• Maxillofacial Plastic and Reconstructive Surgery
• /
• 제34권3호
• /
• pp.220-225
• /
• 2012

Wegener's granulomatosis (WG) is an autoimmune disease of unknown etiology characterized by the triad of necrotizing granulomatous lesion in the upper and lower respiratory tracts or both, disseminated vasculitis involving both small arteries and veins, and necrotizing glomerulonephritis. The most common oral lesions associated with WG are ulceration and strawberry gingivitis. A 47-years old man in medical care associated WG was consulted our Division of Oral and Maxillofacial Surgery for the chief complaint of toothaches. Pre-operative panorama showed the alveolar radiolucency and the loss of lamina dura regarding the left upper teeth. An oropharyngeal magnetic resonance imaging also revealed the increased bone marrow signal intensity on the left maxilla. Under the impression of maxillary osteonecrosis due to WG, maxillary saucerization with removal of involved teeth was performed. We obtained good results and report the first case of WG in Korea, with the review of literatures regarding oral and general systemic features.

• 대한수의학회지
• /
• 제40권3호
• /
• pp.587-599
• /
• 2000

This study was conducted to observe the severity of the disease and pathogenecity of Babesia gibsoni parasite on the splenectomized dogs(SPD) and nonsplenectomized(intact) dogs (NSPD) experimentally infected with B gibsoni. The average prepatent period was 4 days in the SPD and 8 days in the NSPD, respectively. Peak parasitaemia(PE) ranged from 26% to 34% of erythrocytes infected in the SPD and from 4% to 5% in the NSPD. Latent parasitaemia was still detectable 40 days as low as under 1.0% of erythrocytes infected after the initial parasitaemia in the SPD. Blood packed cell volume(PCV) decreased to as little as 6.4% to 6.9% in the SPD. The clinical signs were mild fever and anemia in the NSPD, remissions and exacervations of temperature, intermittent or spike-like increases of temperature, progressive polychromatophilic macrocytic anemia with anisocytosis, icterus, marked loss of appetite, rarely haemoglobinuria, and deep brown-yellowish urine in the SPD. Gross pathologic changes mainly involved slightly enlargement of liver and spleen in the NSPD and marked enlargement of liver in the SPD. Anatomic changes associated with the disease included diffuse periportal and centrilobular hephatitis, and membranoproliferative glomerulonephritis. Hyaline droplets, resulting protein metabolic alterations, were found in the convoluted ephithelium of the kidney. The density of lymphocytes within the liver sinusoids was markedly increased. Aggregates of large monocytes and macrophages were demonstrated in the centrilobular veins of the liver. The density of these cells in the centrilobular veins were greatest in the SPD. The forms of B gibsoni parasite found in the acute stage of SPD were large signet ring form, small signet ring form, pyriform, elongated form, comma form, head-phone form, oval form, peared form, racket-like form, amoeboid form, triangle form, quartered form, dot form, band form and multiple, and rosette form, et al. The severity of the disease and pathogenecity of B gibsoni parasite were mild in the NSPD but fatal in the SPD.

• Childhood Kidney Diseases
• /
• 제21권2호
• /
• pp.69-74
• /
• 2017

Purpose: Kidney transplantation (KT) is an ideal treatment for pediatric patients with end-stage renal disease (ESRD). We report the clinical outcomes of pediatric ESRD patients who underwent KT in a single regional center. Methods: We retrospectively investigated the medical records of 60 pediatric patients who were diagnosed with ESRD and underwent KT in our hospital between January 1985 and June 2016. Results: A total of 60 children and adolescents (40 male, 20 female; mean age, $13.86{\pm}4.26$ years) were included in this study. Six patients (10.0%) underwent KT immediately after receiving the diagnosis of ESRD, while the others underwent KT after dialysis treatment (mean period of dialysis, $368.7{\pm}4,41.8$ days). The mean donor age (50 living-related [83.3%], 10 deceased [16.7%]) was $40.0{\pm}12.85$ years and the male:female ratio was 1.07:1. The most common cause of ESRD was chronic glomerulonephritis. The overall survival rates at 1, 3, and 5 years after KT were 98%, 98%, and 96%, respectively, while the graft survival rates at 1, 3, and 5 years were 93%, 86%, and 68%, respectively. Children who underwent KT before 10 years of age had better monthly growth rates than those who underwent KT later than 10 years of age. Conclusions: KT is performed less frequently in children than in adults, but causes of ESRD vary and clinical outcomes after KT greatly affect the growth and development of pediatric patients. Therefore, further analysis and monitoring of clinical progression after KT in pediatric ESRD patients are necessary.

