• Clinical and Experimental Pediatrics
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• 제50권10호
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• pp.931-937
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• 2007

The hemolytic uremic syndrome (HUS) is a rare disease of microangiopathic hemolytic anemia, low platelet count and renal impairment. HUS usually occurs in young children after hemorrhagic colitis by shigatoxin-producing enterohemorrhagic E. coli (D+HUS). HUS is the most common cause of acute renal failure in infants and young children, and is a substantial cause of acute mortality and morbidity; however, renal function recovers in most of them. About 10% of children with HUS do not reveal preceding diarrheal illness, and is referred to as D- HUS or atypical HUS. Atypical HUS comprises a heterogeneous group of thrombomicroangiopathy (TMA) triggered by non-enteric infection, virus, drug, malignancies, transplantation, and other underlying medical condition. Emerging data indicate dysregulation of alternative complement pathway in atypical HUS, and genetic analyses have identified mutations of several regulatory genes; i.e. the fluid phase complement regulator Factor H (CFH), the integral membrane regulator membrane cofactor protein (MCP; CD46) and the serine protease Factor I (IF). The uncontrolled activation of the complement alternative pathway results in the excessive consumption of C3. Plasma exchange or plasma infusion is recommended for treatment of, and has dropped the mortality rate. However, overall prognosis is poor, and many patients succumb to end-stage renal disease. Clinical presentations, response to plasma therapy, and outcome after renal transplantation are influenced by the genotype of the complement regulators. Thrombotic thrombocytopenic purpura (TTP), another type of TMA, occurs mainly in adults as an acquired disease accompanied by fever, neurologic deficits and renal abnormalities. However, less frequent cases of congenital or hereditary TTP associated with ADAMTS-13 (a disintegrin and metalloprotease, with thrombospondin 1-like domains 13) gene mutations have been reported, also. Recent advances in molecular genetics better allow various HUS to be distinguished on the basis of their pathogenesis. The genetic analysis of HUS is important in defining the underlying etiology, predicting the genotype-related outcome and optimizing the management of the patients.

• 한국임상수의학회지
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• 제29권2호
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• pp.165-168
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• 2012

14년 령의 중성화 하지 않은 수컷 Siberian Husky는 1개월 전부터 혈뇨, 기면, 식욕부진을 보였다. 신체검사상 복부 팽만과 통증을 확인할 수 있었다. 혈액 및 혈청 화학 검사상 빈혈과 질소혈증을 확인할 수 있었다. 방사선 검사 상 신장의 비대를 확인할 수 있었으며 초음파 검사 상 피수질의 경계가 불분명하며 피질의 에코가 증가한 신장을 확인할 수 있었다. 뇨검사 상에서는 혈뇨와 단백뇨를 확인할 수 있었으며 초기의 감별 진단으로는 신부전, 방광염, 신우신염과 종양을 생각했다. 환자의 상태는 내원 후 계속적으로 악화되었으며 폐사했다. 부검 상에서 복강 내 출혈과 양측 신장의 종양을 확인할 수 있었다. 조직검사 상에서 혈관육종을 진단하였다. 본 증례에서는 개에서 비특이적으로 발생하는 양측성의 신장원성 혈관육종에 대해서 다룬다.

• 한국임상약학회지
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• 제21권2호
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• pp.100-105
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• 2011

The perception of the 20 adverse events of targeted therapy for metastatic renal cell carcinoma was compared among medical professionals. Thirty-seven oncologists, 167 nurses and 79 pharmacists participated in the survey, and the response rate was 61.9%, 98.2%, 84.9%, respectively. Results showed that the most serious adverse event was GI perforation (8.83 points, 10 being the most serious), whereas the least serious was anemia (5.32 points). There were significant differences among oncologists, nurses and pharmacists especially for the moderately-serious adverse event such as wound-healing complication and lymphopenia. Adverse Events Composite Score (AECS) for each targeted therapy was calculated by multiplying adverse event incidence rate and seriousness score. Sunitinib had the highest score at 6.86 point and bevacizumab had the lowest at 2.1. Among professional groups oncologists showed the lowest AECS, whereas nurses had the highest. The gap on the perception of the adverse events among medical professionals needs to be reduced to get better outcomes of medical therapies for cancer patients.

• Childhood Kidney Diseases
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• 제6권2호
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• pp.237-242
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• 2002

용혈성 요독 증후군은 용혈성 빈혈, 혈소판 감소증, 급성신부전의 임상양상을 보이는 질환으로서 전형적인 경우 EHEC에 의해 유발되며, 설사(특히 혈변) 등의 전구증상과 연관된다. 비전형적으로 드물게 폐구균 감염으로도 유발되며, 이 경우 매우 불량한 예후를 보이는 것으로 알려져 있고, 아직 국내엔 폐구균 감염과 연관된 HUS에 대해 보고된 바가 없다. 저자들은 폐구균 감염이 진단된 후 급격히 심한 비전형적 용혈성 요독 증후군이 진행되었으나 성공적으로 치료한 1례를 경험하였기에 보고하는 바이다.

