Objective: To explore disease characteristics of primary sclerosing cholangitis (PSC) and inflammatory bowel disease (IBD) and compare the differences between PSC with and without IBD. Methods: Study design was cross sectional. Forty-two patients with PSC who were admitted from January 2000 to January 2021 were included. We analyzed their demographic characteristics, clinical manifestations, concomitant diseases, auxiliary examination, and treatment. Results: The 42 patients were 11-74(43±18) years of age at diagnosis. The concordance rate of PSC with IBD was 33.3%, and the age at PSC with IBD diagnosis was 12-63(42±17) years. PSC patients with IBD had higher incidences of diarrhea and lower incidences of jaundice and fatigue than in those without IBD (all P<0.05). Alanine aminotransferase, total bilirubin, direct bilirubin, total bile acid and carbohydrate antigen 19-9 levels were higher in PSC patients without IBD than in those with IBD (all P<0.05). The positive rates for antinuclear antibodies and fecal occult blood were higher in PSC patients with IBD than in those without IBD (all P<0.05). Patients with PSC complicated with ulcerative colitis mainly experienced extensive colonic involvement. The proportion of 5-aminosalicylic acid and glucocorticoid application in PSC patients with IBD was significantly increased compared with that of PSC patients without IBD (P=0.025). Conclusions: The concordance rate of PSC with IBD is lower at Peking Union Medical College Hospital than in Western countries. Colonoscopy screening may benefit PSC patients with diarrhea or fecal occult blood-positive for early detection and diagnosis of IBD.
Humans ; Child ; Adolescent ; Young Adult ; Adult ; Middle Aged ; Cholangitis, Sclerosing/therapy* ; Cross-Sectional Studies ; Inflammatory Bowel Diseases/diagnosis* ; Colitis, Ulcerative/complications* ; Diarrhea

OBJECTIVES: To investigate the nutritional status and its influencing factors in children with newly diagnosed inflammatory bowel disease (IBD). METHODS: A retrospective analysis was conducted on the clinical data of children who were diagnosed with IBD for the first time in Hunan Children's Hospital from January 2015 to December 2021. Diagnostic delay was defined as the time from the symptom onset to IBD diagnosis being in the upper quartile (P₇₆-P₁₀₀) of all IBD children in the study. Multivariate logistic regression analysis was used to explore the risk factors for emaciation and growth retardation. RESULTS: A total of 125 children with newly diagnosed IBD were included, with Crohn's disease being the main type (91.2%). The rates of emaciation and growth retardation were 42.4% (53 cases) and 7.2% (9 cases), respectively, and the rate of anemia was 77.6% (97 cases). Diagnostic delay was noted in 31 children (24.8%), with the time from the symptom onset to IBD diagnosis of 366 to 7 211 days. Multivariate logistic regression analysis showed that diagnostic delay was a risk factor for emaciation and growth retardation (OR=2.73 and OR=4.42, respectively; P<0.05) and that age was positively associated with emaciation (OR=1.30, P<0.05). CONCLUSIONS Children with newly diagnosed IBD have poor nutritional status, and the rates of anemia, emaciation, and growth retardation are high. Diagnostic delay is associated with malnutrition in children with IBD.
Humans ; Child ; Colitis, Ulcerative/diagnosis* ; Nutritional Status ; Retrospective Studies ; Emaciation/complications* ; Delayed Diagnosis ; Inflammatory Bowel Diseases/complications* ; Malnutrition/complications* ; Growth Disorders/complications*

To analyze the clinical features and prognosis of ulcerative colitis(UC)complicated with acute massive lower gastrointestinal bleeding(LGIB). Methods Eleven patients hospitalized in Peking Union Medical College Hospital from January 2006 to December 2017 for treatment of UC,suffering from acute massive LGIB,were enrolled and descriptively analyzed. Results The proportion of UC patients with acute massive LGIB was 0.7% among all 1486 UC patients hospitalized during the study period.The disease was moderately or severely active in these 11 patients,among whom 9 patients(81.8%)had chronic relapsing pancolitis.Cytomegalovirus infection was present in 5 patients,among whom 4 patients received antiviral treatments.All the 11 patients received treatments including food and water fasting,rehydration,blood transfusion,and use of somatostatin.Four patients received emergency surgical treatment after the first episode of massive bleeding,and 3 of them suffered from re-bleeding after the surgery.Among the remaining seven patients,two underwent emergency total colectomy+subtotal rectectomy+ileostomy and three received elective total resection of colon and rectum or total colectomy+subtotal rectectomy+ileostomy.Thus,9 patients underwent emergency surgery,1 patient did not receive surgey during follow-up,and 1 patient was lost to follow-up. Conclusions Acute massive LGIB is a manifestation of active UC and can be associated with poor prognosis.Optimized perioperative management is important for improving the outcomes of such patients.
Colectomy ; Colitis, Ulcerative ; complications ; diagnosis ; surgery ; Gastrointestinal Hemorrhage ; complications ; surgery ; Humans ; Ileostomy ; Prognosis

INTRODUCTIONThe aim of the study was to determine if age at the creation of an ileal pouchanal anastomosis (IPAA) has an impact on the outcomes in patients with ulcerative colitis (UC).

