Sweet's Syndrome in a Case of Ulcerative Colitis-Case Report and Review of Literature.

ABSTRACT: Sweet syndrome,also known as acute febrile neutrophilic dermatosis , is one of the rare cutaneous association of ulcerative colitis.Only few cases of Sweet syndrome associated with ulcerative colitis have been reported in literature. We herein describe a case of young female with acute exacerbation of ulcerative colitis associated with erythematous , papular skin lesions which on biopsy were consistent with Sweet syndrome. Treatment with intravenous steroids resulted in improvement of ulcerative colitis and disappearance of cutaneous lesions. Cutaneous lesions of Sweet syndrome in ulcerative colitis parallel the bowel disease activity in majority of the cases but sometimes may precede the intestinal symptoms and rarely may appear after procto-colectomy for acute severe ulcerative colitis.Introduction.

Common variable immunodeficiency syndrome with chronic diarrhoea.

Common variable immunodeficiency syndrome (CVID) is a heterogeneous disorder characterised by diminished levels of IgG, IgA and/or IgM, and recurrent bacterial infections. Sinopulmonary infections are most commonly reported followed by gastrointestinal (GI) infections. GI tract represents the largest immune organ with abundance of lymphoid cells, its involvement can manifest variably ranging from asymptomatic involvement to florid symptoms and signs. Diffuse nodular lymphoid hyperplasia (DNLH) of the GI tract is characterised by numerous small polypoid nodules of variable size in the small intestine, large intestine or both. It is commonly seen in association to immunodeficiency states such as CVID, IgA deficiency and chronic infections due to Giardia lamblia and Helicobacter pylori and cryptosporidiosis. Repetitive antigenic stimulation leads to lymphoid hyperplasia. We herein describe a case of DNLH of the intestine and another case of duodenal cytomegalovirus (CMV) infection associated with CVID.

Uncommon cause of dysphagia: paraneoplastic achalasia.

Achalasia secondary to underlying neoplasm is a rare entity. Early recognition of secondary achalasia is important as its treatment involves management of underlying malignancy, while treatment of primary achalasia mainly involves lowering the lower oesophageal sphincter pressure with pneumatic dilatation or Heller's myotomy. We discuss an interesting case of achalasia secondary to non-Hodgkin's lymphoma.

Comparison of endoscopic ultrasound-guided fine-needle aspiration by capillary action, suction, and no suction methods: a randomized blinded study.

BACKGROUND AND STUDY AIM: Different types of endoscopic ultrasound (EUS)-guided fine-needle aspiration (FNA) techniques are used in clinical practice; the best method in terms of outcome has not been determined. The aim of the study was to compare the diagnostic adequacy of aspirated material, and the cytopathological and EUS morphological features between capillary action, suction, and no-suction FNA methods. PATIENTS AND METHODS: This was a prospective, single-blinded, randomized study conducted at a tertiary care hospital. Patients were randomized to the three groups: capillary action, suction, and no suction. A total of 300 patients were included, with 100 patients in each arm. RESULTS: A total of 300 patients (195 males) underwent EUS-FNA of 235 lymph nodes and 65 pancreatic masses (distribution not statistically different between the groups). The mean age was 52 ±â€Š14 years. A 22 gauge needle was used in the majority (93 %) of procedures. There was no statistical difference between the three groups regarding lymph node size at the largest axis and ratio, type of needle, echo features, echogenicity, calcification, necrosis, shape, borders (lymph nodes), number of passes, and cellularity. Diagnostic adequacy of the specimen was 91 %, 91 %, and 94 % in the capillary, suction, and no suction groups, respectively ( P  = 0.67). Significantly more slides and blood clots were generated by the suction method compared with the other methods. CONCLUSION: The capillary action, suction, and no suction methods of EUS-FNA are similar in terms of diagnostic adequacy of the specimen. The suction method has the disadvantages of causing more bleeding and generating more slides.

Gastrointestinal Histoplasmosis and CMV Co-Infection in an Immunocompetent Host.

A middle aged male with no known comorbidities presented with history of colicky abdominal pain, low grade fever and weight loss. Laboratory parameters were normal except low albumin. Imaging showed multiple areas of mural thickening with enhancement in jejunum & ileum. On Colonoscopy there was a thickened and deformed ileum with multiple ulcers. The biopsy showed co-infection of CMV and histoplasma, urine antigen for histoplasma was positive and CMV DNA detected in blood. He was successfully treated with combination of Valgancyclovir and Amphotericin-B followed by itraconazole.

