Esophageal aperistalsis in a patient with lipoid proteinosis

Lipoid proteinosis is a rare disorder with autosomal recessive inheritance, characterized by progressive deposition of hyaline material in the skin, mucous membrane, and different organs of the body, resulting in a multitude of clinical manifestations. A 34-year-old woman presented with hoarseness, dysphagia, eyelid beeding, and acneiform scars on the facial skin and extremities. The patient was diagnosed clinically as having lipoid proteinosis, which was confirmed by laryngeal biopsy. The objective of the present report is to describe this rare entity. This case report also illustrates that lipoid proteinosis may show protean clinical features and yet may remain undiagnosed for many years

Anti-platelet therapy and the risk of upper gastrointestinal bleeding

Background: We aimed to determine the effects of anti-platelet drugs use on adverse outcomes in patients with non-variceal upper gastrointestinal bleeding [UGIB]

Materials and Methods: A historical cohort study was performed on patients with non-variceal UGIB admitted to a tertiary care hospital. Clinical outcomes were compared among users of aspirin and patients who did not receive aspirin. Adverse outcome variables consisted of re-bleeding, need for surgery, and death

Results: Out of 271 patients [77.5% men, mean age 59.5+/-19.0 years] with non-variceal UGIB, 157 [57.9%] did not receive any anti-platelet drugs, 87 [32.1%] received only aspirin, and 27 [10.0%] received dual anti-platelet therapy. The frequency of adverse outcomes was significantly higher in patients who bled while not receiving anti-platelets [31.2% no anti-platelets, 12.6% single anti-platelet agent, and 14.8% on dual anti-platelets, p=0.002]. A significant difference in the duration of admission was not found between the three groups [5.5+/-4.3 in patients with no anti-platelet drugs, 5.6+/-4.6 in patients received single anti-platelet agent, and 5.0+/-4.3 in patients received dual anti-platelets, p =0.84]

Conclusion: Patients with non-variceal UGIB while taking anti-platelet drugs had a lower rate of adverse outcomes compared with non-users of anti-platelets

Tuberculous ventriculitis: a rare complication of central nervous system tuberculosis

Tuberculous ventriculitis is an inflammatory infection of the ventricular system of the brain, and is caused by Mycobacterium tuberculosis. We herein present the case of an immunocompromised patient with brain tuberculomas who developed ventriculitis during treatment. The patient was successfully treated with a high dose of steroid, long-term antituberculosis drugs, and aggressive supportive care

Comparison of oral midazolam and promethazine with oral midazolam alone for sedating children during computed tomography

Both midazolam and promethazine are recommended to be used as sedatives in many studies but each have some side effects that limits their use. Combination therapy as an alternative method, may decreases these limitations. Therefore, this study aimed to compare midazolam with midazolam-promethazine regarding induction, maintenance, and recovery characteristics following pediatric procedural sedation and analgesia. Children under 7 years old who needed sedation for being CT scanned were included in this double-blind randomized clinical trial. The patients were randomly divided into 2 groups: one only received midazolam [0.5 mg/kg], while the other group received a combination of midazolam [0.5 mg/kg] and promethazine [1.25 mg/kg]. University of Michigan Sedation Scale [UMSS] was used to assess sedation induction. In addition to demographic data, the child's vital signs were evaluated before prescribing the drugs and after inducing sedation [reaching UMSS level 2]. The primary outcomes in the present study were onset of action after administration and duration of the drugs' effect. 107 patients were included in the study. Mean onset of action was 55.4 +/- 20.3 minutes for midazolam and 32.5 +/- 11.1 minutes for midazolam-promethazine combination [p<0.001]. But duration of effect was not different between the 2 groups [p=0.36]. 8 [7.5%] patients were unresponsive to the medication, all 8 of which were in the midazolam treated group [p=0.006]. Also in 18 [16.8%] cases a rescue dose was prescribed, 14 [25.9%] were in the midazolam group and 4 [7.5%] were in the midazolam-promethazine group [p=0.02]. Comparing systolic [p=0.20] and diastolic [p=0.34] blood pressure, heart rate [p=0.16], respiratory rate [p=0.17] and arterial oxygen saturation level [p=0.91] showed no significant difference between the 2 groups after intervention. Based on the findings of this study, it seems that using a combination of midazolam and promethazine not only speeds up the sedation induction, but also decreases unresponsiveness to the treatment and the need for a rescue dose

