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2.
Turk J Gastroenterol ; 35(6): 475-480, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-39128088

RESUMO

BACKGROUND/AIMS:  Diverticulosis coli is a common disorder of the colon, and a luminal pressure increase in the colon is a proposed mechanism in disease pathogenesis. Toilet types used around the world can be fundamentally categorized into 2 categories: sitting toilets and squatting toilets. Squatting toilets are shown to lead to better puborectalis muscle relaxation, wider anorectal angle, and require less straining compared to sitting toilets. Stemming from this knowledge, we hypothesized that toilet type would play a role in the complex pathogenesis of diverticulosis and that squatting toilets would lower the risk of diverticula formation. MATERIALS AND METHODS:  This study was conducted at Antalya Training and Research Hospital between January 2023 and July 2023. A 1-page questionnaire consisting of demographic data and bowel habits as well as diverticulosis-related parameters was prepared to gather the study data. Colonoscopy results were matched with corresponding questionnaires. RESULTS:  The study population consisted of 929 patients. Advanced age was found to be a risk factor for diverticulosis. Sitting toilet was also found to be a risk factor for diverticulosis in multivariate logistic regression analysis with an odds ratio of 3.36 (95% CI: 1.684-6.705) (P = .001). CONCLUSION:  The results of this study revealed that toilet type is a determining factor in diverticulosis development, as was hypothesized during the conceptualization of the study. Revealing the potential contribution of the toilet type used to the development of a relatively common and impactful disorder like diverticulosis will lay the bedrock for future studies on the topic.


Assuntos
Diverticulose Cólica , Humanos , Feminino , Masculino , Pessoa de Meia-Idade , Fatores de Risco , Idoso , Inquéritos e Questionários , Diverticulose Cólica/etiologia , Postura Sentada , Adulto , Aparelho Sanitário , Fatores Etários , Colonoscopia , Banheiros , Modelos Logísticos , Idoso de 80 Anos ou mais
4.
Acta Neurol Belg ; 124(3): 981-986, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38526645

RESUMO

Migraine is a common and disabling primary headache disorder and inflammation is a proposed factor in the complex ethiology of the disease. Gasdermin D (GSDMD) is a membrane pore-forming protein acting through the caspase system. End result is cell death caused by leakage of intracellular components to extracellular space which also results in inflammation. Stemming from this knowledge, the potential role of GSDMD in migraine was investigated in this prospective study. This prospective study was conducted between September 2022 to April 2023. 47 patients with migraine were designated as the patient group, whereas 47 healthy volunteers were designated as the control group. Serum GSDMD levels of both groups were compared, with an additional comparison between migraine patients during symptom-free and attack periods. Migraine related characteristics of the patients were also included in the study. Median GSDMD levels of the patient and control group did not reveal a significant difference. Nausea, vomiting and severity of headache were found to be correlated with GSDMD levels in migraine patients. Patients with nausea revealed a higher GSDMD level compared to patients without nausea during both symptom-free and attack periods (p = 0.021 and p = 0.01, respectively). Nausea was correlated to higher GSDMD levels in the patient population during symptom-free period (p = 0.030). The severity of pain was positively correlated with GSDMD levels during the attack period (p < 0.001). Gasdermin family and GSDMD in particular are promising prospects for therapy in a wide spectrum of disorders. Gasdermin proteins are candidates to be the focus for future studies both related to pathogenesis and drug therapy in migraine and varying inflammatory-driven clinical pictures.


Assuntos
Transtornos de Enxaqueca , Proteínas de Ligação a Fosfato , Humanos , Transtornos de Enxaqueca/sangue , Masculino , Feminino , Proteínas de Ligação a Fosfato/sangue , Adulto , Estudos Prospectivos , Pessoa de Meia-Idade , Inflamação/sangue , Proteínas Citotóxicas Formadoras de Poros/sangue , Náusea/etiologia , Adulto Jovem , Gasderminas
5.
Indian J Med Microbiol ; 48: 100553, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38403267

RESUMO

Crohn's disease is a chronic inflammatory disease of the gastrointestinal tract. Immunosuppressive therapy is the main treatment modality in Crohn's disease. Herpes zoster (HZ), caused by Varicella-zoster virus, is a relatively common albeit burdensome clinical picture mainly affecting adult population with immunosuppressive status. In this paper, we aimed to report a Crohn's disease patient with HZ to raise awareness on vaccination. There are commercially available vaccines that are shown to be safe and effective against HZ reactivation. Crohn's disease patients should be evaluated and informed about preventive options against HZ to prevent unwanted HZ-related complications.


