Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019158 (hepatitis)
 30,205 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The syndrome of nodular lymphoid hyperplasia of the small bowel with hypogammaglobulinemia is one of the hypogammaglobulinemic enteropathies. Chronic diarrhea and malabsorption are the most characteristic features of this disease, and they are frequently associated to hypogammaglobulinemia of various types (acquired, congenital non sex-linked) and to selective IgA deficiency. The immunological deficiency gives rise to the more characteristic features of the disease, namely: a) hypogammaglobulinemia; b) respiratory infections and dental caries; c) Giardia lamblia infestation of the small bowel; d) the characteristic radiological features; and, e) the histological aspect of the intestinal mucosa with absence of plasma cells. Periodical follow-up is needed because of the increased incidence of tumors in immunological deficiency states. A new case of nodular lymphoid hyperplasia associated to hemolytic anemia and granulomatous hepatitis is reported, and its possible pathogenesis is discussed.
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PMID:[Nodular lymphoid hyperplasia of the small bowel with IgA deficiency and hemolytic anemia (author's transl)]. 742 63

The zinc, an important enzymatic cofactor, is involved in many metabolic processes. Its deficiency might be due either to malabsorption or to excessive utilization. In the medical literature of the latest 10 years, zinc was considered to play a part in the immune processes. The authors of the present paper intend to study the zinc and immunoglobulin levels in various diseases, i.e., chronic progressive hepatitis, liver cirrhosis (LC), dermatitis, bronchial asthma. This preliminary investigation was carried out in 30 patients with LC in whom serum zinc values were assayed by atomic absorption spectrophotometry and the immunoglobulin levels were determined using the Mancini type simple radial immunodiffusion technique. All these patients presented considerable decrease of serum zinc concentration, the values ranging between 3.06 and 7.65 mumol/l as compared with 19.8 +/- 1.5 mumol/l in the controls, alongside with the increase of immunoglobulins G and M. In the patients treated with Zincum metallicum CH5 it was observed after about 30 days of treatment that the clinical state was considerably improved and IgG and IgM as well as serum zinc had resumed their normal values. This treatment should not be interrupted since in LC, without permanent additional supply, the serum zinc returns rapidly to the initial deficit or even lower.
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PMID:Treatment with zincum metallicum CH5 in patients with liver cirrhosis. Preliminary study. 786 38

Idiopathic neonatal hepatitis is one of the more important causes of neonatal cholestasis. It is regarded one of the clinical presentations of 'idiopathic obstructive cholangiopathy', just like extrahepatic biliary atresia. Is it not possible to discriminate between intrahepatic and extrahepatic causes of neonatal cholestasis, or between idiopathic neonatal hepatitis and metabolic, infectious, or toxic causes, by using clinical or laboratory parameters. Liver histology is slightly more helpful: giant cell formation, focal liver necrosis, and lymphocytic and neutrophilic infiltration may be found in idiopathic neonatal hepatitis. In infectious hepatitis liver pathology mostly is only a lesser part of the symptomatology. Sporadic idiopathic neonatal hepatitis has a better prognosis than familial; about 75% of children with sporadic hepatitis experience complete recovery as compared to less than 25% of children with familial hepatitis. Therapy is confined to the prevention and treatment of complications such as itching, portal hypertension and variceal bleeding, and (fat) malabsorption.
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PMID:[Idiopathic neonatal hepatitis]. 812 25

A 22-year-old Nepali man presented with a 2-month history of fever, ill health, anorexia, loss of weight and diarrhoea. Apart from an ill-defined lower abdominal mass, physical examination was unremarkable. Investigations showed the picture of malabsorption syndrome with no evidence of structural gastro-intestinal tract involvement on barium meal, small bowel and large bowel enema, upper gastro-intestinal endoscopy, colonoscopy and mucous membrane biopsy. Laparoscopy showed typical features of tuberculous peritonitis. Liver biopsy showed tuberculous granulomatous hepatitis, and peritoneal biopsy showed caseating granulomata. The patient responded rapidly to antituberculosis chemotherapy.
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PMID:Malabsorption syndrome complicating tuberculous peritonitis. 944 Oct 65

