Protein-losing enteropathy(PLE) is characterized by loss of serum proteins into the gastrointestinal tract. It may lead to hypoproteinemia and clinically present as protein deficiency edema, ascites, pleural or perica...Protein-losing enteropathy(PLE) is characterized by loss of serum proteins into the gastrointestinal tract. It may lead to hypoproteinemia and clinically present as protein deficiency edema, ascites, pleural or pericardial effusion and/or malnutrition. In most cases the site of protein loss is the small intestine. Here we present an unusual case of severe PLE in a 55-year old female with a one-year history of recurrent diarrhea, crampy abdominal pain, and peripheral edema. Endoscopy and MRI showed a diffuse inflammatory thickening of the sigmoid colon and the rectum. Surgical resection of the involved colon was performed and the symptoms were significantly resolved. The final histologic evaluation confirmed a diagnosis of a pseudomembranous colitis with cap polyposis-like features. Such a cause of PLE has never been described before.展开更多
BACKGROUND The incidence of internal hernias has recently increased in concordance with the popularization of laparoscopic surgery.Of particular concern are internal hernias occurring in Petersen's space,a space t...BACKGROUND The incidence of internal hernias has recently increased in concordance with the popularization of laparoscopic surgery.Of particular concern are internal hernias occurring in Petersen's space,a space that is surgically created after treatment for gastric cancer and obesity.These hernias cause devastating sequelae,such as massive intestinal necrosis,fatal Roux limb necrosis,and superior mesenteric vein thrombus.In addition,protein-losing enteropathy(PLE)is a rare syndrome involving gastrointestinal protein loss,although its relationship with internal Petersen’s hernias remains unknown.CASE SUMMARY A 75-year-old man with a history of laparotomy for early gastric cancer developed Petersen's hernia 1 year and 5 mo after surgery.He was successfully treated by reducing the incarcerated small intestine and closure of Petersen’s defect without resection of the small intestine.Approximately 3 mo after his surgery for Petersen’s hernia,he developed bilateral leg edema and hypoalbuminemia.He was diagnosed with PLE with an alpha-1 antitrypsin clearance of 733 mL/24 h.Double-balloon enteroscopy revealed extensive jejunal ulceration as the etiology,and it facilitated minimum bowel resection.Pathological analysis showed extensive jejunal ulceration and collagen hyperplasia with nonspecific inflammation of all layers without lymphangiectasia,lymphoma,or vascular abnormalities.His postoperative course was unremarkable,and his bilateral leg edema and hypoalbuminemia improved after 1 mo.There was no relapse over the 5-year follow-up period.CONCLUSION PLE and extensive jejunal ulceration may occur after Petersen's hernia.Doubleballoon enteroscopy helps identify and resect these lesions.展开更多
BACKGROUND While primary intestinal lymphangiectasia(PIL)is considered a rare condition,there have been several reported cases in adults.Nevertheless,the absence of clear guidance from diagnosis to treatment and progn...BACKGROUND While primary intestinal lymphangiectasia(PIL)is considered a rare condition,there have been several reported cases in adults.Nevertheless,the absence of clear guidance from diagnosis to treatment and prognosis poses challenges for both physicians and patients.AIM To enhance understanding by investigating clinical presentation,diagnosis,treatment,complications,and prognoses in adult PIL cases.METHODS We enrolled adult patients diagnosed with PIL between March 2016 and September 2021.The primary outcome involved examining the diagnosis and treatment process of these patients.The secondary outcomes included identifying complications(infections,thromboembolism)and assessing prognoses(frequency of hospitalization and mortality)during the follow-up period.RESULTS Among the 12 included patients,peripheral edema(100%)and diarrhea(75%)were the main presenting complaints.Laboratory tests showed that all the pati-ents exhibited symptoms of hypoalbuminemia and hypogammaglobulinemia.Radiologically,the predominant findings were edema of the small intestine(67%)and ascites(58%).The typical endoscopic finding with a snowflake appearance was observed in 75%of patients.Among the 12 patients,two responded positive-ly to octreotide and sirolimus,and eight who could undergo maintenance therapy discontinued subsequently.Complications due to PIL led to infection in half of the patients,thromboembolism in three patients,and one death.CONCLUSION PIL can be diagnosed in adults across various age groups,with different severity and treatment responses among patients,leading to diverse complications and prognoses.Consequently,tailored treatments will be necessary.We anticipate that our findings will contribute to the management of PIL,an etiology of protein-losing enteropathy.展开更多
