成人多发性肌炎或皮肌炎并发慢性假性肠梗阻的临床特点

目的探讨成人多发性肌炎皮肌炎(polymyositisdermatomyositis,PMDM)合并慢性假性肠梗阻(chronic intestinal pseudo-obstruction,CIPO)患者的临床特点及预后,以提高对此类疾病的认识。方法收集北京协和医院2003年11月至2013年10月临床成人PMDM合并CIPO患者的临床资料,回顾性分析及总结临床表现、实验室检查、诊治及转归。结果合并CIPO的成人PM患者2例,DM患者4例,分别占同期住院成人PMDM患者的0.45%(2441)及0.54%(4747)。6例患者男女比例为1∶2,平均年龄(41±15)岁。CIPO均非临床首发症状,发病时间为PMDM诊断后1~10年,部位均位于小肠。83%(56)患者病程中合并出现急性上消化道出血,2例患者经胃镜明确出血部位分别为胃小弯及十二指肠降部,镜下均表现为多发黏膜溃疡出血。6例患者中33%(26)在出现肠梗阻时并无明确肌肉受累表现,67%(46)肌酸激酶、红细胞沉降率及超敏C反应蛋白均处于正常范围。1例患者发病时肌肉活检示小血管炎性病理改变。6例患者中5例接受足量激素(1 mgkg)治疗,2例接受大剂量激素冲击(甲泼尼龙1 gd)联合静脉用人免疫球蛋白治疗,50%(36)患者最终完全缓解。结论成人PMDM患者合并CIPO发病机制尚不清楚,不除外与继发血管炎有关,由于病程中通常合并急性消化道出血,临床处理非常困难,总体死亡率高。早期识别并及时给予大剂量糖皮质激素和免疫抑制剂可能有助于改善预后。     

Achalasia in a Patient with Gastroesophageal Varices: Problematic Treatment Decisions

The coexistence of achalasia and gastroesophageal varices has been reported sporadically in the English medical literature. We report the case of a 60-year-old Hispanic woman with cryptogenic cirrhosis who was referred for a liver transplant evaluation and subsequently developed progressive dysphagia to both solids and liquids as well as substernal chest pain and weight loss. Endoscopy revealed the presence of grade I esophageal varices and large fundic varices, as well as retained liquid and solid food in the distal esophagus. Radiographic and manometric studies were consistent with achalasia. After botulinum toxin (Botox) injections were no longer effective a transjugular intrahepatic portosystemic shunt was performed for portal decompression before proceeding with pneumatic dilation. Optimal treatment of these 2 conditions, when they occur simultaneously, is problematic. We discuss this patient's management and our approach to this infrequent combination of diseases.     

A Coincidental Discovery of a New Stable Variant (Hb Hachioji or HBB: c.187C>T) in a Patient with Chronic Hemolytic Anemia of Unexplained Origin

We report a new hemoglobin (Hb) variant, Hb Hachioji (HBB: c.187C>T), which was detected in a 32-year-old male with hemolytic anemia. The proband had undergone splenectomy in his childhood after being diagnosed with hereditary spherocytosis (HS) with no clinical improvement. A recent study showed that Heinz bodies were frequently observed in his red cells, however, no abnormal band was separated by isoelectric focusing (IEF), and the isopropanol (instability) test was negative. Direct sequencing revealed that the proband was a heterozygous carrier of a novel mutation (GCT>GTT) at codon 62 of the β-globin gene, leading to an alanine to valine substitution. This variant was named Hb Hachioji. Characterization at the mRNA level by cDNA sequencing detected β^Hachioji mRNA, as well as β^A mRNA. Subsequently, study of the proband’s family indicated that his father was a carrier of this Hb variant, although unexpectedly, the father was asymptomatic and clinically healthy. Oxygen affinity measurement of total Hb showed no alteration in the P₅₀ and oxygen equilibrium curve. The presence of Hb Hachioji was confirmed by mass spectrometry (MS). Hb Hachioji comprised approximately 50.0% of the total Hb and was a stable variant. The phenotypic discrepancy between these two carriers suggests that Hb Hachioji may not be associated with the hemolytic involvement in the proband. P4.2Nippon, which is the primary cause of most cases of Japanese HS, was absent in the proband’s parents. The coexistence of glucose-6-phosphate dehydrogenase (G6PD) deficiency was ruled out. Thus, the cause of hemolytic involvement in this patient remains unclear.     

