Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0033687 (proteinuria)
 24,015 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Living related donor (LRD) nephrectomies are controversial due to the risks to the donor and improved cadaveric graft survival using cyclosporine A. Between December 22, 1970, and December 31, 1984, 1096 renal transplants were performed at a single institution, 314 (28.6%) from LRD. The average age was 34.3 years (range: 18-67); none had preoperative hypertension. All nephrectomies were performed transabdominally. Major perioperative complications occurred in 22 (7.0%). These include wound infections (3.5%), pancreatitis (1.0%), injuries to spleen (1.0%) or adrenal gland (0.3%) requiring removal, pneumonitis (0.6%), ulnar nerve palsy (0.6%), femoral artery thrombosis after arteriogram (0.3%), pulmonary embolus (0.3%), and upper pole infarct of contralateral kidney (0.3%). There are six known deaths in this series, none of which were related to the operation. Major late complications were seen in 50 (20.0%) of 250 patients followed for 6 to 175 months (mean 53.1 months). These included definite hypertension (5.6%), suture granuloma (4.4%), incisional hernia (3.6%), proteinuria (2.4%), bowel obstruction (2.0%), nephrolithiasis (1.2%), wound infection (0.4%), scrotal hydrocele (0.4%), and chronic pancreatitis (0.4%). While the risk of hypertension appears to increase as the interval from donation increases, no cases of renal failure after donation have been noted, and negligible proteinuria among those followed long-term has been seen in this series. It is felt that living related kidney donation is justified when the relative is sincerely motivated and well informed prior to donation.
 ...
PMID:Living related kidney donors. A 14-year experience. 352 9

Extramedullary plasmacytoma (EMP) of the small bowel is a rare entity previously reported as a cause of intestinal obstruction or bleeding. A case report of this disease entity presenting as an ileocolic fistula is reported. EMP is diagnosed by the following criteria: 1) absence of paraproteinemia; 2) absence of Bence Jones proteinuria; 3) normal skeletal survey; and 4) normal bone marrow biopsy specimen. Gastrointestinal plasmacytoma often occurs as a manifestation of multiple myeloma. EMP of the gastrointestinal tract is a rare manifestation of the disease, accounting for 13 per cent of all cases of EMP. It is a slow-spreading, radiosensitive tumor with a high tendency toward local recurrence. Surgical excision combined with radiotherapy is the treatment of choice for EMP of the gastrointestinal tract.
 ...
PMID:Extramedullary plasmacytoma of the small intestine: first case report of ileocolic fistula and review of the literature. 731 32

A 46-yr-old multiparous cachetic woman presented with severe hypoalbuminemia in the absence of liver disease, proteinuria, and/or protracted starvation. The clinical presentation and work-up was indicative of protein-losing enteropathy. She developed an acute partial small bowel obstruction, and a presumptive diagnosis of lymphoma of the small intestine was entertained. Surgical resection of the terminal ileum revealed transmural involvement of the bowel by endometriosis. Her postoperative recovery was uneventful, with return of her serum albumin levels to normal.
 ...
PMID:Endometriosis of the small intestine presenting as a protein-losing enteropathy. 842 Feb 54

Chronic idiopathic intestinal pseudo-obstruction (CIIP) is a rare heterogeneous clinical syndrome characterized by recurrent episodes of symptoms and signs of intestinal obstruction in the absence of a mechanically obstructing lesion. Dilatation of other viscera, such as the renal pelvis, ureter or urinary bladder, is identified in a minority of patients. We report the cases of two patients with CIIP presenting with abdominal fullness and constipation. Radiologic examination of the first patient revealed dilatation of the esophagus, stomach, duodenum and bowel loops up to the ascending colon. The nerve conduction velocity study of the right extremities revealed polyneuropathy and urinary bladder manometry revealed poor sensation. The patient had been admitted to our hospital three times for symptomatic relief within the prior six months. During the last admission, his symptoms persisted without response to medical treatment. Soon after discharge, the patient underwent surgery at another hospital and died of nutritional problems. The second patient was transferred to our hospital after an exploratory laparotomy was performed one month earlier. A radiographic examination revealed distention of the stomach, duodenum, small intestine and ascending colon, as well as bilateral hydronephrosis. Rheumatologic examination revealed no evidence of autoimmune disorder. The patient also had heavy proteinuria due to minimal change disease that was proven by renal biopsy. After receiving prokinetic, cathartic and corticosteroid medication for kidney disease, symptoms improved, but hydronephrosis persisted.
 ...
PMID:Chronic idiopathic intestinal pseudo-obstruction. 1074 24

