Adult Multisystem Langerhans Cell Histiocytosis Presenting with Central Diabetes Insipidus Successfully Treated with Chemotherapy

We report the rare case of an adult who was diagnosed with recurrent multisystem Langerhans cell histiocytosis (LCH) involving the pituitary stalk and lung who present with central diabetes insipidus and was successfully treated with systemic steroids and chemotherapy. A 49-year-old man visited our hospital due to symptoms of polydipsia and polyuria that started 1 month prior. Two years prior to presentation, he underwent excision of right 6th and 7th rib lesions for the osteolytic lesion and chest pain, which were later confirmed to be LCH on pathology. After admission, the water deprivation test was done and the result indicated that he had central diabetes insipidus. Sella magnetic resonance imaging showed a mass on the pituitary stalk with loss of normal bright spot at the posterior lobe of the pituitary. Multiple patchy infiltrations were detected in both lung fields by computed tomography (CT). He was diagnosed with recurrent LCH and was subsequently treated with inhaled desmopressin, systemic steroids, vinblastine, and mercaptopurine. The pituitary mass disappeared after two months and both lungs were clear on chest CT after 11 months. Although clinical remission in multisystem LCH in adults is reportedly rare, our case of adult-onset multisystem LCH was treated successfully with systemic chemotherapy using prednisolone, vinblastine, and 6-mercaptopurine, which was well tolerated.

Fusobacterium nucleatum GroEL signaling via Toll-like receptor 4 in human microvascular endothelial cells

The GroEL heat-shock protein from Fusobacterium nucleatum, a periodontopathogen, activates risk factors for atherosclerosis in human microvascular endothelial cells (HMEC-1) and ApoE-/- mice. In this study, we analyzed the signaling pathways by which F. nucleatum GroEL induces the proinflammatory factors in HMEC-1 cells known to be risk factors associated with the development of atherosclerosis and identified the cellular receptor used by GroEL. The MAPK and NF-kappaB signaling pathways were found to be activated by GroEL to induce the expression of interleukin-8 (IL-8), monocyte chemoattractant protein 1 (MCP-1), intercellular adhesion molecule 1 (ICAM-1), vascular cell adhesion molecule 1 (VCAM-1), E-selectin, and tissue factor (TF). These effects were inhibited by a TLR4 knockdown. Our results thus indicate that TLR4 is a key receptor that mediates the interaction of F. nucleatum GroEL with HMEC-1 cells and subsequently induces an inflammatory response via the MAPK and NF-kappaB pathways.

Global Transcriptional Analysis Reveals Upregulation of NF-kappaB-responsive and Interferon-stimulated Genes in Monocytes by Treponema lecithinolyticum Major Surface Protein

MspTL is the major surface protein of Treponema lecithinolyticum associated with periodontitis and endodontic infections. Our recent investigation revealed that MspTL induces proinflammatory cytokines and intercellular adhesion molecule 1 in THP-1 cells and periodontal ligament cells. In this study we conducted oligonucleotide microarray analysis to investigate the global transcriptional regulation in THP-1 cells stimulated with purified recombinant MspTL. MspTL upregulated the expression of 90 genes in THP-1 cells at least four fold, and the functions of these genes were categorized into adhesion, apoptosis/antiapoptosis, cell cycle/growth/differentiation, chemotaxis, cytoskeleton organization, immune response, molecular metabolism, proteolysis, signaling, and transcription. The majority of the modified genes are known to be NF-kappaB-responsive and interferon-stimulated genes (ISGs). The expression of 12 selected genes was confirmed by real-time RT-PCR. Because prostaglandin E2 (PGE2) is an important inflammatory mediator and Cox-2 was found to be induced by MspTL in the microarray analysis, we determined the level of PGE2 in the culture supernatants of MspTL-treated cells and found that MspTL significantly increased PGE2. Our results provide insight into the gene regulation of host cells in response to MspTL, and may contribute to the understanding of the molecular mechanism in periodontitis.

Responses: Risk Factors for Early Development of Macrovascular Complications in Korean Type 2 Diabetes (Korean Diabetes J 33(2):134-142, 2009) / 당뇨병

No abstract available.

Risk Factors for Early Development of Macrovascular Complications in Korean Type 2 Diabetes / 당뇨병