Assuntos
Doença de Crohn , Herpes Zoster , Humanos , Herpes Zoster/prevenção & controle , Vacinação , Herpesvirus Humano 3/imunologia , Adulto , Vacina contra Herpes Zoster/administração & dosagem , Vacina contra Herpes Zoster/imunologia , Masculino , Feminino
6.
Pancreatology ; 24(2): 206-210, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38262841

RESUMO

Acute pancreatitis (AP) is a serious and complex disorder with varying disease course and severity. Early and prompt interventions are crucial in management of AP. Vitamin D, being a prominent actor in calcium metabolism, also takes part in immunity and thus in immune-system related disorders, ranging from infections to cancer. In this study, the role of vitamin D status of a patient on the severity of AP was investigated. This study was conducted between June 2021 to August 2022 with a total of 315 patients. Blood samples were obtained upon admission. A 25-(OH)D3 level less than 10 ng/ml was defined as vitamin D deficiency. 10-19 ng/ml was defined as vitamin D insufficiency whereas 20 ng/ml or above was considered to be sufficient. Scoring systems (Ranson score, CTSI, BISAP, Revised Atlanta Classification (RAC) were applied. Serum 25-(OH)D3 levels of patients with AP were found to be negatively correlated with severity of the disease according to RAC (p < 0.001). In concordance to this finding, both Ranson score and BISAP were found to be statistically significantly related to 25-(OH)D3 levels. Both scoring systems revealed higher scores in patients with insufficient or deficient levels of 25-(OH)D3. Serum 25-(OH)D3 levels were not found to be related to intensive care unit admission or mortality. This study revealed that serum 25-(OH)D3 level is related to the severity of AP. In the future, interventional studies with vitamin D therapy in otherwise serum 25-(OH)D3 deficient AP patients might reveal a new potential therapeutic agent in this mechanically complex, burdensome disorder.


Assuntos
Pancreatite , Humanos , Estudos Prospectivos , Doença Aguda , Vitamina D , Vitaminas/uso terapêutico
8.
BMC Neurol ; 22(1): 187, 2022 May 21.
Artigo em Inglês | MEDLINE | ID: mdl-35597897

RESUMO

BACKGROUND/AIM: White matter lesions (WML) are more frequently observed in migraine patients than in the average population. Associations between Helicobacter pylori (H. pylori) infection and different extraintestinal pathologies have been identified. Here, we aimed to investigate the association between H. pylori infection and WML in patients diagnosed with episodic migraine. MATERIALS AND METHODS: A retrospective study was conducted with 526 subjects with a diagnosis of episodic migraine. Hyperintensity of WML had been previously evaluated in these patients with brain magnetic resonance imaging (MRI) examinations. Previous endoscopic gastric biopsy histopathological examination of the same patients and reports on H. pylori findings were recorded. The demographic characteristics of the patients, such as age, gender and chronic systemic diseases such as hypertension and diabetes mellitus (DM) were recorded. Statistical evaluation was made. RESULTS: Evaluation was made among 526 migraine patients who met the inclusion criteria, comprising 397 (75.5%) females and 129 (24.5%) males with a mean age of 45.57 ± 13.46 years (range, 18-69 years). WML was detected on brain MRI in 178 (33.8%) patients who were also positive for H. pylori (p <  0.05). Subjects who are H. pylori-positive with migraine, WML were observed at a 2.5-fold higher incidence on brain MRI (odds ratio: 2.562, 95% CI 1.784-3.680). WML was found to be more significant in patients with hypertension and migraine than those without (p <  0.001). Older age was also found to be associated with WML (OR = 1.07, 95% CI: 0.01-0.04, p <  0.001). The age (p <  0.001), H. pylori (p <  0.001), hypertension (p <  0.001), and hypertension + DM (p <  0.05), had significant associations in predicting WML according to the multivariate logistic regression analysis. The presence of hypertension had a higher odds ratio value than the other variables. CONCLUSION: It was concluded that H. pylori infection, as a chronic infection, can be considered a risk factor in developing WML in subjects with migraine.


