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

A 5-month-old infant presented with vomiting and severe hypoproteinemia from exsudation of proteins into the digestive tract, confirmed by isotopic tests. The association of hypertrophic edematous gastritis, interstitial jejunitis, and severe blood eosinophilia suggested the diagnosis of an exsudative eosinophil-type gastro-enteropathy. An allergy to cow's milk proteins was suspected because of the onset of the disorders at the time of weaning and the appearance of the eczema during the same period. Clinical, biological, and radiological recovery occurred after an exclusion diet and corticotherapy, and this persisted for 8 months, but the eczema remained. The authors describe a similar case (12) in an infant of 21 months with vomiting edema, and hypoproteinemia due to exsudation of proteins into the digestive tract. Radiological examination revealed the presence of hypertrophic gastritis. A relationship with Mentrier's disease had been suggested but not confirmed because of the rapid favourable outcome after a simple low protein diet.
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PMID:[Hypertrophic gastritis in an infant with eczema and intolerance to cow's milk proteins (author's transl)]. 57 73

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

A female infant with hypoproteinemia and coagulopathy associated with hypertyrosinemia was successfully treated with living-related liver transplantation (LRLT). On the 12th day of life plasma amino acid analysis revealed a marked elevation of tyrosine, so the patient was fed on a low-tyrosine and low-phenylalanine diet. However, hepatosplenomegaly, hypotonia, alopecia, eczema and psychomotor delay did not improve and recurrent episodes of disseminated intravascular coagulation (DIC) caused her condition to deteriorate. Liver biopsy on the 230th day revealed marked fatty change accompanied by mild to moderate cholestasis. Therefore, LRLT from her father was performed on the 286th day resulting in improvement of all the aforementioned signs and symptoms. Despite a thorough examination, no diagnosis of a known disorder could be established. However, her elder brother had also been born with severe hypoproteinemia and coagulopathy, and died of DIC on the second day of life. Thus, the disorder is designated as a new entity, namely 'congenital hypoproteinemia and coagulopathy associated with hypertyrosinemia'.
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PMID:Liver transplantation in a case of hypoproteinemia and coagulopathy. 958 13

We report here a 4-month-old girl with atopic dermatitis accompanied by weight loss, electrolyte disturbance, hypoproteinemia and hypogammaglobulinemia. She has suffered from eczema since one-month of age. Although she was treated with Chinese herbal medicines, including Syosaikotokakikyosekko, Tokishigyakukagoshuyushokyoto and Jumihaidokuto and ibuprofen ointment since three-month of age, she was referred to our hospital due to deteriorated eczema, severe diarrhea and failure to thrive. Laboratory examination revealed hyponatremia, hyperpotassemia, hypoproteinemia, hypogammaglobulinemia and elevated levels of serum IL-18, TARC and fecal EDN. Drug-induced lymphocyte stimulation tests were positive for the prescribed Chinese herbal medicines. Discontinuation of these medicines and application of steroid ointments improved loose bowels and skin lesions as well as laboratory data. It is suggested that the application of inadequate ointment and Chinese herbal medicines exaggerated inflammation in the skin and the intestinal mucosa leading to electrolyte disturbance, hypoproteinemia and hypogammaglobulinemia. Chinese herbal medicines are depicted as an additional therapy in Japanese guideline for atopic dermatitis, whereas their indication to infants with atopic dermatitis should be carefully assessed.
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PMID:[Case of atopic dermatitis in infant treated with Chinese herbal medicines and nsaids ointment, which induced weight loss, electrolyte disturbance and hypoproteinemia]. 2412 59