Jackhammer Esophagus: Current Concepts and Dilemmas.

Jackhammer esophagus (JE) is a recently recognized esophageal motility disorder that is characterized by hypercontractile peristalsis. More than 500 cases have been reported in the literature. Among patients referred for esophageal motility disorders, the prevalence of JE ranges from 0.42% to 9%, with most series describing a prevalence of 2% to 4%. Most cases are women (60.5%). The mean reported age of patients with JE is 65.2 years, and patients commonly have dysphagia (62.8%). Reflux symptoms occur in ∼40% of patients, and chest pain affects more than one-third of patients (36.4%). JE is a heterogenous disorder that is associated with several conditions, including obesity, opioid use, lung transplantation, eosinophilic infiltration of the esophagus, neoplasia, and systemic diseases. The cause and pathogenesis remain unknown, but several observations suggest that it is the result of multiple conditions that likely precipitate increased excitation and abnormal inhibition of neuromuscular function. The natural course of JE also is unknown, but progression to achalasia has been observed in a few patients. Treatment is challenging, in part because of the insufficient understanding of the disorder's underlying mechanisms. Various therapeutic modalities have been used, ranging from observation only to pharmacologic and endoscopic interventions (eg, botulinum toxin injection) to peroral endoscopic myotomy. Treatment efficacy remains largely anecdotal and insufficiently studied.

Lower Urinary Tract and Gastrointestinal Dysfunction Are Common in Early Parkinson's Disease.

PURPOSE: Autonomic dysfunction is a common nonmotor feature and early manifestation of Parkinsons disease (PD). Autonomic dysfunction in PD is associated with a worse prognosis. We sought to characterize autonomic dysfunction and identify associated factors in patients with early PD. METHODS: An observational, cross-sectional, descriptive, and analytical study was conducted to evaluate patients with early PD from the Parkinsons Progression Markers Initiative. We utilized the Scales for Outcomes in Parkinsons Disease-Autonomic dysfunction questionnaire to determine the prevalence and frequencies of autonomic symptomatology. The cohort was grouped into high and low dysautonomic scores. A regression model identified variables that independently explained dysautonomic scores in our early PD cohort. RESULTS: 414 PD patients had a mean age of 61.1 (SD 9.7) years at diagnosis and mean disease duration of 6.7 (SD 6.6) months. Among all patients, 43.7% (181/414) had high dysautonomic scores. Urinary and gastrointestinal symptoms were the most prevalent and frequently reported dysautonomic symptoms. Patients with fatigue (beta = 4.28, p < 0.001), probable rapid eye movement sleep behavior disorder (beta = 2.71, p < 0.001), excessive daytime sleepiness (beta = 1.88,p=0.039), impulsivity and compulsivity (beta = 2.42, p < 0.001), and increasing age (beta = 1.05, p < 0.001) were more likely to have high dysautonomic scores. CONCLUSION: Lower urinary tract and gastrointestinal symptoms are prevalent and frequent in early PD patients. Fatigue, sleep disorders, impulsivity and compulsivity, and age are predictors of autonomic dysfunction. Autonomic symptoms predominated in this group of early PD patients in the disease course and were associated with more severe disease.

Hypertriglyceridemia-induced pancreatitis and risk of persistent systemic inflammatory response syndrome.

BACKGROUND: The mechanisms responsible for the development of acute pancreatitis (AP) and its complications are not fully understood. AIM: To assess the role of clinical and host molecular factors for the development and outcome of persistent systemic inflammatory response syndrome (SIRS) in patients with AP. METHODS: We included 191 patients with AP in the study. The considered variables were demographic characteristics, prognosis and outcome, etiology, laboratory findings and complications. Interleukin (IL) 10 (-1082 G/A, -592 C/A), TNFA-308 (G/A) and ILB-31 (C/T) polymorphisms were determined by pyrosequencing. An amplification refractory mutation system-polymerase chain reaction method was used to genotype the IL8-251 (A/T) polymorphism. RESULTS: Demographic characteristics were not statistically significant risk factors for the acquisition of persistent SIRS in patients with AP. Patients with hypertriglyceridemia were more likely to develop persistent SIRS (P < 0.05). No association with the TNFA, ILB, IL8-251 (A/T) and IL10 single-nucleotide polymorphisms was detected from the allele, genotype or haplotype frequencies. CONCLUSIONS: Patients with hypertriglyceridemia-induced AP were more likely to develop persistent SIRS.