BACKGROUND: The average duration of diabetes and predictive factors of macrovascular complications in Korean diabetic patients remain to be elucidated. This study examines the average duration of diabetes up to the onset of macrovascular complications and clinically important factors of early development of these complications in Korean type 2 diabetic patients. METHODS: Clinical characteristics in type 2 diabetics with (n = 121) and without macrovascular complications (n = 115) were analyzed. In addition, early onset ( 5 years, n = 67) were compared, as were the clinical characteristics between male and female patients in the macrovascular complications group. RESULTS: The average duration of diabetes was 8.7 +/- 7.8 years in the macrovascular complications group. Average age, systolic and diastolic blood pressures and smoking history were all higher in the macrovascular complications group than the control group. However, HbA1c levels and prevalence of microvascular complications were higher in the controls. Average age was lower in the early onset group and many more patients of that group had a smoking history. In the analysis based on sex, marcrovascular complications developed earlier in male patients. In addition, the prevalence of family history of diabetes was higher in males and 77.8% of male patients had a smoking history (female: 3.4%). CONCLUSION: Our study confirms that older age, high blood pressure and smoking history are major risk factors for the development of macrovascular complications. Moreover, a smoking history in males can be both risk and predictive factors for earlier development of macrovascular complications in Korean type 2 diabetic patients. We also found that several clinical characteristics including age, family history of diabetes, hypertension and smoking history, vary between the sexes, and these findings can provide useful indices for the prevention of macrovascular complications.

A case of recurrent flash pulmonary edema after renal artery bypass graft in renal artery stenosis / 대한내과학회지

Renal artery stenosis is a rare cause of acute pulmonary edema. So-called flash pulmonary edema is associated with bilateral renal artery stenosis or stenosis in a single functioning kidney. Flash pulmonary edema has been recognized as an absolute indication for vascular intervention. A 33-year old man was admitted with acute shortness of breath. Renal angiography showed occlusion of the right renal artery. He underwent a right renal artery bypass graft. However, after the renal artery bypass graft, episodes of pulmonary edema recurred. A renal angiography showed complete obstruction of the right renal artery and bypass graft. The left renal angiography showed an intact renal artery and decreased kidney size.

A Case of Duodenal Diverticulum Accompanied with Choledochoduodenal and Pancreaticoduodenal Fistulas / 대한소화기학회지

Choledochoduodenal fistula (CDF) occurring simultaneously with pancreaticoduodenal fistula is extremely rare. CDF has known to be a chronic sequela of cholelithiasis, but it is unknown whether pancreaticoduodenal fistula results from chronic cholelithiasis as well. We report a case of cholelithiasis accompanied with choledochoduodenal and pancreaticoduodenal fistula opening into small suprapapillary diverticulum in a 80-year-old woman.

Treatment of Catheter-related Bacteremia with an Antibiotic Lock Protocol in Hemodilaysis Patients / 대한신장학회잡지

BACKGROUND: Catheter-related bacteremia is a frequent complication among hemodialysis patients using a tunneled cuffed catheter. The standard therapy of catheter-related bacteremia involves both systemic antibiotics and catheter replacement. This study was performed to evaluate the effect of antibiotic lock therapy in conjugation with systemic antibiotics without catheter removal on catheter-related bacteremia. METHODS: Thirty six chronic hemodialysis patients with tunneled cuffed catheter were monitored for infection between July 2001 and July 2005. We analyzed the efficacy of antibiotic lock protocol compared with systemic antibiotics alone. RESULTS: Twenty-nine episodes of catheter-related bacteremia occurred in 27 patients during the study periods. The incidence of catheter-related bacteremia was 1.5 episodes/1000 catheter-days. A single gram-positive coccus grew in the 16 cases (55.2 %), and gram-negative organisms grew in the 69 cases (31.0%). Sixteen of 18 patients (88.9%) treated with antibiotic lock protocol had successful catheter salvage versus only 6 of the 11 patients (54.5%) treated with systemic antibiotics alone (p=0.05). Three patients with Burkholderia pickettii and a patient with Acinetobactor calcoaceticus-baumannii complex were treated with antibiotic lock protocol with systemic ciprofloxacin and imipenem, respectively. CONCLUSION: This study suggests that antibiotic lock protocol in eradicating catheter-related bacteremia is effective treatment without requiring catheter replacement.

A Case of IgA Nephropathy with Systemic Lupus Nephritis / 대한신장학회잡지

Renal involvement is common in systemic lupus erythematosus (SLE). The typical lupus nephropathy demonstrates polyclonal immunoglobulin immune deposits with predominance of IgG, usually heavy polytypic complement factors C1q, C3 and C4. In SLE patients, the superimposition and occurrence of non- lupus nephropathy have rarely been reported. We describe a 28-year-old, 15 weeks pregnant women affected by SLE and IgA nephropathy. She was admitted to our hospital due to generalized edema and arthralgia. The ANA titer was 1: 640, anti-ds DNA levels were 354.2 U/mL and other blood tests included thrombocytopenia and hypoclomplementemia. These clinical and laboratory data allowed the diagnosis of SLE. Renal biopsy showed modest segmental mesangial hypercellularity. Immunofluorescence microscopy revealed distinct mesangial IgA and C3 with absence of IgG, IgM, C1q, and C4. Electron microscopy confirmed the presence of electron-dense deposits throughout the mesangium. These features were consistent with the coexistence of IgA nephropathy. A course of prednisolone (50 mg/day) was given for six months and she responded well with resolution of proteinuria. At the present follow- up time point (48 months), she continues to be treated with prednisolone (5 mg/day); proteinuria and ANA are undetectable.