Primary intestinal lymphangiectasia(PIL) is a rare protein-losing enteropathy with lymphatic leakage into the small intestine. Dilated lymphatics in the small intestinal wall and mesentery are observed in this disease...Primary intestinal lymphangiectasia(PIL) is a rare protein-losing enteropathy with lymphatic leakage into the small intestine. Dilated lymphatics in the small intestinal wall and mesentery are observed in this disease. Laboratory tests of PIL patients revealed hypoalbuminemia, lymphocytopenia, hypogammaglobulinemia and increased stool α-1 antitrypsin clearance. Cell-mediated immunodeficiency is also present in PIL patients because of loss of lymphocytes. As a result, the patients are vulnerable to chronic viral infection and lymphoma. However, cases of PIL with chronic viral infection, such as human papilloma virus-induced warts, are rarely reported. We report a rare case of PIL with generalized warts in a 36-year-old male patient. PIL was diagnosed by capsule endoscopy and colonoscopic biopsy with histological tissue confirmation. Generalized warts were observed on the head, chest, abdomen, back, anus, and upper and lower extremities, including the hands and feet of the patient.展开更多
BACKGROUND Lymphangiomatosis is a multisystem disorder that is rarely localized to the gastrointestinal tract.Lymphangiomatosis usually has no specific clinical presentation and is easily misdiagnosed.A case report an...BACKGROUND Lymphangiomatosis is a multisystem disorder that is rarely localized to the gastrointestinal tract.Lymphangiomatosis usually has no specific clinical presentation and is easily misdiagnosed.A case report and review of the literature on lymphangiomatosis associated with protein-losing enteropathy will help to improve the overall understanding of this disease.CASE SUMMARY We report a case of lymphangiomatosis of the bowel and other solid organs.A 78-year-old man presented with recurrent bowel bleeding and protein-losing enteropathy,as well as cystic lesions in the spleen,liver,and kidney.Imaging examinations revealed many cystic lesions on the spleen,liver,kidney,and thickened wall of the ascending colon,as well as pleural effusion and ascites.Colonoscopy revealed a strawberry mucosa,variable spontaneous bleeding,and surface erosion located in the terminal ileum.Several cystic masses with a translucent and smooth surface as well as diffuse white spots were located in the colon.A laterally spreading tumor(LST)was located in the ascending colon.Pathology indicated highly differentiated adenocarcinoma(LST)and lymphangiomatoid dilation,and D2-40 was positive.The final diagnosis was lymphangiomatosis.The patient underwent surgery for LST and then was administered thalidomide 75-150 mg/d.His condition,however,did not improve.He eventually died 6 mo after the initial diagnosis.CONCLUSION Lymphangiomatosis usually occurs diffusely and can involve many organs,such as the spleen,kidney,liver,lung,mesentery,and bowel.Recurrent bowel bleeding or protein-losing enteropathy is an important indicator that should alert clinicians about the possibility of this disease when it afflicts the bowel.Doctors should improve the medical understanding of lymphangiomatosis.展开更多
A 77-year-old male patient was diagnosed with protein-losing enteropathy as he had diarrhea for 4 years accompanied with repeated and exacerbated diarrhea and edema of the both lower extremities for more than 1 and a ...A 77-year-old male patient was diagnosed with protein-losing enteropathy as he had diarrhea for 4 years accompanied with repeated and exacerbated diarrhea and edema of the both lower extremities for more than 1 and a half years.In this case,warming-needle moxibustion compiled with the abdominal rubbing that patient performed by himself were utilized.Zhongwan(中脘CV12),Guanyuan(关元CV4);bilateral Tianshu(天枢ST25),Zusanli(足三里ST36),Shangjuxu(上巨虚ST37).Xiajuxu(下巨虚ST39)and the point on three cun below ST39 were taken when warming-needle moxibustion was performed.The treatment was given once a day.After 3 weeks'treatment,the frequency of diarrhea was decreased,the edema disappeared,the seralbumin level was increased,and the symptoms of the patient disappered.Follow-up for half a year,the patients had good prognosis.展开更多
