Motivational interviewing for adherence problems in cystic fibrosis

This review focuses on adherence in cystic fibrosis (CF), and the factors known to influence it. In particular, it discusses the importance of effective communication in clinical settings and considers the evidence for the effectiveness of motivational interviewing (MI), to increase adaptation and adherence in physical health and CF. The review also contains an overview of the key concepts of MI, its' practice in medical settings and recommendations on how to adopt MI techniques in the routine care of people with CF. Pediatr Pulmonol. 2010; 45:211–220. © 2010 Wiley‐Liss, Inc.     

Enteric circular muscle dysfunction in the cystic fibrosis mouse small intestine

Background Cystic fibrosis (CF) has multiple effects on the gastrointestinal system, including altered motility. The Cftr knockout mouse model of CF has impaired small intestinal transit but the mechanism is unknown. Methods Behaviour of circular smooth muscle was studied in an organ bath. Expression levels of prostaglandin (PG) degradative genes were measured by quantitative RT‐PCR, and PGE₂ levels were measured by enzyme immunoassay. Key Results Cystic fibrosis circular muscle activity was erratic and had variable frequency of contractions, as compared to WT. The CF tissue was non‐responsive to cholinergic stimulation or direct KCl depolarization. PGE₂ and PGF_2α are significantly elevated in the CF mouse small intestine, and we hypothesized these contribute to impaired smooth muscle activity. After inhibition of PG synthesis, the CF circular muscle exhibited greater cholinergic responsiveness, which was reversed by exogenous PGE₂. PGF_2α enhanced activity of CF tissue only after inhibition of PG synthesis. The enteric microbiota was implicated in PGE₂‐mediated dysmotility because broad spectrum antibiotic treated WT mice, which have slowed transit, exhibit impaired circular muscle activity. This was accompanied by decreased expression of PG degradative genes and increased intestinal PGE₂ levels. Furthermore, administration of oral laxative, which eradicates bacterial overgrowth and improves transit in CF mice, increased expression of PG degradative genes, decreased PGE₂ levels, and improved CF muscle activity. Conclusions & Inferences These results suggest that the enteric microbiota modulates PGE₂ levels in a complex manner, which affects enteric smooth muscle activity and contributes to slower small intestinal transit in CF.     

The effect of proteoglycans inhibited on the neurotropic growth of salivary adenoid cystic carcinoma

J Oral Pathol Med (2010) 40 : 476–482 Objective: To evaluate the relationship between inhibition of proteoglycans which secreted by salivary adenoid cystic carcinoma cell line (SACC‐83) and the neurotropic ability of the tumor cells. Methods: The expression vector of short hairpin RNA (shRNA‐WJ4) targeting xylosyltransferase‐I gene was constructed and transfected into SACC‐83 cells (group SACC83‐WJ4), shRNA‐HK used as negative control was transfected into SACC‐83 cells (group SACC‐83‐HK), SACC‐83 cells without transfection was used as black control (group SACC‐83). The xylosyltransferase‐I gene expression was measured by real‐time PCR. The content of proteoglycans was detected by Blyscan Assay Kit. The effect of down‐regulated proteoglycans on the perineural invasion of nude mice was observed. All data were analyzed by the software spss 13.0. Results: The results showed that the transfected efficiency of shRNA was 43.3%. The expression of xylosyltransferase‐I was inhibited by 43.0% 48 h after transfection of shRNA‐WJ4. The content of proteoglycans was down‐regulated by 30.25% 48 h after transfection. In the neurotropic experiment in vivo of nude mice, the rate of perineural invasion of group SACC‐83‐WJ4 was 33.33%, significantly lower than that of the negative control (100%) and the black control (100%) (P < 0.05). Conclusion: Xylosyltransferase‐I gene of SACC cells was silenced by RNA interference technology, proteoglycans secretion was reduced and neurotropic invasion behavior of SACC was inhibited obviously.     

Clinical classification of congenital extrahepatic portosystemic shunts

Aim: Congenital extrahepatic portosystemic shunt (CEPS) is a rare anomaly in which the enteric blood bypasses the liver and drains into the systemic veins through various venous shunts. Patients with CEPS often have liver tumors and complications such as cardiac or other anomalies, but portosystemic encephalopathy and gastrointestinal bleeding occur only occasionally. The clinical problems differ for each individual with CEPS, and establishing a prognosis can be very difficult. Methods: We reviewed the clinical features of 136 reported cases of CEPS and classified these cases according to their portosystemic shunts. Results: We classified portal blood flow directly into the inferior vena cava (IVC) as type A (88 cases), portal blood flow into the renal vein as type B (36 cases), and portal blood flow into the iliac vein via an inferior mesenteric vein as type C (12 cases). Type A patients were complicated with cardiac anomalies at a higher rate than other types. Type C patients had lower prevalences of cardiac anomalies and portosystemic encephalopathy than the other types, but the prevalence of gastrointestinal bleeding was significantly higher (P < 0.0001). The prognosis of CEPS has improved, and only six deaths have been previously reported, all of which occurred in type A patients. Conclusions: We reviewed the previously reported cases of CEPS. Classification according to the portosystemic shunt system might be useful for investigating the clinical features of CEPS.     

