Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0021843 (bowel obstruction)
 9,927 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Rapunzel Syndrome is a rare trichobezoar, involving strands of swallowed hair extending as a tail through the duodenum, beyond the stomach. Trichobezoar usually occur in patients with history of trichotillomania, characterized by compulsive behavioral disorder of pulling own hairs, combined with trichophagia that consists of ingesting that hairs. It typically occurs in stomach and rarely affects the small intestine. Trichobezoars are more common in adolescent females. Common presentation is gastrointestinal tract obstruction with nausea and vomiting, gut perforation, acute pancreatic necrosis, obstructive jaundice, hypochromic anemia, vitamin B12 deficiency, weight loss, an abdominal mass, or other serious problems. Intestinal obstruction due to trichobezoar is extremely rare. We are here reporting a large trichobezoar in a 3-year-old male child who presented to the emergency department with thread protruding from mouth with no sign of hair loss on body.
 ...
PMID:An unusual case report of rapunzel syndrome trichobezoar in a 3-year-old boy. 2222 71

Protein-losing enteropathy (PLE) is a rare condition characterized by protein loss through the gastrointestinal tract, leading to hypo-proteinemia. Patients may be asymptomatic or present with variety of complications of hypoproteinemia (e.g., oedema, ascites, pleural, and cardial effusions). We describe a case report of a young girl suffering from behavioral disorder since childhood who presented with generalized oedema, hypoproteinaemia, and microcytic hypochromic anemia. In addition, the girl had an intervention for jejunal atresia and intestinal malrotation in her past medical history. Upper gastrointestinal endoscopy revealed a trichobezoar extending from stomach into the small bowel, thus classified as Rapunzel Syndrome (RS), causing mechanical obstruction of intestinal lumen and intestinal lymphatic drainage resulting in a protein-losing enteropathy (PLE). Trichobezoar was successfully removed by a surgical laparotomy resulting in resolution of symptoms and normalization of biochemical parameters. Possibly, previous surgery might have had an influence on intestinal dysmotility and trichobezoar formation. PLE is a very rare presenting symptom of RS, developing as result of intestinal obstruction caused by large trichobezoars. RS has to be considered in patients, especially adolescents, suffering from behavior disorder as trichotillomania and trichophagia. Surgical removal and nutritional supplementation are the gold treatment of large trichobezoar.
 ...
PMID:An Unusual Case of Hypoproteinemia in Childhood: Keep in Mind Trichobezoar. 3219 12