RESUMEN
Wilson's disease typically presents symptoms associated with liver damage or neuropsychiatric disturbances, while endocrinologic abnormalities are rare. We report an unprecedented case of hypopituitarism in a patient with Wilson's disease. A 40-year-old woman presented with depression, general weakness and anorexia. Laboratory tests and imaging studies were compatible with liver cirrhosis due to Wilson's disease. Basal hormone levels and pituitary function tests indicated secondary hypothyroidism and adrenal insufficiency due to hypopituitarism. Brain MRI showed T2 hyperintense signals in both basal ganglia and midbrain but the pituitary imaging was normal. She is currently receiving chelation therapy along with thyroid hormone and steroid replacement. There may be a relationship between Wilson's disease and hypopituitarism. Copper deposition or secondary neuronal damage in the pituitary may be a possible explanation for this theory.
Asunto(s)
Adulto , Femenino , Humanos , Insuficiencia Suprarrenal/diagnóstico , Encéfalo/diagnóstico por imagen , Depresión/etiología , Degeneración Hepatolenticular/complicaciones , Hipopituitarismo/complicaciones , Hipotiroidismo/diagnóstico , Cirrosis Hepática/complicaciones , Imagen por Resonancia Magnética , Esteroides/uso terapéutico , Hormona Liberadora de Tirotropina/uso terapéuticoRESUMEN
Wilson's disease typically presents symptoms associated with liver damage or neuropsychiatric disturbances, while endocrinologic abnormalities are rare. We report an unprecedented case of hypopituitarism in a patient with Wilson's disease. A 40-year-old woman presented with depression, general weakness and anorexia. Laboratory tests and imaging studies were compatible with liver cirrhosis due to Wilson's disease. Basal hormone levels and pituitary function tests indicated secondary hypothyroidism and adrenal insufficiency due to hypopituitarism. Brain MRI showed T2 hyperintense signals in both basal ganglia and midbrain but the pituitary imaging was normal. She is currently receiving chelation therapy along with thyroid hormone and steroid replacement. There may be a relationship between Wilson's disease and hypopituitarism. Copper deposition or secondary neuronal damage in the pituitary may be a possible explanation for this theory.
Asunto(s)
Adulto , Femenino , Humanos , Insuficiencia Suprarrenal/diagnóstico , Encéfalo/diagnóstico por imagen , Depresión/etiología , Degeneración Hepatolenticular/complicaciones , Hipopituitarismo/complicaciones , Hipotiroidismo/diagnóstico , Cirrosis Hepática/complicaciones , Imagen por Resonancia Magnética , Esteroides/uso terapéutico , Hormona Liberadora de Tirotropina/uso terapéuticoRESUMEN
BACKGROUND/AIMS: Globus is a persistent or intermittent non-painful sensation of a lump or foreign body in the throat. Given the benign nature of the condition and the association of gastroesophageal reflux disease, empirical therapy with proton pump inhibitor seems reasonable for patients with typical globus. The aim of this study was to investigate the clinical predictors for symptom response to short-term proton pump inhibitor treatment in patients with globus symptom. METHODS: Fifty-four patients with globus symptom were enrolled prospectively. All patients were treated with pantoprazole 40 mg daily for 4 weeks. Treatment response was defined as a > 50% reduction in symptom scores between symptom assessments. Univariate and multivariate logistic regression analysis between responders and non-responders was performed to identify variables predicting response to pantoprazole treatment. RESULTS: Of the 54 consecutive patients considered, 13 were excluded on the basis of exclusion criteria and/or refusal to participate in the study. Finally, 41 patients were included in this study. After 4-week pantoprazole treatment, 22 patients (53.7%) were classified as responders. On multivariate analysis, the presence of reflux symptom was associated with a higher response rate to 4-week pantoprazole treatment (OR, 68.56; P = 0.043), and long symptom duration (> or = 3 months) were associated with a lower response rate to pantoprazole treatment (OR, 0.03; P = 0.034). CONCLUSIONS: Presence of reflux symptom and short symptom duration were independent predictors of responsiveness to 4-week pantoprazole treatment in patients with globus.
