RESUMO
OBJECTIVE: The aim of the present study was to characterize the pharyngoesophageal segment in laryngectomees who rated themselves as functional tracheoesophageal speakers. METHODS: Voice perceptual assessment, high-resolution videomanometry of swallowing and phonation, and high-speed camera recording during phonation provided information about the anatomy and function of the pharyngoesophageal segment. RESULTS: Fourteen patients were included in the study. The voice assessments presented high intra/inter-listener reliability. We found a significant correlation between roughness and poor voice quality, hyperfunction and poor intelligibility, and poor voice quality, long time since the operation, and old age. High-resolution videomanometry during phonation revealed decreasing mean pressures from the distal esophagus to the pharynx and confirmed low resting pressures at the pharyngoesophageal segment and low esophageal peristaltic contraction pressures after laryngectomy in comparison to normal subjects. The neoglottis shape was mainly circular and presented a strong mucosal wave in most of the patients on the high-speed camera recording. CONCLUSIONS: Perceptual voice assessment and high-speed camera recordings provided baseline information about voice characteristics and vibration regularity of the neoglottis. Additionally, the quantitative measures obtained with high-resolution videomanometry may have clinical applicability as reference data in voice rehabilitation after total laryngectomy.
Assuntos
Carcinoma de Células Escamosas/cirurgia , Esôfago/cirurgia , Neoplasias de Cabeça e Pescoço/cirurgia , Neoplasias Laríngeas/cirurgia , Laringectomia , Laringe Artificial , Fonação , Voz Esofágica , Traqueia/cirurgia , Qualidade da Voz , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Manometria , Pessoa de Meia-Idade , Inteligibilidade da Fala , Voz Alaríngea , Carcinoma de Células Escamosas de Cabeça e PescoçoRESUMO
Dorsal agenesis of the pancreas is a rare congenital disorder. We report a case of a 65-year-old man with mild abdominal pain and insulin-dependent diabetes mellitus. Computed tomography (CT) of the abdomen showed a short pancreas with no pancreatic tissue ventral to the splenic vein. Magnetic resonance cholangiopancreatography (MRCP) visualized the absence of a dorsal duct system and confirmed the suspicion of complete agenesis of the dorsal pancreas. Endoscopic ultrasound (EUS) was also performed to rule out pancreatic malignancy.
Assuntos
Dor Abdominal/etiologia , Anormalidades Congênitas/diagnóstico , Diabetes Mellitus Tipo 1/etiologia , Idoso , Colangiopancreatografia por Ressonância Magnética/métodos , Anormalidades Congênitas/patologia , Diagnóstico Diferencial , Humanos , Masculino , Pâncreas/anormalidades , Pâncreas/diagnóstico por imagem , Pâncreas/patologia , Tomografia Computadorizada por Raios X , UltrassonografiaRESUMO
BACKGROUND: Conditions exhibiting features of two different autoimmune liver diseases are designated overlap syndromes. Variant forms display some, but not all, characteristics of a distinct autoimmune liver disease. We describe transitions over time between variant forms of PBC, i.e. AMA-negative PBC, autoimmune hepatitis (AIH)-PBC overlap and autoimmune cholangitis (AIC) in a large cohort of PBC patients in Sweden. METHODS: We retrieved all patients with variant forms of PBC in six university hospitals in Sweden, covering 60% of the Swedish population. The diagnosis of PBC and its variants was based on laboratory findings and compatible histological features. The revised autoimmune hepatitis scoring system proposed by the International Autoimmune Hepatitis Group was used to establish the diagnosis of AIH. RESULTS: In a population of 800 patients with PBC, we identified 35 (5%) variant forms; 25 patients with AIH-PBC overlap, 8 with AIC and 2 with AMA-negative PBC at the time of our study. The initial diagnoses were PBC (3 patients), AIH (3), AIH-PBC overlap (16), AIC (8) and AMA-negative PBC with (1) or without (4) concomitant AIH. The median follow-up was 125 (41-360) months. Immunosuppression and ursodeoxycholic acid induced a complete or good regression of increased aminotransferases in about half of the patients who were given one or both of these treatments. CONCLUSIONS: Variant forms of PBC are seen in approximately 5% of PBC patients in Sweden. Transition between different forms may occur, emphasizing the value of repeat biopsies, but established overlapping AIH-PBC seems to be stable over time.
