Endoscopic and surgical management of Bouveret's syndrome complicated by gallstone ileus.

Bouveret's syndrome is a rare complication that occurs most commonly in elderly patients with multiple comorbidities. It is secondary to an impacted gallstone causing gastric outlet obstruction from a cholecystoduodenal fistula, and there is no defined standardized management in current literature. A 92-year-old woman presents to our tertiary community hospital with abdominal discomfort concerning for bowel obstruction. Computed tomography revealed pneumobilia with a cholecystoduodenal fistula and a large gallstone in the proximal duodenum causing gastric outlet obstruction. The impacted gallstone failed endoscopic extraction with electrohydraulic lithotripsy, and patient subsequently developed distal gallstone ileus requiring exploratory laparotomy and enterolithotomy. This case report examines the need for early coordinated endoscopic and surgical management of a patient with Bouveret's syndrome complicated by gallstone ileus as it is associated with high morbidity and mortality rates.

[Clinical efficacy of different rehabilitation modes for lumbar degenerative diseases after operation].

OBJECTIVE: To compare clinical effects of different postoperative rehabilitation modes on lumbar degenerative diseases, and explore influence of rehabilitation mode and other factors on postoperative effect. METHODS: From June 2013 to July 2016, totally 900 patients were admitted from nine tertiary hospitals in Beijing to perform single segment bone grafting and internal fixation due to lumbar degenerative diseases were prospectively analyzed. There were 428 males and 472 females, the age of patient over 18 years old, with an average of (51.42±12.41) years old;according to patients' subjective wishes and actual residence conditions, all patients were divided into three groups, named as observation group 1 (performed integrated rehabilitation approach and orthopedic treatment model intervention), observation group 2 (performed integrated rehabilitation approach and orthopedic treatment, classified rehabilitation model intervention), and control group(performed routine rehabilitation model intervention). Visual analogue scale(VAS), Oswestry Disability Index(ODI) and Japanese Orthopaedic Association (JOA) were used to evaluate postoperative efficacy among three groups at 24 weeks. Possible factors affecting the postoperative efficacy including age, age grouping, gender, body mass index (BMI), BMI grouping, education level, visiting hospital, payment method of medical expenses, preoperative complications, preoperative JOA score, clinical diagnosis, surgery section, operative method, intraoperative bleeding volume, postoperative complications and rehabilitation mode were listed as independent variables, and postoperative ODI score at 24 weeks as dependent variables. Univariate analysis was used to analyze relationship between influencing factors and postoperative efficacy. Multiple linear regression was used to analyze relationship between influencing factors, rehabilitation mode and postoperative ODI score at 24 weeks, in further to find out the main reasons which affect postoperative efficacy, and to analyze impact of rehabilitation mode on postoperative efficacy. RESULTS: All patients were followed up for 24 weeks after operation. All incisions healed at stage I with stable internal fixation. (1)Evaluation of postoperative efficacy:① There were no statistical differences in preoperative VAS and ODI among three groups(P>0.05), the degree of pain and dysfunction decreased among three groups after operation, and had differences in postoperative VAS and ODI among three groups (P<0.05). There were no significant differences between observation group 1 and observation group 2(P>0.05); while compared with observation group 1 and control group, observation group 2 and control group, there were significant differences (P<0.05). ②The function among three groups were improved in varying degrees after operation. There was difference in JOA score among three groups before operation and 24 weeks after operation (P<0.05). There were no difference in JOA score among three groups between observation group 1 and observation group 2 (P>0.05);while compared with observation group 1 and control group, observation group 2 and control group, there were significant differences (P<0.05). (2)Influencing factors at 24 weeks after operation:①Univariate analysis showed gender, age, age grouping, education level, preoperative complications, clinical diagnosis, operative section, operative method, preoperative JOA score and rehabilitation mode had statistical significance with postoperative ODI score at 24 weeks (P<0.05). BMI, BMI grouping, payment method of medical expenses, visiting hospital, intraoperative bleeding volume, postoperative complications had no statistical significance with postoperative ODI score at 24 weeks (P<0.05).②Multivariate analysis results showed gender, rehabilitation mode, age, preoperative JOA score entered the equation eventually, stepwise multiple linear equation obtained had statistical significance (F=12.294, P= 0.000). Among rehabilitation mode, standardized regression coefficient of the integrated rehabilitation approach and orthopedic treatment with classified rehabilitation model was absolute value of the largest (0.176), which had the greatest influence on postoperative curative effect. The degree of dysfunction in control group was higher than that in observation group 1 and observation group 2. Postoperative dysfunction was more severe in males than that of in females. Older age has higher degree of dysfunction after operation. Lower preoperative JOA score has higher degree of dysfunction after operation. CONCLUSION: Preoperative JOA score, gender, age could predict postoperative clinical effects of lumbar degenerative diseases in varying degrees treated with single level bone graft fusion and internal fixation. Different rehabilitation modes could improve clinical effects. Intergrated rehabilitation orthopedic treatment model and integrated rehabilitation approach and orthopedic treatment with classifiedrehabilitation model are superior to conventional rehabilitation model in improving patients' postoperative function and relieving pain, which is worthy of promoting in clinical.

