• 한국동물위생학회지
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• 제34권1호
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• pp.31-35
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• 2011

This is a case report that 24 heads of cattle suddenly died without clinical signs in a Hanwoo farm. The cause of death was Clostridium perfringens enterotoxemia resulted from them with feeding leftover food. The clinical signs were observed just before the death; increase of heart rate, shallow and rapid respiration, amyostasia, spasm and so forth. In autopsy, blood coagulation disorder, a little abdominal inflation, hepatomegaly and different size of red spots, congestion hemorrhage and undercurrent of bloody exudation were observed in the entire parts of small and large intestines. C. perfringens were isolated from the substantive organs, and a unique fragment of 405bp C. perfringens was amplified by PCR. Therefore, this case was diagnosed as enterotoxemia caused by ${\alpha}$-toxin of C. perfringens A type.

• 대한수의학회지
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• 제53권3호
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• pp.181-184
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• 2013

Dystrophic calcinosis cutis associated with spontaneous hyperadrenocorticism was diagnosed in a 8-year-old female Chihuahua dog with erythematous, erosive, numerous papules, plaques, and crusts on the bilateral trunk, and inguinal region. Serum biochemical abnormalities included increases in alkaline phosphatase (ALP), alanine aminotransferase (ALT), gamma-glutamyl transpeptidase (GGT), and cholesterol. Radiographs showed mild hepatomegaly and subcutaneous lobulated calcific deposits. Histopathologic examination demonstrated diffuse deposition of basophilic calcified material in the dermis. Von Kossa's stain confirmed calcium deposition. Therapy with diltiazem was useful in resolving calcinosis.

• Pediatric Infection and Vaccine
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• 제9권1호
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• pp.104-109
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• 2002

녹농균은 면역질환이 있거나, 기저의 악성질환이 있을 때 주로 감염되며, 건강한 환아에서 감염되는 경우는 흔하지 않다. 저자들은 특별한 면역질환이나 기저질환 없이 녹농균감염이 간, 신장, 피부 등에 다발적으로 발생하여 간농양과 신농양, 신우신염을 동반한 괴저성 농창으로 진단된 1례를 경험하였기에 보고하는 바이다. 괴저성 농창은 조기 진단과 조기 치료가 예후에 중요한데, 본 증례에서는 비교적 조기 진단과 치료가 이루어져 좋은 예후를 보인 것으로 사료된다.

• 대한유전성대사질환학회지
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• 제13권2호
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• pp.126-130
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• 2013

Galactosemia is a metabolic disorder inherited by the recessive autosome, and appears by the deficiency of one enzyme out of GALT (Galactose-1-Phosphate Uridyltransferase), GALK (galactokinase), and GALE (epimerase) enzymes, among which the GALT deficiency disease is denominated as classical galactosemia and known to have symptoms such as severe nausea, jaundice, hepatomegaly, sucking difficulty and so on. We report the case of a 16-day-old female baby with the new p.A101D mutation together with p.N413d in the GALT gene analysis found in the neonatal screening test and diagnosed to have galactosemia by the GALT deficiency through the enzyme analysis. For the prognosis prediction, the treatment, the genetic counseling and the prenatal diagnosis of the patients, more detailed genetic diagnosis is required by performing GALT gene analysis, and it is deemed to be necessary to analyze the correlation between the phenotype and the genotype of the domestic galactosemia patients.

• Journal of mucopolysaccharidosis and rare diseases
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• 제5권1호
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• pp.1-7
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• 2021

Gaucher disease (GD, OMIM #230800 OMIM#230800) is a rare, autosomal recessive inherited metabolic disorder caused by mutation in GBA1 encoding the lysosomal enzyme, glucocerebrosidase. The deficiency of glucocerebrosidase leads to an accumulation of its substrate, glucosylceramide in macrophages of various tissues. Common clinical manifestations include cytopenia, splenomegaly, hepatomegaly, and bone lesions. The phenotype of GD is classified into three clinical categories: Type 1 (non-neuronopathic) is characterized by involvements on the viscera, whereas types 2 and 3 (neuronopathic) are associated with not only visceral symptoms but also neurological impairment, either severe in type 2 or variable in type 3. A diagnosis of GD can be confirmed by demonstrating the deficiency of acid glucocerebrosidase activity in leukocytes. Mutations in the GBA1 should be identified as they may be of prognostic value in some cases. Biomarkers including Chitotriosidase, CCL18, and glucosylsphingosine (lyso-GL1) are useful in diagnosis and treatment monitoring. Currently available disease-specific treatment in Korea consists of intravenous enzyme replacement therapy and substrate reduction therapy. For enhancing long-term prognosis, the onset of Parkinson's disease and Lewy body dementia, or the occurrence of a blood disease or cancer (hepatocellular carcinoma) should be monitored in older patients. The development of new strategies that can modify the neurological phenotype are expected, especially in Asia including Korea, where the prevalence of neuronopathic GD is relatively higher than that in western countries.

• 한국동물위생학회지
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• 제44권4호
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• pp.299-303
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• 2021

A captive male polar bear (Ursus maritimus) was found dead after a long-term anorexia at a Park Zoo in Republic of Korea. At necropsy, the liver lesion was prominent with hepatomegaly carrying a big mass and numerous small nodules in various sizes. The cut surface of the nodules was creamy white, firm, and solid with umbilicated appearance. Histologically, there was proliferation of hepatocellular neoplastic cells arranged in glandular-like (pseudoglandular) pattern and complete loss of hepatocellular arrangement. The immunoreactivity for neoplastic hepatocyte antigens was positive for hepatocyte paraffin 1, and negative for vimentin, cytokeratin 7 and 19, and carcinoembryonic antigen. Based on those diagnostic features, the neoplasia was diagnosed as hepatocellular carcinoma. To the author's knowledge, this is the first case report of hepatocellular carcinoma in the captive polar bear in Republic of Korea.