Assuntos
Doenças Autoimunes/patologia , Colangite/patologia , Hepatite Autoimune/patologia , Cirrose Hepática Biliar/patologia , Adulto , Idoso , Doenças Autoimunes/tratamento farmacológico , Doenças Autoimunes/imunologia , Biópsia , Colangite/tratamento farmacológico , Colangite/imunologia , Estudos de Coortes , Feminino , Seguimentos , Hepatite Autoimune/tratamento farmacológico , Hepatite Autoimune/imunologia , Humanos , Imunossupressores/uso terapêutico , Cirrose Hepática Biliar/tratamento farmacológico , Cirrose Hepática Biliar/imunologia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Resultado do TratamentoRESUMO
BACKGROUND: Overlap syndrome is a term used for overlapping features of autoimmune hepatitis and primary sclerosing cholangitis or primary biliary cirrhosis and for autoimmune cholangitis. We describe a high prevalence of small duct primary sclerosing cholangitis among patients with overlapping autoimmune hepatitis and primary sclerosing cholangitis. METHODS: We sought to retrieve all patients with overlap syndrome between primary sclerosing cholangitis and autoimmune hepatitis in six university hospitals in Sweden. The revised autoimmune hepatitis scoring system proposed by the International Autoimmune Hepatitis Group was used to establish the diagnosis autoimmune hepatitis. Endoscopic retrograde cholangiography and/or magnetic resonance cholangiography were used to separate the primary sclerosing cholangitis cases diagnosed through liver biopsy into small and large primary sclerosing cholangitis. A histological diagnosis compatible with both autoimmune hepatitis and primary sclerosing cholangitis was required for inclusion. RESULTS: 26 patients fulfilled our criteria for histological overlap of autoimmune hepatitis and primary sclerosing cholangitis, 7 (27%) of which had small duct primary sclerosing cholangitis. The reliability of the diagnosis small duct primary sclerosing cholangitis was supported by a very close similarity between small and large duct primary sclerosing cholangitis patients in clinical and laboratory data, and by a poor response to immunosuppressive therapy in the small duct primary sclerosing cholangitis patients. Patients with large duct overlap syndrome had a good response to immunosuppressive therapy. In both groups, our limited experience from ursodeoxycholic acid was largely poor. CONCLUSIONS: Small duct primary sclerosing cholangitis is prevalent in the overlap syndrome between autoimmune hepatitis and primary sclerosing cholangitis.
Assuntos
Ductos Biliares Intra-Hepáticos/patologia , Colangite Esclerosante/epidemiologia , Colangite Esclerosante/patologia , Hepatite Autoimune/epidemiologia , Hepatite Autoimune/patologia , Adolescente , Adulto , Biópsia , Colangiografia , Feminino , Humanos , Angiografia por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Prevalência , Adulto JovemRESUMO
BACKGROUND & AIMS: The long-term prognosis of patients with small-duct primary sclerosing cholangitis (PSC) remains incompletely characterized. We aimed at determining the natural history and long-term outcomes of a large number of patients with small-duct PSC. METHODS: Data from 83 patients with well-characterized small-duct PSC from several medical institutions in Europe and the United States were combined. Each patient with small-duct PSC was randomly matched to 2 patients with large-duct PSC by age, gender, calendar year of diagnosis, and institution. RESULTS: The median age at diagnosis in both groups was 38 years (61% males). Nineteen (22.9%) of the 83 patients with small-duct PSC progressed to large-duct PSC in a median of 7.4 (interquartile range [IQR], 5.1-14) years. One patient with small-duct PSC who progressed to large-duct PSC was diagnosed with cholangiocarcinoma but after progression to large-duct PSC; 20 patients with large-duct PSC developed cholangiocarcinoma. Patients with small-duct PSC had a significantly longer transplantation-free survival compared with large-duct PSC patients (13 years [IQR, 10-17] vs 10 years [IQR, 6-14], respectively; hazard ratio, 3.04; 95% confidence interval: 1.82-5.06; P < .0001). Two patients with small-duct PSC who underwent liver transplantation had recurrence of small-duct PSC in the graft 9 and 13 years, respectively, after transplantation. CONCLUSIONS: Small-duct PSC is a disease of progressive potential but associated with a better long-term prognosis as compared with large-duct PSC. Small-duct PSC may recur after liver transplantation. Cholangiocarcinoma does not seem to occur in patients with small-duct PSC, unless the disease has progressed to large-duct PSC.