• 보험의학회지
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• 제3권1호
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• pp.149-162
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• 1986

We had experienced very rare a case of renal cell carcinoma through ultrasonic diagnosis, september, 1985 at medical dept. Dae Han Kyoyuk Ins. Company. The conclusions that we gained, making a comparative-analysis of operation's view and ultrasonic view are as follows; 1. Echolucent area which was $0.6cm^2$ size in the center of tumor was shown by ultrasonography, we noticed it occured necrosis or cystic change and the extracts grossly after. operation accorded with ultrasonic view. 2. Tumor was 65mm in diameter on ultrasonography and made clear 55mm in diameter after operation 3. There was not fever, anemia, even typical triad of renal cell carcinoma, blood pressure was within normal limits. 4. The case was stage I by Robson's Modification method. 5. The case was clear cell type by classifying of histology. 6. The affected site was left side and origin was lower pole of the kidney. 7. After radical nephrectomy, until present prognosis was favorable and he exists.

• 한국임상수의학회지
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• 제24권4호
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• pp.647-652
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• 2007

In the present study, we address systemically a case of renal disease developed in a 1 year-old male cocker spaniel dog in terms of clinical signs, clinical pathology and pathological examinations. The animal has been suffered from renal dysfunction signs such as polyuria, anorexia, vomiting, diarrhea and weight loss. The dog was very weak and emaciated and had foamy contents with foul-smell in oral cavity. The animals showed notable decrease in the number of red blood cells and severe decreases of hemoglobin and hematocrit with or without changes of mean corpuscular volume and mean corpuscular hemoglobin concentration values, indicating microcytic or normocytic hypochromatic anemia. In serum chemistry, blood urea nitrogen, creatinine, phosphorous, Na and Cl, which are associated with renal function, were dramatically increased. In addition, alanine aminotransferase, aspartate transferase, alkaline phosphatase, cholesterol, lipase and amylase were also significantly elevated, while K concentration was notably decreased. Urinalysis indicated prominent proteinuria with increase of bilirubin. Despite of symptomatic treatments, the dog was getting worse in healthy condition and dead in the end. At necropsy, both kidneys were brownish, pale, slightly small, and have diffuse, firm and subcapsular pits. Histologically, the kidneys indicated prominent segmental or diffuse interstitial fibrosis in cortex and medulla as well as glomerulonephritis. The clinical signs, clinical pathology and histopathological abnormalities of the young dog presented were consistent with chronic glomerulonephropathy, which was suspected to be a case of familial renal disease in the juvenile cocker spaniel dog.

• Childhood Kidney Diseases
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• 제17권2호
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• pp.149-153
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• 2013

응고성 미세혈관병증은 빠른 진단이 예후에 중요한 인자이나, 현재의 진단 기준에 따라서는 thrombotic thrombocytopenic purpura, haemolytic uremic syndrome 외의 비전형적인 응고성 미세혈관병증의 진단이 늦어짐에 따라 나쁜 예후를 초래하게 되는 경우가 많다고 보고되어 있다. 본 저자들은 시행한 혈액 검사상 용혈의 증거가 없는 빈혈, 혈소판 감소증 그리고 급성 신부전을 보인 소아 환아에서 신조직 검사를 통해 비특이적 응고성 미세혈관병증을 진단받은 1증례를 보고하고자 한다. 14세 여자 환아는 3주간 지속된 발열, 구역과 전신 부종을 주소로 본원으로 전원되었다. 내원하여 시행한 혈액 검사상 빈혈과 혈소판 감소증을 보였으나, 용혈의 증거는 없었으며, 혈정 크레아티닌이 증가되어 있었다. 내원 이후 급성 신부전과 발열은 지속적으로 진행되었으며, 소변 검사상 단백뇨가 발생하였다. 환아는 내원 40일경 신고혈압과 동반된 전신 경련이 5분간 있어 뇌 자기 공명 영상을 촬영하였으며, 가역성 후백질 뇌병증 증후군의 양상을 보여 항 경련제 투여를 시작하였다. 이후 지속되는 혈소판 감소증 및 발열은 고용량 스테로이드 치료를 진행한 후 호전되었으나, 급성 신부전 및 단백뇨가 지속되어 신장 조직 검사를 진행하였으며, 검사 결과상 혈전성 미세혈관병증의 소견을 보였다. 이와 같이 조직검사상에서는 응고성 미세혈관병증을 보이나 전형적인 응고성 미세혈관병증의 혈액학적인 진단 기준이 충족되지 않는 비특이적 형태의 응고성 혈관병증의 효과적인 진단을 위하여 보체 기전이나 ADAMTS 13와 같은 유전자 범위의 보다 활발한 연구를 통한 효과적인 진단 기준의 마련이 되어야 할 것으로 보인다.

