Exocrine Pancreatic Insufficiency is Undiagnosed in Some Patients with Diarrhea-Predominant Irritable Bowel Syndrome Using the Rome IV Criteria.

BACKGROUND AND AIMS: Irritable bowel syndrome (IBS) is one of the most frequent disorders in clinical practice, with a mean 7.6-10.8% worldwide prevalence. A study showed that 6.1% of patients with diarrhea-predominant IBS (IBS-D) had severe exocrine pancreatic insufficiency (EPI). We aimed to identify the prevalence of EPI based on fecal elastase stool testing (Fel-1) in IBS-D and the clinical characteristics that may predict the diagnosis of EPI. METHODS: Patients aged > 18 years presenting to tertiary hospital outpatient clinics with IBS-D completed validated questionnaires and gave a stool sample where Fel-1 concentration was measured. Patients with Fel-1 < 100 µg/g represented EPI and > 100 to < 200 µg/g underwent testing for pancreatic pathology with laboratory and endoscopic ultrasound (EUS) evaluation. RESULTS: One hundred forty patients (mean age 60 years, females 75.7%) were studied. EPI was found in 5% (95% CI 2.2-10.4), and pancreatic steatosis was the main EUS finding (71%). Dyspepsia was an independent factor associated with EPI (OR 34.7; 95% CI 4.95-366.37, p = 0.0007). After pancreatic enzyme replacement therapy (PERT), patients showed a significant improvement in the Bristol stool scale (p < 0.0001), bowel movements per day (p < 0.005), distension score (0.0009), pain score (0.0277) and IBS severity (0.0034). CONCLUSION: EPI is present in 5% of patients who fulfill Rome IV criteria for D-IBS, and dyspepsia was an independent symptom strongly associated with EPI. Pancreatic steatosis was the main endoscopic ultrasound finding. After PERT therapy, patients had significantly improved stool frequency, stool consistency, abdominal pain, distension and IBS severity score.

Analysis of The Left Atrial Function Using Two-Dimensional Strain in Patients with Recent Diagnosis of Hereditary Hemochromatosis.

Hereditary Hemochromatosis (HH) is a genetic condition associated with a systemic iron overload. Heart failure is an important cause of mortality. It has been demonstrated early stages of systolic and diastolic left ventricular dysfunction in previous studies. The aim of the study is to compare the left atrial (LA) function between asymptomatic HH patients and a control group using 2D speckle tracking. Prospective study. LA strain, LA strain rate and LA volumetric parameters during the reservoir, conduit and contraction phases were studied. The LA Stiffness Index was calculated by the ratio between E/e and LA reservoir strain. 30 patients with HH (90% males, 47 ± 18 years old) and 30 healthy controls (85% males, 45 ± 13 years old) were included. LA volume was similar in both groups. No differences were observed in LA ejection fraction (EF), LA passive EF and LA active ejection fraction between both groups. On the contrary, the HH group had lower LA strain during the reservoir (31.5 ± 6.5% vs 38.3 ± 7.9%; P=0.002), and conduit phases (-18 ± 7% vs -23.3 ± 6.4%; P=0.01) and lower LA conduit strain rate (-1.7 ± 0.7 seg-1 vs -2.1 ± 0.5 seg-1; P=0.02) than controls. The LA stiffness index was significantly higher in the HH group (0.25 ± 0.9 vs 0.19 ± 0.6; P=0.01) Early abnormalities in the LA function could be detected by using 2D speckle tracking study despite no evidence of changes in atrial size or volumetric parameters.

Sublingual microcirculatory alterations in cirrhotic patients.

OBJECTIVE: To assess sublingual microcirculation in cirrhotic patients and its relationship to spider angiomas, complications, and outcome. METHODS: Thirty-one cirrhotic patients were prospectively compared to 31 matched controls. Sublingual microcirculation was evaluated by videomicroscopy. We specifically looked for capillaries with increased RBCV, which was defined as a velocity higher than the percentile 100th of controls. RESULTS: Compared to controls, cirrhotic patients showed decreased total and PVD (14.4 ± 2.2 vs 16.0 ± 1.3 and 14.1 ± 2.3 vs 15.9 ± 1.6 mm/mm2 , respectively, P < .001 for both) and increased HFI (0.64 ± 0.39 vs 0.36 ± 0.21, P = .001). They also exhibited high RBCV in 2% of the microvessels (P < .0001). Patients with MELD score ≥10 had higher RBCV than patients with score <10 (1414 ± 290 vs 1206 ± 239 µm/s, P < .05). Patients with spider angiomas showed lower vascular densities. Microcirculation did not differ between survivors and nonsurvivors. CONCLUSIONS: Cirrhosis is associated with microcirculatory alterations that can be easily monitored in the sublingual mucosa. Alterations included decreased density and PPV and hyperdynamic microvessels. The most striking finding, however, was the microvascular heterogeneity. Patients with spider angiomas had more severe alterations. Larger studies should clarify the relationship between microcirculatory abnormalities and outcome.