Assuntos
Colangite Esclerosante/diagnóstico , Colangite Esclerosante/etiologia , Adulto , Causas de Morte/tendências , Colangiopancreatografia Retrógrada Endoscópica , Colangiopancreatografia por Ressonância Magnética , Colangite Esclerosante/mortalidade , Progressão da Doença , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Noruega/epidemiologia , Prognóstico , Estudos Retrospectivos , Fatores de Risco , Taxa de Sobrevida/tendências , Suécia/epidemiologia , Fatores de Tempo , Reino Unido/epidemiologia , Estados Unidos/epidemiologiaRESUMO
OBJECTIVES: A liver biopsy is performed mainly to stage primary sclerosing cholangitis (PSC). In viral hepatitis, alcoholic liver disease and in primary biliary cirrhosis, the ratio of aspartate to alanine aminotransferase (AST/ALT) has been proven to be an indicator of liver cirrhosis. We wanted to test whether or not an AST/ALT ratio >/=1 is an indicator of cirrhosis also in patients with PSC. METHODS: A cohort of 154 patients diagnosed with PSC was studied retrospectively. Laboratory tests and the histological stage were scored. RESULTS: The mean AST/ALT ratio in the cirrhotic patients at the time of the first (n=117) as well as the last (n=72) histological examination was higher (1.3+/-0.5 and 1.6+/-0.7, respectively) than in the non-cirrhotic patients (0.7+/-0.4 and 1.0 +/-0.4, respectively) (P<0.0001 and P=0.0002, respectively). An AST/ALT ratio >/=1 was a strong predictor for liver-related death/orthotopic liver transplantation and liver-related death, being associated with a double and an almost fourfold higher risk, respectively. CONCLUSION: An AST/ALT ratio >/=1 is significantly associated with the presence of cirrhosis and poor outcome in PSC. It may therefore be a valuable non-invasive method for indicating cirrhosis in patients with PSC.
Assuntos
Alanina Transaminase/metabolismo , Aspartato Aminotransferases/metabolismo , Colangite Esclerosante/enzimologia , Cirrose Hepática/enzimologia , Adulto , Biomarcadores/metabolismo , Colangite Esclerosante/complicações , Colangite Esclerosante/diagnóstico , Feminino , Humanos , Cirrose Hepática/diagnóstico , Cirrose Hepática/etiologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos RetrospectivosRESUMO
BACKGROUND/AIMS: Limited systematic data exists on the incidence of drug-induced hepatotoxicity due to disulfiram and the most important prognostic markers. We aimed to determine the nature and frequency of suspected disulfiram hepatotoxicity in Sweden. METHODS: All reports of suspected hepatic adverse drug reactions (ADR) associated with disulfiram received by the Swedish Adverse Drug Reactions Advisory Committee (SADRAC) 1966-2002 were reviewed. Causality assessment was based on the International Consensus Criteria. RESULTS: A total of 82 reports of disulfiram suspected ADRs had at least a possible causal relationship. Eight patients died or underwent liver transplantation (Tx). Mortality or Tx was 16% in patients with jaundice. The median age of the patients (65% males) was 45 years with a median duration of treatment of 42 days. Bilirubin was higher (P<0.0001) in the deceased/transplanted patients compared to surviving patients. No difference was observed in age or duration of therapy between deceased and transplanted and those who recovered. Eosinophilic infiltration in liver biopsies was associated with a favourable outcome, hepatocyte drop-out with a poor outcome. CONCLUSIONS: Disulfiram associated hepatitis has a considerable mortality risk. Histological signs of immunoallergy seem to be common. Bilirubin and hepatocyte drop-out were the only predictors for death or transplantation.