Assuntos
Diabetes Mellitus , Infecções por Helicobacter , Helicobacter pylori , Hipertensão , Transtornos de Enxaqueca , Substância Branca , Adulto , Feminino , Infecções por Helicobacter/complicações , Infecções por Helicobacter/epidemiologia , Infecções por Helicobacter/patologia , Humanos , Hipertensão/complicações , Hipertensão/epidemiologia , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Transtornos de Enxaqueca/diagnóstico por imagem , Transtornos de Enxaqueca/epidemiologia , Transtornos de Enxaqueca/patologia , Estudos Retrospectivos , Substância Branca/patologia
10.
Exp Clin Transplant ; 17(5): 632-637, 2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-31050621

RESUMO

OBJECTIVES: Hepatocellular carcinoma is the most frequent primary malignant tumor of the liver and the third most common cause of all cancer-related mortalities. There is a need to develop new strategies to prevent hepatocellular carcinoma, as the incidence of this cancer continues to increase despite all advancements. In this study, our aim was to determine the effects of propranolol treatment on the incidence of hepatocellular carcinoma in cirrhotic patients waiting for liver transplant. MATERIALS AND METHODS: We retrospectively reviewed the data of patients waiting for liver transplant with cirrhosis due to various causes registered at the Hepatocellular Carcinoma Surveillance Program between June 2011 and December 2017 in our center. These data were compared between patients using propranolol and those not using propranolol. RESULTS: Of the 231 patients, 135 (58.4%) were male and 96 (41.6%) were female. The mean age was 58.1 ± 14 years. We noted that 153 of total patients (66.2%) were using propranolol. Three patients (2%) were using 20 mg propranolol, 125 (81.7%) were using 40 mg propranolol, 10 (6.5%) were using 60 mg propranolol, and 15 (9.8%) were using 80 mg propranolol. Of total patients, 36 (15.6%) developed hepatocellular carcinoma, including in 12 patients (7.8%) using propranolol and 24 patients (30.8%) who did not use this agent (P < .001). Thus, the hepatocellular carcinoma frequency was 5.22 times lower in patients receiving propranolol than in those not receiving propranolol. CONCLUSIONS: Although causes of cirrhosis and initial stages were similar in both groups using and not using propranolol, incidence of hepatocellular carcinoma was significantly lower in the propranolol group than in the group without propranolol. This result showed that propranolol treatment has a protective effect for hepatocellular carcinoma in patients waiting for liver transplant with cirrhosis.


Assuntos
Carcinoma Hepatocelular/epidemiologia , Carcinoma Hepatocelular/prevenção & controle , Cirrose Hepática/cirurgia , Neoplasias Hepáticas/epidemiologia , Neoplasias Hepáticas/prevenção & controle , Transplante de Fígado , Propranolol/uso terapêutico , Adulto , Idoso , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Vigilância da População , Estudos Retrospectivos , Centros de Atenção Terciária , Listas de Espera
11.
Exp Clin Transplant ; 17(1): 52-58, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30719954

RESUMO

OBJECTIVES: The introduction of direct-acting antiviral agents has allowed significant chances for treatment for difficult-to-treat populations. This study aimed to investigate the efficacy, tolerability, and safety of these therapies in both patients with end-stage renal disease and kidney transplant recipients with chronic hepatitis C virus infection. MATERIALS AND METHODS: This study was a retrospective analysis with prospective follow-up of patients. The antiviral combination of ombitasvir 25 mg, paritaprevir 75 mg, ritonavir 50 mg, and dasabuvir 50 mg was prescribed to patients with end-stage renal disease or kidney transplant recipients with noncirrhotic or compensated cirrhotic liver disease. The other antiviral combination consisted of sofosbuvir 400 mg and ledipasvir 90 mg, which was recommended to patients with decompensated cirrhosis or those who could not tolerate the first combination regimen. Ribavirin was given to all patients with genotype 1a hepatitis C virus infection. All clinical and laboratory data were recorded at week 4, at end of the treatment, and at 12 weeks after completion of treatment. RESULTS: In terms of efficacy, sustained virologic response at 12 weeks was achieved in 94% of patients in the end-stage renal disease group and 92% of patients in the kidney transplant group. In terms of tolerability, antiviral treatment was well tolerated in both groups. Cardiac arrest and cerebrovascular accident were seen in the end-stage renal disease group; severe mucositis and glossitis were seen in the kidney transplant group. Hospitalization was needed in 2 patients for treatment of drug interactions with tacrolimus and sirolimus. Renal allograft function worsened in 2 patients, with 1 patient having biopsyproven antibody-mediated rejection. CONCLUSIONS: We observed great efficacy and safety in both kidney transplant recipients and patients with end-stage renal disease with these agents in treatment of chronic hepatitis C. However, clinicians should remain aware of drug interactions and adverse events in this fragile patient population.