Whilst ascites is a common presenting complaint in patients with decompensated chronic liver disease and disseminated malignancy,in Crohn's disease however,it is exceptionally rare.We describe a patient with no pr...Whilst ascites is a common presenting complaint in patients with decompensated chronic liver disease and disseminated malignancy,in Crohn's disease however,it is exceptionally rare.We describe a patient with no prior history of inflammatory bowel or liver disease,presenting with rapid onset gross ascites and scrotal swelling.Further investigations revealed severe hypoalbuminemia and transudative ascitic fluid with normal other liver function tests and a negative liver screen.Computed tomography revealed widespread ascites and pleural effusions with no features of malignancy or portal hypertension,and a small bowel barium series showed features of fistulating small bowel Crohn's disease.An ileo-colonoscopy confirmed the presence of terminal ileal inflammatory stricture.The patient's clinical condition and serum albumin improved with a combination of diuretics,elemental diet,antibiotics and oral 5-aminosalicylic acid therapy.展开更多
BACKGROUND Primary intestinal lymphangiectasia(PIL)is a rare protein-losing enteropathy characterized by abnormally dilated lymphatic structures,resulting in leakage of lymph(rich in protein,lymphocytes,and fat)from t...BACKGROUND Primary intestinal lymphangiectasia(PIL)is a rare protein-losing enteropathy characterized by abnormally dilated lymphatic structures,resulting in leakage of lymph(rich in protein,lymphocytes,and fat)from the intestinal mucosal and submucosal layers and thus hypoproteinemia,lymphopenia,hypolipidemia,and pleural effusion.CASE SUMMARY A 19-year-old Chinese male patient complained of recurrent limb convulsions for the last 1 year.Laboratory investigations revealed low levels of calcium and magnesium along with hypoproteinemia and high parathyroid hormone levels,whereas gastroscopy exhibited chronic non-atrophic gastritis and duodenal lymphatic dilatation.Subsequent gastric biopsy showed moderate chronic inflammatory cell infiltration distributed around a small mucosal patch in the descending duodenum followed by lymphatic dilatation in the mucosal lamina propria,which was later diagnosed as PIL.The following appropriate mediumchain triglycerides nutritional support significantly improved the patient’s symptoms.CONCLUSION Since several diseases mimic the clinical symptoms displayed by PIL,like limb convulsions,low calcium and magnesium,and loss of plasma proteins,it is imperative to conduct a detailed analysis to avoid any misdiagnosis while pinpointing the correct clinical diagnosis and simultaneously ruling out other clinical aspects in the reported cases without any past disease history.A careful assessment should always be made to ensure an accurate diagnosis in a timely manner so that the patient can be delivered quality health services for a positive health outcome.展开更多
Protein losing enteropathy(PLE), a very rare disease with hypoproteinemia and edema as its characteristics, is caused by various diseases resulting in protein depletion from the gut. The diagnosis is relatively diffic...Protein losing enteropathy(PLE), a very rare disease with hypoproteinemia and edema as its characteristics, is caused by various diseases resulting in protein depletion from the gut. The diagnosis is relatively difficult due to its complex pathogeneses.The present paper reported a case whose symptom started with acute diarrhea and hypoproteinemia.Gastrointestinal endoscopies showed digestive ulcersandcolonpolyp.Thetreatmentscontainedalbumin infusion, Chinese herbal decoction and other symptomatic therapies. The hypoproteinemia become even worse and edema occurred after 4 days' treatment.Alargerdoseofalbumininfusion(40-60g/d) and modified herbal decoctions were prescribed. A final diagnosis of eosinophilic gastroenteritis(EG) complicated with PLE was confirmed by histopathological examination of a repeated gastroscopy. After three weeks' treatment, the serum albumin level was raised and the edema subsided gradually. In conclusion, herbs may have an effect on PLE patients, but PLE resulting from EG is very complex and easy to misdiagnose, especially in atypical conditions. Further studies are required to find the exact mechanisms.展开更多