Assuntos
Dissuasores de Álcool/efeitos adversos , Doença Hepática Induzida por Substâncias e Drogas , Dissulfiram/efeitos adversos , Hepatopatias/patologia , Adulto , Sistemas de Notificação de Reações Adversas a Medicamentos/estatística & dados numéricos , Bilirrubina/sangue , Biópsia , Causalidade , Doença Hepática Induzida por Substâncias e Drogas/diagnóstico , Doença Hepática Induzida por Substâncias e Drogas/mortalidade , Doença Hepática Induzida por Substâncias e Drogas/patologia , Feminino , Hepatócitos/patologia , Humanos , Icterícia/induzido quimicamente , Icterícia/diagnóstico , Icterícia/patologia , Icterícia/cirurgia , Fígado/patologia , Hepatopatias/epidemiologia , Hepatopatias/cirurgia , Transplante de Fígado/patologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Suécia/epidemiologiaRESUMO
BACKGROUND & AIMS: There is no medical treatment of proven benefit for primary sclerosing cholangitis. This study aimed at studying the effect of a higher dose of ursodeoxycholic acid than previously used on survival, symptoms, biochemistry, and quality of life in this disease. METHODS: A randomized placebo-controlled study was performed in tertiary and secondary gastroenterology units. A total of 219 patients were randomized to 17 to 23 mg/kg body weight per day of ursodeoxycholic acid (n = 110) or placebo (n = 109) for 5 years. Follow-up data are available from 97 patients randomized to ursodeoxycholic acid and for 101 randomized to placebo. Quality of life was assessed by using the Medical Outcomes Study 36-item Short-Form Health Survey. RESULTS: The combined end point "death or liver transplantation" occurred in 7 of 97 (7.2%) patients in the ursodeoxycholic acid group vs 11 of 101 (10.9%) patients in the placebo group (P = .368; 95% confidence interval, -12.2% to 4.7%). The occurrence of liver transplantation as a single end point showed a similar positive trend for ursodeoxycholic acid treatment (5/97 [5.2%] vs 8/101 [7.9%]; 95% confidence interval, -10.4% to 4.6%). Three ursodeoxycholic acid and 4 placebo patients died from cholangiocarcinoma, and 1 placebo patient died from liver failure. Alkaline phosphatase and alanine aminotransferase tended to decrease during the first 6 months. There were no differences between the 2 groups in symptoms or quality of life. Analyses of serum ursodeoxycholic acid concentration gave no evidence that noncompliance may have influenced the results. CONCLUSIONS: This study found no statistically significant beneficial effect of a higher dose of ursodeoxycholic acid than previously used on survival or prevention of cholangiocarcinoma in primary sclerosing cholangitis.
Assuntos
Colagogos e Coleréticos/administração & dosagem , Colangite Esclerosante/tratamento farmacológico , Ácido Ursodesoxicólico/administração & dosagem , Adulto , Alanina Transaminase/sangue , Fosfatase Alcalina/sangue , Ácidos e Sais Biliares/sangue , Colagogos e Coleréticos/efeitos adversos , Colangite Esclerosante/mortalidade , Feminino , Seguimentos , Humanos , Falência Hepática/mortalidade , Falência Hepática/cirurgia , Transplante de Fígado , Masculino , Pessoa de Meia-Idade , Pacientes Desistentes do Tratamento , Qualidade de Vida , Análise de Sobrevida , Resultado do Tratamento , Ácido Ursodesoxicólico/efeitos adversosRESUMO
There are varying reports on the prevalence of risk factors in porphyria cutanea tarda (PCT). We reviewed 84 patients with PCT in a restricted uptake area in Gothenburg, Sweden and evaluated different potential risk factors for the disease and complications. Besides a thorough medical history, the patients were investigated with urinary porphyrin analyses, transferrin saturation, ferritin and liver tests. Subsamples of patients were tested for antibodies to hepatitis C virus (n = 68), haemochromatosis gene mutations (n = 58) and with the oral glucose tolerance test (n = 31). We found a prevalence of about 1 patient with PCT in 10 000 inhabitants. Nineteen (23%) patients reported heredity for PCT. Identified risk factors were alcohol abuse (38% of male patients), oestrogen treatment (55% of female patients), anti-hepatitis C virus positivity (29% of male patients), diabetes (17%) or impaired glucose tolerance (45% of tested patients) and haemochromatosis gene mutations (57% of tested patients). All patients positive for anti-hepatitis C virus belonged to the non-hereditary group. During follow-up we observed a high incidence of stroke, no case of hepatocellular carcinoma and a normal life expectancy.