Assuntos
Anilidas/uso terapêutico , Antivirais/uso terapêutico , Carbamatos/uso terapêutico , Hepacivirus/efeitos dos fármacos , Hepatite C Crônica/tratamento farmacológico , Falência Renal Crônica/complicações , Falência Renal Crônica/cirurgia , Transplante de Rim , Compostos Macrocíclicos/uso terapêutico , Ritonavir/uso terapêutico , Sulfonamidas/uso terapêutico , Uracila/análogos & derivados , 2-Naftilamina , Adulto , Idoso , Anilidas/efeitos adversos , Antivirais/efeitos adversos , Carbamatos/efeitos adversos , Ciclopropanos , Interações Medicamentosas , Quimioterapia Combinada , Feminino , Genótipo , Hepacivirus/genética , Hepatite C Crônica/complicações , Hepatite C Crônica/diagnóstico , Hepatite C Crônica/virologia , Humanos , Imunossupressores/efeitos adversos , Falência Renal Crônica/diagnóstico , Transplante de Rim/efeitos adversos , Lactamas Macrocíclicas , Compostos Macrocíclicos/efeitos adversos , Masculino , Pessoa de Meia-Idade , Prolina/análogos & derivados , Estudos Prospectivos , Estudos Retrospectivos , Ribavirina/uso terapêutico , Fatores de Risco , Ritonavir/efeitos adversos , Sulfonamidas/efeitos adversos , Resposta Viral Sustentada , Fatores de Tempo , Resultado do Tratamento , Uracila/efeitos adversos , Uracila/uso terapêutico , Valina
15.
Springerplus ; 5: 128, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-26933627

RESUMO

Experience with endoscopic retrograde cholangiopancreatography in the pediatric population is limited. The aim of this study was to evaluate the outcomes of ERCP in the pediatric population performed by adult gastroenterologists with standard duodenoscopes. This study is a structured retrospective review of endoscopic reports, computerized and paper medical records, and radiographic images of patients under the age of 18 who underwent ERCP for any indication at a tertiary referral centre. Data regarding demographic characteristics and medical history of patients, indications, technical success rate, final clinical diagnosis, and complications were analyzed. Forty-eight children with a mean age of 13 years (range 2-17) underwent a total of 65 ERCPs. The indications of ERCP were as follows; suspected choledocholithiasis (55 %), post-liver transplantation anastomotic biliary strictures (21 %), post-surgical bile duct injury (10 %), choledochal cyst (2 %), recurrent or chronic pancreatitis (10 %), and trauma (2 %). The cannulation success rate in the overall procedure was 93.8 %. Therapeutic interventions were performed in 70.7 % of patients. Post ERCP pancreatitis was the most common complication occurring in 9.2 % of patients, and no procedure related mortality occurred. When performed by well-trained adult gastroenterologists, the use of endoscopic retrograde cholangiopancreatography with standard duodenoscopes is safe in pediatric population.

