Cases reported "Hypoproteinemia"

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11/66. Protein-losing gastropathy associated with autoimmune disease: successful treatment with prednisolone.

    We report a patient with protein-losing gastropathy probably associated with autoimmune disease, in whom prednisolone treatment was highly effective. A 45-year-old woman was admitted to our hospital with edema of the face and lower extremities. blood examination revealed hypoproteinemia, hypoalbuminemia, and hypercholesteremia. Renal biopsy revealed no definite findings of lupus nephritis, including vasculitis. A diagnosis of protein-losing gastropathy was made on the basis of increased alpha1-antitrypsin clearance and 99mTc-labeled human serum albumin scintigram showing abnormal radioactivity in the stomach. Endoscopic gastric biopsies revealed nonspecific inflammation, but marked intramural edema. Based on a slight elevation of antinuclear antibody level, autoimmune disease was suspected to be involved in this patient. Administration of prednisolone, as a diagnostic therapy, alleviated the hypoproteinemia, hypoalbuminemia, and hypercholesteremia. These findings suggest that an autoimmune mechanism could have been involved in this case of protein-losing gastropathy. ( info)

12/66. Two autopsy cases of diffuse gastrointestinal polyposis with ectodermal changes. Cronkhite-canada syndrome.

    Two autopsy cases of Cronkhite-canada syndrome were reported. The caused of hypoproteinemia, electrolyte imbalance and ectodermal changes were discussed with reference to previously reported cases. The mechanism of protein loss was probably due to outflow into the intestinal lumen of the mucous substance in the cystically dilated glands, directly and/or indirectly followed by loss of mucosal surface. Electrolyte imbalance probably developed from gastrointestinal loss as well as poor substitution. The ectodermal changes were probably not a subsequent part of the emaciation or hypoproteinemis, but an inherent part of this disease. Therapy, whether substitution or surgical procedure, should be selected in order to control the general condition of the patient. ( info)

13/66. Giant hypertrophic gastritis and acute hepatitis associated with cytomegalovirus infection.

    A 38-year-old man developed prominent hypoproteinemia after acute elevation of serum transaminase levels. Giant hypertrophy of the gastric mucosa, a short serum albumin half-life, and the absence of massive hepatocyte necrosis established the diagnosis of protein-losing gastropathy. The hypoproteinemia, gastric fold hypertrophy and hepatitis remitted spontaneously within 4 months. A high antibody titer against cytomegalovirus suggested an association between the viral infection and the patient's disease. ( info)

14/66. Studies of tryptophan and albumin metabolism in a patient with carcinoid syndrome, pellagra, and hypoproteinemia.

    Detailed studies of protein metabolism were undertaken in a patient with pellagra and hypoproteinemia associated with the carcinoid syndrome both before and after treatment. The synthesis of albumin improved from 82 mg per kg per day to 135 mg per kg per day with little change in the daily excretion of 5-hydroxyindole acetic acid. After treatment with nicotinamide the patient made good progress with a complete resolution of the signs of pellagra and protein malnutrition. These results support the hypothesis that a reduced availability of the essential amino acid L-tryptophan may limit the synthesis of albumin and nicotinic acid in patients with the carcinoid syndrome who become anoretic. ( info)

15/66. edema, anemia, hypoproteinemia, and acrodermatitis enteropathica: an uncommon initial presentation of cystic fibrosis.

    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. ( info)

16/66. Angioimmunoblastic lymphadenopathy with dysproteinemia: thoracic involvement.

    Angioimmunoblastic lymphadenopathy (AILD) is a rare condition, which is difficult to diagnose as it mimics tuberculosis or lymphoma both clinically and radiologically. A case of AILD with pulmonary involvement that was initially mistaken for tuberculosis on fine needle aspiration cytology and put on antituberculous treatment for three months, is presented here. The case was subsequently diagnosed to lymph node biopsy as one of AILD. ( info)

17/66. Clinical heterogeneity of neonatal intrahepatic cholestasis caused by citrin deficiency: case reports from 16 patients.

    A deficiency of citrin, which is encoded by the SLC25A13 gene, causes both adult-onset type II citrullinemia (CTLN2) and neonatal intrahepatic cholestasis (NICCD). We analyzed 16 patients with NICCD to clarify the clinical features of the disease. Severe intrahepatic cholestasis with fatty liver was the most common symptom, but the accompanying clinical features were variable, namely; suspected cases of neonatal hepatitis or biliary atresia, positive results from newborn screening, tyrosinemia, failure to thrive, hemolytic anemia, bleeding tendencies and ketotic hypoglycemia. Laboratory data showed elevated serum bile acid levels, hypoproteinemia, low levels of vitamin k-dependent coagulation factors, and hypergalactosemia. Hypercitrullinemia was detected in 11 out of 15 patients examined. Most of the patients were given a lactose-free and/or medium chain triglycerides-enriched formula and lipid-soluble vitamins. The prognosis of the 16 patients is going fairy well at present, but we should observe these patients carefully to see if they manifest any symptom of CTLN2 in the future. ( info)

18/66. Spontaneous pregnancy and partial recovery of pituitary function in a patient with Sheehan's syndrome.

    Sheehan's syndrome is caused by pregnancy-related hemorrhage leading to ischemic necrosis of the anterior pituitary gland and hypopituitarism. Spontaneous pregnancy in Sheehan's syndrome is very rare. We report the case of a patient with Sheehan's syndrome who suffered from anterior pituitary insufficiency, but with sparing of gonadotropic function. The patient became pregnant spontaneously and, after her second delivery, thyrotropic function recovered. However, the patient's growth hormone and cortisol levels remained unresponsive to an insulin-tolerance test. This case demonstrates that pituitary function may recover from less extensive pituitary ischemia. We emphasize the importance of early identification of pregnancy in such cases. It is crucial to institute adequate hormone-replacement therapy during pregnancy, since hypopituitarism is associated with high fetal and maternal morbidity and mortality. ( info)

19/66. A patient with eosinophilia, hypoalbuminemia and abdominal pain.

    strongyloides stercoralis infections frequently present with eosinophilia and abdominal pain. Since the gastrointestinal symptoms are non-specific, only 15 percent of these patients are correctly considered to have an infectious enteritis or intestinal parasite. In fact, the initial diagnosis is peptic ulcer disease in most patients. The clinical course may be indolent, or patients may develop a sudden catastrophic illness, particularly following the administration of corticosteroids. ( info)

20/66. A patient with protein-losing enteropathy associated with systemic lupus erythematosus.

    A 46-year-old woman previously diagnosed as having systemic lupus erythematosus presented with severe hypoalbuminemia and anasarca. She was demonstrated to have protein-losing enteropathy without any other active symptoms of SLE. Her bowel habit was normal and endoscopic examination revealed non-specific colitis and a small ulcer in the duodenum. Serum biochemistry showed an abnormal profile of the serum protein, including severe hyperlipoproteinemia and hyperfibrinogenemia. The process of protein-losing was not selective in terms of the molecular size. All of these symptoms and the abnormalities in laboratory data were improved by corticosteroid therapy. ( info)
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