Assuntos
Porfiria Cutânea Tardia/epidemiologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Feminino , Ferritinas/sangue , Predisposição Genética para Doença , Hemocromatose/genética , Humanos , Testes de Função Hepática , Masculino , Pessoa de Meia-Idade , Porfiria Cutânea Tardia/sangue , Porfiria Cutânea Tardia/etiologia , Porfiria Cutânea Tardia/genética , Porfiria Cutânea Tardia/patologia , Porfiria Cutânea Tardia/urina , Porfirinas/urina , Prevalência , Estudos Retrospectivos , Fatores de Risco , Suécia/epidemiologiaRESUMO
BACKGROUND: Fatigue has been reported to be common in patients with primary biliary cirrhosis (PBC). Limited data exist on comparison with fatigue in the general population and comparison with patients with other chronic gastrointestinal disorders are lacking. METHOD: We enrolled 96 patients with PBC (87 females); mean age 63 (range 34-65) who completed the Fatigue Impact Scale (FIS). In comparison we included matched controls from the general population, patients with organic (OGD) and functional GI disorders (FGD). Liver function test and the latest liver biopsy were analysed and correlated with fatigue scores. RESULTS: The mean duration of PBC was 7.4 years, the mean bilirubin 13 micromol/l. Twelve per cent of patients had cirrhosis, 29% were in stage I on Ludwig's histology score and 30% and 29% were in stages II and III, respectively. The PBC patients had a median FIS total score of 29 in comparison with 38 in GP controls (P<0.05). Patients with OGD and FGD had more severe fatigue (FIS total score 67 and 59 (P<0.01 compared with PBC)). Fatigue in the PBC patients did not correlate with liver tests and histology stage. CONCLUSION: PBC patients had less severe fatigue measured with the FIS than controls from the GP and patients with OGD and FGD. This study also confirms results of other studies showing no correlation with fatigue in PBC and liver disease parameters. These results argues strongly against fatigue as a specific symptom in PBC.
Assuntos
Fadiga/etiologia , Cirrose Hepática Biliar/complicações , Adulto , Idoso , Depressão/complicações , Depressão/fisiopatologia , Depressão/psicologia , Fadiga/fisiopatologia , Fadiga/psicologia , Feminino , Gastroenteropatias/complicações , Gastroenteropatias/fisiopatologia , Gastroenteropatias/psicologia , Humanos , Síndrome do Intestino Irritável/complicações , Síndrome do Intestino Irritável/fisiopatologia , Síndrome do Intestino Irritável/psicologia , Cirrose Hepática Biliar/fisiopatologia , Cirrose Hepática Biliar/psicologia , Masculino , Fadiga Mental/etiologia , Fadiga Mental/fisiopatologia , Fadiga Mental/psicologia , Pessoa de Meia-Idade , Testes Psicológicos , Qualidade de VidaRESUMO
BACKGROUND & AIMS: Iron overload may be carcinogenic. Patients with hereditary hemochromatosis (HH) are reportedly at a 20-200-fold risk of intrahepatic cancer, but the reported risks for nonhepatobiliary cancers are conflicting. The risk of cancer in heterozygous individuals (estimated allele frequency, 1/10 to 1/20) is unknown. This study aimed to better assess these risks. METHODS: We performed a population-based cohort study of 1847 Swedish patients with HH and 5973 of their first-degree relatives using nationwide, population-based health and census registers. We used standardized incidence ratios (SIRs) as relative risk. RESULTS: With 62 liver cancers and 128 nonhepatobiliary cancers, patients with HH were at a 20-fold risk of liver cancer (SIR, 21; 95% confidence interval [CI], 16-22) but an almost unaltered risk of all other cancers (SIR, 1.2; 95% CI, 1.0-1.4), including nonelevated risks for several gastrointestinal tract cancers. At 10 years of follow-up, the absolute risk of liver cancer was 6% among men and 1.5% among women. With 21 liver cancers and 508 nonhepatobiliary cancers, first-degree relatives were at an unaltered risk of extrahepatic cancer (SIR, 1.0; 95% CI, 0.9-1.1, including unelevated risks for gastrointestinal cancers) but at a modest and historic increased risk of hepatobiliary cancer (SIR, 1.5; 95% CI, 1.0-2.4), the histopathologic spectrum of which differed from the patients. CONCLUSIONS: Patients (particularly men) with HH are at increased risk for hepatocellular cancer, although the magnitude of the risk is lower than previous estimates. Overall cancer risk in first-degree relatives does not seem to be increased.