MATERIALS AND METHODSA retrospective review of all patients who underwent IPAA for UC from 1999 to 2011 was performed. Long-term functional outcome was assessed using both the Cleveland Clinic and St Mark's incontinence scores.

RESULTSEighty-nine patients, with a median age of 46 (range, 16 to 71) years, formed the study group. The median duration of disease prior to their pouch surgery was 7 (0.5 to 39) years. There were 57 (64%) patients who were aged ≤50 years old and 32 (36%) who were >50 years old. Fifty-seven (64%) patients developed perioperative complications of which 51 (89.5%) were minor. High ileostomy output (n = 21, 23.6%) and urinary symptoms (n = 13, 14.6%) were the most commonly encountered complications. The older patients were more likely to have an ASA score ≥3 and a longer length of stay. Although there was a higher incidence of complications in the older group of patients, the difference was not statistically significant. There were no significant differences in the incidence of severe complications. Forty-nine (55%) patients completed our questionnaire on the evaluation of their functional outcomes. There were no significant differences in the Cleveland Clinic and St Mark's incontinence scores between the older (n = 19, 38.8%) and younger (n = 30, 61.2%) patients. There were also no significant differences in the frequency of bowel movements during the day or overnight after sleep between the 2 groups.

CONCLUSIONIPAA procedure for patients with UC can be safely performed. Long-term functional outcome is not significantly influenced by the age at which the IPAA was created.

Adolescent ; Adult ; Age Factors ; Aged ; Colitis, Ulcerative ; surgery ; Colonic Pouches ; Fecal Incontinence ; diagnosis ; epidemiology ; etiology ; Female ; Follow-Up Studies ; Humans ; Incidence ; Male ; Middle Aged ; Postoperative Complications ; diagnosis ; epidemiology ; Proctocolectomy, Restorative ; Retrospective Studies ; Treatment Outcome ; Young Adult

A 45-year-old-male who had underlying ulcerative colitis and presented with fever and dry cough. Initially, the patient was considered to have invasive aspergillosis due to a positive galactomannan assay. He was treated with amphotericin B followed by voriconazole. Nevertheless, the patient deteriorated clinically and radiographically. The lung biopsy revealed eosinophilic pneumonia, and ELISA for Toxocara antigen was positive, leading to a diagnosis of pulmonary toxocariasis. After a 10-day treatment course with albendazole and adjunctive steroids, the patient recovered completely without any sequelae. Pulmonary toxocariasis may be considered in patients with subacute or chronic pneumonia unresponsive to antibiotic agents, particularly in cases with eosinophilia.
Albendazole/therapeutic use ; Animals ; Anthelmintics/therapeutic use ; Anti-Inflammatory Agents/therapeutic use ; Antigens, Helminth/analysis ; Colitis, Ulcerative/*complications ; Diagnosis, Differential ; Enzyme-Linked Immunosorbent Assay ; Humans ; Lung/pathology ; Lung Diseases, Parasitic/*diagnosis/*pathology ; Male ; Middle Aged ; Pulmonary Aspergillosis/diagnosis/pathology ; Steroids/therapeutic use ; Toxocara/*isolation & purification ; Toxocariasis/*diagnosis/*pathology ; Treatment Outcome

No abstract available.
Amyloidosis/complications/*diagnosis/pathology ; Colitis, Ulcerative/diagnosis ; Colonoscopy ; Gastrointestinal Hemorrhage/*etiology ; Heart Ventricles/radiography ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged

OBJECTIVELeukocyte adhesion deficiency type 1 (LAD-I) is rare. We present 1 case of LAD-I patient diagnosed by gene analysis. His clinical manifestations and genetic mutation features are analyzed in this article.

METHODThe clinical material of the LAD-I patient who was diagnosed by gene analysis was retrospectively analyzed.

RESULTThe patient was a 2-month-old boy. He had a complaint of recurrent fever and cough for 30 days. Pulmonary CT indicated a small to moderate quantity pleural effusion on the right side. His peripheral blood leukocyte and C-reactive protein (CRP) was always significantly higher than normal. After hospitalization he had diarrheal diseases, routine stool test showed 2 RBC cells/high power (HP), WBC 30 cells/HP, stool cultures were negative, digestive tract ultrasonography showed an array of defects, in the sigmoid colon and rectal mucosa suggestive of ulcerative colitis. He was treated with cefoperazone and sulbactam and vancomycin. He had a history of impetigo in his neonatal period and without delayed umbilical cord exfoliation. His family history was normal. ITGB2 genetic mutation analysis revealed a homozygous mutation (1062A > T). His parents did not participate in this study. He had no fever but had diarrheal disease after 1 month of follow up.

CONCLUSIONThis patient had suffered from impetigo, pleural effusion, diarrheal diseases, markedly increased peripheral white blood cell and ITGB2 genetic mutation analysis showed that homozygous mutation (1062A > T). He received a diagnosis of LAD-I.

