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

A 64-year-old man presented with leg edema and hypoproteinemia. His alpha-1 antitrypsin clearance rate of 174 mL/day indicated the presence of protein-losing enteropathy (PLE). Computed tomographic scans demonstrated thickened ileal wall and mesenteric edema. Angiography revealed occlusion in a peripheral branch of the superior mesenteric vein. Furthermore, both the patient and his son had low protein C levels. The patient was treated successfully with partial resection of the ileum. Histologic examination of the resected ileum revealed multiple erosions and submucosal fibrosis with organized venous thrombi in the mesenteric veins. This is the first case report of PLE caused by mesenteric venous thrombosis, and our findings suggest that serum protein was lost through erosion of the ileum caused by ischemia due to mesenteric venous thrombosis.
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PMID:Protein-losing enteropathy caused by mesenteric venous thrombosis with protein C deficiency. 1063 22

Although hypotension is a potential complication for all procedures involving extracorporeal circulation, including plasmapheresis, the effects of serial double-filtration plasmapheresis (DFP) on hemodynamic status have rarely been reported. Blood pressure (BP) and pulse rate (PR) were prospectively monitored at 30-minute intervals (baseline, M30, M60, M90, and Ml20) during procedures for 20 myasthenia gravis patients who underwent one course of five consecutive DFP treatments on alternate days, with hemodynamic parameters recorded and analyzed for all sessions. To evaluate the hemodynamic influence of protein loss resulting from serial DFP treatment, additional analysis of serum protein levels including albumin and globulin was conducted before and after the entire course of treatment. Longitudinal analysis on the systolic BP (SBP) changes over five sessions revealed that the SBP at baseline and at M30 dropped significantly during the third and fourth sessions, in comparison to the first (P < 0.05). By contrast, SBP at M120 rose significantly (P < 0.05) after the second session of treatment. A similar trend was revealed for the diastolic BP (DBP) with a significant fall recorded at baseline and at M30 for the fourth session. The PR did not differ significantly during consecutive DFP treatments. Globulin removal rates were correlated significantly with falls in SBP (r(2) = 0.250, P = 0.048) and DBP (r(2) = 0.405, P = 0.008). However, analogous albumin removal rate was not correlated with these hemodynamic parameters. In conclusion, our results confirm that hypoproteinemia is an important factor for contributing to unstable hemodynamics during serial DFP.
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PMID:Hemodynamic study of serial double-filtration plasmapheresis. 1194 4

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.
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PMID:Strategies to treat protein-losing enteropathy. 1199 60

As a complication of atopic dermatitis (AD), the incidence of hypoproteinemia is increasing among infants with severe AD in Japan. It can be a life-threatening condition owing to hypovolemic shock as a result of hypoproteinemia and vascular infarction as a result of thrombocythemia. However, the pathophysiology of this condition remains unclear. The objectives of the present study were two-fold. The first objective was to determine the main route of protein loss, i.e. through the damaged skin or the gastrointestinal tract, or as a result of insufficient food intake. The second objective was to identify whether allergy or infection was the cause of severe skin inflammation. Fifteen patients with AD were enrolled who had serum protein levels of 3.2-5.8 g/dl. Specific immunoglobulin E (IgE) and skin test to allergens, stool eosinophils, alpha1-antitrypsin clearance, skin Staphylococcus aureus colonization and superantigens (SAgs) produced by the organism, serum SAg-specific IgE antibodies, serum interleukin (IL)-5, IL-6, IL-12, and interferon-gamma (IFN-gamma) were evaluated. Prominent serous skin discharge was seen in all of the patients and was found to have almost the same protein concentration as serum. Marked thrombocytosis, with a maximum of 1,060 x 103/ml, was seen. Skin culture revealed S. aureus colonization in all patients. SAg-producing S. aureus were found in 84.6% of the patients. The concentration of serum IL-5 was significantly increased and correlated well with the blood eosinophil count. Hence, the main route of protein loss was believed to be through damaged skin. The cause of severe inflammation was thought to be a combination of allergic inflammation and skin colonization by SAg-producing S. aureus. Serum cytokines showed a T helper 2 (Th2) T-cell-mediated pattern. To prevent hypovolemic shock, vascular occlusion, and growth retardation, it is of vital importance to diagnose hypoproteinemia at an early stage and start appropriate therapy.
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PMID:Hypoproteinemia in severe childhood atopic dermatitis: a serious complication. 1239 Apr 45

Plasmanate(R), a human-serum protein solution, appears to have all the attributes of an ideal plasma expander. Freedom from infection, immediate availability in a clear, stable solution and the apparent absence of antigenic properties are particularly valuable qualities. The efficacy and safety of Plasmanate was clinically demonstrated in the treatment of 125 infants and children. This solution seems especially effective in the treatment of acute shock states and for the physiologic correction of hypoproteinemia. Comparison with other plasma expanders makes Plasmanate the agent of choice in the initial treatment of shock states in pediatrics.
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PMID:Plasmanate: a new plasma substitute for pediatric therapy. 1358 42