• Journal of Microbiology and Biotechnology
• /
• 제25권2호
• /
• pp.296-301
• /
• 2015

Chronic kidney diseases are based on uncontrolled immunological and inflammatory responses to pathophysiological renal circumstances such as glomerulonephritis, which is caused by immunological mechanisms of glomerular inflammation with increased production of renal pro-inflammatory cytokines. Pentoxifylline (PTX) exhibits anti-inflammatory properties by inhibiting cytokine and chemokine production through aggregation of erythrocytes and thrombocytes. We synthesized a series of 1,7-substituted methylxanthine derivatives by the Traube purine reaction, and the formation of purine ring was completed through nitrosation, a reduction of the nitroso to the amine by catalytic hydrogenation as derivatives of PTX. Then we studied biological activities such as renal anti-inflammatory effects of the synthesized compounds in the production of cytokines such as nitric oxide (NO), interleukin (IL)-1β, IL-6, and tumor necrosis factor-α (TNF-α) and of chemokines such as monocyte chemoattractant protein-1 and IL-8 in Raw 264.7 and HK-2 cells. Renal antiinflammatory activities of this novel series of N-1 and N-7-substituted methylxanthine showed that the N-7 methyl-group-substituted analogs (S7b) showed selective 61% and 77% inhibition of the production of NO and IL-8. The other replacement of the N-1-(CH₂)₄COCH₃ roup, as in the case of compound S6c, also showed an effective 50% and 77% inhibition of TNF-α and IL-8 production in LPS-stimulated Raw 264.7 and HK-2 cells.

• Advances in Traditional Medicine
• /
• 제9권1호
• /
• pp.20-38
• /
• 2009

The objectives of this study were to determine the effect of herb formula HDDM, a modification of Huangdan decoction that has been shown to be effective in the treatment of glomerulonephritis and chronic renal failure, on the blood glucose levels in multiple low doses (MLD; 50 mg/kg for five consecutive days) of streptozotocin (STZ)-treated female B6C3F1 mice. Initial studies were performed to compare diabetes induction in five strains (e.g., B6C3F1, NOD, CD-1, C3H/HeN and C57BL/6) of mice by MLD-STZ, and immune changes following the treatment. The results suggested that the order of susceptibility to diabetes induction was NOD $\approx$ CD-1 > B6C3F1 $\approx$ C3H > C57BL/6. Furthermore, STZ modulation of T cell development, differentiation and activation might play a role in diabetes induction by MLD-STZ treatment. MLD-STZ-induced diabetes in female B6C3F1 mice was moderate, which allowed the evaluation of drug-induced protection or exacerbation of diabetes to be performed. As such, modulation of blood glucose by HDDM, which consisted of Da Huang (Radix Et Rhizoma Rhei), Huang Qi (Radix Astragali Seu Hedysari), Dan Shen (Radix Salviae Miltiorrhizae), Yin Yang Huo (Herba Epimedii), Yi Yi Ren (Semen Coicis or Coix lacryma-jobi), Mai Dong (Radix Ophiopogonis) and Shan Zhu Yu (Fructus Corni), was evaluated in MLD-STZ-treated female B6C3F1 mice. The results suggested that HDDM could lower the blood glucose levels, but it had no immunomodulatory activities. Additionally, HDDM-treated mice exhibited improved glucose tolerance. In conclusion, these studies have suggested that MLD-STZ-induced diabetes in female B6C3F1 mice is a useful model to evaluate drug modulation of diabetes, and that the herb formula HDDM possesses anti-diabetic effects.

• Tuberculosis and Respiratory Diseases
• /
• 제53권4호
• /
• pp.463-469
• /
• 2002

저자들은 비폐색과 두통을 주소로 내원한 49세 남자에서 단백뇨 및 혈뇨와 병발된 다발성 폐결절을 발견하였고, c-ANCA와 조직검사를 통해 부비동, 폐, 신장을 침범한 Wegener씨 육아종증을 진단하고, cyclophosphamide와 스테로이드 병합투여로 증세가 호전되고, 흉부 방사선 사진에서도 호전된 소견을 보이는 환자를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

