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)

This report deals with a 26-year-old white woman exhibiting signs of both Kwashiorkor (marasmus, pallor, hypopigmentation of hair and hepatomegaly) and acrodermatitis enteropathica (eczematous dermatitis predominantly on acral areas). Clinical and laboratory examinations excluded malabsorption syndrome and glucagonoma syndrome and revealed hypoproteinemia and marked zinc deficiency. Psychiatric examination disclosed anorexia nervosa. Substitution therapy led to rapid clearing of the skin lesions.
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PMID:Kwashiorkor-like zinc deficiency syndrome in anorexia nervosa. 9 54

The pathogenesis of jejunoileal bypass-induced liver disease was investigated in the rat model. Male Sprague-Dawley rats were subjected to 90% jejunoileal bypass and compared to rats having undergone 90% jejunoileal resection, to ad libitum and pair-fed controls and to weight-matched (underfed) controls. After 8 weeks the animals were killed and selected analyses performed. Several indications of liver dysfunction were observed in the bypass rats including hepatomegaly, hypotriglyceridemia, hypoproteinemia, elevated SGOT levels, and markedly decreased levels of cytochrome P-450. All of these abnormalities with the exception of elevated SGOT levels and decreased serum proteins were not observed to the same degree in animals in which the defunctionalized bowel was resected. Rats which were underfed (weight matched) did not develop any of the abnormalities of liver injury demonstrated in the bypass rats. Multiple factors appear to be responsible for the production of bypass-induced liver disease, but the defunctionalized bowel plays an important role.
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PMID:Role of defunctionalized bowel in jejunoileal bypass-induced liver disease in rats. 51 91

Failure to thrive may not be a result of organ disease, physical abuse, or intentional neglect. We describe an infant who developed kwashiorkor with a high-fat, low-protein, nondairy coffee creamer diet. The elimination diet was administered on the advice of a family friend for a facial rash. The child presented at 10 months of age with decreased weight for height, rash, hepatomegaly, edema, hypoproteinemia, hypoalbuminemia, anemia, hypoglycemia, and evidence of hepatic sequestration of lipids. A rapid recovery of biochemical abnormalities was evident on reinstitution of a full diet. An intellectual assessment at age 5 years showed normal results.
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PMID:Nondairy-creamer-induced kwashiorkor: 5-year follow-up. 186 86

The rat was used as an animal model to explore the mechanism responsible for the development of hepatomegaly and hypoproteinemia which commonly occur after jejunoileal bypass. Sprague-Dawley rats. 300 to 350 g, were divided into three groups of 12 animals. Six of the 12 rats per group served as study animals and six as controls. The first six were subjected to 90% jejunoileal bypass and the six controls were sham-operated and pair-fed. In the second group, six animals were subjected to 90% jejunoileal resection and six controls were sham-operated and pair-fed. Six animals in the third group were underfed so that their weights mimicked that of the bypassed animals and six controls were fed ad libitum. After 8 wk the animals were killed. Liver weights, hepatic protein content, and serum protein and triglycerides were determined. Synthesis and secretion of proteins and glycoproteins were measured using incorporation of 14C-leucine and 14C-glucosamine, respectively, into hepatic and medium proteins by liver slices. Bypassed animals demonstrated hepatomegaly, decreased serum proteins and triglycerides, and increased hepatic protein content. While both protein and glycoprotein synthesis remained normal, the secretion of these proteins into the medium appeared to be impared. Comparable changes did not occur after jejunoileal resection or after underfeeding. This study suggests that the impairment of glycoprotein and protein secretion may be a contributing factor in the increased liver weight and protein content in conjunction with decreased serum protein observed in the bypassed rat.
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PMID:Hepatic protein synthesis and secretion after jejunoileal bypass in the rat. 684 85

The features of 41 proven or suspected cases of pancreatic glucagonoma and one possible case of renal glucagonoma have been reviewed. Glucagonoma is one form of islet cell neoplasm and involves pancreatic alpha cells. It may occur more frequently in women and is more likely to be malignant than insulinoma. Patients may present with glucose intolerance, an erythematous, eczematous dermatitis, glossitis, stomatitis, vaginitis and unexplained weight loss. Anemia, hypoproteinemia, hypoaminoacidemia and hypolipidemia may also be present. Malignant glucagonoma metastasizes frequently to liver. An evaluation for possible glucagonoma may be considered in a patient with the characteristic eczematous dermatitis, glossitis or stomatitis and glucose intolerance, an unusual or atypical history of diabetes mellitus, or hepatomegaly with other characteristics of glucagonoma. Initial evaluation may include measurement of fasting plasma glucagon concentration, and an oral glucose tolerance test with measurements of plasma glucose and glucagon levels. Extreme fasting hyperglucagonemia, and a paradoxical rise in plasma glucagon concentrations after glucose ingestion should strongly suggest the presence of glucagonoma. Radiographic demonstration of pancreatic glucagonoma is best carried out by celiac arteriography. Surgical excision of the tumor is the treatment of choice. Nonresectable lesions may respond to chemotherapy with streptozotocin. Treatment for the various dermatologic or metabolic complications of glucagonoma which include glucose intolerance, hypoproteinemia, hypocholesterolemia and anemia may not be satisfactory. Glucose intolerance is usually mild and may be adequately treated with dietary or insulin therapy. Rarely, glucagonoma with massive destruction of the pancreas or other factors may induce severe glucose intolerance. In contrast, the anemia, skin rash, and hypoproteinemia do not respond to conservative therapies tested thus far. Glucagonoma is a model for studying the importance of glucagon in causing the hyperglycemia of diabetes mellitus. Study of patients with glucagonoma does suggest that glucagon has some role in the etiology of hyperglycemia in diabetic states; however, as in studies on diabetes, investigations on glucagonoma do not demonstrate that glucagon has a primary role in producing severe glucose intolerance.
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PMID:Clinical and metabolic aspects of glucagonoma. 698 81