[Usefulness of gastric emptying scintigraphy for the evaluation and management of scleroderma related gastroparesis]. / Utilidad de la medición del vaciamiento gástrico por centellografía con 99mTc para la evaluacion y el manejo de la gastroparesia en la esclerodermia.

Scleroderma is a chronic autoimmune disease of unknown cause characterized by fibrotic skin and multiple organs involvement, including the gastrointestinal tract. It occurs mainly in women between 35 and 65 years of age. It is classified as limited or diffuse based on the extent of skin involvement. Gastrointestinal dysmotility is observed in up to 90% of patients with a diffuse and limited scleroderma. It may involve any segment of the gastrointestinal tract from the esophagus to the anus and is related to collagen deposition at the level of enteric and vascular smooth muscle. Gastroparesis is a condition characterized by abnormal gastric motility, delay gastric emptying, in the absence of a mechanical obstruction to outflow. Gastric scintigraphy with radiolabeled solid food is the gold standard for the diagnosis of gastroparesis. Two cases of patients with systemic scleroderma and severe gastroparesis are presented in order to discuss the diagnostic and therapeutic approach, emphasizing the utility of gastric emptying scintigraphy.

[Prolonged survival after local excision of a rectal leiomyosarcoma]. / Sobrevida prolongada luego de la resección local de un leiomiosarcoma de recto.

Leiomyosarcoma is a stromal tumor, originated from smooth muscle cells. The pathologic diagnosis represents a challenge in terms of differentiation from leiomyomas and gastrointestinal stromal tumors (GIST), defined by immunohistochemistry techniques. Its location in the rectum is extremely rare. So, management is not standardized. However, in the largest published series it was found that the abdominoperineal resection leads to better results in the prevention of local recurrence compared with local excision. We present here the case of a 44-year-old woman, whose first clinical manifestation of the disease was fever of prolonged course. A 5 cm leiomyosarcoma was diagnosed at 4 cm from the anal margin. A local transanal resection was performed. The patient is free of disease after 8 years.

Familial association in autoimmune liver disease.

The occurrence of autoimmune liver disease in members of the same family is hardly a frequent observation in clinical practice. In a group of 204 cases of primary biliary cirrhosis (PBC) (196 women) and 219 of type 1 autoimmune hepatitis (AIH) (183 women), seen from 1985 to 2000, family occurrence of autoimmune liver disease was investigated. Diagnosis of both entities was based on clinical criteria, immunological studies and liver biopsy. Six families were identified with 2 members each presenting with autoimmune liver disease. In 4 of them the index case had an AIH. This association was observed between mother and daughter in 3 instances. In the remaining AIH index case the association found was with a PBC in her sister. In the other two families the index cases were PBC. In one of them, PBC and AIH association were observed in sisters. Lastly, in another case, an antimitochondrial (AMA) negative variant of PBC was detected in mother and her daughter. The low frequency of family association observed in this cohort could be due to the fact that only symptomatic cases were included. Concurrent autoimmune manifestations were confirmed in 5 members of 6 families (42%). Our results, given the concurrence of both liver diseases in the same family, suggest a link among diverse entities of the autoimmune lineage. The frequency of AIH family association seems to be more prominent in this series than that of PBC. It is also shown that family association in the case of an AMA-negative variant of PBC is feasible, thus confirming that no substantial differences exist between the latter and AMA-positive PBC.

Familial association in autoimmune liver disease

The occurrence of autoimmune liver disease in members of the same family is hardly a frequent observation in clinical practice. In a group of 204 cases of primary biliary cirrhosis (PBC) (196 women) and 219 of type 1 autoimmune hepatitis (AIH) (183 women), seen from 1985 to 2000, family occurrence of autoimmune liver disease was investigated. Diagnosis of both entities was based on clinical criteria, immunological studies and liver biopsy. Six families were identified with 2 members each presenting with autoimmune liver disease. In 4 of them the index case had an AIH. This association was observed between mother and daughter in 3 instances. In the remaining AIH index case the association found was with a PBC in her sister. In the other two families the index cases were PBC. In one of them, PBC and AIH association were observed in sisters. Lastly, in another case, an antimitochondrial (AMA) negative variant of PBC was detected in mother and her daughter. The low frequency of family association observed in this cohort could be due to the fact that only symptomatic cases were included. Concurrent autoimmune manifestations were confirmed in 5 members of 6 families (42). Our results, given the concurrence of both liver diseases in the same family, suggest a link among diverse entities of the autoimmune lineage. The frequency of AIH family association seems to be more prominent in this series than that of PBC. It is also shown that family association in the case of an AMA-negative variant of PBC is feasible, thus confirming that no substantial differences exist between the latter and AMA-positive PBC.