16.
Exp Clin Transplant ; 13 Suppl 3: 36-40, 2015 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-26640908

RESUMO

OBJECTIVES: We investigated the effect of hepatitis B virus in kidney transplant patients in terms of patient care and survival. MATERIALS AND METHODS: We retrospectively analyzed kidney transplant patients from 1993 to 2013. A control group with negative serology was selected. The hepatitis B virus-positive group was divided into 2 subgroups based on serologic status, treatments, and treatment responses. Group A had viral suppression, and group B had hepatitis B virus DNA persistence. Overall and allograft survival rates were compared. RESULTS: We identified 32 hepatitis B virus-positive and 74 hepatitis B virus-negative patients. Positive group was treated with lamivudine (n = 23), lamivudine plus entecavir (n = 4), lamivudine plus tenofovir (n = 4), or lamivudine plus entecavir and tenofovir (n = 1). In group A (n = 15), antiviral treatment was given based on the presence of either hepatitis B surface antigen with negative hepatitis B virus DNA (n = 11) or hepatitis B virus DNA positivity (n = 4). Group B patients (n = 17) received antiviral treatment for persistence of hepatitis B virus DNA (n = 7) or for viral reactivation (ie, recurrence of hepatitis B virus DNA) (n = 10). Groups A and B did not differ significantly in terms of graft or overall survival. Liver biopsy was performed in 17 patients; 3 patients had high-grade fibrosis or cirrhosis, and 14 patients had normal histology or mild hepatitis. Median graft survival time was longer in positive group (69.5 mo vs 54 mo; P = .007). Five- and 10-year overall survival rates were comparable (89%-84% vs 96%-96%; P = .107). CONCLUSIONS: Hepatitis B virus-positive kidney transplant patients have increased liver transaminase levels, longer graft survival times, and similar median overall survival rates compared with hepatitis B virus-negative patients.


Assuntos
Sobrevivência de Enxerto , Vírus da Hepatite B/patogenicidade , Hepatite B/virologia , Falência Renal Crônica/cirurgia , Transplante de Rim , Adulto , Idoso , Antivirais/uso terapêutico , Feminino , Hepatite B/complicações , Hepatite B/diagnóstico , Hepatite B/tratamento farmacológico , Vírus da Hepatite B/efeitos dos fármacos , Humanos , Estimativa de Kaplan-Meier , Falência Renal Crônica/complicações , Falência Renal Crônica/diagnóstico , Falência Renal Crônica/mortalidade , Transplante de Rim/efeitos adversos , Transplante de Rim/mortalidade , Masculino , Pessoa de Meia-Idade , Sistema de Registros , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Adulto Jovem
17.
Exp Clin Transplant ; 13 Suppl 3: 55-7, 2015 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-26640913

RESUMO

OBJECTIVES: We aimed to investigate the colonoscopy findings in patients after kidney transplant. MATERIALS AND METHODS: We retrospectively analyzed kidney transplant patients who had colonoscopy examinations for various indications between 2011 and 2015. RESULTS: Eighty-one patients (25 women and 56 men) with a mean age of 39 years (range, 18-64 y) were identified. Mean follow-up after transplant was 9 years (range, 1-29 y). The most common indications for colonoscopy were diarrhea (41%), anemia (29%), gastrointestinal bleeding (12%), abdominal pain (12%), and unexplained weight loss (6%). Either colitis or ileitis or both were diagnosed in 20 patients (25%), whereas polyps were found in 9 patients (11%). One patient presented with hematochezia, which was diagnosed as cytomegalovirus colitis. The remaining cases of colitis or ileitis were diagnosed as nonspecific inflammation. Indications for colonoscopy were not correlated with age, duration after transplant, or use of immunosuppressive drugs. A subgroup analysis for mycophenolate-induced colitis found that 88% of patients used mycophenolate, but presence of colitis or ileitis had no statistical correlation with its use. In patients with poor gastrointestinal symptoms, the only significant predictor of presence of colitis or ileitis was a high C-reactive protein value (> 5 mg/dL; P = .02). CONCLUSIONS: Incidence of colitis and/or ileitis is a relatively common finding in patients after kidney transplant. Opportunistic infections, mycophenolate use, and mild degree of indeterminate colitis or ileitis disease may be the underlying condition. Cytomegalovirus infection should be screened in all recipients because it may cause serious complications or death in chronically immunocompromised patients.


Assuntos
Colite/patologia , Colo/patologia , Colonoscopia , Infecções por Citomegalovirus/patologia , Ileíte/patologia , Íleo/patologia , Pólipos Intestinais/patologia , Transplante de Rim/efeitos adversos , Infecções Oportunistas/patologia , Adolescente , Adulto , Colite/imunologia , Colite/virologia , Colo/imunologia , Colo/virologia , Infecções por Citomegalovirus/imunologia , Infecções por Citomegalovirus/virologia , Feminino , Humanos , Ileíte/imunologia , Íleo/imunologia , Hospedeiro Imunocomprometido , Imunossupressores/efeitos adversos , Pólipos Intestinais/imunologia , Masculino , Pessoa de Meia-Idade , Infecções Oportunistas/imunologia , Infecções Oportunistas/virologia , Valor Preditivo dos Testes , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Adulto Jovem
18.
Exp Clin Transplant ; 13 Suppl 3: 93-6, 2015 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-26640924

