Hypopituitarism Presenting as Adrenal Insufficiency and Hypothyroidism in a Patient with Wilson's Disease: a Case Report

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.

Hypopituitarism Presenting as Adrenal Insufficiency and Hypothyroidism in a Patient with Wilson's Disease: a Case Report

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.

Clinical Predictors for Response to Proton Pump Inhibitor Treatment in Patients With Globus

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.

Primary Squamous Cell Carcinoma of the Liver Initially Presenting with Pseudoachalasia

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.

A Case of Carney Complex with Multiple Spinal Fractures / 대한내과학회지

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.

A Case of Benign Biliary Stricture as a Complication of Photodynamic Therapy for Biliary Papillomatosis / 대한소화기내시경학회지

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.

Diagnosis and Treatment of Gastric MALT Lymphoma / 대한소화기학회지

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.

Predictive Value of Post-Transplant Bone Marrow Plasma Cell Percent in Multiple Myeloma Patients Undergone Autologous Transplantation

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.

A Case of Duodenal Perforation Caused by Biliary Plastic Stent Treated with Approximation using Endoclip and Detachable Snare / 대한소화기학회지

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.

Toxic Epidermal Necrolysis Associated with Sorafenib and Tosufloxacin in a Patient with Hepatocellular Carcinoma

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.

Comparative analysis of the clinical features and prognosis of extrapulmonary tuberculosis according to the renal function / 대한내과학회지

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.

Can Cilostazol Improve the Patency Rate of Native Arteriovenous Fistula in Hemodialysis Patients? / 대한신장학회지

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.

A Case of Eosinophilic Esophagitis Found Incidentally during the Evaluation of a Gastric Submucosal Tumor / 대한소화기내시경학회지

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.

Osteomyelitis on Maxilla Caused by Arsenic Trioxide

Long-term evaluation of mandibular condyle fractures