Duodenal amyloidosis secondary to ulcerative colitis

Amyloidosis is defined as the extracellular deposition of non-branching fibrils composed of a variety of serum-protein precursors. Secondary amyloidosis is associated with several chronic inflammatory conditions, such as rheumatologic or intestinal diseases, familial Mediterranean fever, or chronic infectious diseases, such as tuberculosis. Although the association of amyloidosis with inflammatory bowel disease is known, amyloidosis secondary to ulcerative colitis (UC) is rare. A 36-year-old male patient with a 15-year history of UC presented with nausea, vomiting, and abdominal pain. He had been treated with infliximab for 6 years. At the time of admission, he had been undergoing treatment with mesalazine and adalimumab since the preceding 5 months. Esophagogastroduodenoscopy showed mucosal erythema, edema, and erosions with geographic ulcers at the 2nd and 3rd portions of the duodenum. Duodenal amyloidosis was diagnosed using polarized light microscopy and Congo red stain. Monoclonal gammopathy was not detected in serum and urine tests, while the serum free light chain assay result was not specific. An increase in plasma cells in the bone marrow was not found. Secondary amyloidosis due to UC was suspected. The symptoms were resolved after glucocorticoid therapy.

Infective Endocarditis Presenting as Endogenous Endophthalmitis Secondary to Streptococcus agalactiae in a Healthy Adult: Case Report and Literature Review / 감염과화학요법

Endogenous endophthalmitis secondary to group B Streptococcus (GBS) is extremely rare, particularly in healthy adults. However, the visual prognosis is poor. We report the first South Korean case of GBS infective endocarditis presenting as endogenous endophthalmitis and skin and soft tissue infection. Cultures of blood, vitreous humor, and pus from skin aspirates yielded a penicillin-susceptible serotype V strain of Streptococcus agalactiae. After 6 weeks, the patient completely recovered from GBS infective endocarditis. However, despite early antibiotic treatment and early surgical intervention, the patient's right eye developed phthisis bulbi and was a candidate for evisceration.

A Case of Simultaneous Resection of Recurrent Combined Hepatocellular Cholangiocarcinoma and Hypovascular Hepatocellular Carcinoma

Liver cancer is more complex to treat compared to cancers in other organs, since liver function should be considered. In addition, only a few patients can be applied curative treatment due to advanced stage at diagnosis. Therefore, early stage detection is important and has been increased through screening and surveillance programs using image modalities recently. However, it is still difficult to diagnose small or hypovascular hepatocellular carcinoma (HCC) even using advanced image modalties. In particular, hypovascular HCCs do not show arterial contrast enhancement which is a typical finding of HCC on computed tomography (CT) and magnetic resonance imaging (MRI). Those also account for a considerable portion of early HCC. We present 54 yearsold man who had recurrent hypervascular and hypovascular nodules on three phase CT and gadoxetic acid-enhanced MRI. The nodules were removed by surgical resection and confirmed as combined hepatocellular-cholangiocarcinoma and well differentiated HCC respectively.

Cyanoacrylate injection versus band ligation for bleeding from cardiac varices along the lesser curvature of the stomach

BACKGROUND/AIMS: Practice guidelines recommend endoscopic band ligation (EBL) and endoscopic variceal obturation (EVO) for bleeding from esophageal varices and fundal varices, respectively. However, the optimal treatment for bleeding from cardiac varices along the lesser curvature of the stomach (GOV1) remains undefined. This retrospective study compared the efficacy between EBL and EVO for bleeding from GOV1. METHODS: Patients treated by EBL or EVO via cyanoacrylate injection for bleeding from GOV1 were enrolled. Patients diagnosed with hepatocellular carcinoma or treated with endoscopic injection sclerotherapy were excluded. RESULTS: The study included 91 patients treated for bleeding from GOV1. The mean age was 56.3±10.9 years (mean±SD), and 78 of them (85.7%) were men. Overall, 51 and 40 patients were treated with EBL and EVO, respectively. A trend for a higher hemostasis rate was noted in the EVO group (100%) than in the EBL group (82.6%, P=0.078). Varices rebled in 15 patients during follow-up. The rebleeding rate was significantly higher in the EBL group than in the EVO group (P=0.004). During follow-up, 13 patients died (11 in the EBL group and 2 in the EVO group); the survival rate was marginally significant between two groups (P=0.050). The rebleeding-free survival rate was significantly higher in the EVO group than in the EBL group (P=0.001). CONCLUSIONS: Compared to EBL, EVO offered significantly lower rebleeding rates, significantly higher rebleeding-free survival rates, and a trend for higher hemostasis and survival rates. EVO appears to be the better therapeutic option for bleeding from GOV1.