Behavioral Treatment of Intractable Chest Pain in a Patient with Vigorous Achalasia

Chest pain is a major symptom of patients diagnosed with esophageal motility abnormalities. Motility disorders of the esophagus are also associated with elevated scores on measures of somatic anxiety and depression. In spite of this relationship between psychological characteristics and esophageal motility disturbances, few attempts have been made to treat complaints of chest pain in patients with esophageal motility disorders using psychological methods. This report describes the successful use of a behavioral pain management program for the treatment of persistent chest pain in a patient diagnosed with vigorous achalasia who was previously treated with pneumatic dilatation and a long Heller myotomy. This is the first report on the use of psychotherapy in treating chest pain associated with vigorous achalasia, and suggests that, in the etiology and treatment of chest pain in patients with esophageal motility disturbances, psychological influences may be more important than has generally been recognized. No long-term relationship between esophageal motility disturbances and complaints of chest pain was found.     

Severity of Airflow Obstruction in Chronic Obstructive Pulmonary Disease (COPD): Proposal for a New Classification

Current classifications of Chronic Obstructive Pulmonary Disease (COPD) severity are complex and do not grade levels of obstruction. Obstruction is a simpler construct and independent of ethnicity. We constructed an index of obstruction severity based on the FEV₁/FVC ratio, with cut-points dividing the Burden of Obstructive Lung Disease (BOLD) study population into four similarly sized strata to those created by the GOLD criteria that uses FEV₁. We measured the agreement between classifications and the validity of the FEV₁-based classification in identifying the level of obstruction as defined by the new groupings. We compared the strengths of association of each classification with quality of life (QoL), MRC dyspnoea score and the self-reported exacerbation rate. Agreement between classifications was only fair. FEV₁-based criteria for moderate COPD identified only 79% of those with moderate obstruction and misclassified half of the participants with mild obstruction as having more severe COPD. Both scales were equally strongly associated with QoL, exertional dyspnoea and respiratory exacerbations. Severity assessed using the FEV₁/FVC ratio is only in moderate agreement with the severity assessed using FEV₁ but is equally strongly associated with other outcomes. Severity assessed using the FEV₁/FVC ratio is likely to be independent of ethnicity.     

Small Cell Carcinoma of the Esophagus in a Patient with Longstanding Primary Achalasia

Achalasia is believed to be a predisposing factor for the development of esophageal cancer. Small cell carcinoma of the esophagus is a rare neoplasm, with fewer than 150 cases having been reported in the world literature, and it has been described only once previously in a patient with longstanding achalasia. We describe a case of an 85-yr-old woman with long-term primary achalasia who developed primary small cell carcinoma of the esophagus. We hypothesize that this patient's recurrent, worsening dysphagia is related to a paraneoplastic phenomenon. We discuss this association and review the literature.     

Beneficial Effects of Naloxone in a Patient with Intestinal Pseudoobstruction

A 15-day course of Naloxone treatment was given to a patient with intestinal pseudoobstruction who had previously undergone subtotal colectomy with terminal ileostomy for invalidating constipation. The effects of the drug were assessed according to symptoms, by recording the myoelectric activity of the stomach, and by measuring gastric emptying of a radiolabeled solid-liquid meal and the intestinal transit time of radiopaque markers. All tests were performed 1) at baseline; 2) after 2 wk with Naloxone 1.6 mg subcutaneous per day; and 3) after 8 days of placebo. Results showed that before treatment gastric emptying of solids was delayed, emptying of liquids was normal, myoelectric activity of the stomach was normal, small intestinal transit time of radiopaque markers was considerably increased while ileal output was markedly decreased. After Naloxone, gastric emptying of solids was markedly accelerated, emptying of liquids remained normal, gastric electrical spiking activity increased, small intestinal transit time strikingly decreased, and ileal output increased. After placebo, a tendency to return to pretreatment values was observed. This observation suggests that Naloxone may be helpful in the treatment of some patients with intestinal pseudoobstruction.     

