Clinical characteristics of paroxysmal nocturnal hemoglobinuria (PNH) complicated with ischemic bowel disease / 中华内科杂志

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired hematopoietic stem cell disease. Gastrointestinal involvement is rarely seen in PNH. This study aims to analyze the clinical features in PNH patients complicated with ischemic bowel disease. Clinical date of 6 patients were collected at Peking Union Medical College Hospital from January 2010 to December 2020. The clinical manifestations, laboratory tests,imaging, endoscopic,and histopathological features and treatment were analyzed.Five in 6 patients were men, with a median age of 31 years old at onset. Most of disease course were recurrent episodes of chronic disease, with abdominal pain (5/6) and gastrointestinal bleeding (5/6). Laboratory examinations showed pancytopenia, reticulocytosis, elevated serum lactate dehydrogenase, high D-dimer and C-reactive protein levels in all patients. Multiple segments of small intestine were the most commonly involved and colon was also affected. Abdominal CT scan showed thickening and roughness or exudation of the intestinal wall (6/6), increased mesenteric density or “comb sign”(4/6), and cholestasis or gallbladder stones (5/6). Endoscopic manifestations included irregular shallow ulcers in the annular cavity (5/6), swelling mucosa with well-defined margins (6/6). Pathological biopsy revealed chronic inflammation of mucosa. The efficacy of steroids combined with anticoagulant therapy was better than that of steroids alone. Ischemic bowel disease in PNH patients is different from typical ischemic enteritis. Young patients, involvement of intestine with multiple segments are common characteristics. The anticoagulant is an essential agent for these patients.

Clinical characteristics of ischemic bowel disease in elderly patients / 中华全科医师杂志

Objective To analyze the clinical characteristics of ischemic bowel disease in elderly patients.Methods The clinical data of 83 patients with ischemic bowel disease, including 48 patients aged ≥60 years (elderly group ) and 35 patients aged ＜60 years ( control group ), admitted in our hospital from January 2011 to June 2016 were retrospectively analyzed.The clinical characteristics , underlying diseases, examination methods and treatment were compared between the two groups .Results There were no significant differences in gender , occupation, urban or rural origin between the two groups (P＞0.05).The incidence of acute mesenteric ischemia ( AMI) in elderly group was significantly higher than that in the control group [22.9%(11/48) vs.5.7%(2/35), χ2＝4.534, P ＝0.033];while the incidence of ischemic colitis (IC) in control group was higher than that in the elderly group [94.3%(33/35) vs. 70.8%(34/48), χ2＝7.154, P＝0.007].Elderly group was more prone to misdiagnosis as compared with the control group [58.3%(28/48) vs.34.3%(12/35), χ2＝4.688, P ＝0.030] .Abdominal pain, diarrhea and hematochezia were the most common clinical manifestations of the two groups .Moderate pain and dull pain were the main symptoms of the elderly group accounting for 75.0%(36/48), while colic was the main symptoms of the control group accounting for 65.7%(23/35).The proportion of elderly patients with hypertension, coronary heart disease and cerebral infarction was higher than that of the control group [70.8%(34/48)vs.25.7%(9/35),62.5%(30/48) vs.20.0%(7/35),16.7%(8/48) vs.0(0/35), χ2＝16.504,14.798,4.684,all P＜0.05].The number of patients receiving colonoscopic examination was lower in the elderly group than that in the control group [52.1%(25/48) vs.94.3%(33/35), χ2＝17.127, P＝0.000 ] .The overall effective rate of medical treatment for elderly patients was 90.2%(37/41), mostly of them were chronic mesentery ischemia (CMI) and IC patients; while 11 cases of AMI patients had poor outcomes of medical and surgical treatment including 5 fatal cases.Conclusions The incidence and mortality of AMI in elderly patients with ischemic bowel disease are higher , while CMI and IC have good prognosis.Timely diagnosis, appropriate treatment may improve the cure rate and reduce mortality for elderly patients with AMI.