Microsporidia are ubiquitous in nature. Several clinical syndromes have been associated with microsporidiosis, especially in HIV-infected individuals, and include enteropathy, keratoconjunctivitis, sinusitis, tracheobronchitis, encephalitis, interstitial nephritis, hepatitis, cholecystitis, osteomyelitis, and myositis. Diarrhea and malabsorption are the most common clinical problems. Enterocytozoon bieneusi is the most common microsporidial cause of intestinal disease. A second species, Encephalitozoon intestinalis (originally named Septata intestinalis) is associated with disseminated as well as intestinal disease. Microsporidiosis has been seen worldwide, and is recognized as a frequent enteric infection in patients with AIDS. The pathogenesis of intestinal disease is related to excess death of enterocytes as a result of cellular infection. Clinically, microsporidiosis most often presents with diarrhea and weight loss as a result of small intestinal injury and malabsorption. However, microsporidia have been detected in virtually all organs, and may provoke symptoms related to their specific localization. The diagnosis of microsporidiosis is made histologically, either from tissue biopsies or secretions. While transmission electron microscopy was required for diagnosis in the past, special stains and light microscopy, as well as immunohistochemical and molecular techniques are capable of providing a firm diagnosis. Therapeutic options are limited. Enc. intestinalis responds well to albendazole, while no antiparasitic therapy has documented efficacy in Ent. bieneusi infections.
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PMID:Clinical syndromes associated with microsporidiosis. 955 78

Necrolytic migratory erythema is characterized by waves of irregular erythema in which a central bulla develops, and subsequently erodes and becomes crusted. It usually occurs in patients with an alpha-islet cell tumor of the pancreas. However, necrolytic migratory erythema has also been observed in patients without an associated glucagonoma. We describe a woman with iatrogenic necrolytic migratory erythema. She received intravenous glucagon for hypoglycemia associated with an insulin-like growth factor II-secreting hemangiopericytoma. After chemotherapy, she developed necrolytic migratory erythema. The characteristics of the previously reported patients with nonglucagonoma-associated necrolytic migratory erythema are reviewed. In patients with nonglucagonoma-associated necrolytic migratory erythema, the dermatosis-related conditions most commonly observed were celiac disease or malabsorption, cirrhosis, malignancy, and pancreatitis; less common conditions included hepatitis, inflammatory bowel disease, heroin abuse, and odontogenic abscess. Although the pathogenesis of necrolytic migratory erythema remains unknown, hyperglucagonemia appears to have had a causative role in the development of this dermatosis in our patient. Patients who develop necrolytic migratory erythema should be evaluated for the presence of a glucagonoma; if a glucagonoma is ruled out, evaluation for other conditions known to occur with necrolytic migratory erythema, such as liver disease, malabsorptive disorders, and nonislet-cell tumors is warranted.
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PMID:Iatrogenic necrolytic migratory erythema: a case report and review of nonglucagonoma-associated necrolytic migratory erythema. 959 6

Asymptomatic persistent hypertransaminasemia unrelated to hepatitis viral infection is a common cause of referral to the hepatologist. Less frequent liver diseases should then be considered, as well as extrahepatic-origin hypertransaminasemia. Celiac disease, although it has repeatedly been reported as a cause of persistent hypertransaminasemia, is often not included in its differential diagnosis in the absence of the classic malabsorption syndrome. We present the cases of four patients sent to a liver unit for evaluation of persistent hypertransaminasemia in whom celiac disease was finally discovered. Our report highlights the importance of including celiac disease in list of conditions potentially responsible for chronic hypertransaminasemia of unknown cause.
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PMID:Persistent hypertransaminasemia as the presenting feature of celiac disease. 1020 90