Plummer-Vinson Syndrome with Simultaneous Mid-esophageal Growth.

Patterson Brown Kelly or Plummer-Vinson syndrome is characterized by dysphagia, iron deficiency anemia and post-cricoidal esophageal web. Waldenstorm introduced the term 'sideropenic dysphagia' because of absence of stainable iron in the bone marrow. There is increased incidence of upper aero-digestive tract carcinoma in patients with Plummer-Vinson syndrome has been well established. The reported rates range from 4% to 16%, with almost all cases occurring at the postcricoid location.We have reported here a case of a 48-year-old woman with dysphagia , upper esophageal web and iron deficiency anemia . Dilatation of esophageal web with subsequent endoscopy showed mid- esophageal growth which on biopsy showed squamous cell carcinoma.

Isolated splenic tuberculosis diagnosed by endoscopic ultrasound-guided fine needle aspiration.

Our patient was a 48-year-old female, who presented with history of persistent low-grade fever and weight loss. The CT scan of the abdomen revealed multiple hypodense lesions in spleen. No primary focus of infection was detected in any other organs. Endoscopic ultrasound-guided fine needle aspiration of splenic lesion revealed granulomatous inflammation. The patient was started on anti-tuberculous therapy. There is a diagnostic possibility of splenic tuberculosis even in immunocompetent individuals and we chose a combination anti-tuberculous therapy as the first line treatment with consideration of splenectomy depending on the response.

Gastric Angioinvasive Mucormycosis in Immunocompetent Adult, A Rare Occurrence.

Mucormycosis is a rare, opportunistic fungal infection that occurs almost exclusively in immunocompromised hosts such as patients with diabetes mellitus, leukemia, lymphoma, renal disease, septicemia, burns, malnutrition, and following long-term treatment with steroids and antibiotics. Based on the clinical presentation and involvement, mucormycosis is classified as six major forms, namely, rhinocerebral, pulmonary, cutaneous, gastrointestinal (GI), disseminated and miscellaneous, with rhinocerebral and pulmonary being the common forms. GI mucormycosis is rare, accounting for only 7% of all cases; however, the mortality rate is as high as 85%. Here we report a case of a young immunocompetent male who developed gastric invasive mucormycosis during an acute illness and succumbed to it despite all supportive care.

Impact of endoscopic ultrasound-guided fine-needle aspiration in prospective liver transplant recipients with hepatocellular carcinoma and lymphadenopathy.

BACKGROUND: Diagnosis of metastatic disease is important in patients with cirrhosis and hepatocellular carcinoma (HCC) to prevent futile liver transplantation. Some of these patients have metastatic lymphadenopathy; however, it is difficult to perform percutaneous fine-needle aspiration due to presence of collateral and anatomic location. Endoscopic ultrasound (EUS)-guided fine-needle aspiration (FNA) of lymph nodes offers several advantages like real-time vision, proximity to target, and avoidance of collaterals. AIM: The aim of this study was to look for metastatic lymphadenopathy by EUS-guided FNA (EUS-FNA) in prospective liver transplant recipients with HCC. METHODS: A prospective study was conducted from January 2013 to January 2016 at a tertiary care center. All prospective liver transplant recipients with HCC had PET-CT and bone scan to look for metastatic disease. EUS-FNA was done in patients with abdominal or mediastinal lymphadenopathy and no evidence of extrahepatic disease. Data is shown as median (25-75 interquartile range). RESULTS: EUS-guided FNA was done for 50 patients (42 abdominal and 8 mediastinal lymph nodes), age 57 (53-62) years, Child-Turcotte-Pugh 7 (6-9), and model for end-stage liver disease 10 (7-16). FNA material was adequate in 92% patients, metastasis in 15 (30%), granulomatous lymphadenopathy in 4 (8%), and reactive change in 27 patients (54%). The material was inadequate for diagnosis in 4 (8%) patients. Thus, EUS-guided FNA precluded transplantation in 30% of patients with lymphadenopathy, and 4 (8%) patients received anti-tubercular therapy before liver transplantation. CONCLUSION: In patients with HCC and lymphadenopathy, EUS-guided FNA detected metastatic disease and precluded liver transplantation in approximately one third of patients.