Mycophenolate mofetil as an alternative treatment for autoimmune hepatitis

Autoimmune hepatitis (AIH) is an immune-mediated chronic liver disease characterized by hepatocellular inflammation, necrosis, and fibrosis, which can progress to cirrhosis and fulminant hepatic failure. The standard treatment for AIH includes corticosteroids alone or in combination with azathioprine. Although most patients achieve remission using the standard regimen, some patients do not respond due to either drug intolerance or refractory disease; in such cases alternative immunosuppressive agents should be explored. The second-line therapies are cyclophilin inhibitors such as cyclosporine A or tacrolimus, and nowadays mycophenolate mofetil (MMF) is widely used if azathioprine-based therapies are not tolerated. Although these are recommended as an alternative to the first-line regimen, there is insufficient evidence for the efficacy of second-line therapies, with the evidence based mainly on expert opinion. Therefore, we report an AIH patient receiving the standard regimen in whom remission did not occur due to side effects to azathioprine, but was successfully treated with MMF in combination with corticosteroids as an alternative to the standard regimen.

A Case of Esophageal Carcinosarcoma Treated by Endoscopic Resection

Esophageal carcinosarcoma is a rare malignant neoplasm that is composed of both carcinomatous and sarcomatous components. A 78-year-old man with esophageal carcinosarcoma presented with dysphagia, and was treated by endoscopic resection. Although surgery is the standard treatment for esophageal carcinosarcoma, endoscopic resection is an excellent alternative when the tumor is superficial and has no metastasis.

A Case of Non-Hodgkin's Lymphoma of the Ocular Adnexa and Lung in a 17-year-old with Primary Sjogren's Syndrome

Sjogren's syndrome (SS) is a chronic autoimmune disorder characterized by lymphocyte-mediated destruction of exocrine glands, which produces classical symptoms of dry eyes and dry mouth. Aside from the clinical manifestations associated with exocrine glands, extraglandular features of SS include a major long-term concern for development of lymphoma. The lifetime risk of non-Hodgkin's lymphoma (NHL) in an SS patient is approximately 5% to 10%, 20 times higher than that of the normal population. This case report describes a rare occurrence of NHL in the eyelid and lung of an adolescent female with SS, whose disease activity had been monitored closely. This is the first reported case in Korea.

Hepatitis B surface antigen titer is a good indicator of durable viral response after entecavir off-treatment for chronic hepatitis B