Amyloidosis is a pathological process which encompasses a spectrum of diseases that result from extracellular deposition of pathological fibrillar proteins. Clinical presentations vary depending on the organs involved. There is no documented case of amyloidosis presenting as small bowel encapsulation. A previously healthy 62-year-old man developed a small bowel obstruction in 1997. At surgery, a peculiar membrane encasing his entire small bowel was discovered. This appeared to have no vascularity and was removed without difficulty, exposing a grossly normal bowel. Histopathology revealed thick bands of collagen overlying the peritoneal surface, which was congo red positive and showed apple green birefringence. The findings were consistent with encapsulating peritonitis due to amyloidosis. There was no history or symptoms of any chronic inflammatory condition and he became symptom-free postoperatively. An abdominal fat pad biopsy failed to demonstrate amyloidosis. Endoscopic duodenal biopsies revealed classical primary amyloidosis. Quantitative immunoglobulins, lactate dehydrogenase, C3, C4 and beta-2 microglobulin were normal. Protein electrophoresis identified monoclonal paraprotein, immunoglobulin G lambda 3.7 g/L. Bone marrow biopsy and aspirate revealed only a mild plasmacytosis (5% to 10%). Echocardiogram and skeletal survey were normal. He had mild proteinuria. Complete blood count, C-reactive protein, calcium, albumin and total protein were normal. No specific therapy was instituted. In January of 1998 the patient remained asymptomatic with no gastrointestinal, cardiovascular or constitutional symptoms. He had developed nephrotic range proteinuria (3.95 g/24 h), microalbuminuria, hypoalbuminemia and a renal biopsy consistent with renal amyloidosis. In 1999 there was an increase in the monoclonal paraprotein (6.2 g/L). The remaining investigations were normal except for an echocardiogram which showed left ventricular hypertrophy but a normal ejection fraction and no diastolic dysfunction. He went on to have high-dose chemotherapy and an autologous stem cell transplant in September, 2000. He has subsequently developed renal insufficiency. To our knowledge this is the first reported case of primary amyloidosis presenting as small bowel obstruction from encapsulating peritonitis.
 ...
PMID:Primary amyloidosis presenting as small bowel encapsulation. 1505 90

The Rapunzel syndrome is a rare complication of gastric trichobezoar, which may be long insidious. Systemic hair eating gradually leads to bezoar growth, gastritis, gastric mucosal ulcerations, and evacuatory disorders. The Rapunzel syndrome may cause acute and chronic bowel obstruction, peritonitis, pancreatitis, appendicitis, anemia, hypoalbuminemia, and allergic manifestations. Neither proteinuria nor nephrotic syndrome is depicted in any of the 38 Rapunzel syndrome cases described in the literature. The authors present the first case of gastric trichobezoar extending to the small bowel (its total length was 118 cm), which gave rise to chronic recurrent partial bowel obstruction, causing intoxication nephrotic syndrome in a 20-year-old women with trichotillomania. The nephrotic syndrome became a reason for her admission to a nephrology department and had specific features: it was unaccompanied by hypercholesterolemia, it rapidly regressed and completely disappeared after surgical removal of the trichobezoar weighing 1980 g.
 ...
PMID:[Nephrotic syndrome in a female patient with Rapunzel syndrome]. 2580 45