The prevalence and clinical characteristics of glucose metabolism disorders in patients with liver cirrhosis. A prospective study.

AIMS: To define the prevalence and clinical characteristics of glucose metabolism disorders (GMD) in patients with compensated liver cirrhosis (LC). MATERIAL AND METHODS: Fasting plasma glucose (FPG) levels were measured to 130 patients with clinically stable LC. Oral glucose tolerance tests (OGTT) and fasting plasma insulin determinations were performed to patients with normal FPG. Insulin resistance (IR) was calculated with HOMA2-IR index. GMD were classified according to FPG and OGTT tests results and to the chronologic relation between diagnosis of diabetes mellitus (DM) and LC as follows: type-2 DM (T2DM), hepatogenous diabetes (HD) and impaired glucose tolerance. Patients from all groups were compared. RESULTS: The prevalence of GMD were as follows: T2DM in 25 patients (19.2%, 95% CI 12.5-25.9), HD in 28 (21.5%, 95% CI 14.5-28.5) and IGT in 36 (38.5%, 95% CI 30.1-46.7). The total of patients with GMD was 79.2% (95% CI 72.3-86.1). In 41% of cases GMD were subclinical and 48.7% of patients had IR. Patients with T2DM had a higher number of variables with significant differences compared with the other groups (more marked compared to the patients without GMD). The only differences between the patients with T2DM and HD were hypercreatininemia: 1.14 ± 0.53 vs. 0.84 ± 0.22 mg/dL (p = 0.005) and family history of DM: 8 (32%) vs. 2 (7%) (p = 0.02). CONCLUSION: Almost 80% of patients with compensated LC had GMD. Half of them were subclinical. The patients with T2DM had marked clinical differences compared to patients from the other groups, particularly renal impairment.

Nonvariceal upper gastrointestinal bleeding in patients with liver cirrhosis. Clinical features, outcomes and predictors of in-hospital mortality. A prospective study.

BACKGROUND: Clinical and endoscopic features of cirrhotic patients with nonvariceal upper gastrointestinal bleeding (NVUGIB) have been rarely reported and clinical outcomes and predictors of mortality have not been evaluated. AIMS: 1) To describe the clinical features; 2) To define the clinical outcomes; and 3) To identify the predictors of in-hospital mortality of cirrhotic patients with NVUGIB. METHODS: One hundred sixty cirrhotic patients with NVUGIB were prospectively studied. Clinical features, endoscopic findings, clinical outcomes and in-hospital mortality rate were studied. Predictors of death were identified by means of univariate and multiple logistic regression analysis. RESULTS: The mean age was 56.5 ± 14.4, male gender prevailed. Alcohol was the most frequent etiology. Hemodynamic instability was reported in 29.4%. Mean serum hemoglobin was 9.5 ± 3.3 g/dL and blood transfusions were required in 59.4%. Gastroduodenal ulcers were the most frequent source of bleeding (50.6%). In endoscopy "high-risk" bleeding stigmata (HRBS) at the ulcer base were found in 53.1%. All patients with HRBS received endoscopic treatment. Rebleeding occurred in 3 patients (1.9%) and mortality was of 13.8%. By univariate analysis: Cryptogenic etiology, BUN, hypoalbuminemia, active bleeding at ulcer base, and endoscopic treatment were predictors of mortality. However, only cryptogenic etiology, hypoalbuminemia and active bleeding at ulcer base were independent predictors of death in multivariate analysis. CONCLUSIONS: Gastroduodenal ulcers as a source of NVUGIB are frequent in cirrhotic patients. They were severe; half of them had HRBS, and required frequently endoscopic treatment. In-hospital mortality of these patients seemed to be greater than that of non-cirrhotic patients, and it was significantly related to cryptogenic etiology of cirrhosis, renal dysfunction, severe hepatic failure, and active bleeding ulcers on admission to the hospital.

Predictors of in-hospital mortality in patients with non-variceal upper gastrointestinal bleeding.