Giant adominal wall hernia in a patient with cryptogenic cirrhosis

Abdominal wall hernias are common problems found in patients with cirrhosis because of persistently high intra-abdominal pressure. When abdominal hernias are neglected in such patients, they may become larger and could result in cosmetic problems and pressure effects that are also difficult to treat. We found a voluminous mass in the anterior abdominal wall of a 40-year-old patient with cirrhosis. The patient was operated on for acute cholecystitis 12 years earlier. Abdominal computed tomography revealed an epigastric hernia presenting as a grossly distended hernia sac filled with serous fluid and intestinal loops. The patient was not operated on and was discharged with sodium-restricted diet and diuretics

Fulminant hepatic failure due to primary hepatic lymphoma: a case report

Primary hepatic lymphoma is a rare malignancy usually presenting with symptoms of fever, hepatomegaly, jaundice and weight loss. This picture mimics infectious and inflammatory disorders and thus delays the diagnosis. Here, we present a 47-year old man with prolonged fever who underwent several investigations and, in the meantime, developed fulminant hepatic failure before the diagnosis could be reached

[Prevalence of consanguineous marriage among parents of deaf and normal children in Ardabil, North Western Iran]

Having healthy non-handicapped children plays a major role in mental health of the family and decreases family and society's costs. While consanguineous marriage could lead to expression of recessive genes and a variety of handicaps including deafness, the aim of present study was to scrutinize the prevalence of consanguineous marriage among parents of deaf and normal children as well as its relationship with deafness. In this study, 467 couples parenting normal children were selected by cluster sampling from elementary, guidance and high schools of Ardabil city and 423 couples parenting disabled children were selected non-randomly among which 130 had deaf children. Descriptive statistics was used to determine the prevalence of consanguineous marriage and chi-square test to compare prevalence of consanguineous marriage among parents of normal and deaf children. Descriptive analyses showed that 80 out of 130 [61.54%] parents who had deaf children have had consanguineous marriage. Furthermore data analysis demonstrated that prevalence of consanguineous marriage was significantly higher among parents of deaf children [p<0.001]. Consanguineous marriage plays a major role in expression of recessive genes and could lead to development of various handicaps including deafness. Increasing couples' awareness about consequences of consanguineous marriage and conducting genetic counseling are indispensable.

Endoscopic ultrasonography in patients with chronic liver disease: a case control study

The portal system and azygos vein are the main drainage systems during portal hypertension. This study aims to compare the diameter of these veins by endoscopic ultrasonography [EUS] in patients with and without chronic liver disease [CLD]. During one year, patients with CLD enrolled as the study group. Patients who underwent EUS for other reasons during the same period served as controls. In cases with CLD, we assessed the relationship between degrees of hepatic dysfunction [Child-Pugh class], history of variceal bleeding, presence of hyponatremia, thrombocytopenia, and endoscopic grading of varices with the sizes of the portal, splenic, and azygos veins on EUS. During the study period, there were 63 patients [20 females and 43 males] with CLD and 85 control subjects [42 females and 43 males] enrolled. The mean ages of cases was 45.60 +/- 14 years and controls was 48.5 +/- 15 years. The most common cause of CLD was post-necrotic cirrhosis due to hepatitis B virus. Patients with CLD had significantly higher mean portal, splenic, and azygos vein diameters than the control group [p < 0.001]. With azygos, portal, and splenic vein diameters of 10, 11 and 9 mm, sensitivity for the diagnosis of portal hypertension was 66%, 71%, and 66%, while specificity was 94%, 99% and 99%, respectively. Splenic and portal vein dilation, and thrombocytopenia significantly correlated with variceal bleeding [p < 0.05]. EUS allows for the collection of valuable quantitative data from the portal system, the diagnosis of portal hypertension, and follow up of patients with CLD

Diagnostic accuracy of endoscopic ultrasonography in patients with inconclusive magnetic resonance imaging diagnosis of biliopancreatic abnormalities.

Aim To determine the sensitivity and specificity of endoscopic ultrasonography (EUS) in patients with inconclusive magnetic resonance imaging/magnetic resonance cholangiopancreatography (MRI/MRCP) in pancreatobiliary abnormalities. Methods During 10 months, patients with pancreatobiliary diseases referred to endoscopic retrograde cholangiopancreatography (ERCP) because of inconclusive MRI/MRCP diagnosis were scheduled to undergo endoscopic ultrasonography. Patients were divided into four major groups: patients with (i) resectable periampullary neoplasms who were referred to a surgeon, (ii) unresectable periampullary cancer who underwent ERCP for biliary stenting, (iii) bile duct stone who were referred to ERCP for stone extraction, and (iv) normal pancreatobiliary tract. Reference standards for comparison were ERCP, surgery, a biopsy confirming malignancy, or the clinical course during follow up (at least 12 months) in cases without evidences of malignancy. Results One hundred and seven patients (51 men; mean [SD] age 60.0 [15.5]) were included in the study. Final diagnoses were common bile duct (CBD) stone (n=24), periampullary neoplasms (n=46), others (n=23) and no pathologic findings (n=14). EUS determined the staging for clinical decision-making in 47 patients with neoplasms which showed that tumors in 34 patients (79.1%) were unresectable (advanced stage). After EUS, 47 patients (43.9%) did not require ERCP. The accuracy of EUS for the diagnosis of CBD stone and periampullary neoplasms were 96.3% and 99.1%, respectively. Conclusions EUS is a useful modality in cases of inconclusive MRI/MRCP indicating pancreatobiliary disorders.