Two patients with choledochal cyst who needed an emergent biliary drainage are presented. Case 1: Spontaneous rupture of the cyst was demonstrated by hepatobiliary scintigram in a 21-month-old girl and an emergent T-tube drainage relieved her symptom. Acute obstruction by protein plugs was considered to have caused rapid expansion of the cyst, which resulted in rupture eventually. Case 2: Marked hypoproteinemia was demonstrated in a 29-month-old boy with choledochal cyst. Bile drainage through Foley catheter promptly made the serum protein level elevated above normal range. Portal hypertension due to severely dilated cyst may have increased ascites amount, which caused extracellular fluid shift and protein to be lost into ascites. In both cases the excision of the extrahepatic bile duct and hepaticojejunostomy was carried out successfully later. Treatment of 20 pediatric cases in our institute and the literature were reviewed. Bile drainage would be safer in emergency condition even though it has been suggested that reconstructive surgery may be tolerable. Definitive surgery should be regarded as a procedure with some risk of postoperative complications because asymptomatic cases operated electively had serious ones. Treatment strategy of patients with choledochal cyst is not straightforward and should be arranged based upon their conditions.
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PMID:Emergent biliary drainage for choledochal cyst. 1595 91

Protein-losing enteropathy (PLE) with hypoproteinemia is an uncommon but serious complication of congestive heart failure. Reports of patients with PLE resulting from severe tricuspid regurgitation (TR) caused by trauma are rare. A 66-year-old male diabetic patient had a chest contusion as a result of a road traffic accident, and one year later suffered from progressively generalized edema. Examination revealed severe TR with a high central venous pressure and PLE with serum protein deficiency. Treatment with albumin administration and diuretic therapy proved ineffective, and consequently the TR was corrected by valve replacement. Postoperatively, the serum protein level gradually returned to normal. Surgical intervention successfully improved this patient with severe TR and PLE resistant to medical treatment.
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PMID:Traumatic tricuspid regurgitation resulting in protein-losing enteropathy: a case report. 1635 69

In this study, 73 cows with different types of traumatic reticuloperitonitis (TRP) and sequellae along with 25 healthy buffalo cows were used to evaluate the total blood serum protein and protein electrophoresis. According to rumenotomy or necropsy findings, the affected buffalo cows were classified into acute local TRP (AL-TRP), chronic local TRP (CL-TRP), reticular abscess (RA), acute diffuse TRP (AD-TRP), purulent pericarditis (PPC) and fibrinous pericarditis (FPC). Polyclonal gammopathy was characteristic for the electrophoretogram of buffalo cows with CL-TRP (55%), RA (61.1%) and PPC (62.5%). Various degrees of hypoalbuminaemia, hyper-alpha-globulinaemia and low A/G ratios were the associated changes in all cases of TRP and sequellae. Hyper-beta-globulinaemia was noticed in cases with CL-TRP, RA and PPC. Hyper-gamma-globulinaemia was evident in cases with AL-TRP, CL-TRP, RA and PPC. Hyperproteinaemia was noticed in buffalo cows with local complications of TRP including AL-TRP, CL-TRP and RA. Hypoproteinaemia associated with severe hypoalbuminaemia (>50% reduction compared to controls) and very low A/G ratios (<0.4) characterized buffalo cows with AD-TRP, PPC or FPC. In conclusion, the concentrations and electrophoretic patterns of serum proteins in buffaloes differ according to the anatomical location of the foreign body and the associated pathological lesions.
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PMID:Comparison of blood serum proteins in water buffaloes with traumatic reticuloperitonitis and sequellae. 1815 61

We present a case of pemphigus vulgaris (PV) accompanied with protein-losing gastroenteropathy (PLE). A 9-year-old girl developed multiple oral ulcerations and erosions. She was first treated with oral antibiotics and a topical steroid without improvement. Laboratory data showed eosinophilia (absolute eosinophil count 1.08 x 10(9)/L) and hypoproteinemia (total serum protein 3.9 g/dL, albumin 2.2 g/dL). A biopsy specimen from the ileum showed intense eosinophil infiltration and albumin scintigraphy demonstrated protein exduation from the same site. Endoscopic examination of the oesophagus showed multiple ulcerations and erosions, and biopsy specimen showed eosinophilic spongiosis and immunohistologic staining demonstrated deposits of IgG and C3 in the intercellular space. Antidesmoglein-3 antibody elevated, she was diagnosed as PV complicated with PLE. Immunofluorescence study of a biopsy specimen from the terminal ileum showed no significant immunoglobulin or complement deposition, and autoantibody against intestinal mucosa was unclear in this case. Gastrointestinal evaluations should be considered in patients with hypoproteinemia associated with PV.
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PMID:Pemphigus vulgaris as a possible cause of protein-losing gastroenteropathy: a case report. 1830 20

A case of large ameloblastoma of the mandible with severe hypoproteinemia is reported. The patient, a 53-year-old man, had a large swelling (14 x 11 x 10 cm) from the right cheek to the submandible causing severe hypoproteinemia. The tumor was removed when the serum protein level had recovered to 5.3g/dl following administration of 50 ml of 20% albumin for 4 days. After the operation, his hypoproteinemia, which was thought to be caused by leakage of plasma through the oral fistula of the ameloblastoma, rapidly improved. This article describes preoperative and postoperative findings and the clinical course of the patient.
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PMID:Large ameloblastoma of the mandible with hypoproteinemia. 1855 69


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