Blood pressure circadian rhythms and adverse outcomes in type 2 diabetes patients diagnosed with orthostatic hypotension.

AIMS/INTRODUCTION: Patients with diabetes frequently develop orthostatic hypotension (OH). The present study was designed to examine the relationship of blood pressure (BP) circadian rhythms and outcomes in diabetes with OH. MATERIALS AND METHODS: In the present study, 173 inpatients with type 2 diabetes were enrolled. Patients were divided into an OH group and a non-OH group according to the BP changes detected in the supine and standing position. Then, 24-h ambulatory BP was monitored. Patients were followed up for an average of 45 ± 10 months post-discharge. Outcomes - death and major adverse cardiac and cerebrovascular events, including heart failure, myocardial infarction and stroke - were recorded. RESULTS: There were 61 patients (35.26%) in the OH group and 112 patients (64.74%) in the non-OH group. In the OH group, the night-time systolic BP and night-time diastolic BP were higher, the blood BP rhythms were predominantly of the riser type (67.21%). OH was as an independent marker of riser type circadian rhythm (adjusted odds ratio 4.532, 95% confidence interval 2.579-7.966). In the OH group, the incidence rates of mortality, and major adverse cardiac and cerebrovascular events were increased significantly compared with those in the non-OH group (11.48 vs 2.68%, P = 0.014; 37.70 vs 8.93%, P < 0.01). CONCLUSIONS: In patients who had type 2 diabetes diagnosed with OH, the BP circadian rhythm usually showed riser patterns, and they had increased rates of mortality, and major adverse cardiac and cerebrovascular events.

Vitamin D Level is Associated with Increased Left Ventricular Mass and Arterial Stiffness in Older Patients with Impaired Renal Function.

BACKGROUND Impaired renal function is common among older patients. Deficiency of vitamin D is a frequent phenomenon among patients with impaired renal function, who are likely to develop cardiovascular diseases. This study aimed to explore the association of 25 (OH) D levels with left ventricular mass and arterial stiffness in older patients with impaired renal function. MATERIAL AND METHODS Based on their admission estimate glomerular filtration rate (eGFR), 273 inpatients (≥65 years) were allocated into a normal eGFR group (≥60 ml/min) and an impaired eGFR group (<60 ml/min). The 25 (OH) D levels were measured and the left ventricular mass index (LVMI) was estimated. Pulse wave velocity (PWV) was used to explore arterial stiffness. RESULTS The 25 (OH) D levels of patients in the impaired eGFR group were significantly lower than in the normal eGFR group [(11.92±6.01) µg/L vs. (18.14±8.07) µg/L, p<0.05). LVMI and PWV were both significantly higher in the impaired eGFR group than in the normal eGFR group [(104.89±33.50) g/m2 vs. (92.95±18.95) g/m2, P<0.05; (15.99±3.10) m/s vs. (13.62±2.90) m/s, P<0.05]. After adjusting for age, sex, eGFR, cardiovascular risk factors, serum calcium, and iPTH levels, the inverse association between LVMI and 25 (OH) D, PWV, and 25 (OH) D were statistically significant. CONCLUSIONS Vitamin D level is lower in older patients with impaired renal function. Lower vitamin D levels were correlated with higher left ventricular mass and increased arterial stiffness in older patients.