[Predictors of boredom in adolescents]

Boredom is a mental state that can have serious impact on different aspects of life, including: profession and education. The purpose of this study was to identify those factors associated with boredom in a sample of Persian adolescences. In a correlational study, 182 students [96 girls and 82 boys], aged between 12 to 18 years who were studying in secondary and high school level of education in Khoram Abad city, center of Lorestan [a western province of Iran] were selected using a cluster random sampling in 2009. The participants were asked to fill in boredom assessment scale [BSA] and boredom factor questionnaire [BFQ] included 4 components: home factors, school factors, community factors and educational items. Several Pearson correlation and regression analyses were applied on the data. It was shown that home factor [beta=0.23] and school factor [beta=0.24] were significantly correlated with boredom [P<0.01]. Home factor [beta=0.39] was a robust predictors of boredom in girls, while school factor [beta=0.30] was a strong predictor of boredom in boys [P<0.01]. Major components of home factor were: strictness of parents, parental dispute and conflict, more spare time without any job to do, and lack of pastime and recreational activities, and for school factor, component were: strict and serious behaviors of teachers and inappropriate homework. Inadequate interpersonal relationships and inflexible teaching or parenting style appeared to be the main predictors of boredom in adolescents. These variables should be taken in account while dealing with boredom in adolescents in order to prevent destructive consequences

Comparison of metronidazole and placebo in control of gaseous symptoms in patients with functional bowel disorders; a randomized double- blind controlled study

To compare the efficacy of metronidazoie versus placebo in the control of gaseous symptoms in patients with functional bowel disease. In the absence of organic or systemic diseases, all cases with chief complaints of bloating and normal laboratory tests were consecutively enrolled in this double-blind study. Lactase deficiency and bacterial overgrowth were ruled out by the lactose breath test. Patients were randomly assigned to receive either metronidazole or placebo. Demographic characteristics as well as frequency and severity of the patients' scores [mean total symptom score] before and after therapy, their compliance and drug adverse effects were evaluated. A 50% decrease in the total symptom scores was defined as effective treatment. During one year, 46 patients [17 males, 29 females, mean age: 38.9 +/- 9.9 years] were enrolled in the study. A total of 23 patients received metronidazole [cases] and 23 received placebo [controls]. Two patients in the metronidazole group did not tolerate the drug and one patient in the placebo group did not continue with follow-up. Patients responded similarly to both regimens: 59% of patients in the placebo group and 52.2% of patients in the metronidazole group had a 50% decrease in their total symptom score [p = 0.64]. Side effects of metronidazole were frequent, but tolerable. Bad taste in the mouth and anorexia were the most common complaints in the metronidazole group. This study showed no difference between placebo and metronidazole in relief of bloating and other related complaints in patients with functional bowel disease

Long-term follow-up of common bile duct diameter after endoscopic sphincterotomy in patients with common bile duct stones.

Background and Aims To determine the time to normalization of common bile duct (CBD) diameter after endoscopic sphincterotomy and stone extraction in patients with choledocholithiasis. Methods Patients with CBD dilation due to choledocholithiasis were enrolled. CBD diameter was measured by transabdominal ultrasonography before, and repeated after one, three, six and twelve months after endoscopic sphincterotomy and stone extraction, until normalization of CBD diameter. Results Of 115 cases enrolled over a 36-month period, CBD diameter reversed to normal in 71 (61.7%) patients after one month. Of the remaining 44 patients, CBD diameter reversed to normal in 36 patients (including 3 in whom repeat ERCP revealed choledocholithiasis) at the end of three months. CBD diameter had not reversed to normal diameter in 8 (18.2%) patients; none of these patients had symptoms. Two of them had asymptomatic dilated CBD after 6 months with no abnormal liver function tests (LFT); the duct reversed to normal at the last follow-up (month 12). Conclusions Asymptomatic CBD dilation may persist in a minority of patients (18% at the end of 3 months) after removal of CBD stones. A dilated CBD can be attributed to retained choledocholithiasis within the first month, if it is associated with symptoms and abnormal LFT.