Asian Continental Ancestry Group ; Colitis, Ulcerative ; diagnosis ; etiology ; Cytoskeletal Proteins ; genetics ; DNA Mutational Analysis ; Flow Cytometry ; Homozygote ; Humans ; Infant ; Leukocyte Count ; Leukocyte-Adhesion Deficiency Syndrome ; complications ; diagnosis ; genetics ; Male ; Muscle Proteins ; genetics ; Pleural Effusion ; diagnosis ; etiology ; Point Mutation ; genetics ; Polymerase Chain Reaction ; Retrospective Studies

Takayasu arteritis (TA) is a chronic vasculitis that affects the aortic arch and its primary branches. Ulcerative colitis (UC) is an inflammatory bowel disease of unknown etiology. Patients diagnosed with both TA and UC have rarely been reported. The pathogenesis of TA and UC is uncertain, but cell-mediated mechanisms play an important role in both diseases, and a genetic factor is thought to have an effect on the coincidence of these two diseases. We herein report a 38-year-old female with TA who had a history of UC with optic neuritis. We believe that this is the first case of the coexistence of TA and UC in Korea.
Adult ; Anti-Inflammatory Agents/therapeutic use ; Aortography/methods ; Colitis, Ulcerative/*complications/diagnosis/drug therapy ; Colonoscopy ; Female ; Humans ; Immunosuppressive Agents/therapeutic use ; Optic Neuritis/*complications/diagnosis/drug therapy ; Positron-Emission Tomography ; Republic of Korea ; Steroids/therapeutic use ; Takayasu Arteritis/*complications/diagnosis/drug therapy ; Tomography, X-Ray Computed ; Treatment Outcome

A 21-year-old man admitted complaining of sudden severe epigastric pain for 1 day. He had been diagnosed as ulcerative colitis (UC) and taking mesalazine for two months. UC was in nearly complete remission at admission. He never drank an alcohol, and serum amylase was 377 IU/L. CT scan showed inferior vena cava (IVC) thrombosis in addition to mild acute pancreatitis. To evaluate the cause of acute pancreatitis and IVC thrombosis, magnetic resonance cholangiopancreatogram (MRCP), endoscopic ultrasonogram (EUS), lower extremity Doppler ultrasonogram (US) and blood test of hypercoagulability including factor V, cardiolipin Ab, protein C, protein S1, antithrombin III, and anti phospholipids antibody were performed. There was no abnormality except mild acute pancreatitis and IVC thrombosis in all the tests. He was recommended to stop taking mesalazine and start having anticoagulation therapy. After all symptoms disappeared and amylase returned normal, rechallenge test with mesalazine was done. Flare-up of abdominal pain occurred and the elevation of serum amylase was observed. Ulcerative colitis came to complete remission with short-term steroid monotherapy. Acute pancreatitis and IVC thrombosis were completely resolved after 3-month anticoagulation therapy with no more mesalazine. We postulated that IVC thrombosis occurred due to hypercoagulable status of UC and intra-abdominal inflammation caused by mesalazine-induced pancreatitis.
Acute Disease ; Amylases/blood ; Anti-Inflammatory Agents, Non-Steroidal/*adverse effects/therapeutic use ; Anticoagulants/therapeutic use ; Cholangiopancreatography, Magnetic Resonance ; Colitis, Ulcerative/complications/*diagnosis/drug therapy ; Endosonography ; Humans ; Male ; Mesalamine/*adverse effects/therapeutic use ; Pancreatitis/chemically induced/*diagnosis/ultrasonography ; Tomography, X-Ray Computed ; Ultrasonography, Doppler ; *Vena Cava, Inferior/ultrasonography ; Venous Thrombosis/complications/*diagnosis/drug therapy ; Young Adult

A case of hemophagocytic syndrome associated with ulcerative colitis is very rare. A 32-year-old man visited the hospital complaining of fever and severe abdominal pain for 7 days. He was diagnosed to have ulcerative colitis 2 years ago and had been treated with sulfasalazine. Three months ago, he had abdominal pain, weight loss, and hematochezia, so prednisolone and mercaptopurine were added to the treatment. On admission, the physical examination showed splenomegaly. Peripheral blood counts revealed pancytopenia, and bone marrow aspirate smears showed many histiocytes with active hemophagocytosis. There was no evidence of viral and bacterial infections and other neoplasms, which were commonly associated with hemophagocytic syndrome. He was successfully treated with high dose steroid. We report this case along with a review of the related literatures.
6-Mercaptopurine/therapeutic use ; Adult ; Anti-Inflammatory Agents/therapeutic use ; Anti-Inflammatory Agents, Non-Steroidal/therapeutic use ; Bone Marrow Cells/pathology ; Colitis, Ulcerative/complications/*diagnosis/drug therapy ; Colonoscopy ; Dexamethasone/therapeutic use ; Humans ; Immunosuppressive Agents/therapeutic use ; Lymphohistiocytosis, Hemophagocytic/complications/*diagnosis/drug therapy ; Male ; Prednisolone/therapeutic use ; Sulfasalazine/therapeutic use ; Syndrome ; Tomography, X-Ray Computed