Over-the-scope clip placement for closure of gastrointestinal fistula, postoperative leaks and refractory gastrointestinal bleed.

INTRODUCTION: The over-the-scope clip (OTSC) has been successfully used in the closure of fistula, perforation, dehiscence, and endoscopic hemostasis. We describe our experience with the OTSC application. METHODS: Between April 2014 and April 2015, seven patients underwent OTSC application. In four patients, OTSC was applied for the closure of esophageal fistula, one had OTSC closure of persistent gastrocutaneous fistula after percutaneous endoscopic gastrostomy removal, and OTSC was applied in duodenum in two patients, for duodenal Dieulafoy's lesion after failed conventional endotherapy and massive rebleed in one and duodenal perforation in another. RESULTS: All procedures had technical success with no immediate complication related to OTSC application. Patients were followed up for every month with mean duration of follow up 10.2 months. One patient with bronchoesophageal fistula had development of another fistulous opening above the site of OTSC placement, which was successfully closed with another OTSC. One patient had superficial esophageal wall ulcer opposite the OTSC but it healed spontaneously. CONCLUSION: OTSC provided safe and successful closure in a number of settings.

Impact and safety of endoscopic ultrasound guided fine needle aspiration on patients with cirrhosis and pyrexia of unknown origin in India.

BACKGROUND AND AIMS: Etiologic diagnosis of pyrexia of unknown origin is important in patients with cirrhosis for optimal management and to prevent flare up of infectious disease after liver transplantation. However, there is very limited literature available on this subject. The present study aimed to examine the safety and impact of endoscopic ultrasound (EUS) guided fine needle aspiration (FNA) in patients with cirrhosis. METHODS: The study was conducted between January 2014 and January 2016 at a tertiary care center. A total of 50 (47 lymph nodes, 3 adrenal) EUS guided FNAs were performed in 46 patients. Data are presented as median (25 - 75 IQR). RESULTS: The study included 46 patients (40 males) whose mean age was 47.9 ±â€Š11.1 (SD) years; mean Child-Turcotte-Pugh (CTP) score and mean MELD (Model for End-Stage Liver Disease) score were 10 (8 - 11) and 18 (12 - 20), respectively. The Child Pugh class was A in 4, B in 14, and C in 28 (including three patients with adrenal FNAs). Indications for FNA were pyrexia of unknown origin and lymphadenopathy on CT imaging. The cytopathological diagnoses were metastatic disease in 1 (adrenal), granulomatous change in 10 (6 positive with acid fast bacilli stain), histoplasmosis in three (two adrenals, one lymph node), 32 lymph nodes were reactive and four lymph node FNAs showed inadequate cellularity. The pathologic nodes had significantly lower long-to-short axis ratio [1.25 (1.09 - 1.28) versus 1.46 (1.22 - 1.87), P = 0.020]; a higher proportion of hypoechoic echotexture (5 versus 3, P = 0.017), and sharply defined borders (4 versus 2, P = 0.029). Complications included mild hepatic encephalopathy related to sedation in two patients with Child's C status. CONCLUSION: EUS guided FNA is safe in patients with cirrhosis and modified the management in 14/46 (30.4 %) patients.

Gastric lipoma: an unusual cause of dyspeptic symptoms.

Gastric lipomas are rare tumours accounting for 2-3% of all benign gastric tumours. These are usually submucosal in origin but in rare cases may be subserosal in origin. Although a majority of gastric lipomas are asymptomatic and usually detected incidentally, occasionally these can cause abdominal pain, dyspeptic disorders, obstruction, invagination and haemorrhages. In the literature, only 200 cases of gastric lipomas have been reported so far. We report a case of a 56-year-old female with a submucosal symptomatic gastric lipoma presenting with disabling dyspeptic symptoms.

Pancreaticopericardial fistula: a rare complication of chronic pancreatitis.

Pancreaticopericardial fistula (PPF) is an extremely rare clinical problem encountered in patients with chronic pancreatitis. The diagnosis should be suspected if a patient presents with pericardial effusion on a background of chronic pancreatitis. Significantly raised amylase in the pericardial fluid offers an important clue for the diagnosis. CT is the initial imaging modality to look for pancreatic and pericardial changes. The therapeutic options include medical, endoscopic or surgical interventions. Medical and endoscopic therapies are the preferred modes of treatment while surgery is reserved for those who fail these measures.

