A Case of Lower GI Bleeding from Portal Hypertensive Colopathy Successfully Treated with Octreotide Administration and Endoscopic Hemoclipping / 대한소화기내시경학회지

Cirrhotic patients with portal hypertension are often found to have changes in their colonic mucosa. Such mucosal changes are termed portal hypertensive colopathy. Most patients with portal hypertension remained asymptomatic but some may show massive bleeding. The mainstay of treatment for portal hypertensive gastropathy include non-surgical methods such as octreotide injection, endoscopic hemostasis, and interventional methods such as TIPS. However, treatment for portal hypertensive colopathy remained unresolved. The authors here report a case of a 41 year old male with liver cirrhosis admitted for fever and abdominal pain, who reported an episode of hematochezia in the course of admisssion period. Subsequent colonoscopy revealed angiodysplasia-like lesions throughout the entire colon. We observed that such lesions were the source of hematochezia and that direct clipping with octreotide injection was successful in controlling the bleeding.

Therapeutic Efficacy of Anagrelide for Thrombocytosis / 대한혈액학회지

BACKGROUND: Thrombocytosis can result in life-threatening thrombotic or hemorrhagic events. Anagrelide acts exclusively on megakaryocytes and has been reported as an useful agent in controlling thromobocytosis associated with chronic myeloproliferative disorders. METHODS: Seven patients with essential thrombocythemia and three with chronic myelogenous leukemia were enrolled and early responses and adverse effects of anagrelide were retrospectively analyzed. The drug was started with a dose of 2 mg/day with increases of 0.5 mg/day every 5~7 days as needed. RESULTS: Anagrelide in starting doses of 2 mg/day reduced the platelet count by 50%, or to less than 600,000/mm3, for at least 28 days in 7 of the 9 (78%) evaluable patients. Adverse effects of the drug were observed in 5 patients and generally well tolerated; headache in 4, gastrointestinal troubles in 2, palpitations and chest tightness in 1, and tinnitus in 1. Changes in hemoglobin or white blood cell counts in peripheral blood were minimal and tolerable. CONCLUSION: The present study shows that anagrelide is a useful platelet-lowering agent in whom hydroxyurea or interferon has failed. Long-term efficacy and adverse effects of the drug remain to be determined.

A case of X-linked agammaglobulinemia with deletion of introns 15-18 of Btk gene mediated by Alu-Alu recombination / 대한내과학회지

X-linked agammaglobulinemia (XLA) is characterized by early onset of recurrent bacterial infection, markedly reduced levels of all major classes of immunoglobulins in the serum and few mature B cells in the blood. XLA is known to be associated with mutations in Bruton's tyrosin kinase (Btk). The Btk protein consists of 5 functional domains; the pleckstrin homology (PH) domain, the Tec homology (TH) domain, the Src homology 3 (SH3) domain, the SH2 domain, and the kinase (SH1) domain. Mutations in all domains of the Btk gene have been shown to cause XLA. The large number of Alu elements within the human genome provides abundant opportunities for unequal homologous recombination events between Alu repeats, resulting in human disease. We present a case of XLA with deletion of introns 15-18 of Btk gene which were mediated by an Alu-Alu recombination event.

A case of X-linked agammaglobulinemia with deletion of introns 15-18 of Btk gene mediated by Alu-Alu recombination / 대한내과학회지

X-linked agammaglobulinemia (XLA) is characterized by early onset of recurrent bacterial infection, markedly reduced levels of all major classes of immunoglobulins in the serum and few mature B cells in the blood. XLA is known to be associated with mutations in Bruton's tyrosin kinase (Btk). The Btk protein consists of 5 functional domains; the pleckstrin homology (PH) domain, the Tec homology (TH) domain, the Src homology 3 (SH3) domain, the SH2 domain, and the kinase (SH1) domain. Mutations in all domains of the Btk gene have been shown to cause XLA. The large number of Alu elements within the human genome provides abundant opportunities for unequal homologous recombination events between Alu repeats, resulting in human disease. We present a case of XLA with deletion of introns 15-18 of Btk gene which were mediated by an Alu-Alu recombination event.

Elevated monocyte chemoattractant protein-1 in patients with Behcet's disease / 대한내과학회지

BACKGROUND: Monocyte chemoattractant protein-1 (MCP-1) belongs to C-C subfamily of chemokines, which stimulates the migration of monocytes. MCP-1 exerts various effects on the monocytes, including the induction of integrin and tissue factor, and synthesis of proinflammatory cytokines and arachidonic acid. In this study, we measured the MCP-1 levels in patients with Behcet's disease and evaluated the associations between the levels of MCP-1 and the level of other chemokines and various clinical features of Behcet's disease. METHODS: Serum samples were obtained from 67 patients with Behcet's disease and 30 healthy controls. Simultaneously, whole blood was isolated from patients (n=25) with Behcet's disease and healthy controls (n=11) and cultured in 24 well plates for 48 hours in the absence or presence of lipopolysaccharide (LPS) 5 microgram/mL, phytohaemagglutinin (PHA) 5 microgram/mL, phorbol 12-myristate 13-acetate (PMA) 50 ng/mL + ionomycin 5 microgram/mL. The MCP-1 concentrations were measured in the sera and culture supernatants by enzyme-linked immunosorbent assay (ELISA). RESULTS: The levels of serum MCP-1 were 2.5 times higher in patients with Behcet's disease than healthy controls. The patients with Behcet's disease had also higher levels of MCP-1 in the culture supernatants of whole blood cells, stimulated with LPS, but not with either PHA or PMA plus ionomycin, compared to healthy controls. Serum MCP-1 levels (n=67) were strongly correlated with serum RANTES, MIP-1alpha, IL-8 levels in Behcet's disease. In addition, the production of MCP-1 by whole blood culture from Behcet's disease patients (n=25) were also correlated well with those of RANTES, MIP-1alpha, and IL-8, when stimulated with LPS. However, MCP-1 levels in the sera and culture supernatants did not show any association with various clinical features of Behcet's disease including oral ulcer, genital ulcer, erythema nodosum, arthritis, uveitis, intestinal involvement, central nervous system involvement, and vascular thrombosis. CONCLUSION: In the sera and culture supernatants of whole blood stimulated with LPS, MCP-1 levels were higher in patients with Behcet's disease than controls and correlated well with RANTES, MIP-1alpha, IL-8 levels. These results suggest that the activation and migration of monocytes triggered by the increased production of MCP-1 may play a role in the pathogenesis of Behcet's disease.