[Clinical and biopsy-based myopathological features of 5 cases with centronuclear myopathy].

OBJECTIVE: To analyze the clinical and pathological features of the centronuclear myopathy (CNM) in 5 Chinese patients and evaluate their diagnostic and differential diagnostic value. METHODS: A standard series of histochemical and enzymohistochemical investigations were performed on all muscle specimens of CNM cases obtained via biopsy. The clinical manifestations and myopathological features of 5 CNM patients were retrospectively analyzed. RESULTS: The age of onset ranged from 3 to 12 years. All patients primarily presented with limb girdle muscle weakness. In 3 patients extraocular muscles, facial muscles and cervical muscles were affected, respectively. The proximal muscles were affected more seriously than the distal and the lower limbs more seriously than the upper. Tendon reflex was reduced and no evident muscular atrophy was seen. The course of the disease ranged from 4 to 46 years and progressed slowly. The ability of walking could be maintained for many years and the fast movements such as running and jumping were impaired early. The serum creatine kinase (CK) level was normal or elevated slightly. Electromyography showed myopathic pattern in all cases. Two patients (mother and son) were from the same family and the son's two siblings had similar symptoms indicating autosomal dominant inherited pattern. There was mild variation in fiber size and most small fibers were round. Interstitial tissue increase slightly. Fibers with centrally placed nuclei accounted for 23% - 93%. Neither necrotic and regenerated fibers nor infiltration of inflammatory cells were seen. Type I fiber predominance and hypotrophy were present in all patients. Abnormal arrangement of the sarcoplasmic strands in appearance of "spokes of a wheel", increased oxidative enzyme activity around centronuclear and perinuclear halo were observed in 2 patients by NADH-TR staining. CONCLUSIONS: For the patients who had the onset during the childhood and presented with slow progressive limb girdle muscle weakness, disability of fast movements and normal serum CK level, the possibility of benign congenital myopathy should be considered. High percentage of centronuclear fibers as well as type I fiber predominance and hypotrophy in muscle biopsy pathology may provide a morphological evidence for the definite diagnosis of CNM.

[Clinical and pathological features and prognosis of Chinese patients with distal myopathy with rimmed vacuoles: study of 17 cases].

OBJECTIVE: To clarify the clinical and pathological features and prognosis of Chinese patients with distal myopathy with rimmed vacuoles (DMRV). METHODS: The clinical data of 17 Chinese DMRV patients with the courses of disease of 1-21 years, 5 males and 12 females, aged 28.9 (19-41), were collected. Biopsy of muscle specimens of 17 Chinese DMRV patients were summarized retrospectively. Muscle specimens were collected from the biceps brachii, tibialis anterior, gastrocnemius, or quadriceps femoris and underwent light microscopy. Eight muscle specimens underwent electron microscopy. 11 patients were followed up for 4 months to 15 years. RESULTS: The age of onset ranged from 5 to 40 years (averaging 23 years). Distal muscle weakness and atrophy of the lower extremities, especially anterior tibial muscle, was predominant in the early stage. Proximal and trunk muscles were involved in the advanced stage. Quadriceps femoris were slightly involved. The striking and characteristic pathological finding was the presence of rimmed vacuoles in atrophic muscle fibers with little evidence of necrotic or regenerative processes. Electron microscopy showed accumulation of myeloid structure and cytoplasmic or intranuclear tubofilamentous inclusion bodies. Although atrophy and weakness of the leg muscle appeared as initial symptoms, severe generalized skeletal muscle involvement with sparing of the facial, extraocular, bulbar, intercostals, and diaphragm muscles was recognized in the advanced stage. The patients became non-ambulant about 7-10 years after the onset of the disease. They lost the self-care ability and the quality of their life was rather low. CONCLUSION: The clinical and pathological features of the Chinese DMRV patients are basically similar to those of the Japanese patients. With the disease progressing slowly, the patients become wheelchair-bound and lose the self-care ability. As to daily life, the prognosis of DMRV is extremely poor.

[Dysferlin expression in limb-girdle muscular dystrophy and Miyoshi myopathy: analysis of 45 cases].

