、处方2组和阳性对照组。处方1组虾苗死亡率显著低于阳性对照组(P<0.05);处方2组、处方3组和20%氟苯尼考对照组虾苗死亡率低于阳性对照组,但差异不显著(P>0.05)。由此可见,处方1可显著降低患病虾苗死亡率。组织病理学和超微组织病理学研究显示,相比于其他感染组,处方1组虾苗肝胰腺病变程度明显较轻,肠道结构较为完整;虾苗肝小管上皮细胞的细胞膜、细胞核正常,线粒体、内质网丰富且基本正常。表明口服处方1可对患病虾苗的肝胰腺和肠道组织起到良好的保护作用,并优于处方2、处方3和20%氟苯尼考粉。综上所述,处方1(诃子30 g、五味子20 g、香附20 g)对溶藻弧菌感染的虾苗保护和治疗效果良好。本研究结果可为研制防控虾苗BVS专用中药提供依据,助力对虾养殖绿色高质量发展。