文摘Protein-losing enteropathy(PLE) is characterized by loss of serum proteins into the gastrointestinal tract. It may lead to hypoproteinemia and clinically present as protein deficiency edema, ascites, pleural or pericardial effusion and/or malnutrition. In most cases the site of protein loss is the small intestine. Here we present an unusual case of severe PLE in a 55-year old female with a one-year history of recurrent diarrhea, crampy abdominal pain, and peripheral edema. Endoscopy and MRI showed a diffuse inflammatory thickening of the sigmoid colon and the rectum. Surgical resection of the involved colon was performed and the symptoms were significantly resolved. The final histologic evaluation confirmed a diagnosis of a pseudomembranous colitis with cap polyposis-like features. Such a cause of PLE has never been described before.
文摘BACKGROUND The incidence of internal hernias has recently increased in concordance with the popularization of laparoscopic surgery.Of particular concern are internal hernias occurring in Petersen's space,a space that is surgically created after treatment for gastric cancer and obesity.These hernias cause devastating sequelae,such as massive intestinal necrosis,fatal Roux limb necrosis,and superior mesenteric vein thrombus.In addition,protein-losing enteropathy(PLE)is a rare syndrome involving gastrointestinal protein loss,although its relationship with internal Petersen’s hernias remains unknown.CASE SUMMARY A 75-year-old man with a history of laparotomy for early gastric cancer developed Petersen's hernia 1 year and 5 mo after surgery.He was successfully treated by reducing the incarcerated small intestine and closure of Petersen’s defect without resection of the small intestine.Approximately 3 mo after his surgery for Petersen’s hernia,he developed bilateral leg edema and hypoalbuminemia.He was diagnosed with PLE with an alpha-1 antitrypsin clearance of 733 mL/24 h.Double-balloon enteroscopy revealed extensive jejunal ulceration as the etiology,and it facilitated minimum bowel resection.Pathological analysis showed extensive jejunal ulceration and collagen hyperplasia with nonspecific inflammation of all layers without lymphangiectasia,lymphoma,or vascular abnormalities.His postoperative course was unremarkable,and his bilateral leg edema and hypoalbuminemia improved after 1 mo.There was no relapse over the 5-year follow-up period.CONCLUSION PLE and extensive jejunal ulceration may occur after Petersen's hernia.Doubleballoon enteroscopy helps identify and resect these lesions.
文摘BACKGROUND While primary intestinal lymphangiectasia(PIL)is considered a rare condition,there have been several reported cases in adults.Nevertheless,the absence of clear guidance from diagnosis to treatment and prognosis poses challenges for both physicians and patients.AIM To enhance understanding by investigating clinical presentation,diagnosis,treatment,complications,and prognoses in adult PIL cases.METHODS We enrolled adult patients diagnosed with PIL between March 2016 and September 2021.The primary outcome involved examining the diagnosis and treatment process of these patients.The secondary outcomes included identifying complications(infections,thromboembolism)and assessing prognoses(frequency of hospitalization and mortality)during the follow-up period.RESULTS Among the 12 included patients,peripheral edema(100%)and diarrhea(75%)were the main presenting complaints.Laboratory tests showed that all the pati-ents exhibited symptoms of hypoalbuminemia and hypogammaglobulinemia.Radiologically,the predominant findings were edema of the small intestine(67%)and ascites(58%).The typical endoscopic finding with a snowflake appearance was observed in 75%of patients.Among the 12 patients,two responded positive-ly to octreotide and sirolimus,and eight who could undergo maintenance therapy discontinued subsequently.Complications due to PIL led to infection in half of the patients,thromboembolism in three patients,and one death.CONCLUSION PIL can be diagnosed in adults across various age groups,with different severity and treatment responses among patients,leading to diverse complications and prognoses.Consequently,tailored treatments will be necessary.We anticipate that our findings will contribute to the management of PIL,an etiology of protein-losing enteropathy.