Asunto(s)
Humanos , 2-Piridinilmetilsulfinilbencimidazoles , Cuerpos Extraños , Reflujo Gastroesofágico , Modelos Logísticos , Análisis Multivariante , Faringe , Estudios Prospectivos , Inhibidores de la Bomba de Protones , Bombas de Protones , Protones , Negativa a Participar , Sensación , Evaluación de SíntomasRESUMEN
Pseudoachalasia secondary to primary squamous cell carcinoma (SCC) of the liver is extremely rare and has not been reported until now. Here, we report a unique case of primary SCC of the liver initially presenting with progressive dysphagia along with short periods of significant weight loss. A 58-year-old man initially presented with progressive dysphagia along with significant weight loss over brief periods of time. The radiographic and manometric findings were consistent with achalasia. Subsequent esophagogastroduodenoscopy revealed a moderately dilated esophagus without evidence of neoplasm or organic obstruction. However, firm resistance was encountered while traversing the esophagogastric junction (EGJ), although no mucosal lesion was identified. Due to the clinical suspicion of the presence of a malignant tumor, endoscopic ultrasonography (EUS) and computed tomography scans of the chest and abdomen were obtained. A huge hepatic mass with irregular margins extending to the EGJ was found. EUS-guided fine-needle aspiration was performed, and the mass was diagnosed as a primary SCC of the liver by immunohistochemical staining.
Asunto(s)
Humanos , Persona de Mediana Edad , Abdomen , Biopsia con Aguja Fina , Carcinoma de Células Escamosas , Trastornos de Deglución , Endoscopía del Sistema Digestivo , Endosonografía , Acalasia del Esófago , Unión Esofagogástrica , Esófago , Hígado , Tórax , Pérdida de PesoRESUMEN
Endoscopic retrograde biliary drainage (ERBD) is useful for the palliative decompression of biliary obstruction. However, the complications of ERBD include cholangitis, hemorrhage, acute pancreatitis, obstruction of the stent, and duodenal perforation. Pressure necrosis on the duodenal mucosa by the stent may contribute to perforation. Although duodenal perforation following ERBD is very rare compared to other complications, it can result in a fatal outcome. Recent reports describe nonsurgical treatment for small gastrointestinal perforation with localized peritonitis and suggest that endoclipping may be appropriate in the management of a well selected group of patients with iatrogenic perforation. We describe a case of duodenal perforation secondary to ERBD that was successfully treated with approximating using endoclip and detachable snare.
Asunto(s)
Femenino , Humanos , Persona de Mediana Edad , Conductos Biliares Extrahepáticos , Enfermedades de las Vías Biliares/complicaciones , Colangiopancreatografia Retrógrada Endoscópica , Drenaje , Enfermedades Duodenales/diagnóstico , Neoplasias de la Vesícula Biliar/diagnóstico , Perforación Intestinal/diagnóstico , Plásticos , Stents/efectos adversos , Tomografía Computarizada por Rayos XRESUMEN
Gastric mucosa-associated lymphoid tissue (MALT) lymphoma represents approximately 40% of gastric lymphomas, and its incidence is increasing. An early diagnosis for gastric MALT lymphoma is important, but not easy due to non-specific symptoms and endoscopic findings. Diagnosis is based on the histopathologic evaluation of multiple, deep and repeated biopsies taken from normal and any abnormal appearing sites of the stomach. In addition, the presence of Helicobacter pylori (H. pylori) infection must be determined to determine therapeutic approach. Endoscopic ultrasonography (EUS) is essential for the evaluation of regional lymph nodes and the depth of tumor invasion in the gastric wall, for predicting response to H. pylori eradication, and for monitoring tumor regression or recurrence. The eradication of H. pylori is recommended as an initial treatment for low-grade gastric MALT lymphoma with H. pylori infection. Both radiation therapy and chemotherapy are suitable alternative options for H. pylori-negative, refractory, or high-grade gastric MALT lymphoma. But, the role of surgery is diminishing. After treatment, strict endoscopic regular follow-up including EUS is recommended with multiple biopsies. However, controversy remains regarding the best diagnosis, treatment and follow-up strategy for this disease.
Asunto(s)
Humanos , Biopsia con Aguja Fina , Endosonografía , Gastroscopía , Infecciones por Helicobacter/complicaciones , Linfoma de Células B de la Zona Marginal/complicaciones , Neoplasias Gástricas/complicacionesRESUMEN
Biliary papillomatosis is a rare disease with a high risk of recurrence and malignant transformation. Therapeutic options include partial hepatectomy, Whipple's procedure and liver transplantation. If there is no surgical option left due to several reasons, local palliative procedures such as biliary stenting and drainage for the treatment of cholestasis are considered, but tumor growth cannot be influenced. Photodynamic therapy might be a new additional, palliative option for patients with biliary papillomatosis who are not eligible for surgery. Benign biliary stricture is a rare complication of photodynamic therapy. We report here a case of a 63-year-old male who developed benign biliary stricture after photodynamic therapy using the photosensitizer photofrin.
