Get your own Free Home PageCRYPTOGENIC HEPATITIS MASKING THE DIAGNOSIS OF ORNITHINE TRANSCARBAMYLASE DEFICIENCY.
Zammarchi E; Donati MA; Filippi L; Resti M, Department of Pediatrics, University of Florence, Italy. J Pediatr Gastroenterol Nutr, 1996 May, 22:4, 380-3
We describe three children with transaminase elevations and hepatic insufficiency who were given the diagnosis of cryptogenic hepatitis after the more common viral and metabolic diseases of the liver had been excluded. However, further laboratory investigations showed hyperammonemia, low blood urea levels, elevated plasma glutamine levels, and low citrulline levels. Urinary excretion of orotic acid was higher than normal, with absent urinary homocitrulline and normal fractional tubular reabsorption of lysine, ornithine, and arginine. These findings suggest the diagnosis of ornithine transcarbamylase deficiency. We emphasize the importance of investigating possible urea cycle disorders by determining ammonia plasma levels, both at baseline and after a protein load; urinary and plasma amino acids; and urinary orotic acid in all patients with liver disease of indeterminate etiology.
DIRECT DETECTION OF MYCOBACTERIUM TUBERCULOSIS USING POLYMERASE CHAIN REACTION ASSAY AMONG PATIENTS WITH HEPATIC GRANULOMA
Diana E. Alcantara-Payawal, Masayuki Matsumura, Yasushi Shiratori, Takehito Okudaira, Roy Gonzalez, Roland A. Lopez, Jose D. Sollano and Masao Omata
Background:In liver tuberculosis, demonstration of acid bacilli by conventional methods remains futile. Since the definitive diagnosis of liver tuberculosis is based on the histologic evidence of granulomatous process with caseation necrosis, seen in only a third of cases, the diagnosis is made retrospectively by response to empirical anti-tuberculous drug therapy.
Aims:Our objective is to establish a polymerase chain reaction assay for detection of Mycobacterium tuberculosis affecting the liver using the paraffin-embedded liver biopsy specimens obtained from patients with hepatic granulomas.
Methods:As positive control, patients having either rising-dbl-quote-leftdefinitive' (n=8) or rising-dbl-quote-leftpresumptive' (n=9) tuberculosis on the basis of clinical, microbiological, histologic data and their positive response to empirical treatment of anti-tuberculous drugs were used. Patients with hepatic granulomas secondary to schistosomiasis (n=6), sarcoidosis (n=2) and other liver diseases (n=10) were used as negative control.
Results: Of those patients who were diagnosed as having rising-dbl-quote-leftdefinitive' and rising-dbl-quote-leftpresumptive' liver tuberculosis, positivity by one-step polymerase chain reaction was 100% and 44%, respectively. Using the nested polymerase chain reaction, positivity increased to 78% with rising-dbl-quote-leftpresumptive' liver tuberculosis. In contrast, the polymerase chain reaction assays were negative among all patients with hepatic granuloma due to non-tuberculous-in-origin and other liver diseases.
Conclusions: The overall positivity of this polymerase chain reaction
assay (88%) compares favorably with that of other conventional
methods (12%). Thus, this polymerase chain reaction assay may
be a reliable diagnostic tool for liver tuberculosis in a patient
population in which the prevalence of diseases associated with
hepatic granuloma is common.
CHRONIC EPSTEIN-BARR VIRUS INFECTION: A CAUSE OF GRANULOMATOUS HEPATITIS?
Biest S; Schubert TT; University of Missouri Kansas City School of Medicine, Gastroenterology Section, Truman Medical Center. J Clin Gastroenterol, 1989 Jun, 11:3, 343-6
A 37-year-old man had fever, weight loss, malaise, right upper quadrant pain, lymphadenopathy, and a twofold elevation of serum alkaline phosphatase, transaminases, and gamma glutamyl transpeptidase. Granulomas were found on liver biopsy after 2 ½ months of illness. Treatment with isoniazid and rifampin for 2 months did not lead to improvement in fever or symptoms, but prednisone caused a prompt resolution. Positive IgM antibodies to the viral capsule antigen followed by marked elevation of the IgG fraction suggest chronic Epstein-Barr virus infection to be the etiology. The patient was asymptomatic without treatment 14 months after onset of the illness.
CHRONIC Q FEVER OF PREGNANCY PRESENTING AS COXIELLA BURNETII PLACENTITIS: SUCCESSFUL OUTCOME FOLLOWING THERAPY WITH ERYTHROMYCIN AND RIFAMPIN.
Bental T; Fejgin M; Keysary A; Rzotkiewicz S; Oron C; Nachum R; Beyth Y; Lang R; Department of Medicine, Meir General Hospital, Kfar Saba, Israel. Clin Infect Dis, 1995 Nov, 21:5, 1318-21
Chronic Q fever has been associated with endocarditis, granulomatous hepatitis, and osteomyelitis but only rarely with pregnancy. The apparent predilection of Coxiella burnetii, the organism causing Q fever, for the human placenta suggests that chronic Q fever of pregnancy is due to placentitis. We describe a patient with chronic, clinically apparent Q fever in pregnancy and a successful outcome. The diagnosis was made both by serology and by isolation of C. burnetii from the patient's serum and placenta. Therapy with erythromycin and rifampin contributed to the delivery of a healthy baby. The mother's infection was clinically cured by subsequent therapy with doxycycline and rifampin.
