Neuroendocrine Tumors in the Stomach, Duodenum, and Pancreas Accompanied by Novel MEN1 Gene Mutation / 대한소화기학회지

Multiple endocrine neoplasia type 1 (MEN1) syndrome is a relatively rare disease, characterized by the occurrence of multiple endocrine tumors in the parathyroid and pituitary glands as well as the pancreas. Here, we report a case of MEN1 with neuroendocrine tumors (NETs) in the stomach, duodenum, and pancreas. A 53-year-old man visited our hospital to manage gastric NET. Five years prior to his visit, he had undergone surgery for incidental meningioma. His brother had pancreatic nodules and a history of surgery for adrenal adenoma. His brother's daughter also had pancreatic nodules, but had not undergone surgery. The lesion was treated by endoscopic submucosal dissection and diagnosed as a grade 1 NET. Another small NET was detected in the second duodenal portion, resected by endoscopic submucosal dissection, which was also diagnosed as a grade 1 NET. During evaluation, three nodules were detected in the pancreas, and no evidence of pituitary, parathyroid tumors, or metastasis was observed. After surgery, the pancreatic lesions were diagnosed as NETs, with the same immunohistochemical patterns as those of the stomach and duodenum. Genetic testing was performed, and a heterozygous mutation was detected in the MEN1 gene, which is located on 11q13.

An Intractable Caustic Esophageal Stricture Successfully Managed with Sequential Treatment Comprising Incision with an Insulated-Tip Knife, Balloon Dilation, and an Oral Steroid

Bougie or balloon dilation is a good short-term treatment for caustic esophageal strictures, although recurrence after dilation occurs in approximately 30% of these cases. Therefore, long-term treatment options are required in some cases, and endoscopic incisional therapy has been used for patients with an anastomotic stricture in the gastrointestinal tract. A 58-year-old woman presented with severe swallowing difficulty because of a caustic esophageal stricture, which was caused by accidental exposure to anhydrous acetic acid at infancy. She had undergone several previous bougie and balloon dilations but the stricture did not improve. We performed sequential treatment comprising incision with an insulated-tip knife, balloon dilation, and an oral steroid, which resulted in the patient’s symptoms markedly improving. Thus, we report this case of an intractable caustic esophageal stricture, which was successfully treated using combined endoscopic sequential treatment.

The Experience and Management of Two Cases of Metformin-associated Lactic Acidosis

Metformin, a dimethylbiguanide, is an oral antihyperglycemic drug used in treatment of type 2 diabetes mellitus. It has been reported that metformin may be associated with lactic acidosis in patients with clinical conditions such as renal failure and heart failure. Metformin-associated lactic acidosis (MALA) is a rare, but serious complication with a mortality rate of approximately 30~50%. Therefore, an aggressive treatment strategy including hemodialysis is recommended for these patients. Although continuous renal replacement therapy (CRRT) has been administered in hemodynamically unstable patients with MALA, there are few case reports describing the use of CRRT as a therapeutic modality in Korea. Here, we describe the case histories of two MALA patients who underwent treatment with CRRT.

Two Successfully Treated Cases of Pulmonary Fibrosis Due to Paraquat Poisoning

Paraquat poisoning can cause severe multiple organ failure involving the kidneys, liver, lungs, adrenals, and central nervous system. The toxic effect of paraquat on the lung manifests as pulmonary edema, hypoxia, respiratory failure, and pulmonary fibrosis. However, optimal guidelines for treatment of lung fibrosis following paraquat ingestion are not available. We experienced two cases, a 45-year-old Korean male and a 66-year-old Korean male, who visited the emergency center because of paraquat poisoning. They initially received methylprednisolone pulse therapy and cyclophosphamide. Then they experienced pulmonary fibrosis approximately 10 days after admission during renal recovery. Although steroid pulse therapy with cyclophosphamide was reported to reduce mortality due to paraquat poisoning, the side effects of cyclophosphamide treatment were concerning in our patients, who had already received cyclophosphamide. Therefore, we decided to repeat steroid pulse therapy without cyclophosphamide. Fortunately, pulmonary fibrosis in these two patients resolved after repeated steroid pulse therapy. Thus, steroid pulse therapy alone could benefit patients with lung fibrosis, who have already received steroid and cyclophosphamide treatment. Herein, we report on two cases of pulmonary fibrosis due to paraquat poisoning that were treated successfully with repeated steroid pulse therapy.

A Case of Cryptococcal Meningitis Mimicking Hepatic Encephalopathy in a Patient with Liver Cirrhosis Caused by Chronic Hepatitis C / 대한소화기학회지

Cryptococcus neoformans, an encapsulated fungus, is an important opportunistic pathogen that can cause meningitis in immunocompromised patients. Since patients with cryptococcemia have high mortality, it is essential to make an early diagnosis and promptly initiate antifungal therapy. However, it is often very difficult to differentiate between cryptococcal meningitis and hepatic encephalopathy in patients with liver cirrhosis, and there is delay in making the diagnosis. Therefore, these patients have a particularly grave prognosis and consequently many patients die before culture results become available. In one study, starting antifungal therapy within 48 hours of the blood culture was associated with improved survival, but patients with liver cirrhosis were significantly less likely to receive antifungal therapy within 48 hours compared to those without liver cirrhosis. Recently, the authors experience a case of a 68-year-old woman with liver cirrhosis who presented with fever and a drowsy mental status. She had a previous history of having been admitted for infection-associated hepatic encephlopathy. Cryptococcal meningitis and cryptococcemia were diagnosed by spinal puncture and culture of cerebrospinal fluid. In spite of adequate treatment, the patient developed multi-system organ failure and eventually expired. Herein, we report a case of cryptococcal meningitis mimicking hepatic encephalopathy in a patient with liver cirrhosis.

Idiopathic retroperitoneal fibrosis associated with Hashimoto's thyroiditis in a patient with a single functioning kidney

Retroperitoneal fibrosis (RPF) is a rare disease characterized by the presence of fibroinflammatory tissue around the abdominal aorta and ureteral entrapment in most cases. Idiopathic RPF is frequently reported in association with autoimmune diseases; however, there have been few reports of idiopathic RPF associated with Hashimoto's thyroiditis. Here, we report a case of idiopathic RPF with Hashimoto's thyroiditis in a patient with a single functioning kidney, which was successfully treated by corticosteroid therapy and transient intraureteral stent insertion with a double-J catheter.