Asunto(s)
Humanos , Masculino , Persona de Mediana Edad , Colestasis , Constricción Patológica , Éter de Dihematoporfirina , Drenaje , Hepatectomía , Trasplante de Hígado , Papiloma , Fotoquimioterapia , Enfermedades Raras , Recurrencia , StentsRESUMEN
This is the first case report to describe a 44-year-old woman with a history of advanced hepatocellular carcinoma who developed toxic epidermal necrolysis (TEN) clinically after taking 400 mg sorafenib (Nexavar(R), BAY 43-9006) and tosufloxacin orally once per day. Both sorafenib and tosufloxacin were eventually discontinued, and the TEN resolved with corticosteroids and supportive treatment. Clinical physicians should be aware of this possible complication so that early interventions can be made.
Asunto(s)
Adulto , Femenino , Humanos , Corticoesteroides , Bahías , Carcinoma Hepatocelular , Intervención Educativa Precoz , Síndrome de Stevens-Johnson , Fluoroquinolonas , Naftiridinas , Niacinamida , Compuestos de FenilureaRESUMEN
BACKGROUND/AIMS: Autologous stem cell transplantation (ASCT) has become the treatment of choice for patients with multiple myeloma (MM). Studies have shown that maintenance treatment with interferon-alpha is associated with improved survival rates following ASCT. However, despite these recent advances in regimes, relapses are inevitable; thus, the prediction of relapse following ASCT requires assessment. METHODS: We retrospectively analyzed 39 patients who received ASCT between 2003 and 2008. All patients received chemotherapy with vincristine, adriamycin, and dexamethasone (VAD), and ASCT was performed following high-dose melphalan conditioning therapy. We evaluated the influence of the post-transplant day +14 (D+14) bone marrow plasma cell percent (BMPCp) (> or = 2 vs. or = 50 vs. or = 50% at diagnosis, CR after 3 cycles of VAD therapy, del (13q) by fluorescence in situ hybridization, and BMPCp > or = 2% at post-transplant D+14 were correlated with PFS and OS. A multivariate analysis revealed that a post-transplant D+14 BMPCp > or = 2% (PFS, hazard ratio [HR] = 4.426, p = 0.008; OS, HR = 3.545, p = 0.038) and CR after 3 cycles of VAD therapy (PFS, HR = 0.072, p = 0.014; OS, HR = 0.055, p = 0.015) were independent prognostic parameters. CONCLUSIONS: Post-transplant D+14 BMPCp is a useful parameter for predicting the outcome for patients with MM receiving ASCT.
Asunto(s)
Adulto , Femenino , Humanos , Masculino , Persona de Mediana Edad , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Médula Ósea/patología , Terapia Combinada , Trasplante de Células Madre Hematopoyéticas , Mieloma Múltiple/mortalidad , Células Plasmáticas/patología , Valor Predictivo de las Pruebas , Estudios Retrospectivos , Trasplante AutólogoRESUMEN
Carney complex is an autosomal dominant syndrome characterized by multiple neoplasias, including myxomas and endocrine tumors, spotty pigmentation, and schwannomas. Primary pigmented nodular adrenal dysplasia (PPNAD) is a cause of Cushing syndrome, independently of adrenocorticotropic hormone. A 21-year-old man was referred for evaluation with multiple spinal compression fractures and adenomas affecting both adrenal glands. Plasma and urinary cortisol levels were elevated and were not suppressed by the administration of a high dose of dexamethasone. The patient was diagnosed with Carney complex manifesting as Cushing syndrome due to PPNAD with multiple pigmented skin lesions. Left adrenalectomy was performed because the nodule on the left adrenal gland was markedly larger than the other nodules. Subsequently, right adrenalectomy was also performed. Pathological analysis revealed that the adrenal glands contained multiple dark brown and black nodules. We describe herein our recent experience of a case of Carney complex manifesting as Cushing syndrome due to PPNAD, with lentigines on the lips, and review the published literature on this subject.