• Clinical and Experimental Pediatrics
• /
• 제60권8호
• /
• pp.266-271
• /
• 2017

Purpose: The aim of this study was to assess the clinical characteristics of hypertensive encephalopathy according to the underlying etiologies in children. Methods: We retrospectively evaluated 33 pediatric patients who were diagnosed as having hypertensive encephalopathy in Chonbuk National University Children's Hospital. Among the patients, 18 were excluded because of incomplete data or because brain magnetic resonance imaging (MRI) was not performed. Finally, 17 patients were enrolled and divided into a renal-origin hypertension group and a non-renal-origin hypertension group according to the underlying cause. We compared the clinical features and brain MRI findings between the 2 groups. Results: The renal group included renal artery stenosis (4), acute poststreptococcal glomerulonephritis (2), lupus nephritis (2), and acute renal failure (1); the nonrenal group included essential hypertension (4), pheochromocytoma (2), thyrotoxicosis (1), and acute promyelocytic leukemia (1). The mean systolic blood pressure of the renal group ($172.5{\pm}36.9mmHg$) was higher than that of the nonrenal group ($137.1{\pm}11.1mmHg$, P<0.05). Seizure was the most common neurologic symptom, especially in the renal group (P<0.05). Posterior reversible encephalopathy syndrome (PRES), which is the most typical finding of hypertensive encephalopathy, was found predominantly in the renal group as compared with the nonrenal group (66.6% vs. 12.5%, P<0.05). Conclusion: We conclude that the patients with renal-origin hypertension had a more severe clinical course than those with non-renal-origin hypertension. Furthermore, the renal-origin group was highly associated with PRES on brain MRI.

• Childhood Kidney Diseases
• /
• 제24권1호
• /
• pp.36-41
• /
• 2020

Purpose: Hepatitis B virus (HBV) infection is among etiologies of secondary membranous nephropathy (MN) in pediatric patients. We evaluated expression of phospholipase A2 receptor (PLA2R), a specific target antigen of primary MN, in pediatric HBV-related MN. Methods: We retrospectively reviewed patients with biopsy-proven HBV-related MN from the renal biopsy registry and electronic medical records of Severance Hospital, Seoul, Korea, from 1993 to 2004. Paraffin-embedded human kidney tissues were retrieved and immunohistochemically stained for PLA2R. Results: Ten pediatric patients with 13 biopsied specimens were reviewed. The predominant pathological stage was stage II-III, and second was stage II. The intensity of staining for IgG was greatest, with less intense staining for IgM, IgA, C3, C4, and C1q. All the patients had angiotensin-converting enzyme inhibitor combined with glucocorticoid, and four patients converted to cyclosporine treatment from glucocorticoid monotherapy. Urinalysis of all the patients normalized after variable period. PLA2R staining was demonstrated in the outer glomerulus in 3 out of 13 biopsies, 2 of which were obtained from the same patient over a 5-year interval. Conclusions: PLA2R was expressed in a small number of cases diagnosed as pediatric HBV-related MN, indicating that some HBV-related MN cases may be primary MN concurrent with HBV infection.

• Childhood Kidney Diseases
• /
• 제11권1호
• /
• pp.118-125
• /
• 2007

Wegener 육아종증은 괴사성 육아종을 형성하는 작은 혈관의 혈관염으로, 주로 상기도와 하기도, 신장의 3기관을 침범한다. 주로 성인기에 발생하나 소아에서도 발생할 수 있으며, 대부분의 임상 증상은 성인과 소아에서 비슷하나, 몇몇 임상증상에서는 성인과 차이를 보인다. Wegener 육아종증은 소아에서 호흡기 감염성 질환으로 생각되기 쉬우므로 진단이 지연되어, 만성 사구체신염이 말기신부전 등까지 진행할 수 있다. 저자들은 여러 장기를 침범하여 흔하지 않은 임상 양상을 보인 Wegener 육아종증 13세 환아를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• Journal of Yeungnam Medical Science
• /
• 제21권1호
• /
• pp.101-107
• /
• 2004

Diffuse alveolar hemorrhage is a rare but serious and frequently life-threatening complication of a variety of conditions. The first goal in the management of patients with diffuse alveolar hemorrhage is to achieve or preserve stability of the respiratory status. Subsequently, the differential diagnosis is aimed at the identification of a remediable cause of the alveolar hemorrhage. The most common causes of diffuse alveolar hemorrhage with glomerulonephritis are microscopic polyangiitis and Wegener's granulomatosis, followed by Goodpasture syndrome and systemic lupus erythematosus. Microscopic polyangiitis (MPA) is a distinct systemic small vessle vasculitis affecting small sized vessels with few or no immune deposits and with no granulomatosus inflammation. The disease may involve multiple organs such as kidney, lung, skin, joint, muscle, gastrointestinal tract, eye, and nervous system. MPA is strongly associated with antineutrophil cytoplasmic autoantibody (ANCA) that is a useful serological diagnostic marker for the most common form of necrotizing vasculitis. Our report concerns a case of microscopic polyangiitis with diffuse alveolar hemorrhage in a 54-year-old man. He was admitted to our hospital due to dyspnea upon exertion and recurrent hemoptysis. Laboratory findings showed hematuria, proteinuria and deterioration of renal function. In the chest CT scan, diffuse ground glass appearance was seen in both lower lungs. A lung biopsy revealed small vessel vasculitis with intraalveolar hemorrhage and showed a positive reaction to against perinuclear ANCA. The patient was treated with prednisolone and cyclophosphamide. Chest infiltration decreased and hemoptysis and hypoxia improved. He is still being followed up in our hospital with a low dose of prednisolone.