文摘Primary intestinal lymphangiectasia(PIL) is a rare protein-losing enteropathy with lymphatic leakage into the small intestine. Dilated lymphatics in the small intestinal wall and mesentery are observed in this disease. Laboratory tests of PIL patients revealed hypoalbuminemia, lymphocytopenia, hypogammaglobulinemia and increased stool α-1 antitrypsin clearance. Cell-mediated immunodeficiency is also present in PIL patients because of loss of lymphocytes. As a result, the patients are vulnerable to chronic viral infection and lymphoma. However, cases of PIL with chronic viral infection, such as human papilloma virus-induced warts, are rarely reported. We report a rare case of PIL with generalized warts in a 36-year-old male patient. PIL was diagnosed by capsule endoscopy and colonoscopic biopsy with histological tissue confirmation. Generalized warts were observed on the head, chest, abdomen, back, anus, and upper and lower extremities, including the hands and feet of the patient.
基金The National Natural Science Foundation of China,No.81502025China Postdoctoral Science Foundation,No.2018M632631.
文摘BACKGROUND Lymphangiomatosis is a multisystem disorder that is rarely localized to the gastrointestinal tract.Lymphangiomatosis usually has no specific clinical presentation and is easily misdiagnosed.A case report and review of the literature on lymphangiomatosis associated with protein-losing enteropathy will help to improve the overall understanding of this disease.CASE SUMMARY We report a case of lymphangiomatosis of the bowel and other solid organs.A 78-year-old man presented with recurrent bowel bleeding and protein-losing enteropathy,as well as cystic lesions in the spleen,liver,and kidney.Imaging examinations revealed many cystic lesions on the spleen,liver,kidney,and thickened wall of the ascending colon,as well as pleural effusion and ascites.Colonoscopy revealed a strawberry mucosa,variable spontaneous bleeding,and surface erosion located in the terminal ileum.Several cystic masses with a translucent and smooth surface as well as diffuse white spots were located in the colon.A laterally spreading tumor(LST)was located in the ascending colon.Pathology indicated highly differentiated adenocarcinoma(LST)and lymphangiomatoid dilation,and D2-40 was positive.The final diagnosis was lymphangiomatosis.The patient underwent surgery for LST and then was administered thalidomide 75-150 mg/d.His condition,however,did not improve.He eventually died 6 mo after the initial diagnosis.CONCLUSION Lymphangiomatosis usually occurs diffusely and can involve many organs,such as the spleen,kidney,liver,lung,mesentery,and bowel.Recurrent bowel bleeding or protein-losing enteropathy is an important indicator that should alert clinicians about the possibility of this disease when it afflicts the bowel.Doctors should improve the medical understanding of lymphangiomatosis.
文摘A 77-year-old male patient was diagnosed with protein-losing enteropathy as he had diarrhea for 4 years accompanied with repeated and exacerbated diarrhea and edema of the both lower extremities for more than 1 and a half years.In this case,warming-needle moxibustion compiled with the abdominal rubbing that patient performed by himself were utilized.Zhongwan(中脘CV12),Guanyuan(关元CV4);bilateral Tianshu(天枢ST25),Zusanli(足三里ST36),Shangjuxu(上巨虚ST37).Xiajuxu(下巨虚ST39)and the point on three cun below ST39 were taken when warming-needle moxibustion was performed.The treatment was given once a day.After 3 weeks'treatment,the frequency of diarrhea was decreased,the edema disappeared,the seralbumin level was increased,and the symptoms of the patient disappered.Follow-up for half a year,the patients had good prognosis.