Assuntos
Carcinoma Hepatocelular/etiologia , Hemocromatose/genética , Neoplasias Hepáticas/etiologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Carcinoma Hepatocelular/genética , Feminino , Hemocromatose/complicações , Proteína da Hemocromatose , Antígenos de Histocompatibilidade Classe I/genética , Humanos , Neoplasias Hepáticas/genética , Masculino , Proteínas de Membrana/genética , Pessoa de Meia-Idade , Mutação , RiscoRESUMO
BACKGROUND/AIMS: This study aims at describing the natural history and outcome of small duct primary sclerosing cholangitis (PSC). METHODS: Thirty-two patients with small duct PSC were studied. The average time taken for diagnosis was 69 (1-168) months. The median follow-up time was 63 (1-194) months. RESULTS: All patients including one who underwent liver transplantation because of end-stage liver disease and hepatocellular carcinoma were alive at follow-up. None developed cholangiocarcinoma. In 27 patients repeated cholangiographic examinations were done after a median time of 72 (12-192) months from first ERCP. Four developed features of large duct PSC. CONCLUSIONS: Small duct PSC rarely progresses to large bile duct PSC and it seems to have a benign course in most patients and no development of cholangiocarcinoma was found.
Assuntos
Colangite Esclerosante/diagnóstico , Colangite Esclerosante/epidemiologia , Adolescente , Adulto , Idoso , Antimetabólitos/uso terapêutico , Azatioprina/uso terapêutico , Neoplasias dos Ductos Biliares/epidemiologia , Ductos Biliares Extra-Hepáticos , Ductos Biliares Intra-Hepáticos , Colangiocarcinoma/epidemiologia , Colangite Esclerosante/tratamento farmacológico , Progressão da Doença , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Risco , Esteroides/uso terapêutico , Suécia/epidemiologia , Resultado do TratamentoRESUMO
BACKGROUND/AIMS: To assess the risk of hepatic and extrahepatic malignancies in a large cohort of Swedish primary sclerosing cholangitis (PSC) patients compared with that of the general Swedish population. METHODS: The study cohort comprised 604 PSC patients identified between 1970 and 1998. Follow-up was provided through linkages to the Swedish Cancer and Death registries. Cumulative incidence of malignancies and standard incidence ratio were calculated with the incidence rates in the Swedish population, taking into account: sex, age and calendar year as comparison group. RESULTS: Median time of follow-up was 5.7 years (range 0-27.8). Seventy-nine percent had concomitant inflammatory bowel disease. The cause of death was cancer in 44%. The frequency of hepatobiliary malignancies was 13.3% (81/604). Thirty-seven percent (30/81) of all hepatobiliary malignancies were diagnosed less than 1 year after the diagnosis of PSC. The risk for hepatobiliary malignancy was increased 161 times, for colorectal carcinoma 10 times and for pancreatic carcinoma 14 times, compared with that of the general population. CONCLUSIONS: In this national-based study including the largest cohort of PSC patients ever presented, the frequency of cholangiocarcinoma is 13%. The risk of hepatobiliary carcinoma is constant after the first year after PSC diagnosis with an incidence rate of 1.5% per year. The risk of pancreatic carcinoma is increased 14 times compared with the general Swedish population. These results are suggestive of an increased risk of pancreatic carcinoma in patients with PSC.