OBJECTIVE: to determine the independent predictors of in-hospital death of Hispanic patients with nonvariceal upper gastrointestinal bleeding (NVUGB). EXPERIMENTAL DESIGN: prospective and observational trial. PATIENTS: in a period between 2000 and 2009, all patients with NVUGB admitted to our hospital were studied. Demographical and clinical characteristics, endoscopic findings and laboratory tests were evaluated χ² and Mann-Whitney U analyses were per-formed for comparisons, and binary logistic regression was employed to identify independent predictors of in-hospital mortality. RESULTS: 1,067 patients were included, 65% male with a mean age of 58.8 years. Mean number of comorbidities per patient was 1.6 ± 0.76. The most frequent cause of bleeding were gastric and duodenal ulcers (55.4%); 278 patients (25.8%) received endoscopic treatment of which 69.1% had combined therapy. Rebleeding occurred in 36 patients (3.4%) of which 50% died. In-hospital mortality was 10.2%, of which only 3.1% was associated to bleeding. When comparing causes of death among patients with and without comorbidities, only hypovolemic shock was found significative (48.3 vs. 25%; p = 0.020). Binary logistic regression found that the number of comorbidities, Rockall scale score; serum albumin < 2.6 g/dL on admission; rebleeding and length of hospital stay were independent risk factors of in-hospital mortality. CONCLUSION: the number of comorbidities, the Rockall scales core, an albumin level < 2.6 g/dL, the presence of rebleeding and hospital stay were predictors of in-hospital mortality in patients with NVUGB.

Prevalence of minimal hepatic encephalopathy in cirrhotic patients.

Background. Minimal hepatic encephalopathy (MHE) has implications for health-related quality of life as well as for survival of cirrhotic patients, but a standardized diagnostic test is not available. Objective. To determine the prevalence of MHE among cirrhotic patients by using the psychometric hepatic encephalopathy score (PHES) system and the critical flicker frequency (CFF) test to diagnose MHE and to identify factors that influence the results of these tests. Material and methods. From April 2007 to March 2008, PHES and CFF tests were performed on patients with cirrhosis but no overt hepatic encephalopathy. Descriptive statistics were used to express the results and the Spearman correlation was used to evaluate CFF and PHES results according to age and education level. Results. We studied 104 patients. The prevalence of MHE was 55.8% (n = 58) based on a positive result for either the PHES or the CFF test, 32.7% (n = 34) based on positive PHES results alone, 34.6% (n = 36) based on positive CFF test results alone and 11.5% (n = 12) based on a positive result for both tests. According to PHES, the incidence of MHE was correlated with education level (r = 0.333, p = 0.001), but not with age. According to CFF, the incidence of MHE was correlated with age (r = -0.93, p = 0.049), but not with education level. Conclusion. The prevalence of MHE was similar to that previously reported. Patient literacy influences MHE diagnosis with PHES but not with CFF. CFF is a simple and feasible method that identifies patients with MHE who may benefit from treatment independently of their education level.

Hepatitis C virus infection in health-care settings: medical and ethical implications.

Hepatitis C is a major public health issue. It infects about 200 million people worldwide and is a major cause of chronic liver disease. Its transmission in medical facilities is a topic of increased concern, as outbreaks of the disease had raised the attention of media and medical authorities. To date, evidence suggests that infection from in which a health-care worker is involved is mostly result of bad injecting practices as well as the result of shared medical devices. Furthermore, the infection caused by physicians is rare and very few well documented cases exist on the literature. Among countries, different definitions and legislation exist, in that mode that the responsibility of this issue almost is an obligation of individual institutions. Nonetheless, Hepatitis C virus transmission in medical facilities is an important source of new cases, and as treatments options are very limited, it's recommendable that institutions as well as governments implement policies to avoid Hepatitis C spread in a almost fully preventable setting.

Human leukocyte antigens among primary biliary cirrhosis patients born in Mexico.

BACKGROUND AND AIM: Primary biliary cirrhosis (PBC) is a chronic cholestatic disease of autoimmune origin and has a genetic component. Although it has been reported that the prevalence of the HLA-DRB1*08 allele is high in various populations, the prevalence of HLA alleles in Mexican PBC patients has not been described. The aim of this study was to quantify the prevalence of HLA-B/-DR alleles in Mexican PBC patients. MATERIALS AND METHODS: A case-control transversal study was performed during January and July 2008 with adult patients diagnosed with PBC. Cases were defined as subjects with PBC and controls were obtained from healthy subjects evaluated for bone marrow transplantation. Laboratory was performed at the moment of diagnosis. HLA-B/-DR allele frequency was obtained by gene counting and allele presence was determined by PCR-SSP procedure. Descriptive statistics between groups was evaluated by Chi-square with Yates correction. RESULTS: Nine patients (seven females and two males, mean +/- SD age = 57.5 +/- 10.5 years) were studied. The most prevalent alleles were HLA-DRB1*01 (n = 4), DRB1*04 (n = 4), B*39 (n = 34), B*14 (n = 3), and B*51 (n = 2). Linkage disequilibrium was detected for alleles HLA-B*39/DRB1*04 (n = 3), HLA-B*14/HLA-DRB1*01 (n = 2), and B*51-DRB1*04 (n = 1). IN CONCLUSION: Mexican PBC patients express genes of native and Mediterranean origin.