Azathioprine is commonly prescribed for autoimmune hepatitis and inflammatory bowel disease. An acute gastroenteritis-like syndrome has been ascribed to azathioprine use, but chronic diarrhea has not. We report a patient with autoimmune hepatitis who developed severe small-bowel villus atrophy and chronic diarrhea after azathioprine was initiated (50 mg/day). We present a case report of a patient followed up prospectively. Duodenal mucosal histology and expression of brush border enzyme dipeptidyl peptidase IV and peptide transporter PepT1 messenger RNA levels were determined before and after azathioprine discontinuation. Chronic diarrhea developed several weeks after the initiation of azathioprine and resulted in micronutrient depletion and severe protein-calorie malnutrition, which was unresponsive to oral pancreatic enzyme therapy or a gluten-free diet. Severe malabsorption required parenteral nutrition support for longer than 1.5 years; this was complicated by unstable blood glucose control, acute calculous cholecystitis, catheter sepsis, and severe venous thrombosis. When the temporal association between azathioprine and diarrhea was identified, the drug was tapered while the patient consumed an unrestricted diet. Within 2 weeks after azathioprine was discontinued, diarrhea had completely resolved, and parenteral nutrition was discontinued. Mucosal biopsies obtained before and 4 months after azathioprine discontinuation showed complete reversal of severe duodenal villus atrophy and marked up-regulation of mucosal dipeptidyl peptidase IV and PepT1 messenger RNA. The patient has subsequently maintained normal liver function tests on low-dose prednisone alone, with normal stools and stable nutritional status for longer than 4 years. Azathioprine can induce severe small-bowel villus atrophy, diarrhea, and malabsorption that is reversible with drug discontinuation.
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PMID:Severe villus atrophy and chronic malabsorption induced by azathioprine. 1280 28

Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is caused by mutations in the autoimmune regulator (AIRE) gene, which has a central function in maintaining immunological tolerance. A number of conditions with proven or likely autoimmune pathogenesis occur in APECED: hypoparathyroidism, adrenocortical insufficency, candidiasis, hypogonadism, type 1 diabetes, hypothyroidism, hypophysitis, hepatitis, malabsorption, nail dystrophy, enamel hypoplasia and keratopathy. It is not clear which factors are responsible for variation in clinical picture of APECED, but human leukocyte antigen (HLA) genotype may be important. The authors report the first description of a case of primary pulmonary hypertension (PPH) in patient with APECED, caused by R257X mutation in AIRE. The HLA genotype of the patient (DRB1*01/DRB1*11, DQB1*0301/DQB1*0501) has been previously reported as a predisposing factor to PPH. The findings from this study, provided that other similar cases are reported, suggest that immune deregulation plays a role in the pathogenesis of primary pulmonary hypertension.
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PMID:Fatal primary pulmonary hypertension in a 30-yr-old female with APECED syndrome. 1458 26

Autoimmune polyglandular syndrome type 1 (APS-1) is an autosomal recessive disorder characterized by chronic mucocutaneous candidiasis, autoimmune hypoparathyroidism, and primary adrenal insufficiency. It has recently been associated with mutations of a single gene found on chromosome 21, designated AutoImmune Regulator (AIRE). We report two patients with APS-1 referred to our hospital for evaluation. The first patient was an 11-year-old girl with hypoparathyroidism, infectious or immunological malabsorption, and autoimmune hepatitis. Hypoparathyroidism associated with other processes with a probable autoimmune origin suggested APS-1. Genetic study was performed revealing deletion of 13 base pairs in exon 8 of the AIRE gene. The second patient was a 17-year-old girl with autoimmune hepatitis, hypoparathyroidism, mucocutaneous candidiasis, nail dystrophy, and obliterating bronchiolitis with a probable autoimmune origin. We suspected APS-1 and genetic study was performed. The only finding was an AIRE gene polymorphism. In conclusion, the presence of a single disease criterion is sufficient to suspect APS-1 and to indicate genetic study. Further studies are required to confirm the involvement of other genes in the development of this disease.
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PMID:[AIRE gene mutation in polyglandular syndrome type 1]. 1679 67


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