Laparoscopic management of primary hepatic pregnancy

A 30-year-old woman presented with epigastric pain with elevated serum human chorionic gonadotropin level (hCG), absence of intrauterine gestational sac and absence of an abnormal adnexal mass on pelvic ultrasonography. Laparoscopy revealed a ruptured hepatic ectopic pregnancy. This was removed by laparoscopic suctioning and haemostasis secured with Surgicel^® Fribrilla™ Absorbable Hemostat. Intramuscular methotrexate was administered post-operatively. Patient recovered uneventfully and serum hCG returned to normal.     

从临床流行病学调查探讨肝气郁结辨证标准

目的:通过临床流行病学调查,探讨肝气郁结辨证标准。方法:采用流行病学整群抽样法,从湖南中医药大学第一附属医院第二附属医院及第三附属医院2000年1月-2001年12月所有住院病例中,查找临床中医证候诊断属肝气郁结及其兼证者,填写调查表格,运用SPSS软件进行统计分析。结果:本组612例肝气郁结及相兼证候病例的常见症状中,胸胁、乳房、少腹胀痛481例,占78.59%;纳差450例,占73.53%;神疲乏力407例,占66.50%。纳差及神疲乏力分别排在肝气郁结及相兼证侯常见症状的第2位及第3位。而出现咽有梗阻感症状及巅顶头痛症状的病例分别仅2例,出现痛经、闭经或月经先后不定期症状的病例16例,且主要存在于妇科疾病中,三项分别仅占总例数的0.33%、0.16%和2.61%。结论:纳差及神疲乏力症状应纳入肝气郁结辨证标准,咽有梗阻感、巅顶头痛及痛经、闭经或月经先后不定期等特征性症状应归于兼症之列。本法为中医证侯学规范化研究的有效方法,比较客观、真实,符合临床实际情况。     

Ovarian tumor in a 12‐year old female with severe hypothyroidism: A case of Van Wyk and Grumbach syndrome

We report a 12‐year‐old female presenting with an abdominal tumor. Diagnostic workup revealed giant bilateral ovarian cysts, severe hypothyroidism as well as an elevation of CA 125. We refrained from ovariectomy, which would be necessary for a malignant tumor, in view of an evident Van Wyk and Grumbach syndrome. The patient promptly responded to L ‐thyroxine with complete regression of all symptoms. Hypothyroidism should be considered in the evaluation of ovarian cysts. Although the Van Wyk and Grumbach syndrome is rare, it is crucial to rule it out in order to avoid unnecessary ovarian surgery when thyroid replacement is completely sufficient. Pediatr Blood Cancer 2009;52:677–679. © 2009 Wiley‐Liss, Inc.     

Biliary deposition of Liesegang rings presenting as a polypoid mass in the liver: Previously unrecognized lesion

Liesegang rings (LR) are acellular, laminated structures that may be deposited within and around cysts and inflamed or necrotic tissue. Previous reports have shown that the kidney and breast are commonly affected organs of LR. Herein is reported a rare case of biliary deposition of LR presenting as a tumor-like polypoid mass in the liver. A 70-year-old man was found to have a cystic lesion, measuring 3.0 cm in diameter, in the lateral segment of the liver. The lesion was accompanied by a solid mass, 1.8 cm in diameter, within the cystic cavity. Lateral segmentectomy of the liver was performed because clinical examinations could not exclude the possibility of hepatobiliary cystadenoma or cystadenocarcinoma. Pathology of the resected specimen indicated a soft polypoid mass, connecting to the cystically dilated bile duct with elongated stalk-like structures. Histologically the surface of the mass was covered by non-neoplastic biliary-type epithelium. Beneath the epithelium, extensive deposition of numerous ring-like laminated structures, which exhibited an identical appearance to LR, was observed. This is a unique and previously unrecognized lesion involving the occurrence of LR deposition in the hepatobiliary tracts, which further formed a grossly visible mass resembling a hepatic tumor.