Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0020639 (hypoproteinemia)
 1,134 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Of 300 congenital malformations of the lymphatics of the small intestine investigated, 120 were operated upon. Intestinal lymphography shows no injection of the cisterna chyli and histology proves that the mesenteric lymph nodes are abnormal. The induced hyperlipidemia test permits a biochemical diagnosis. Modifications of the flow of the chyle secondary to the hypoplasia of the cisterna chyli were studied: (1) in the abdominal cavity, (2) in the extraperitoneal region and the lower limb, (3) in the thorax, especially the chyle drainage channels from the diaphragm towards the cervical region. Our investigations have established that the following diseases are produced by malformation of the lymphatics of the small intestine: protein losing enteropathy, chyloperitoneum, chyluria, lymphedema with chyle reflux, chylothorax, chylopericardium, chyle reflux in the pulmonary lymphatics, hypoproteinemia and food allergies. A better understanding of the pathophysiology of the malformations of the intestinal lymphatics permits a more rational treatment of the diseases produced by this anomaly.
J Cardiovasc Surg (Torino)
PMID:Congenital malformation of the lymphatics of the small intestine. 201 16

Protein-losing enteropathy (PLE), excessive serum protein loss within the gastrointestinal tract, after Fontan operation is a poorly understood disorder. Reported to occur anywhere from weeks to years after Fontan operation, there are no identifiable risk factors for its development, and its clinical manifestations vary widely from significant morbidity and mortality to mild-to-moderate hypoproteinemia with minimal functional impairment. Treatment strategies, tailored to the severity of the disease, include symptomatic relief with diuretics and supplemental protein, attempts at halting intestinal protein leak using steroids or heparin, and alteration of cardiovascular physiology via fenestration creation, atrial pacing, or heart transplantation. A better understanding of the pathophysiology of PLE will allow the development of more effective treatment modalities. We hypothesize an abnormality of local intestinal circulation in patients with PLE that may be related to low cardiac output. Compensatory flow redistribution takes place under conditions of low cardiac output. We studied superior mesenteric artery flow using Doppler ultrasound in 40 patients after Fontan operation, 13 of whom had clinical signs of PLE, and compared them with 25 normal control patients. Diastolic velocities were lower in Fontan subjects, and the ratio of systolic-to-diastolic velocities and the resistance index were higher in Fontan patients compared with the control group. Patients with PLE after Fontan operation had higher systolic-to-diastolic velocities and resistance index than patients with Fontan and no active PLE. However, subjects with Fontan circulation but without PLE had higher indices of mesenteric resistance than the normal controls, suggesting an abnormality of the mesenteric circulation even in those without overt signs of PLE. It is plausible to postulate that activation of the renin-angiotensin system with increased levels of circulating angiotensin II may be responsible for the increase in mesenteric vascular resistance seen after Fontan operation, thereby placing these patients at risk for development of PLE.
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2002
PMID:Strategies to treat protein-losing enteropathy. 1199 60