CLINICAL ASPECTS OF GIANT CELL HEPATITIS
Maier KP; Fachbereich Gastroenterologie, Universität Tübingen. Schweiz Rundsch Med Prax, 1994 Nov 1, 83:44, 1235-6
Giant-cell hepatitis in adults is a rare event resembling either chronic non-A, non-B or autoimmune chronic active hepatitis. The etiology is unknown. Paramyxoviruses may be implicated. The clinical picture varies remarkably from mild chronic liver disease to subacute hepatic failure. Liver biopsy is diagnostic, showing giant cells as the common pathological finding. Due to the unknown cause of the disease, treatment is symptomatic. Steroids are applied, as in the case of autoimmune disease, with remarkable results in some patients. In fulminant cases, however, liver transplantation is the only option for cure.
SUBFULMINANT SYNCYTIAL GIANT CELL HEPATITIS: recurrence after liver transplantation treated with RIBAVIRIN
François Durand, Claude Degott, Alain Sauvanet, Georges Molas, Christian Sicot, Patrick Marcellin, JacquesBelghiti, Serge Erlinger, Jean-Pierre Benhamou and Jacques Bernuau; Journal of Hepatology Volume 26 - issue 3( March 1997 ) - page 722 - 726
We report the case of an adult patient affected by acute syncytial giant cell hepatitis which had a subfulminant course leading to liver transplantation. Syncytial giant cell hepatitis recurred after transplantation and was efficiently treated with ribavirin. In this patient, the recurrence of the disease, the presence of filamentous strands on electron microscopy during both bouts of hepatitis and the efficacy of ribavirin on post-transplantation hepatitis suggest that the disease was caused by an original virus. This observation also suggests that early administration of ribavirin in patients affected by acute syncytial giant cell hepatitis of unknown origin could avoid liver transplantation.
COELIAC DISEASE:
GLUTEN-SENSITIVE ENTEROPATHY
Vajro P; Fontanella A; Mayer M; De Vincenzo A; Terracciano LM; D'Armiento M; Vecchione R Department of Pediatrics, Pediatric Gastroenterology and Hepatology, II Faculty of Medicine, University of Naples, Italy. J Pediatr, 1993 Mar, 122:3, 416-9
Six children in whom long-standing hypertransaminasemia of unknown
cause led to an initial diagnosis of chronic or protracted cryptogenic
hepatitis were found to have asymptomatic celiac disease. Administration
of a gluten-free diet caused a prompt improvement of both hepatic
and intestinal biochemical/histologic abnormalities. Hepatic damage
may be another "atypical" form of celiac disease in
children.
HEPATIC INJURY IN ADULT COELIAC DISEASE.
Hagander B; Berg NO; Brandt L; Nordén A; Sjölund K; Stenstam M Lancet, 1977 Aug 6, 2:8032, 270-2
In an attempt to determine the frequency of liver injury in adult coeliac disease (A.C.D.) the case records of 74 consecutive patients were examined. In 13 cases histological sections of the liver were available and in 5 of these there were signs of reactive hepatitis. Histological signs of distinct hepatic injury with cirrhosis and/or chronic active hepatitis were found in 7 other patients. In 5 of these serum-IgA was normal, whereas 16 out of 20 control patients with liver cirrhosis not associated with A.C.D. had raised serum-IgA. Serum-aspartate-aminotransferase and serum-alanine-aminotransferase were determined in 53 patients; 29 had raised concentrations. In 19 patients serum-aminotransferases were repeatedly determined before and during the dietary regimen and there was a significant reduction in enzyme concentrations during treatment. The median concentration of serum-alkaline-phosphatase was also reduced during treatment but not significantly. The histological evidence of liver injury in 16% and the abnormal liver-function tests in 39% of the patients indicate that hepatic injury is common in A.C.D. Since liver-function tests or liver biopsy specimens were available for only about two-thirds of the patients, liver damage in A.C.D. may be more common than indicated by these results. The effect of a gluten-free diet on aminotransferase concentrations indicates that the liver injury may be reversible and suggests that in some A.C.D. patients progressive liver damage may be prevented by suitable treatment. Since A.C.D. is not always recognised, the diagnosis should be considered in patients with liver disease of unknown aetiology.
PERSISTENT HYPERTRANSAMINASEMIA AS THE PRESENTING FINDING OF CHILDHOOD MUSCLE DISEASE.
Rutledge J; Andersen J; Fink CW; Cook J; Strickland A; Clin Pediatr (Phila), 1985 Sep, 24:9, 500-3
Four children with isolated, persistent elevations of serum transaminases were investigated for hepatic disease and followed for 4 to 24 months before serum creatine kinase determinations were obtained and found to be markedly elevated. Evidence of muscle disease was obtained by close questioning, retrospectively, and mild abnormalities were found on physical examination. Review of laboratory tests in our center for 6 months revealed 30 additional cases of anicteric hypertransaminasemia (20% of those with elevated enzymes), only two of which were unexplained by the admitting diagnosis. Serum transaminase values are elevated in a variety of diseases of different organ systems. Creatine kinase determinations may provide the clue to the diagnosis of occult muscle disease in some children with unexplained anicteric hypertransaminasemia.
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