BACKGROUND/AIMS: Clear indicators for stopping antiviral therapy in chronic hepatitis B (CHB) patients are not yet available. Since the level of hepatitis B surface antigen (HBsAg) is correlated with covalently closed circular DNA, the HBsAg titer might be a good indicator of the off-treatment response. This study aimed to determine the relationship between the HBsAg titer and the entecavir (ETV) off-treatment response. METHODS: This study analyzed 44 consecutive CHB patients (age, 44.6±11.4 years, mean±SD; men, 63.6%; positive hepatitis B envelope antigen (HBeAg) at baseline, 56.8%; HBV DNA level, 6.8±1.3 log₁₀ IU/mL) treated with ETV for a sufficient duration and in whom treatment was discontinued after HBsAg levels were measured. A virological relapse was defined as an increase in serum HBV DNA level of >2000 IU/mL, and a clinical relapse was defined as a virological relapse with a biochemical flare, defined as an increase in the serum alanine aminotransferase level of >2 × upper limit of normal. RESULTS: After stopping ETV, virological relapse and clinical relapse were observed in 32 and 24 patients, respectively, during 20.8±19.9 months of follow-up. The cumulative incidence rates of virological relapse were 36.2% and 66.2%, respectively, at 6 and 12 months, and those of clinical relapse were 14.3% and 42.3%. The off-treatment HBsAg level was an independent factor associated with clinical relapse (hazard ratio, 2.251; 95% confidence interval, 1.076–4.706; P=0.031). When patients were grouped according to off-treatment HBsAg levels, clinical relapse did not occur in patients with an off-treatment HBsAg level of ≤2 log10 IU/mL (n=5), while the incidence rates of clinical relapse at 12 months after off-treatment were 28.4% and 55.7% in patients with off-treatment HBsAg levels of >2 and ≤3 log₁₀ IU/mL (n=11) and >3 log₁₀ IU/mL (n=28), respectively. CONCLUSION: The off-treatment HBsAg level is closely related to clinical relapse after treatment cessation. A serum HBsAg level of <2 log₁₀ IU/mL is an excellent predictor of a sustained off-treatment response in CHB patients who have received ETV for a sufficient duration.

A Case of Management for Hepatocellular Carcinoma with Lung Metastasis

Liver cancer is the 2nd most common cause of cancer related death in Korea. Especially, patients who present extrahepatic spread of hepatocellular carcinoma (HCC) have a shorter life expectancy (50% survival at 1 year and less than 4 months of median overall survival). Molecular target agent like sorafenib was usually mentioned as a treatment for them, but that was still not firmly established. We present a 75 year-old who had expanding nodular type of HCC. The mass was removed by resection and radiofrequency ablation. However, lung metastasis were revealed shortly after surgery. That lesions were treated with lenvatinib and systemic chemotherapy.

A Case of Gynecomastia Induced by Use of Doxazosin / 대한내과학회지

Doxazosin is an adrenergic alpha-1 receptor antagonist used to treat lower urinary tract symptoms that are common in prostatic hyperplasia. To our knowledge, few cases of gynecomastia and mastodynia, as a complication of adrenergic alpha-1 receptor antagonist, have been reported to date; no cases have been reported in Korea. We describe a case involving a 78-year-old man treated for prostatic hyperplasia with 13 months of doxazosin. He complained about unilateral gynecomstia and mastodynia. Five months after the discontinuation of doxazosin, the gynecomastia was significantly improved. This is the first reported case of gynecomastia and mastodynia associated with doxazosin use in Korea.

Coarctation of the Aorta Treated with Stenting in a Patient with Turner Syndrome / 대한내과학회지

Cardiovascular malformations, such as bicuspid aortic valve and coarctation of the aorta, are more prevalent in patients with Turner syndrome than in the general population. Here, we describe the case of a 35-year-old female with Turner syndrome who was admitted to our hospital with a fever caused by infective endocarditis. Aortic coarctation was diagnosed using computed tomography. The patient was treated with antibiotics and a stent was then implanted. Endovascular therapy using stents is a safe and effective treatment option for aortic coarctation in patients with Turner syndrome.

A Case of Relapsing Polychondritis Associated with Ankylosing Spondylitis

Relapsing polychondritis is an uncommon disease associated with inflammation in cartilaginous tissues throughout the body, particularly affecting the cartilaginous structures of ears, nose, joints, and respiratory tract. Several autoimmune diseases, including vasculitis, are associated with the concurrent relapsing polychondritis. However, ankylosing spondylitis primarily affecting the sacroiliac joints and spine is rare in patients with relapsing polychondritis. We report on a 54-year-old man with concurrently relapsing polychondritis and ankylosing spondylitis.