RESUMO

OBJECTIVES: We report the outcomes of endoscopic retrograde cholangiopancreatography procedures performed for diagnostic and therapeutic purposes in patients who had undergone kidney transplant. MATERIALS AND METHODS: We retrospectively evaluated the records of kidney transplant patients for January 1993 to December 2014. Endoscopic retrograde cholangiopancreatography was carried out using an Olympus JF240 duodenoscope (tip outer diameter 12.6 mm, working channel diameter 3.2 mm). The procedures were performed by Department of Anesthesiology staff while the patients were deeply sedated, given a combination of midazolam plus propofol. RESULTS: Data from 21 kidney transplant patients (16 men and 5 women; mean age at endoscopic retrograde cholangiopancreatography 42.6 ± 23.4 y) were evaluated. A total of 23 endoscopic retrograde cholangiopancreatography procedures were performed. The indications were choledocholithiasis in 6 patients (28.6%), common bile duct dilatation plus liver enzyme elevations in 4 patients (19%), liver enzyme elevation alone in 4 (19%), biliary necrotizing pancreatitis in 2 (9.6%), and cholangitis in 5 (23.8%). Hepatobiliary ultrasonography findings showed that 3 patients (14.3%) had absence of gallbladder owing to cholecystectomy, 14 (66.7%) had gallstones, 1 (4.7%) had gallstones with cholecystitis findings, and 3 (14.3%) were normal. Endoscopic retrograde cholangiopancreatography findings were normal in 4 patients (19%), showed cholangitis in 2 (9.6%), choledocholithiasis in 10 (47.6%), bile duct wall irregularities in 2 (9.6%), dilated common bile duct in 2 (9.6%), and cholangiocarcinoma in 1 (4.7%). Sphincterotomy was performed in 16 patients. None of the patients who underwent endoscopic retrograde cholangiopancreatography developed any complications, for example, acute pancreatitis, bleeding, duodenal, or bile duct perforation. CONCLUSIONS: In kidney transplant patients, endoscopic retrograde cholangiopancreatography is safe and able to provide substantial information for managing biliopancreatic diseases.


Assuntos
Doenças Biliares/diagnóstico , Doenças Biliares/terapia , Colangiopancreatografia Retrógrada Endoscópica , Transplante de Rim , Pancreatopatias/diagnóstico , Pancreatopatias/terapia , Adulto , Idoso , Colangiopancreatografia Retrógrada Endoscópica/efeitos adversos , Colangiopancreatografia Retrógrada Endoscópica/instrumentação , Sedação Profunda , Duodenoscópios , Desenho de Equipamento , Feminino , Humanos , Hipnóticos e Sedativos/administração & dosagem , Masculino , Midazolam/administração & dosagem , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Propofol/administração & dosagem , Estudos Retrospectivos , Fatores de Risco , Resultado do Tratamento , Turquia , Adulto Jovem
19.
Exp Clin Transplant ; 13 Suppl 3: 107-9, 2015 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-26640927

RESUMO

We present a rare case of progressive familial intrahepatic cholestasis within a family. A 34-yearold female became a living-related liver transplant donor for her son, who had the disease. Nine years after the transplant, the mother developed severe intrahepatic cholestasis, for which she was evaluated after using an oral contraceptive drug. She presented with jaundice, pruritus, and increased bilirubin levels, together with elevated gamma glutamyl transferase and alkaline phosphatase levels. A liver biopsy revealed findings consistent with intrahepatic cholestasis. However, despite follow-up management and cessation of the insulting drug, her total bilirubin count continuously increased to 20 mg/dL and was accompanied by intractable pruritus. A total of 9 plasmapheresis sessions were performed, and she was started on a regimen of ursodeoxycholic acid (13 mg/kg/d) and cholestyramine (4 g, 3 times daily). The clinical and laboratory picture dramatically improved following cessation of the oral contraceptive, plasmapheresis sessions, and drug treatment. The patient's cholestasis normalized within 3 months, and she recovered uneventfully. A genetic analysis of the whole family revealed that both parents were heterozygous for the mutation c.124G>A in ABCB11, and the son was homozygous for this mutation. These findings supported varying degrees of bile salt export pump deficiency in the family members. Defective bile salt excretory system function can result in a wide spectrum of clinical presentations, ranging from progressive familial intrahepatic cholestasis requiring liver transplant to late-onset drug-induced cholestasis. Our findings suggest that, in a heterozygous carrier of a progressive familial intrahepatic cholestasis mutation, drug-induced cholestasis is responsive to treatment, after which the clinical picture can normalize within 3 months.