Familial association in autoimmune liver disease

The occurrence of autoimmune liver disease in members of the same family is hardly a frequent observation in clinical practice. In a group of 204 cases of primary biliary cirrhosis (PBC) (196 women) and 219 of type 1 autoimmune hepatitis (AIH) (183 women), seen from 1985 to 2000, family occurrence of autoimmune liver disease was investigated. Diagnosis of both entities was based on clinical criteria, immunological studies and liver biopsy. Six families were identified with 2 members each presenting with autoimmune liver disease. In 4 of them the index case had an AIH. This association was observed between mother and daughter in 3 instances. In the remaining AIH index case the association found was with a PBC in her sister. In the other two families the index cases were PBC. In one of them, PBC and AIH association were observed in sisters. Lastly, in another case, an antimitochondrial (AMA) negative variant of PBC was detected in mother and her daughter. The low frequency of family association observed in this cohort could be due to the fact that only symptomatic cases were included. Concurrent autoimmune manifestations were confirmed in 5 members of 6 families (42). Our results, given the concurrence of both liver diseases in the same family, suggest a link among diverse entities of the autoimmune lineage. The frequency of AIH family association seems to be more prominent in this series than that of PBC. It is also shown that family association in the case of an AMA-negative variant of PBC is feasible, thus confirming that no substantial differences exist between the latter and AMA-positive PBC. (AU)

Familial association in autoimmune liver disease.

The occurrence of autoimmune liver disease in members of the same family is hardly a frequent observation in clinical practice. In a group of 204 cases of primary biliary cirrhosis (PBC) (196 women) and 219 of type 1 autoimmune hepatitis (AIH) (183 women), seen from 1985 to 2000, family occurrence of autoimmune liver disease was investigated. Diagnosis of both entities was based on clinical criteria, immunological studies and liver biopsy. Six families were identified with 2 members each presenting with autoimmune liver disease. In 4 of them the index case had an AIH. This association was observed between mother and daughter in 3 instances. In the remaining AIH index case the association found was with a PBC in her sister. In the other two families the index cases were PBC. In one of them, PBC and AIH association were observed in sisters. Lastly, in another case, an antimitochondrial (AMA) negative variant of PBC was detected in mother and her daughter. The low frequency of family association observed in this cohort could be due to the fact that only symptomatic cases were included. Concurrent autoimmune manifestations were confirmed in 5 members of 6 families (42

). Our results, given the concurrence of both liver diseases in the same family, suggest a link among diverse entities of the autoimmune lineage. The frequency of AIH family association seems to be more prominent in this series than that of PBC. It is also shown that family association in the case of an AMA-negative variant of PBC is feasible, thus confirming that no substantial differences exist between the latter and AMA-positive PBC.

Distribucion de los genotipos del virus de la hepatitis C en una poblacion argentina de drogadictos endovenosos / Distribution of the genotypes of Hepatitis C virus in a Argentine population of intravenous drug addicts

El antecedente de drogadicción endovenosa constituye un factor de riesgo poco frecuente en pacientes (pts) con infección crónica por el virus C (HCV) en la Argentina, representando en nuestro servicio menos del 10 por ciento. Nos propusimos determinar la prevalencia de los diferentes genotipos (Gt) del HCV en un grupo de pts con hepatitis crónica por HCV con antecedentes de drogadicción endovenosa. Un total de 68 pts con antecedentes de drogadicción endovenosa y hepatitis crónica HCV fueron comparados con 68 pts de igual edad y sexo pero sin el antecedente de drogadicción. La biopsia hepática fue realizada en todos los pts. La genotipificación del HCV fue efectuada por INNO LiPA (Innogenetics). Para el análisis estadístico se empleó el test de Student. La edad media en ambos grupos fue de 35 + 7.8 años correspondiendo 50 pts al sexo masculino. No se observaron diferencias entre ambos grupos en la prevalencia de los GT 1a, 2a/c e infecciones mixtas. El Gt 1b fue más frecuente en el grupo control 26/68 (38,2 por ciento) que en el de drogadictos 13/68 (19.1 por ciento) (p = 0.0228). También fue observada una diferente prevalencia en el GT3, presente en 29/68 (42.6 por ciento) de los drogadictos y en 8/68 (11.8 por ciento) del grupo control (p = 0.0001). El Gt1a fue el segundo más frecuente en el grupo con antecedentes de drogadicción 18/68 (26.5 por ciento). La infección simultánea con el HIV fue observada en 8 pts con antecedentes de drogadicción y en ninguno del grupo control. La biopsia hepática mostró una mayor prevalencia de lesiones leves en el grupo control 39/68 (57.3 por ciento) que en los pts con antecedentes de drogadicción 22/68 (32.4 por ciento) (p = 0.0058). En los pts infectados con el Gt3 la hepatitis crónica severa y cirrosis fueron más frecuentes en los pts con antecedentes de drogadicción. Se concluye que en nuestro medio el Gt 3 es el más prevalente en los pts con antecedentes de drogadicción endovenosa. Las formas de hepatitis leves son menos frecuentes en los pts con antecedentes de drogadicción. A pesar del pequeño número de pts coinfectados con el HIV es importante notar que el 25 por ciento de ellos presentaron hepatitis crónica severa o cirrosis.