Hematemesis: Unusual presentation of isolated gastric tuberculosis.

A 25-year-old male presented with hematemesis, epigastric pain, and melena. He had dyspepsia with significant weight loss for 3 months period. On clinical examination, he was pale with no organomegaly or lymphadenopathy. The X-ray chest was normal, and ultrasound abdomen was normal. Upper GI endoscopy revealed nodularity and ulceration along proximal part of lesser curvature of the stomach. CT scan abdomen showed thickening of lesser curvature just below gastro-esophageal junction. The biopsies were negative for malignancy. Repeat upper GI endoscopy showed a nonhealing ulcer, on repeat well biopsies taken from the base of ulcer primary gastric tuberculosis was diagnosed. It showed many epithelioid cell granulomas and multinucleated giant cells with caseous necrosis on histology. Acid-fast bacilli on Zeil Neelsen staining and TB PCR were positive for Mycobacterium tuberculosis. He was put on four-drug anti-tuberculous treatment. On follow-up, the patient gradually improved and regained weight. Repeat upper GI endoscopy done after 8 weeks showed healing of the ulcer with decrease in nodularity.

Removable self-expanding metal stents insertion for the treatment of perforations and postoperative leaks of the esophagus.

BACKGROUND: Esophageal rupture, spontaneous or iatrogenic, is associated with significant morbidity and mortality. The current study aims at highlighting the various clinical scenarios, where esophageal fully covered self-expanding removable metal stents (FCSEMS) can be used in esophageal rupture. METHODS: In patients who underwent insertion of FCSEMS between January 2013 and June 2014, all data regarding demographics, indications, insertion, removal, and outcomes were studied retrospectively. RESULTS: Seven patients underwent the placement of esophageal covered SEMS. Two patients had Boerhaave syndrome, two had leak following the repair of aortic aneurysm, one had extensive esophageal injury following transesophageal echocardiography, one had carcinoma esophagus with tracheaesophageal fistula, and one had dehiscence of esophagogastric anastomosis. Stent insertion was successful in all the patients; one had stent migration which was managed endoscopically. Two patients died due to underlying illness; the rest had successful removal of stents after 8-10 weeks and good outcomes. CONCLUSION: Esophageal FCSEMS placement is safe and effective modality in management of patients with esophageal rupture.

Endoscopic ultrasound characteristics of tubercular lymphadenopathy in comparison to reactive lymph nodes.

AIM: Tuberculosis is a common disease in India with significant morbidity and mortality. Limited data is available on the description of tubercular lymphadenopathy on endoscopic ultrasound. METHODS: Retrospective data of 116 lymph nodes in 113 patients was evaluated at a tertiary care center. Lymphadenopathy in the mediastinum and abdomen were included. The study was aimed at identifying the endoscopic ultrasound (EUS) features of tubercular lymphadenopathy and comparing them with reactive lymphadenopathy in patients with pyrexia of unknown origin. RESULTS: The following features were suggestive of tubercular lymphadenopathy (n = 55) as compared to reactive lymphadenopathy (n = 61): hypoechoic echotexture (94.5% vs. 75.4%, p 0.004), patchy anechoic/hypoechoic areas (30.2% vs. 0%, p = 0.000), calcification (24.5% vs. 0%, p = 0.000), sharply demarcated borders (34.5% vs. 9.8%, p = 0.001), pus like material on aspirate (18.2% vs. 0%, p 0.000), and conglomeration of lymph nodes (10.9% vs. 0%, p = 0.009). The tubercular lymph nodes were significantly larger than reactive nodes at long axis and short axis diameter (2.4 ± 1.1 vs. 1.6 ± 0.6 cm, p < 0.001 and 1.5 ± 0.7 vs. 0.9 ± 0.3 cm, p = 0.001 respectively). On cytopathological examination, presence of necrosis (92.7% vs. 0%, p = 0.000) and granulomas (78.1% vs. 0%, p = 0.000) favored tubercular as compared to reactive lymphadenopathy. CONCLUSION: EUS features like hypoechoic echotexture, patchy anechoic/hypoechoic areas, calcification, sharply demarcated borders, conglomeration, purulent aspirate, larger size, and cytopathological presence of necrosis/granulomas are suggestive of tubercular as compared to reactive lymphadenopathy.