文摘Whilst ascites is a common presenting complaint in patients with decompensated chronic liver disease and disseminated malignancy,in Crohn's disease however,it is exceptionally rare.We describe a patient with no prior history of inflammatory bowel or liver disease,presenting with rapid onset gross ascites and scrotal swelling.Further investigations revealed severe hypoalbuminemia and transudative ascitic fluid with normal other liver function tests and a negative liver screen.Computed tomography revealed widespread ascites and pleural effusions with no features of malignancy or portal hypertension,and a small bowel barium series showed features of fistulating small bowel Crohn's disease.An ileo-colonoscopy confirmed the presence of terminal ileal inflammatory stricture.The patient's clinical condition and serum albumin improved with a combination of diuretics,elemental diet,antibiotics and oral 5-aminosalicylic acid therapy.
文摘BACKGROUND Primary intestinal lymphangiectasia(PIL)is a rare protein-losing enteropathy characterized by abnormally dilated lymphatic structures,resulting in leakage of lymph(rich in protein,lymphocytes,and fat)from the intestinal mucosal and submucosal layers and thus hypoproteinemia,lymphopenia,hypolipidemia,and pleural effusion.CASE SUMMARY A 19-year-old Chinese male patient complained of recurrent limb convulsions for the last 1 year.Laboratory investigations revealed low levels of calcium and magnesium along with hypoproteinemia and high parathyroid hormone levels,whereas gastroscopy exhibited chronic non-atrophic gastritis and duodenal lymphatic dilatation.Subsequent gastric biopsy showed moderate chronic inflammatory cell infiltration distributed around a small mucosal patch in the descending duodenum followed by lymphatic dilatation in the mucosal lamina propria,which was later diagnosed as PIL.The following appropriate mediumchain triglycerides nutritional support significantly improved the patient’s symptoms.CONCLUSION Since several diseases mimic the clinical symptoms displayed by PIL,like limb convulsions,low calcium and magnesium,and loss of plasma proteins,it is imperative to conduct a detailed analysis to avoid any misdiagnosis while pinpointing the correct clinical diagnosis and simultaneously ruling out other clinical aspects in the reported cases without any past disease history.A careful assessment should always be made to ensure an accurate diagnosis in a timely manner so that the patient can be delivered quality health services for a positive health outcome.
基金Supported by National Natural Science Foundation of China(No.81573806,81774066)National Science and technology support program for 12th Five-Year(No.2013BAI02B05)Beijing Key Laboratory of Functional Gastrointestinal Disorders Diagnosis and Treatment of Traditional Chinese Medicine(No.BZ0374)
文摘Protein losing enteropathy(PLE), a very rare disease with hypoproteinemia and edema as its characteristics, is caused by various diseases resulting in protein depletion from the gut. The diagnosis is relatively difficult due to its complex pathogeneses.The present paper reported a case whose symptom started with acute diarrhea and hypoproteinemia.Gastrointestinal endoscopies showed digestive ulcersandcolonpolyp.Thetreatmentscontainedalbumin infusion, Chinese herbal decoction and other symptomatic therapies. The hypoproteinemia become even worse and edema occurred after 4 days' treatment.Alargerdoseofalbumininfusion(40-60g/d) and modified herbal decoctions were prescribed. A final diagnosis of eosinophilic gastroenteritis(EG) complicated with PLE was confirmed by histopathological examination of a repeated gastroscopy. After three weeks' treatment, the serum albumin level was raised and the edema subsided gradually. In conclusion, herbs may have an effect on PLE patients, but PLE resulting from EG is very complex and easy to misdiagnose, especially in atypical conditions. Further studies are required to find the exact mechanisms.