Computed tomography in the diagnosis of portal venous and intestinal wall gas in patients with ischemic bowel disease / 中华肝胆外科杂志

Objective To investigate radiological features on computed tomography (CT) in the di agnosis of portal venous and intestinal wall gas in patients with ischemic bowel disease.Methods The clinic-pathological data of 17 patients with portal venous and intestinal gas associated with ischemic bowel diseases from Wenzhou People's Hospital (n =6),Yueqing People's Hospital (n =5),Shanghai Xuhui Dahua Hospital (n =3) and the Second Affiliated Hospital of Wenzhou Medical University (n =3) from January 2013 to October 2016 were analysed retrospectively.All the patients have been fasting for 8 h prior to CT scans.Enhanced CT study was performed following routine CT with no abdominal pressure for breath less scanting.Portal venous gas,intestinal wall gas,intestinal thickness and density,mesentery thickness,celiac effusion,and severity of intestinal wall enhancement were recorded.Results All the 17 patients ex perienced abdominal distension and pain.Additionally,nausea and vomiting was observed in 9 patients,di arrhea in 7,melena in 7,periumbilical tenderness in 11 and rebound tenderness in 8.CT scans of these 17 patients showed portal venous gas,including massiveprune-tree signs of hepatic vein and portal vein (n =11) and scanty gas shadows in distal hepatic vein (n =6).Intestinal gas sign was determined in all these patients (n =17),including single bubble shadow (n =8),multiple bubble shadow (n =7),and band-shaped bubble (n =2).Furthermore,CT study indicated extensive intestinal wall thickening with edema (n =13),predominate luminal extension of thinner bowels (n =4),scanty celiac effusion (n =3).Enhanced CT scans demonstrated 8 patients with decreased enhancement of intestinal wall and mesentery with diseases,target and halo signs observed in enhanced scans.Conclusions Portal venous and intestinal wall gas may demonstrate distinctive CT imaging.CT study could have superior sensitivity and spe cialty in clinical diagnoses of ischemic bowel diseases.

Analysis for abdomen CT characteristics of patients with ischemic bowel disease / 解放军医学杂志

Objective To investigate the abdomen CT characteristics of patients with ischemic bowel disease (ICBD).Methods CT imaging data of ICBD patients from January 2008 to December 2013 in the Chinese PLA General Hospital were retrospectively analyzed to find CT imaging features in intestinal lesions associated with ICBD death.Results In CT imaging analysis,151 patients including acute superior mesenteric artery thromboembolism (ASMATE,n=51),acute superior mesenteric vein thrombosis (ASMVT,n=53),non-occlusive mesenteric ischemia (NOMI,n=8),chronic mesenteric ischemia (CMI,n=10) and ischemic colitis (IC,n=29) were divided into survival group (n=115) and death group (n=36).In comparison with the survival group,the death group had higher incidence of abdomenal effusion,portomesenteric gas,pneumatosis intestinalis and pneumoperitoneum (P＜0.001,P＜0.001,P＜0.001,P=0.003).Conclusion The ascites,portomesenteric vein gas,pneumatosis intestinalis and pneumoperitoneum in bowel CT may be associated with the death of ICBD patients.

Value of plasma D-dimer in early diagnosis of acute ischemic bowel disease / 国际检验医学杂志

Objective To study the value of D‐dimer for early diagnosing ischemic bowel disease(IBD) and to find more econom‐ic and more effective detection means for its diagnosis and control .Methods Ninety‐five patients with suspected acute IBD in our hospital from October 2014 to October 2015 were selected as the observation group ,and contemporaneous 74 individuals undergoing routine physical examination were selected as the control group .The differences in D‐dimer levels were compared between the con‐trol group and the patients with definitely diagnosed IBD in the observation group on 1 ,7 ,14 d after admission .At the same time the differences in initial WBC level and fecal occult blood were compared between the two groups .The sensitivity and specificity of plas‐ma D‐dimer level for early diagnosing IBD were calculated and their clinical application value was summarized .Results (1) Accord‐ing to plasma D‐dimer results ,the 74 cases of acute IBD were diagnosed and 21 cases were negative ,while 80 positive cases and 15 negative cases were diagnosed by adopting the CT inferior mesenteric arterial angiography and multislice spiral CT ,the sensitivity of plasma D‐dimer detection was 88 .75% and specificity was 80 .0% .(2) The D‐dimer level ,WBC count and fecal occult blood on 1 d after admission in the observation group were (5 480 .12 ± 876 .32)μg/L ,(17 .37 ± 2 .21) × 109/L and 81 .3% respectively ,which were far higher than those in the healthy control group ,the differences were statistically significant (P<0 .05) .(3)The D‐dimer levels on 1 ,7 ,14 d after admission in the observation group were significantly higher than those in the control group (P<0 .05) , while with the treatment duration extension ,the plasma D‐dimer level was gradually decreased .Conclusion The D‐dimer detection has a relatively higher sensitivity and specificity in the early diagnosis of acute IBD and has better clinical application value .