• 한국임상약학회지
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• 제14권2호
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• pp.61-70
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• 2004

Heptaplatin is a new platinum derivative with antitumor activity against gastric cancer. Preclinical studies showed that it is less toxic than other platinum analogues. The purpose of this study is to evaluate the efficacy and toxicity of the combination therapy of heptaplatin and 5-fluorouracil in Korean advanced gastric cancer patients. This study was investigated retrospectively. The patients group consisted of 65 advanced gastric cancer patients with no prior radiotherapy. All patients received heptaplatin $400\;mg/m^2$ by 2-3 hour infusion on Day 1 and 5-FU $1000\;mg/m^2by 12-24 hour continuous infusion for 5 days. After the first cycle, subsequent doses were adjusted according to the toxicity. Courses were repeated every 28 days. As results, objective response occurred in 16 patients $(24.6\%)$. Two were complete and 14 were partial response. Median progression free survival was 32 weeks with $29\%$ of patients progression free at 1 year. The most common hematologic toxicity was anemia. Grade 3 or 4 anemia was seen at $2.7\%$ of treatment cycles. Grade 3 or higher leucopenia was seen at $1.2\%$ of cycles. Grade 3 or 4 neutropenia and thrombocytopenia occurred at $6.1\%\;and\;1.5\%$ of cycles, respectively. The most common nonhematologic toxicity was proteinuria. Though no patients experienced grade 3 or 4 proteinuria, proteinuria was a considerable factor for this chemotherapy. Grade 3 or higher gastrointestinal toxicities were nausea and vomiting ($4.6\%$ of patients) and diarrhea ($1.5\%$ of patients). Grade 2 renal toxicity with elevation of serum creatinine was seen in $0.3\%$ of cycles, which is less than that of other platinum analogues. This study showed that combination therapy of heptaplatin and 5-FU have modest antitumor activity against advanced gastric cancer without severe renal toxicity.

• Clinical and Experimental Pediatrics
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• 제52권10호
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• pp.1061-1068
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• 2009

만성 신질환 환아들의 치료는 신대체 요법과 더불어 적절한 식이 요법, 수분, 전해질 및 산-염기의 관리, 신장 골형성 장애의 예방 및 치료, 빈혈과 고혈압, 성장 지연의 치료 등으로 이루어진다. 식이 요법은 환아들의 성장이 충분히 이루어질 수 있도록 탄수화물, 지방, 단백질 및 열량의 공급이 적절하게 이루어져야 한다. 수분, 전해질 및 산-염기의 상태를 확인하고 적절하게 관리해야 한다. 혈청 칼슘, 인, 부갑상선 호르몬 농도가 사구체 여과율에 따른 적절한 목표치 안에서 유지될 수 있도록 인 결합 제제와 비타민 D 제제를 투여하여 신장 골형성 장애를 예방하여야 한다. 적혈구 생산 촉진제와 철분 제제를 투여하여 혈색소 값을 11-12 g/dl로 유지하여야 한다. 수분 및 염분의 균형과 적절한 항고혈압제의 투여로 고혈압을 조절해야 한다. 사람 재조합 성장 호르몬을 투여하여 최종 성인 키를 호전시키도록 한다.

• Biomolecules & Therapeutics
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• 제6권3호
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• pp.312-316
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• 1998

Efficacy and in vivo bioassay of recombinant human erythropoietin (rho-EPO), was investigated. Efficacy studies were conducted in normal, and cisplatin-induced anemic rats. Normal and anemic animals were treated intravenously with rhu-EPO for 5 days, and the changes in the number of red blood cells (RBC), hemoglobin concentration (Hb), hematocrit value (Hct) and percentage reticulocyte value (Ret, reticulocyte/RBC) were examined. In normal rats, rho-EPO significantly increased RBC, Hb, Hct and Ret at the doses of 50∼ 1,250 lU/kg/day in a dose-dependent fashion. Cisplatin-induced anemic rats showed significant increase of RBC, Hb, Hct and Ret after administration of rho-EPO (50-200 lU/kg/day) in a dose-dependent manner. These changes of hematological parameters disappeared gradually after cessation of the treatment. The in vivo bioassay results in polycythemic mice showed that rho-EPO had 90% of bioactivity compared to NIBSC standard rhu-EPO. These results suggest that rho-EPO might be useful for the therapy of anemia originated from renal failure and chemotherapy-induced anemia.