Assuntos
Transportadores de Cassetes de Ligação de ATP/genética , Doença Hepática Induzida por Substâncias e Drogas/genética , Colestase Intra-Hepática/genética , Anticoncepcionais Orais Hormonais/efeitos adversos , Transplante de Fígado/métodos , Doadores Vivos , Mutação , Membro 11 da Subfamília B de Transportadores de Cassetes de Ligação de ATP , Adulto , Bilirrubina/sangue , Biomarcadores/sangue , Biópsia , Doença Hepática Induzida por Substâncias e Drogas/diagnóstico , Doença Hepática Induzida por Substâncias e Drogas/terapia , Colestase Intra-Hepática/diagnóstico , Análise Mutacional de DNA , Feminino , Predisposição Genética para Doença , Hepatectomia , Heterozigoto , Humanos , Fenótipo , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento
20.
Exp Clin Transplant ; 13 Suppl 3: 131-6, 2015 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-26640933

RESUMO

OBJECTIVES: Spontaneous bacterial peritonitis, unless originating from surgery or an intra-abdominal source, is an infection diagnosed by neutrophil counts greater than 250/mm³ in ascites. Spontaneous bacterial peritonitis is the most common infection among patients hospitalized with cirrhosis, with a prevalence of 9% and a risk of development among all patients with cirrhosis within 1 year of 10%. No valid parameters have been defined to predict the mortality related to spontaneous bacterial peritonitis. Unless it is treated, the mortality rate as a result of spontaneous bacterial peritonitis is 50%, and serious complications may arise. MATERIALS AND METHODS: Medical records from 29 patients on the deceased-donor transplant waiting list and receiving treatment at the Baskent University Hospital Gastroenterology Clinic for cirrhotic ascites infection between 1996 and 2013 were analyzed. Demographic information, paracentesis findings, clinical follow-up, and treatment results were reviewed and collected from patient medical records, with data recorded to the research form. RESULTS: In our patient group, 72.4% were men and the average age was 46.6 years. Most of our patients were at advanced stage, with 55.2% having a Child-Pugh score of C and an average Model for End-Stage Liver Disease score of 17 ± 4.1. We found that 34.5% of the patients received prophylactic treatment for spontaneous bacterial peritonitis, 72.4% received a proton pump inhibitor, and 82.8% had treatment with intravenous albumin support at the time of diagnosis. Albumin treatment showed no effect on mortality. Mortality rate among patients with Child-Pugh score of C was 81.3%. CONCLUSIONS: Existence of chronic renal failure, liver graft surgery, and hepatocellular cancer did not seem to have a significant effect on patient mortality. The albumin treatment protocol showed no significant difference despite common belief among physicians.


Assuntos
Ascite/mortalidade , Infecções Bacterianas/mortalidade , Doença Hepática Terminal/mortalidade , Hospitalização , Cirrose Hepática/mortalidade , Peritonite/mortalidade , Administração Intravenosa , Adulto , Idoso , Albuminas/administração & dosagem , Ascite/diagnóstico , Ascite/microbiologia , Ascite/terapia , Infecções Bacterianas/diagnóstico , Infecções Bacterianas/microbiologia , Infecções Bacterianas/terapia , Doença Hepática Terminal/diagnóstico , Doença Hepática Terminal/etiologia , Doença Hepática Terminal/cirurgia , Feminino , Hospitais Universitários , Humanos , Cirrose Hepática/complicações , Cirrose Hepática/diagnóstico , Cirrose Hepática/cirurgia , Transplante de Fígado/mortalidade , Masculino , Prontuários Médicos , Pessoa de Meia-Idade , Paracentese , Peritonite/diagnóstico , Peritonite/microbiologia , Peritonite/terapia , Inibidores da Bomba de Prótons , Sistema de Registros , Estudos Retrospectivos , Fatores de Risco , Índice de Gravidade de Doença , Resultado do Tratamento , Turquia , Listas de Espera/mortalidade
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