Twenty children with constrictive pericarditis are reported on. Tuberculous etiology was established in five, could not be excluded in 7, while the etiology could not be identified in 8 cases. The main symptoms were venous congestion involving the pulmonary and systemic circulation (hepatomegaly, pulmonary congestion), pericardial click at the apex, abnormalities of repolarization in the ECG. X-ray evidence of pericardial calcification and a "quiet heart" were occasionally found. Hypoproteinaemia, hypalbuminaemia and ascites, signs of an associated protein losing enteropathy may occur in constrictive pericarditis, but are not invariably present. Pericardiectomy is the most adequate form of treatment. Sixteen of the 17 cases in whom the operation was performed recovered completely; one child died.
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PMID:Chronic constrictive pericarditis in children - etiology, clinical picture and treatment. A report of 20 cases. 723 57

Hepatic disease was diagnosed in 11 mynah birds. The most common clinical signs were dyspnea, weight loss, and abdominal swelling, usually accompanied by ascites. Radiography (9 birds) revealed hepatomegaly and ascites in most cases, and laboratory testing (6 birds) revealed hypoproteinemia and high activity of liver enzymes in all cases. Histologic examination (6 birds) revealed variable amounts of iron granule accumulation in hepatocytes, indicating hemochromatosis. Therapy consisted of administration of diuretics and abdominocentesis in dyspneic birds. All birds died, with survival time ranging from 1 day to 1 year.
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PMID:Hepatopathy associated with excessive iron storage in mynah birds. 732 6

A 7-year-old boy was admitted for RVOT restenosis. The patient was born following an unremarkable pregnancy and delivery. He was carried out Jatene's procedure for TGA at 23rd day after birth. At 19 month of age, he was removed pulmonary stenosis using patch angioplasty with mono cusp. When he was 6 year of age, the onset of edem, ascites and hepatomegaly were pointed out. At 7 year of age, he was admitted for edema, hypoproteinemia and protein loosing enteropatchy. After he was treated medical therapy, cardiac catheterization showed markedly right atrial and right ventricular hypertension and ventricular septal defect (VSD). Cine angiography suggested the right ventricular outflow tract stenosis, pulmonary valve and tricuspid valve insufficiency. On August 1, 1996, the patient underwent surgical repair: direct closure of VSD, aortic homograft (20mm) replacement for RVOT stenosis and tricuspid valve annuloplasty by DeVega's procedure. Postoperative course was uneventful. At 23rd postoperative day, cardiac catheterization and cine angiography showed no problems. He was not treated with anticoagulation.
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PMID:[A case report: right ventricular outflow tract (RVOT) reconstruction using homograft for RVOT restenosis after Jatene's procedure of transposition of the great arteries (TGA)]. 952 29

A major outbreak of dengue hemorrhagic fever (DHF) affected more than 10,000 people in Delhi and neighboring areas in 1996. The outbreak started in September, peaked in October to November and lasted till early December. The clinical and laboratory data of 515 adult patients admitted to Lok Nayak Hospital, New Delhi were reviewed. Fever (100%), myalgias and malaise (96%), abdominal pain (10.2%) and vomiting (8.7%) were the prominent presenting features. Hemorrhagic manifestations were seen in all patients- a positive tourniquet test (21.2%), scattered petechial rash (23.07%), confluent rash (2.7%), epistaxis (38.4%), gum bleeds (28.06%) and hematemesis (22.86%) being the major bleeding manifestations. Hepatomegaly was observed in 96% of the patients. Laboratory investigations revealed thrombocytopenia, hemoconcentration and leukopenia. Serological confirmation with a microcapture ELISA technic was done in 143/515 patients. The mortality rate was 6.6% and, multiple bleeding manifestations, severe thrombocytopenia, hypoproteinemia and dengue shock syndrome (DSS) were associated with a higher mortality.
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PMID:The 1996 outbreak of dengue hemorrhagic fever in Delhi, India. 1043 46

Cystic fibrosis is a genetic disorder characterized by chronic obstructive pulmonary disease, pancreatic exocrine deficiency, and abnormally high sweat electrolyte concentrations. Less frequently, the presenting features in infants may include edema, anemia, hypoproteinemia, and acrodermatitis enteropathica. Liver involvement may produce hepatomegaly and mild elevation of transaminases. This clinical symptom usually presents within the first 6 months of life and is associated with a high morbidity and mortality. Early recognition and institution of appropriate nutritional supplementation and pancreatic enzymes is essential to improve outcome. Since the sweat test may be falsely negative, emergency physicians must maintain a high index of suspicion to make the diagnosis of cystic fibrosis in an infant who presents with edema, anemia, hypoproteinemia, and acrodermatitis enteropathica.
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PMID:Edema, anemia, hypoproteinemia, and acrodermatitis enteropathica: an uncommon initial presentation of cystic fibrosis. 1475 9


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