Asunto(s)
Humanos , Adulto Joven , Adenoma , Glándulas Suprarrenales , Adrenalectomía , Hormona Adrenocorticotrópica , Complejo de Carney , Síndrome de Cushing , Dexametasona , Fracturas por Compresión , Hidrocortisona , Lentigo , Labio , Mixoma , Neurilemoma , Pigmentación , Plasma , Piel , Fracturas de la Columna VertebralRESUMEN
BACKGROUND/AIMS: There is an increased risk of tuberculosis (TB) with impaired cellular immunity and extrapulmonary TB is more common in patients with chronic kidney disease. We explored the clinical features and treatment outcomes of extrapulmonary TB according to renal function. METHODS: This retrospective study reviewed the medical records of patients diagnosed with extrapulmonary TB between January 2003 and December 2007. We classified the patients into two groups using the glomerular filtration rate (eGFR), estimated using the Modification of Diet in Renal Disease (MDRD) formula cut-off of 60 mL/min/1.73 m2 and evaluated their clinical features, treatment outcome and mortality (Group I vs. Group II, > or = 60 mL/min/1.73 m2). RESULTS: The mean eGFR of Groups I (n=30) and II (n=312) was 34+/-19 and 102+/-26 mL/min/1.73 m2, respectively. The pleura was the most frequent site of TB in both groups (Group I, 30.0% vs. Group II, 28.2%; p=0.379). There was no treatment failure or recurrence in either group. The mortality was higher in Group I (22.2% vs. 2.8%; p<0.01). In a multivariate analysis, eGFR<60 mL/min/1.73 m2 was an independent risk factor for mortality (HR=11.51, CI 2.512-52.741; p=0.002). CONCLUSIONS: Mortality related to extrapulmonary TB was higher in patients with impaired kidney function and kidney function was an independent predictor. However, there was no difference in treatment failure and recurrence according to renal function.
Asunto(s)
Humanos , Dieta , Tasa de Filtración Glomerular , Inmunidad Celular , Riñón , Registros Médicos , Análisis Multivariante , Pleura , Pronóstico , Recurrencia , Insuficiencia Renal Crónica , Estudios Retrospectivos , Factores de Riesgo , Insuficiencia del Tratamiento , Resultado del Tratamiento , TuberculosisRESUMEN
PURPOSE: Vascular access failure is the most common reason for hospitalization among hemodialysis (HD) patients. Cilostazol, which has antiplatelet action and vasodialtory effects, significantly reduces the risk of restenosis after percutaneous coronary intervention in many patients. We conducted this study to evaluate the relationship between the use of antiplatelet agents, especially cilostazol, and arteriovenous fistula (AVF) patency in HD patients. METHODS: A total of 241 patients underwent native AVF creation from January 2001 to December 2008. Among these patients, we selected 86 patients excluding 38 patients (15.8%) with primary technical failure, 49 patients without complete data and 68 patients used cilostazol less than 1 month. Demographic characteristics, medication history and fistula failure rate were collected and analyzed to elucidate the effect of cilostazol to native AVF. RESULTS: From all groups, AVF failure occurred in 24 patients (27.9%). 28 patients received cilostazol (62.3 %) and mean duration of cilostazol therapy was 229.5+/-115.7 days. All patients were classified into two groups according to cilostazol (Cilostazol [n=28, 32.6%] vs. non-Cilostazol [n=58, 67.4%]. There was no statisticallly significant difference in failure rate between the two groups (32.1% vs. 25.9%, p=0.543). In diabetes group, patients who received statin have much lower AVF failure rate (0% vs. 32.4%, p=0.024). Logistic regression analysis showed that female was independent risk factor for access failure (HR 5.549, CI 1.104-27.877, p=0.037). CONCLUSION: Cilostazol and other antiplatelet agent had a no significant association with AVF patency. Female was an independent risk factor for access failure.
Asunto(s)
Femenino , Humanos , Fístula Arteriovenosa , Fístula , Hospitalización , Inhibidores de Hidroximetilglutaril-CoA Reductasas , Modelos Logísticos , Intervención Coronaria Percutánea , Inhibidores de Agregación Plaquetaria , Diálisis Renal , Factores de Riesgo , Tetrazoles , Grado de Desobstrucción VascularRESUMEN
Over the last several years, eosinophilic esophagitis in adults is an increasingly recognized disease in various parts of world. A 77-year-old male with a gastric submucosal tumor was referred to our department for endoscopic ultrasonography. During the examination, there was some resistance in inserting the echoendoscope into the esophagus. Detailed endoscopic examination of the esophagus showed narrow lumen and scattered whitish plaques, and biopsy of the esophagus revealed more than 20 eosinophils per high-power field. We diagnosed this case as eosinophilic esophagitis and treated successfully by topical steroid. We report a case of eosinophilic esophagitis found incidentally during endoscopic ultrasonography for a gastric submucosal tumor.
Asunto(s)
Adulto , Anciano , Humanos , Masculino , Biopsia , Endosonografía , Esofagitis Eosinofílica , Eosinófilos , EsófagoRESUMEN
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Humanos , Arsénico , Arsenicales , Odontólogos , Encía , Maxilares , Maxilar , Necrosis , Osteomielitis , Óxidos , PulpotomíaRESUMEN