A Rare Cause of Dysphagia--Kommerell's Diverticulum.

A right-sided aortic arch with an aneurysm of the aberrant subclavian artery is a rare disease. We report a case of Kommerell's diverticulum of an aberrant left subclavian artery in a patient with a right-sided aortic arch with associated ventricular septal defect. Fewer than 50 cases have been reported in literature so far. Our patient presented with short duration of dysphagia without any syncope or left subclavian steal syndrome. The major morbidity was caused by Barrett's oesophagus with reflux and a mixed paraoesophageal and hiatal hernia. There was associated psoriasis. An attempt at repair was not undertaken because of the high operative risk and a small aneurysm. Left thoracotomy for direct repair of Kommerell's diverticulum is a simple and safe method.

Thrombophilia Profile in Budd-Chiari Syndrome and Splanchnic Vein Thrombosis: A Study from Western India.

AIM: To estimate the prevalence of inherited prothrombotic risk factors in patients with splanchnic venous thrombosis (SVT) and Budd-Chiari syndrome (BCS) and to compare the risk factor profiles between these two groups. METHODS: In this prospective study, patients with abdominal venous thrombosis were studied. The patients were divided into two groups on the basis of the veins involved; splanchnic venous thrombosis group [portal (PVT), splenic, superior mesenteric veins (SMV)] and Budd-Chiari group (hepatic vein, IVC thrombosis). Thrombophilia profile including protein C, protein S, antithrombin III, factor V Leiden mutation, activated protein C, factor VIII level, CD55, CD59, IgM cardiolipin, IgG cardiolipin, anti-ß2 glycoprotein, JAK2 mutation, homocysteine levels, MTHFR and lupus anticoagulant was done in all patients. RESULTS: Out of 30 patients, 23 patients had SVT, 7 had BCS, including 2 of the 23 patients with SVT had mixed venous thrombosis, PVT and SMV thrombosis. Risk factors were found in 21/30 (70%) patients [17/23 (73.9%) of PVT. 4/7 (57.1% of BCS] and multiple risk factors were overall present in 8/23(34.7%) patients of SVT. CONCLUSIONS: Hereditary risk factors play an important role in the etiopathogenesis of abdominal venous thrombosis and hyperhomocysteinemia and protein S deficiency are the most common risk factors.

Sequential therapy versus standard triple-drug therapy for Helicobacter pylori eradication: a randomized study.

BACKGROUND: Antimicrobial resistance has decreased eradication rates for Helicobacter pylori infection worldwide. A sequential treatment schedule has been reported to be effective, but studies published to date were performed in Italy. We undertook this study to determine whether these results could be replicated in India. METHODS: A randomized, open-labeled, prospective controlled trial comparing sequential vs. standard triple-drug therapy was carried out at Lokmanya Tilak Municipal General Hospital, Mumbai. Two hundred and thirty-one patients with dyspepsia were randomized to a 10-day sequential regimen (40 mg of pantoprazole, 1 g of amoxicillin, each administered twice daily for the first 5 days, followed by 40 mg of pantoprazole, 500 mg of clarithromycin, and 500 mg of tinidazole, each administered twice daily for the remaining 5 days) or to standard 14-day therapy (40 mg of pantoprazole, 500 mg of clarithromycin, and 1 g of amoxicillin, each administered twice daily). RESULTS: The eradication rate achieved with the sequential regimen was significantly greater than that obtained with the triple therapy. Per-protocol eradication rate of sequential therapy was 92.4% (95% CI 85.8-96.1%) vs. 81.8% (95% CI 73.9-87.8%) (p = 0.027) for standard drug therapy. Intention-to-treat eradication rates were 88.2% (95% CI 80.9-93.0%) vs. 79.1% (95% CI 71.1-85.4%), p = 0.029, respectively. The incidence of major and minor side effects between therapy groups was not significantly different (14.6% in the triple therapy group vs. 23.5% in sequential group, p = 0.12). Follow up was incomplete in 3.3% and 4.7% patients in standard and sequential therapy groups, respectively. Sequential therapy includes one additional antibiotic (tinidazole) that is not contained in standard therapy. CONCLUSIONS: Sequential therapy was significantly better than standard therapy for eradicating H. pylori infection.