Distribucion de los genotipos del virus de la hepatitis C en una poblacion argentina de drogadictos endovenosos / Distribution of the genotypes of Hepatitis C virus in a Argentine population of intravenous drug addicts

El antecedente de drogadicción endovenosa constituye un factor de riesgo poco frecuente en pacientes (pts) con infección crónica por el virus C (HCV) en la Argentina, representando en nuestro servicio menos del 10 por ciento. Nos propusimos determinar la prevalencia de los diferentes genotipos (Gt) del HCV en un grupo de pts con hepatitis crónica por HCV con antecedentes de drogadicción endovenosa. Un total de 68 pts con antecedentes de drogadicción endovenosa y hepatitis crónica HCV fueron comparados con 68 pts de igual edad y sexo pero sin el antecedente de drogadicción. La biopsia hepática fue realizada en todos los pts. La genotipificación del HCV fue efectuada por INNO LiPA (Innogenetics). Para el análisis estadístico se empleó el test de Student. La edad media en ambos grupos fue de 35 + 7.8 años correspondiendo 50 pts al sexo masculino. No se observaron diferencias entre ambos grupos en la prevalencia de los GT 1a, 2a/c e infecciones mixtas. El Gt 1b fue más frecuente en el grupo control 26/68 (38,2 por ciento) que en el de drogadictos 13/68 (19.1 por ciento) (p = 0.0228). También fue observada una diferente prevalencia en el GT3, presente en 29/68 (42.6 por ciento) de los drogadictos y en 8/68 (11.8 por ciento) del grupo control (p = 0.0001). El Gt1a fue el segundo más frecuente en el grupo con antecedentes de drogadicción 18/68 (26.5 por ciento). La infección simultánea con el HIV fue observada en 8 pts con antecedentes de drogadicción y en ninguno del grupo control. La biopsia hepática mostró una mayor prevalencia de lesiones leves en el grupo control 39/68 (57.3 por ciento) que en los pts con antecedentes de drogadicción 22/68 (32.4 por ciento) (p = 0.0058). En los pts infectados con el Gt3 la hepatitis crónica severa y cirrosis fueron más frecuentes en los pts con antecedentes de drogadicción. Se concluye que en nuestro medio el Gt 3 es el más prevalente en los pts con antecedentes de drogadicción endovenosa. Las formas de hepatitis leves son menos frecuentes en los pts con antecedentes de drogadicción. A pesar del pequeño número de pts coinfectados con el HIV es importante notar que el 25 por ciento de ellos presentaron hepatitis crónica severa o cirrosis. (AU)

Ketoconazole-induced liver damage

Five cases (four females, one male) of ketoconazole-related liver damage are presented, two of whom died. All patients received ketoconazole (400 mg/day) for various mycoses. In the four women the first signs of hepatotoxicity appeared after four weeks of therapy. One fatal case developed massive necrosis with fulminant liver failure and the other, submassive necrosis. In four cases cholestasis was a prominent finding. Biochemical evidence of biliary stasis may persist for several months, as occurred in the three surviving patients of our series. The two fatal cases continued receiving the drug in spite of its adverse effects. Consequently, repeated evaluation is recommended to detect early signs of liver involvement.

Ketoconazole-induced liver damage

Five cases (four females, one male) of ketoconazole-related liver damage are presented, two of whom died. All patients received ketoconazole (400 mg/day) for various mycoses. In the four women the first signs of hepatotoxicity appeared after four weeks of therapy. One fatal case developed massive necrosis with fulminant liver failure and the other, submassive necrosis. In four cases cholestasis was a prominent finding. Biochemical evidence of biliary stasis may persist for several months, as occurred in the three surviving patients of our series. The two fatal cases continued receiving the drug in spite of its adverse effects. Consequently, repeated evaluation is recommended to detect early signs of liver involvement. (AU)