Iranian Registry of Crohn's and Colitis: study profile of first nation-wide inflammatory bowel disease registry in Middle East

BACKGROUND/AIMS: A recent study revealed increasing incidence and prevalence of inflammatory bowel disease (IBD) in Iran. The Iranian Registry of Crohn's and Colitis (IRCC) was designed recently to answer the needs. We reported the design, methods of data collection, and aims of IRCC in this paper. METHODS: IRCC is a multicenter prospective registry, which is established with collaboration of more than 100 gastroenterologists from different provinces of Iran. Minimum data set for IRCC was defined according to an international consensus on standard set of outcomes for IBD. A pilot feasibility study was performed on 553 IBD patients with a web-based questionnaire. The reliability of questionnaire evaluated by Cronbach's α. RESULTS: All sections of questionnaire had Cronbach's α of more than 0.6. In pilot study, 312 of participants (56.4%) were male and mean age was 38 years (standard deviation=12.8) and 378 patients (68.35%) had ulcerative colitis, 303 subjects (54,7%) had college education and 358 patients (64.74%) were of Fars ethnicity. We found that 68 (12.3%), 44 (7.9%), and 13 (2.3%) of participants were smokers, hookah and opium users, respectively. History of appendectomy was reported in 58 of patients (10.48%). The most common medication was 5-aminosalicylate (94.39%). CONCLUSIONS: To the best of our knowledge, IRCC is the first national IBD registry in the Middle East and could become a reliable infrastructure for national and international research on IBD. IRCC will improve the quality of care of IBD patients and provide national information for policy makers to better plan for controlling IBD in Iran.

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

Achalasia in a patient with multiple myeloma: a case report

Achalasia is a medical condition presenting with dysphagia to both liquids and solids. Although it is usually a primary disease, there are cases in which achalasia occurs secondary to other conditions. Despite its presentation as a secondary condition in a number of diseases, it is very rare for achalasia to be associated with multiple myeloma especially as the presenting symptom when the patient's disease has not been diagnosed yet. Initial manifestations of multiple myeloma include array of symptoms such as back pain, bone lesions, and anemia, but as mentioned before it is extremely rare for it to initially present with achalasia. Here we describe a man who initially presented with dysphagia and further studies confirmed the diagnosis of achalasia. Later he was diagnosed as having multiple myeloma following other presentations of the disease, thus making the dysphagia caused by achalasia his presenting symptom of multiple myeloma

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

Ulcerative colitis following orthotopic cardiac transplantation

Inflammatory bowel disease following a solid organ transplantation while the patient is re-ceiving immunosuppressive therapy is a rare phenomenon. Here we present a 48-year-old man who underwent cardiac transplantation 9 years earlier and was receiving cyclosporine as immunosuppressive therapy since then, presenting with complaints of rectorrhagia and diarrhea. In follow-up, he was diagnosed as having ulcerative colitis. We also reviewed the literature for similar cases, which yielded very few similar ones

Leech induced pyoderma gangrenosum in an ulcerative colitis patient: a case report

Pyoderma gangrenosum [PG] is a painful skin lesion that results from excessive inflammatory response to a host of traumatic, inflammatory, or neoplastic processes in susceptible individuals. A clear pathogenetic mechanism as well as an exhaustive list of potential triggers for PG is yet to be fully characterized. This case documents the occurrence of pyoderma gangrenosum following leech-therapy in a patient who is a known case of ulcerative colitis and it deserves attention because leeches have been part of medical armamentarium since ancient times and have re-emerged in the last century relying on their ancient charm and modern research revealing potential benefits of several bioactive substances in their saliva

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

young woman with multiple liver cysts

A 29-year-old housewife presented with abdominal pain and abnormal results on liver function tests [table 1]. The patient was from a rural area [Garmsar, Iran] and there was no family history of a similar disease. Her family owned various animals most notably jackals, dogs, and cats. The animals were not receiving regular veterinary check-ups, preventive care, or even vaccinations. The patient underwent a liver sonography and multiple echogenic liver cysts were reported. Consequently, an abdominal CT scan was done which showed multiple cysts in her liver [figure 1]. The radiological findings suggested multiple cystic lesions of the liver and patient's history raised the suspicion of hydatid disease. To confirm this diagnosis, a serological study of IgG ELISA for echinococcal antigens was done which yielded positive results. A surgical consultation was done; however, the medical treatment was selected by the patient. Therefore, treatment with albendazole was started