Patient Assessment of Chronic Illness Care (PACIC) and Improved Patient-centered Outcomes for Chronic Conditions

Background The Patient Assessment of Chronic Illness Care (PACIC) has potential for use as a patient-centered measure of the implementation of the Chronic Care Model (CCM), but there is little research on the relationship between the PACIC and important behavioral and quality measures for patients with chronic conditions. Objective To examine the relationship between PACIC scores and self-management behaviors, patient rating of their health care, and self-reported quality of life. Design Cross-sectional survey with a 61% response rate. Participants Included in the survey were 4,108 adults with diabetes, chronic pain, heart failure, asthma, or coronary artery disease in the Kaiser Permanente Medical Care program across 7 regions nationally. Measurements The PACIC was the main independent variable. Dependent variables included use of self-management resources, self-management behaviors such as regular exercise, self-reported adherence to medications, patient rating of their health care, and quality of life. Results PACIC scores were significantly, positively associated with all measures (odds ratio [ORs] ranging from 1.20 to 2.36) with the exception of self-reported medication adherence. Conclusions Use of the PACIC, a practical, patient-level assessment of CCM implementation, could be an important tool for health systems and other stakeholders looking to improve the quality of chronic disease care. Electronic supplementary material The online version of this article (doi: ) contains supplementary material, which is available to authorized users.     

Magnetic Compression Duct-to-duct Anastomosis for Biliary Obstruction in a Patient with Living Donor Liver Transplantation

Magnetic compression anastomosis (MCA) is a minimally invasive method of performing choledochocholedochostomy without surgery in patients with biliary stricture or obstruction. We describe a successful case involving magnetic compression duct-to-duct biliary reconstruction in right-lobe living donor liver transplantation (RL-LDLT). Endoscopically, a samarium-cobalt (Sm-Co) rare-earth magnet was placed at the superior site of obstruction via the percutaneous transhepatic biliary drainage route, and another Sm-Co magnet was also placed at the inferior site of obstruction with the aid of an endoscope. MCA techniques enabled complete anastomosis without procedure-related complications. In conclusion, the MCA technique is a revolutionary method of performing choledochocholedochostomy in patients with biliary obstruction after LDLT.     

Improvement of the Guide Wire in the Long Intestinal Tube Used for Endoscopic Intubation in Patients with Intestinal Obstruction

Abstract: In 1980, an endoscopic intubation method utilizing a long intestinal tube for ileus was developed. However, with this method the success rate of jejunal intubation was 89.4%, and the intubation time was 17.5±8.1 minutes (Mean±S. D.). These results were unsatisfactory as “rapid and reliable intubation” was the initial objective. Because the earliest guide wire was excessively elastic and therefore pliable, it formed a loop in the stomach. The guide wire was then improved by increasing its rigidity by approximately 20%. With this improvement, the force needed to advance the guide wire can now be effectively transmitted to its tip. As a result, the initial objective of improving the rate of jejunal intubation with the long tube (to 100%) and shortening the intubation time (to 8.9±3.9 min.) was achieved.     

Extrahepatic Portal Vein Obstruction and Portal Vein Thrombosis in Special Situations: Need for a New Classification

Extrahepatic portal vein obstruction is a vascular disorder of liver, which results in obstruction and cavernomatous transformation of portal vein with or without the involvement of intrahepatic portal vein, splenic vein, or superior mesenteric vein. Portal vein obstruction due to chronic liver disease, neoplasm, or postsurgery is a separate entity and is not the same as extrahepatic portal vein obstruction. Patients with extrahepatic portal vein obstruction are generally young and belong mostly to Asian countries. It is therefore very important to define portal vein thrombosis as acute or chronic from management point of view. Portal vein thrombosis in certain situations such as liver transplant and postsurgical/liver transplant period is an evolving area and needs extensive research. There is a need for a new classification, which includes all areas of the entity. In the current review, the most recent literature of extrahepatic portal vein obstruction is reviewed and summarized.