[Plasma levels of the NT-pro BNP in patients presenting with heart failure]

In view of the high morbidity and mortality of patients with congestive heart failure, early diagnosis is of paramount importance. To assess the diagnostic and prognostic utility of plasma levels of the NT-Pro BNP in patients presenting with heart failure this study was carried out. This cross-sectional study was performed on patients with heart failure admitted in the cardiology ward of Taleghani Medical Center during a certain period from 2005- 2006. Subjects with acute coronary syndrome, hyperthyroidism, renal failure [GFR<60], severe valvular stenosis, severe pulmonary disease, ARDS, hepatic cirrhosis, primary hyperaldosteronism and the candidates for heart transplant were excluded from the study. Blood samples were collected and plasma levels of NT-Pro BNP were measured by ELISA method [Biomedica- crop, Bratislava Slovakia]. Results were documented and analyzed using SPSS software. Mann- Whitheny and KrusKall-Wallis tests were utilized for analysis. Values were analyzed for confounding variables [e.g age, sex, blood pressure, and ischemic heart disease and NYHA class]. 79 patients full filled the criteria of inclusion. 70% were males and mean age of patients was 63 +/- 14 years. Mean plasma NT-Pro BNP level was 421 +/- 387. In subjects <60 years of age, the mean levels were 309 +/- 300, and among those >/= 60 years it was 486 +/- 419, [P<0.05]. With advancing clinical stage, the mean plasma level of NT-Pro BNP increased 6 folds, [p<0.01], whereas with advancing NYHA class it increased 4 folds [P<0.01]. It seems that plasma NT-Pro BNP level is a suitable indicator of the severity of heart failure

[Causes of minimal bright red bleeding per rectum in patients who referred to the gastroenterology clinic of Loghman Hakim Hospital [September 2003-March 2006]]

Minimal bright red bleeding per rectum [MBRBPR] is a common complaint in adult patients. Most studies have not been performed in patients with strictly minimal BRBPR, and there are no evidence-based recommendations for this group of patients. Patients with complaints as small amounts of red blood after wiping or a few drops of blood in the toilet bowl after defecation not intermixed with stool were consecutively enrolled. Those with a history of inflammatory bowel disease, colorectal cancer, adenomatous polyps, significant weight loss, anemia, strong family history of colorectal cancer and not consenting for colonoscopy were excluded. Patients underwent total colonoscopy or rectosigmoidoscopy plus double contrast barium enema [age>40] or a minimum of rectosigmoidoscopy [to the splenic flexure in patients aged < 40]. two-hundred twenty patients were eligible in the study period. Normal findings were found in 23 [10.5%]. There was at least one distal lesion in the remaining patients. From 11 patients with a proximal lesion, 10 had the same lesion in distal colon and there was only one patient with an adenomatous polyp near the hepatic flexure and hemorrhoids. In patients with anorectal pathologies [n=189], there was a concomitant colonic lesion in 25 [13.2%]. All patients with clinically significant lesions [IBD, polyp or cancer] located over 30 cm from anus were aged more than 40 years. The causative lesions for MBRBPR are located in the distal colon. Rectoscopy [40 years or less] or flexible rectosigmoidoscopy [over 40 years] seem to be the appropriate evaluation in these patients

Oral omeprazole in patients undergoing combination endoscopic therapy for bleeding peptic ulcers A: prospective double-blind randomized study

Endoscopic therapies can decrease the morbidity of patients with high risk peptic ulcer. The aim of this study was to evaluate the beneficial effects of oral omeprazole therapy in patients with bleeding peptic ulcer who received combined endoscopic treatment [epinephrine injection and Argon Plasma Coagulation]. Eighty six patients with bleeding from gastric, duodenal or stomal ulcers and endoscopic stigmata of recent bleeding were enrolled in our study. All patients received injection of epinephrine [1:10,000] and also their ulcers were treated with Argon Plasma Coagulator. The patients then randomly assigned to receive oral omeprazole [40 mg every 12 hours] or placebo. Five [11.6%] of 43 patients in the placebo group had rebleeding; but no rebleeding was detected among 43 patients in omeprazole group [p= 0.05]. One patient in the Placebo group underwent surgery for control of his rebleeding; but none of the patients in omeprazole group needed surgery. One patient in the placebo group and none of the patients in the omeprazole group died. The average hospital stay was 5 days in the omeprazole group and 5.8 days in the placebo group. Addition of oral omeprazole to combined endoscopic therapy significantly reduces recurrent bleeding rates