OBJECTIVE: To clarify the expression patterns of dysferlin in limb-girdle muscular dystrophy (LGMD) and Miyoshi myopathy (MM), and to investigate the frequency and clinicopathologic features of dysferlinopathy. METHODS: The expressing patterns of dysferlin were analyzed by immunohistochemistry, with a set of antibodies against dystrophin, alpha-sarcoglycan and dysferlin, in the biopsied muscle specimens from 45 patients with LGMD or MM diagnosed on the basis of clinical manifestations and muscle pathological features. The specimens with abnormal dysferlin expression shown by IHC were further analyzed with Western blotting for a quantitative evaluation. RESULTS: Eight patients were proved to be primary dysferlinopathy according to total dysferlin deficiency or a significant decrease of dysferlin (less than 15% that of normal value). The clinical manifestations of 5 of the 8 dysferlinopathy patients were consistent with those of typical MM, and the other 3 were diagnosed as with LGMD. All patients had an average onset at the age of 18.8 years. Two of them had family history, and one patient had consanguineous mating parents, meaning an autosomal recessive inheritance pattern. The serum CK levels were 6240 IU/L on average. EMG showed myogenic patterns in all patients. Muscular pathology showed typical changes of muscular dystrophy in all patients. Focal or scattered inflammatory cellular infiltrations were found in 3 cases. CONCLUSION: The clinical and pathological features of dysferlinopathy are nonspecific. Inflammatory cellular infiltrations are relatively common in biopsied muscles of dysferlinopathy patients, which may cause misdiagnosis of inflammatory myopathy. Identification of dysferlin expression by IHC and Western blotting are essential for the diagnosis of dysferlinopathy and differential diagnosis of inflammatory myopathy.

Plasma concentration of Fas/Fas ligand and left ventricular function in response to metoprolol in conjunction with standard treatment.

Recent studies suggest that cardiac myocyte apoptosis contributes to the progress of CHF (congestive heart failure). In the present study, we tested the hypothesis that metoprolol in conjunction with the standard treatment regime for CHF [an ACE (angiotensin-converting enzyme) inhibitor, diuretics and digoxin] may significantly reduce the plasma concentrations of the apoptotic mediators sFas (soluble Fas) and sFasL (soluble Fas ligand) in patients with CHF. An ELISA was used to determine the plasma concentrations of sFas and sFasL in 106 patients with stable CHF at recruitment. Echocardiography was performed at baseline and after 1 year of treatment with metoprolol in conjunction with the standard treatment regime for CHF (i.e. an ACE inhibitor, diuretics and digoxin). The dose of metoprolol was doubled on a biweekly basis up to 50 mg twice a day or maintained at the maximum tolerated dose. Data after 1 year were available for 92 patients and were analysed. The plasma concentrations of sFas and sFasL in patients with CHF decreased significantly (P<0.01) after 1 year of treatment with metoprolol in conjunction with the standard treatment regime compared with at baseline (5.4+/-0.2 compared with 3.2+/-0.1 ng/ml respectively for sFas, and 52.1+/-2.3 compared with 26.7+/-1.0 pg/ml respectively for sFasL). Compared with baseline, after 1 year of treatment with metoprolol in conjunction with the standard treatment regime there were significant improvements in LV (left ventricular) ejection fraction (from 32.6+/-0.9 to 51.5+/-0.8%; P<0.01), LV end-diastolic dimension (from 69.8+/-0.6 to 57.7+/-0.3 mm; P<0.01), LV end-systolic dimension (from 53.9+/-0.6 to 40.5+/-0.5 mm; P<0.01), LV end-diastolic volume (from 254.7+/-5.0 to 164.1+/-2.2 ml; P<0.01) and LV end-systolic volume (from 142.0+/-4.2 to 72.2+/-2.0 ml; P<0.01). In addition, the distance walked in a 6-min walk test increased markedly (P<0.01) from 260.3+/-5.2 m at baseline to 440.9+/-5.7 m after 1 year of treatment. In conclusion, we have demonstrated that metoprolol in conjunction with an ACE inhibitor, diuretics and digoxin in patients with CHF can lead to a reverse in LV remodelling potentially through its anti-apoptotic effects.