[Type 1 Arnold Chiari malformation, syringomielia, syringobulbia and IV ventricle entrapment]. / Malformación de Arnold-Chiari tipo I, siringomielia, siringobulbia y atrapamiento del ventrículo IV.

Arnold-Chiari Malformation (ACM) was first described by Hans Chiari in 1890. Four types of this malformation are recognized, of those, type I is the most common among adults. It is caused by an elongation of the cerebellum into the conic projections that accompany the brain stem within the spinal channel. It is mostly congenital but "acquired" forms can be seen in the context of infections or posterior fossa tumors. Clinically, it can present as an asymptomatic finding, but it can produce brain stem compression. Syringomielia--cavitation of the brain stem central areas--is associated with MAC type I in 75 to 85% of cases. Clinical signs include pain, weakness, extremity parestesia. A shunt to the lateral ventricles can produce IV ventricle entrapment and is associated with cerebrospinal fluid blockage. We describe a case of MAC type I associated with the presence of syringomielia, syringobulbia and IV ventricle entrapment.

Mortality trends for liver cancer in Mexico from 2000 to 2006.

UNLABELLED: Hepatocellular carcinoma (HCC) is the most common primary liver cancer, with an estimated incidence of half a million new cases per year around the world. Furthermore, HCC is the third greatest cause of cancer-related death in the world, and most of these deaths are registered in developing countries. Recently it has been suggested that Hispanics in the United States have high rates of HCC, but no information regarding this is available in Mexico. The aim of this study was to investigate recent trends (2000-2006) in HCC mortality rates in Mexico. METHODS: Data on national mortality (death certificates) reported for the years 2000-2006 by the Health Ministry of Mexico were analyzed (www.salud.gob.mx). HCC as a cause of death was analyzed. Mortality rates were calculated for all population ages. Causes of death related to HCC were selected in accordance with the International Classification of Diseases, 10th Revision, Liver Cancer (C22.0, C22.7, C22.9). RESULTS: We found that age-adjusted mortality rates were remarkably higher in men than in women in the period 2000-2006. In addition, we found an increase in the general mortality rates of HCC from 4.1 per 100,000 in 2000 to 4.7 per 100,000 in 2006. CONCLUSIONS: The results of this study suggest an increase in the mortality rate for HCC in the period 2000-2006. HCC will become a significant cause of morbidity and mortality in the near future.

Malformación de Arnold-Chiari tipo I, siringomielia, siringobulbia y atrapamiento del ventrículo IV / Type 1 Arnold Chiari malformation, syringomielia, syringobulbia and IV ventricle entrapment

La malformación de Arnold Chiari (MAC) fue descrita inicialmente por Hans Chiari en 1890. Existen cuatro tipos, de los cuales el tipo I es el más frecuente en adultos y se encuentra por la elongación de las amígdalas y las divisiones mediales del lóbulo inferior del cerebelo hacia las proyecciones cónicas que acompañan al bulbo raquídeo en el canal espinal. Su origen es congénito, aunque puede presentarse como una forma adquirida en el contexto de infecciones o tumores de fosa posterior. Clínicamente puede comportarse de manera asintomática o dar sintomatología de compresión de bulbo raquídeo. La siringomielia —cavitación de las áreas centrales de la médula espinal— se asocia a la MAC tipo I; en 75 a 85% de los casos se manifiesta con dolor, debilidad o parestesias de extremidades. El ventrículo IV atrapado se debe a un cortocircuito a los ventrículos laterales y se asocia a bloqueo de la circulación del líquido cefalorraquídeo. Se describe un caso de MAC tipo I asociado a siringomielia, siringobulbia y ventrículo IV atrapado.

