Intraabdominal lymphangiomyoma in an infant with protein-losing enteropathy and hemihypertrophy

Lymphangiomyoma is an extremely rare tumor occurring exclusively in women of reproductive age. The tumor is characterized by proliferation of immature smooth muscle along the lymphatic vessels of the abdomen, thorax and lung. Although lymphangiomyoma has been reported in a young girl and a girl infant, none has been reported in boys. We report herein a case of lymphangiomyoma in a two-year-old boy. The unusual presentation in this patient was that the tumor arose from the small bowel mesentery without any evidence of lung involvement. The tumor was extirpated and lymphangiomyomatosis was confirmed pathologically.     

儿童蛋白丢失性肠病

蛋白丢失性肠病或称蛋白丢失性胃肠病是指各种原因所致的血清蛋白质从胃肠道过量丢失的一组疾病。发病机制主要包括黏膜糜烂渗出、通透性增加及肠淋巴管阻塞。临床表现随基础疾病不同而有很大差异，常见的有慢性腹泻、水肿、营养不良等。实验室检查以血清白蛋白、球蛋白均减低为主要特征。24 h α1抗胰蛋白酶清除率升高或淋巴核素肠蛋白示踪显像可作为肠道蛋白丢失的依据。治疗包括膳食疗法及针对基础疾病的治疗。     

Protein-losing enteropathy in prolonged post-ischemic ileitis

A 10-year-old boy developed internal herniation of the small intestine associated with significant ischemia, which was reduced without resection. Severe diarrhea, hypoalbuminemia, and lymphopenia ensued while he was receiving total parenteral nutrition. Resolution of symptoms occurred only after resection of an area of ileum, which revealed persistent inflammation and atrophy. Postischemic epithelial cell regeneration of the intestine is discussed in light of this patient's protein-losing enteropathy.     

Protein-losing enteropathy during chronic liquid pericarditis]

Severe hypo-albuminemia associated with a decrease in P.B.I., gamma-globulins and plasmatic ceruloplasmin occurred in a 2 1/2 year old girl with chronic pericarditis since the age of 8 months. This was related to a protein-losing enteropathy which was confirmed by histological examination. Venous hypertension, secondary to the chronic pericarditis may explain the enteropathy.     

Protein-losing enteropathy following Fontan operation for a complex heart defect

A 12 year old boy developed protein losing enteropathy 6 months after surgery for a severe cardiac malformation complex by a modified Fontan procedure. Gastrointestinal protein loss was due to sustained venous pressure elevation. Conservative therapy with diet, diuretics and albumininfusions remained ineffective. The patient died from right atrial and pulmonary thrombembolism 10 months after surgery.     

Protein-losing gastroenteropathy and retinitis associated with cytomegalovirus infection in an immunocompetent infant: a case report

A 6-week-old immunocompetent girl developed protein-losing gastroenteropathy (PLGE) and retinitis associated with cytomegalovirus (CMV) infection. At presentation, CMV antigenaemia (6 cells/46,000 white blood cells) and its DNA were detected in the patients blood and in the mothers milk. Intravenous ganciclovir and -globulin rapidly ameliorated all symptoms and CMV antigenaemia disappeared. No immunological defects were identified in this patient. To the best of our knowledge, this case involves the youngest known immunocompetent patient demonstrating CMV-induced PLGE and retinitis. Conclusion:breast-feeding by a cytomegalovirus-positive mother can be a primary cause of early onset cytomegalovirus infection in infants.Abbreviations CMV cytomegalovirus - PLGE protein-losing gastroenteropathy     

Protein-losing enteropathy detected by Tc-99m-MDP abdominal scintigraphy

This case report describes the incidental finding of intestinal leakage during bone scan imaging with Tc-99m methylene diphosphonate (MDP) in a patient with previously diagnosed protein-losing enteropathy (PLE). Most radiopharmaceuticals reported in the literature that are useful for making this diagnosis are not generally available for clinical use in the United States. This report suggests that Tc-99m-MDP is useful for confirming the diagnosis of PLE although further investigations are indicated to determine the accuracy of this method.     

Protein-losing enteropathy detected by 99mTc-labeled human serum albumin abdominal scintigraphy

99mTc-labeled human serum albumin (HSA) abdominal imaging is a new way of demonstrating gastrointestinal protein loss. We present two children with hypoalbuminemia of obscure etiology in whom albumin loss was localized in the gastrointestinal tract with 99mTc-HSA scintigraphy and the loss correlated with abnormal fecal alpha 1-antitrypsin excretion. In the absence of gastrointestinal blood loss or contamination of the 99mTc-HSA with free pertechnetate, significant activity accumulating in the gastrointestinal tract and moving with its contents is thought to represent 99mTc-HSA leakage into bowel lumen. Abdominal imaging with 99mTc-HSA has a low radiation burden to the patient and is readily available, relatively inexpensive, and easily performed. It can be used as a screening test for the detection of protein-losing enteropathy. It also offers the potential of being able to localize the site of protein loss visually within the gastrointestinal tract.     

儿童蛋白丢失性胃病1例报告

No abstract available     

A case of protein-losing enteropathy caused by intestinal lymphangiectasia in a preterm infant

Intestinal lymphangiectasia is characterized by obstruction of lymph drainage from the small intestine and lacteal dilation that distorts the villus architecture. Lymphatic vessel obstruction and elevated intestinal lymphatic pressure in turn cause lymphatic leakage into the intestinal lumen, thus resulting in malabsorption and protein-losing enteropathy. Intestinal lymphangiectasia can be congenital or secondary to a disease that blocks intestinal lymph drainage. We describe the first case of intestinal lymphangiectasia in a premature infant. The infant presented with peripheral edema and low serum albumin; high fecal concentration of alpha(1)-antitrypsin documented intestinal protein loss. Endoscopy showed white opaque spots on the duodenal mucosa, which indicates dilated lacteal vessels. Histology confirmed dilated lacteals and also showed villus blunting. A formula containing a high concentration of medium chain triglycerides resulted in a rapid clinical improvement and normalization of biochemical variables. These features should alert neonatologists to the possibility of intestinal lymphangiectasia in newborns with hypoalbuminemia and peripheral edema. The intestinal tract should be examined for enteric protein losses if other causes (ie, malnutrition and protein loss from other sites) are excluded. The diagnosis rests on jejunal biopsy demonstrating dilated lymphatic lacteal vessels.     

Chronic diarrhea, ascites, and protein-losing enteropathy in an infant with hepatic venous outflow obstruction after liver transplantation

Hourigan SK, Anders RA, Mitchell SE, Schwarz KB, Lau H, Karnsakul W. Chronic diarrhea, ascites, and protein‐losing enteropathy in an infant with hepatic venous outflow obstruction after liver transplantation. Abstract: An 18‐month‐old female status post‐orthotopic liver transplant for biliary atresia presented nine months after transplant with severe diarrhea and intolerance of feeds. She was found to have a PLE as evidenced by a low serum albumin and a persistent elevation of fecal A1AT. Investigation eventually revealed that the cause of the PLE was a stricture at the anastomosis site between the hepatic vein and inferior cava, supported by resolution of the PLE after venoplasty of the stricture. The patient has subsequently required several repeat venoplasties for recurrence of her symptoms correlating with recurrence of the stricture. This is a very rare presentation of hepatic venous outflow obstruction. Moreover, normal duplex ultrasound imaging of liver vasculature and her unusual presentation led to a delay in her diagnosis highlighting the need for an increased index of suspicion.