RESUMO
The Dubin-Johnson syndrome is a hereditary deficiency in the excretion ofconjugated bilirrubin by hepatocytes characterized by chronic hyperbilirubinemia, alteration in coproporphyrin metabolism, and intracellular deposition of a dark melanin-like pigment giving the liver a typical black cast. We report a 28-year-old male patient who presented conjunctival jaundice and conjugated-hyperbilirubinemia without no other alteration in hepatic biochemistry. The diagnosis of this syndrome was perfomed by using the low-risk methods of laparoscopy-facilitated hepatic biopsy and oral cholecystography In contrast, we avoided the classical Bromsulphalein test because of potential severe side effects. We stress here the current importance of these tests for confirming the diagnosis. By using this methodology, we were not able to quantify the isomeric profile of the urinary coproporphyrins nor 99mTc-HIDA cholescintigraphy. In conclusion, we confirm the utility of hepatic biopsy with the aid of laparoscopy and oral cholecystography for the diagnosis of the Dubin-Johnson syndrome on the basis of their effectiveness and relative lack of complications.
Assuntos
Hiperbilirrubinemia Hereditária/diagnóstico , Adulto , Doenças da Túnica Conjuntiva/diagnóstico , Coproporfirinas/urina , Humanos , Icterícia Idiopática Crônica/diagnóstico , Fígado/patologia , Masculino , SíndromeRESUMO
BACKGROUND AND OBJECTIVES: The aim of this study was to investigate the occurrence of hypertransaminasemia (hTAMSemia) as an indicator of liver damage and to establish the association of this hepatotoxicity with exposure to aromatic hydrocarbons (benzene, toluene, and xylene [BTX]) among workers in a petrochemical company. PATIENTS AND METHOD: The medical records of 167 industrial employees, 95 with hydrocarbon exposure (EHCs) and 72 without exposure (NEHCs) were reviewed. Age, sex, number of years employed, body mass index, and biochemical and hematological parameters were evaluated. Employees with previous liver disease, diabetes mellitus, or alcohol intake (> 50 g/day) were excluded. In employees with hTAMSemia, we performed a proteinogram and hepatic ultrasonography and tested blood samples for prothrombin and hepatitis B and C markers. Within this subgroup, 3 workers were excluded (due to serum markers of hepatitis B virus in 2 and refusal to participate in 1), leaving a total of 92 in the EHC group. Finally, the working environment was screened for volatile contaminants. RESULTS: Twenty-seven employees from the EHC group (29.4%) and 1 from the NEHC group (1.4%) had hTAMSemia (p = 0.001). The remaining biochemical tests and parameters measured showed no significant differences between the two groups. Comparison between the EHC subgroup of 27 workers showing hTAMSemia and the remainder of the EHC group with normal values (65 workers) revealed no differences in the other parameters measured. Of the 27 workers of the former subgroup, 14 (51.9%) showed ultrasonographic images compatible with a fatty liver. One worker (1.4%) in the NEHC group showed hTAMSemia and ultrasonography compatible with fatty liver. The environmental levels of BTX during the 9 months of the study remained below the maximum values permitted by law in Argentina (benzene, 1.5 ppm., toluene 10 ppm and xylene 18.5 ppm). The odds ratio of developing hTAMSemia in the EHC group was 27.7 (p = 0.002). CONCLUSIONS: Occupational exposure to aromatic hydrocarbons may cause liver damage. The liver is more vulnerable to these hydrocarbons than bone marrow. These conclusions would argue for a modification of the environmental regulations currently in force within the petroleum refineries in Argentina.
Assuntos
Indústria Química , Doença Hepática Induzida por Substâncias e Drogas/etiologia , Hidrocarbonetos Aromáticos/toxicidade , Doenças Profissionais/induzido quimicamente , Adulto , Doença Hepática Induzida por Substâncias e Drogas/sangue , Humanos , Pessoa de Meia-Idade , Transaminases/sangueRESUMO
Introducción: el anismo se define como el aumento de presión del conducto anal durante la defecación. Es causa de costirpación crónica al producir obstrucción del tracto de salida. El mecanísmo fisiopatogénico es la contracción paradojal del esfínter anal o el puborrectal. Se asocia a otros desordenes pelvirrectales. Existe una alta prevalencia en pacientes con historia de abuso sexual o situaciones de estrés. Paciente y métodos: Los pacientes fueron estudiados según protocolo (historia clínica y estudios funcionales anorrectales) con posterior evaluación de la respuesta terapéutica al biofeedback (BFB). Resultados: Sobre 207 pacientes constipados se diagnosticaron 31 casos de anismo (15 por ciento) de los cuales 19 recibieron BFB. La prevalencia fue en mujeres (80 por ciento), la media de edad fue de 46 años (R=19-73), el 94 por ciento de los pacientes presentaron sintomas de obstrucción del tracto de salida... (AU)
Assuntos
Humanos , Masculino , Feminino , Pessoa de Meia-Idade , Doenças do Ânus/fisiopatologia , Constipação Intestinal/diagnóstico , Constipação Intestinal/terapia , MétodosRESUMO
Introducción: el anismo se define como el aumento de presión del conducto anal durante la defecación. Es causa de costirpación crónica al producir obstrucción del tracto de salida. El mecanísmo fisiopatogénico es la contracción paradojal del esfínter anal o el puborrectal. Se asocia a otros desordenes pelvirrectales. Existe una alta prevalencia en pacientes con historia de abuso sexual o situaciones de estrés. Paciente y métodos: Los pacientes fueron estudiados según protocolo (historia clínica y estudios funcionales anorrectales) con posterior evaluación de la respuesta terapéutica al biofeedback (BFB). Resultados: Sobre 207 pacientes constipados se diagnosticaron 31 casos de anismo (15 por ciento) de los cuales 19 recibieron BFB. La prevalencia fue en mujeres (80 por ciento), la media de edad fue de 46 años (R=19-73), el 94 por ciento de los pacientes presentaron sintomas de obstrucción del tracto de salida...