• Asian Pacific Journal of Cancer Prevention
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• 제17권10호
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• pp.4699-4711
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• 2016

Background: In the last decade, it has become clear that change of gene expression may alter the hematopoietic cell quiescent state and consequently play a major role in leukemogenesis. WT1 is known to be a player in acute myeloid leukemia (AML) and FOXP3 has a crucial role in regulating the immune response. Objectives: To evaluate the impact of overexpression of WT1and FOXP3 genes on clinical course in adult and pediatric AML patients in Egypt. Patients and methods: Bone marrow and peripheral blood samples were obtained from 97 de novo non M3 AML patients (63 adult and 34 pediatric). Real-time quantitative PCR was used to detect overexpression WT1 and FOXP3 genes. Patient follow up ranged from 0.2 to 39.0 months with a median of 5 months. Results: In the pediatric group; WT1 was significantly expressed with a high total leukocyte count median 50X109/L (p=0.018). In the adult group, WT1 had an adverse impact on complete remission induction, disease-free survival and overall survival (p=0.02, p=0.035, p=0.019 respectively). FOXP3 overexpression was associated with FAB subtypes AML M0 +M1 vs. M2, M4+M5 (p =0.039) and the presence of hepatomegaly (p=0.005). Conclusions: WT1 and FOXP3 overexpression has an adverse impact on clinical presentation, treatment response and survival of pediatric and adult Egyptian AML patients.

• 대한미생물학회지
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• 제15권1호
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• pp.63-69
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• 1980

C. parvum은 면역학적(免疫學的) 연구(硏究)에 immunomodulating agent로서 광범위(廣範圍)하게 이용(利用)되고 있기 때문에 C. parvum이 SRBC에 대(對)한 면역반응(免疫反應)에 미치는 영향(影響) 및 그 기전(機轉)을 알아보고자 본(本) 실험(實驗)을 실시(實施)한 결과(結果) 다음과 같은 성적(成績)을 얻었다. C. parvum이 SRBC에 대(對)한 DTH에 미치는 영향(影響)은 C. parvum 투여시기(投與時期)에 따라 상이(相異)한 결과(結果)를 나타내었는데 C. parvum을 SRBC로 면역전(免疫前)에 정맥내(靜脈內)로 투여(投與)했을 때 유의(有意)있는 지연성(遲延性) 과민반응(過敏反應)(DTH)의 감소(減少)를 보였으며 이러한 현상(現象)은 cyclophosphamide로 처리(處理)했을 때 완전(完全)히 소실(消失)되었다. 또한 C. parvum은 S. typhimurium의 증식(增殖)을 억제(抑制)하고 비장(脾臟) 및 간장종대(肝臟腫大)를 야기(惹起)시켰는데 이러한 사실(事實)로 미루어 C. Parvum의 DTH 감소현상(減少現象)은 suppressor T 세포(細胞) 또는 활성화(活性化)된 대식세포(大食細胞)의 중개(仲介)에 의한 것으로 사료(思料)되었다. C. parvum은 SRBC에 대(對)한 항체가(抗體價)에 유의(有意)있는 영향(影響)을 미치지 못하였다.

• Parasites, Hosts and Diseases
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• 제51권6호
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• pp.735-738
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• 2013

Eosinophilic meningitis, caused by the nematode Angiostrongylus cantonensis, is prevalent in northeastern Thailand, most commonly in adults. Data regarding clinical manifestations of this condition in children is limited and may be different those in adults. A chart review was done on 19 eosinophilic meningitis patients aged less than 15 years in Srinagarind Hospital, Faculty of Medicine, Khon Kaen University, Thailand. Clinical manifestations and outcomes were reported using descriptive statistics. All patients had presented with severe headache. Most patients were males, had fever, nausea or vomiting, stiffness of the neck, and a history of snail ingestion. Six patients had papilledema or cranial nerve palsies. It was shown that the clinical manifestations of eosinophilic meningitis due to A. cantonensis in children are different from those in adult patients. Fever, nausea, vomiting, hepatomegaly, neck stiffness, and cranial nerve palsies were all more common in children than in adults.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제17권4호
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• pp.239-247
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• 2014

Purpose: There are no studies of hepatic glycogen storage diseases (GSDs) other than type I and III in Korea. We aimed on investigating the characteristics of hepatic GSDs in Korea diagnosed and followed at a single center. Methods: We retrospectively analyzed patients who were diagnosed as GSD and followed at Samsung Medical Center from January, 1997 to December, 2013. Clinical manifestations, laboratory results, treatment, and prognosis were investigated. Results: Twenty-one patients were included in the study. The types of 17 patients were confirmed by enzyme activity tests and/or gene analysis. GSD Ia was diagnosed in 7 patients (33.3%), Ib in 1 patient (4.8%), III in 2 patients (9.5%), IV in 1 patient (4.8%), and IX in 6 patients (28.6%). Types other than GSD I constituted 52.9% (9/17) of the patients diagnosed with a specific type of hepatic GSD. The median age at presentation was 2 years. Hepatomegaly was observed in 95.2%, elevated liver transaminases in 90.5%, and hyperlactacidemia in 81.0% of the patients. The duration for follow-up was $77{\pm}62.0$ months. Uncooked corn starch was initiated in all the patients. No mortality was observed during the follow-up period, and liver transplantation was performed in 14.3%. Conclusion: Types other than GSD I comprised more than half of the patients diagnosed with a specific type of hepatic GSD. Clinical suspicion and thorough evaluation of hepatic GSDs in Korea should be focused not only on GSD I, but also on other types.