Arnold-Chiari Malformation (ACM) was first described by Hans Chiari in 1890. Four types of this malformation are recognized, of those, type I is the most common among adults. It is caused by an elongation of the cerebellum into the conic projections that accompany the brain stem within the spinal channel. It is mostly congenital but [quot ]acquired[quot ] forms can be seen in the context of infections or posterior fossa tumors. Clinically, it can present as an asymptomatic finding, but it can produce brain stem compression. Syringomielia--cavitation of the brain stem central areas--is associated with MAC type I in 75 to 85% of cases. Clinical signs include pain, weakness, extremity parestesia. A shunt to the lateral ventricles can produce IV ventricle entrapment and is associated with cerebrospinal fluid blockage. We describe a case of MAC type I associated with the presence of syringomielia, syringobulbia and IV ventricle entrapment.

Gallbladder disease in patients with primary sclerosing cholangitis.

BACKGROUND/AIMS: Gallbladder abnormalities may be part of the spectrum in primary sclerosing cholangitis (PSC). The aim of the present study was to evaluate the occurrence and prognostic importance of gallbladder abnormalities in patients with PSC. METHODS: Presence of gallbladder abnormalities was assessed in 286 patients with PSC treated at the Liver Unit, Karolinska University Hospital, Huddinge, between 1970 and 2005. RESULTS: One or more gallbladder abnormalities were found in 41% of the patients. Gallstones were found in 25% and cholecystitis in 25%. Cholecystitis among patients with extrahepatic involvement of PSC (30% (65/214)) was significantly higher than among those with intrahepatic involvement (9% (6/70)) (P<0.0001). A gallbladder mass lesion with a mean size of 21 (+/-9) mm (S.D.) was found in 18 (6%) patients, in 56% (10/18) of whom it constituted gallbladder carcinoma. In 9 patients without a gallbladder mass lesion, histological re-evaluation disclosed epithelial dysplasia of the gallbladder. CONCLUSIONS: Gallbladder disease is common in patients with PSC. Dysplasia and carcinoma are commonly found in gallbladder epithelium, suggesting that regular examination of the gallbladder in PSC patients could be of value for early detection of a gallbladder mass lesion. Cholecystectomy is recommended when such a lesion is detected, regardless of its size.

Gallstones are associated with carotid atherosclerosis.

BACKGROUND/AIMS: Gallstone disease (GD) and cardiovascular disease (CD) are common diseases worldwide with considerable economical impact and they are strongly associated. Carotid atherosclerosis is an excellent marker of risk for CD like stroke and myocardial infarction. The aim of this study was to assess the association between gallstones and carotid atherosclerosis. METHODS: A cross-sectional study was conducted. We evaluated subjects with ultrasonographical evidence of GD and asymptomatic subjects without such evidence. Anthropometric, clinical and biochemical variables were collected. The Metabolic syndrome was evaluated using adult treatment panel III criteria. Carotid artery intima-media thickness (CIMT) was determined by a standard ultrasound protocol. Insulin-like growth factor-1 (IGF-1) serum levels were measured in all subjects. RESULTS: We studied 191 subjects: 62 subjects with GD (53.2% males) and 129 asymptomatic subjects without GD (65.9% males). Subjects with GD exhibited a higher body mass index, body fat percent, insulin serum levels and CIMT (P<0.05 for all). The prevalence of GD was higher in subjects with a CIMT>0.75 independently of other factors [odds ratio (OR) 2.12, 95% confidence interval (CI) 1.04-4.34; P=0.039], and for every 0.1 mm increase in CIMT the independent probability to be a case of GD increased by a factor of 1.25 (95% CI 1.02-1.53; P=0.027). IGF-1 levels did not differ among groups. CONCLUSIONS: Subjects with GD exhibit greater carotid atherosclerosis, and therefore have a higher risk for stroke and myocardial infarction.

Eosinophilic gastroenteritis: a review.

Eosinophilic gastroenteritis is a rare benign disease characterized by tissue eosinophilic infiltration that may involve several digestive tract layers. Also known as allergic, or eosinophilic allergic, gastroenteropathy, it usually involves the stomach and small intestine: rarely the colon. It may or may not be accompanied by higher counts of eosinophils in the peripheral blood. The main clinical manifestations depend on the site affected. It has been classified according to clinical and pathological features, and the symptoms depend on the patient's immunological response to several cytokines released by eosinophils. Because of lack of understanding of the etiology and triggering factors, treatment is based mainly on corticosteroids; although other drugs acting on the immune system have been tested, the results are not always satisfactory. This review focuses on the epidemiology, pathophysiology, clinical features, and treatment of this hitherto under-diagnosed disease.