Acute Renal Failure and Ischemic Bowel Disease Complicated by Acute Pyelonephritis in a Patient with Systemic Lupus Erythematosus / 대한신장학회잡지

Acute infection increases disease activity in patients with systemic lupus erythematosus(SLE) and causes life threatening complication such as acute renal failure or ischemic bowel disease. We here report a case of acute renal failure and ischemic bowel disease complicated by acute pyelonephritis in a patient with SLE. A 19-year-old woman was admitted for high fever and right flank pain. Urine examination revealed acute pyelonephritis. Thrombocytopenia, proteinuria, positive antinuclear antibody and anti-dsDNA, false positive VDRL confirmed SLE. The pyelonephritis improved with antibiotic treatment, but oliguria and abdominal pain and ascites newly developed. Kidney biopsy and abdominal computed tomography revealed lupus nephritis type IV and ischemic bowel disease, respectively. After methylprednisolone and cyclophosphamide treatment, the patient improved.

Rectal Obstruction Caused by Ischemic Colitis with Angiodysplasia: A case report

Angiodysplasia is a vascular lesion of the gut, which reveals intestinal bleeding as a major symptom. It is the cause of as much as 5~6% of the intestinal bleeding of unkown origin. Pathologically, it reveals anomalous submucosal vascular overgrowth, which is characterized by tortuous, dilated and thin-walled vessels. Because the lesion is mainly submucosal. It is hard to diagnosis with endoscopy and even in laparotpmy. Angiography has a low detection rate for angiodysplasia, but some of this typical findings are delayed excretion of dye into the venous structure around the lesion, vascular tuft in the arterial phase, and accelerated dye secretion into venous structure in the situation of arteriovenous malformation. Endoscopy, especially colonoscopy is so effective a diagnostic method as to make interventional therapy. Conjugated estrogen therapy is effective and is regarded as choice of treatment in the not-life-threatening intestinal bleeding caused by angiodysplasia. Surgery is only indicated in the case of uncontrolled bleeding. Intestinal obstruction has rarely been reported as another manifestation of intestinal angiodysplasia. Therefore pathologic definition should be confirmed, and research for pathophysiology of mucosal hypertrophy in the angiodysplasia is needed.

A Clinical Review of Ischemic Rowel Disease / 대한내과학회지

OBJECTIVE: Despite increased awareness of the fatality of mesenteric ischemia, the diagnosis seldom is made prior to the onset of gangrene. The multiplicity of etiologic factors, the many varied presentations, and splanchnic vasoconstriction all affect the extent of ischemic injury, adding to the complexity of the clinical problem. Extensive acute processes are still catastrophic illnesses with a high mrotality, but there is a potential for both better diagnisis and therapy with an improved outcome. Discussion of the pathophysiology, diagnosis, and treatment of this entity will be presented. METHODS: A Retrospective review of our experience with ischemic bowel disease was made. Ten consecutive clinical cases admitted in ChungNam National University Hospital from October 1990 to April 1994 were observed. RESULTS: 1) We experienced 5 patients with arterial embolic occlusion, 1 patient with venous thrombosis and 4 patients with colonic ischemia. 2) The peak ages were 6th decade and 8th decade. 3) The major clincal symptoms and signs were abdominal pain (100%), abdominal tenderness (70%), melena (70%), nausea (60%), fever (50%), vomiting (40%) and abdominal distension (30%). 4) On laboratory findings, there were leukocytosis (80%) and thrombocytopenia (20%) 5) In plain film, there were ileus (70%), edematous intestinal wall (50%), mucosal edema (30%), thumb printing (10%) and gasless abdomen (10%). Among the 5 cases performed abdominal CT, there were thickening of intestinal wall in 4 cases, narrowing of intestinal lumen in 2 case and ascites in 3 cases. 6) Bowel resections were perfomed in 7 cases and supportive care was performed in 3 cases. 7) The overall mortality rate was 30%, CONCLUSION: An oggressive approach in patients suspected of having ischemic bowel is indicated if the diagnosis is to be made before necrosis has occurred. A high index of suspicion, early angiography, correction of the underlying cardiac disease, treatment of splanchnic vasoconstriction, surgical revascularization, and resection of gangrenous bowel are necessery if there is to be a significant reduction in the high mortality rates associated with mesenteric ischemia.