• Title/Summary/Keyword: Skin abnormalities

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A Case Report for a Korean Patient with Mucopolysaccharidosis IIIA Confirmed by Biochemical and Molecular Genetic Investigation (생화학적 검사 및 분자유전학적 검사에 의해 뮤코다당증 제3A형으로 진단된 한국인 환자의 증례 보고)

  • Kim, Borahm;Cho, Sung Yoon;Sohn, Young Bae;Park, Hyung-Doo;Lee, Soo-Youn;Song, Junghan;Jin, Dong-Kyu
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.15 no.1
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    • pp.44-48
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    • 2015
  • Mucopolysaccharidosis (MPS) IIIA is a lysosomal storage disorder caused by abnormalities of the enzyme Heparan N-sulfatase that is required for degradation of heparan sulfate. The patient in this study was a 5 year-old boy who presented with macrocephaly and developmental delay. Urinary excretion of glycosaminoglycan was increased (26 g/moL creatinine, reference range: <7 g/moL creatinine) and a distinct band of heparan sulfate was shown in electrophoresis. Heparan N-sulfatase activity was significantly decreased in skin fibroblasts (0.2 pmoL/min/mg protein, reference range: 9-64 pmoL/min/mg protein). PCR and direct sequencing analysis of the SGSH gene showed compound heterozygous mutations: c.1040C>T (p.S347F) and c.703G>A (p.D235N). This is the first report for a Korean patient with MPS IIIA who was confirmed by biochemical investigation and molecular genetic analyses.

Assessment of Systemic Arterial Thromboembolism with Multi-Slice Spiral CT in a Dog (개에서 다중나선형 CT촬영에 의한 동맥혈전색전증의 평가)

  • Shin, Seung-Ho;Lee, Ki-Chang
    • Journal of Veterinary Clinics
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    • v.24 no.2
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    • pp.208-213
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    • 2007
  • A 15 kg 6-year-old intact male Jindo dog with a history of a respiratory distress, hindlimb paralysis with necrosed skin of dorsal digit for three weeks was referred to Animal Medical Center, Chonbuk National University. Heartworm infection was identified by kit examination. In plain thoracic radiographs, dilated pulmonary arteries reverse D sign and focal interstitial pattern was compatible with heartworm infection and possible pulmonary thromboembolism. Abdominal radiographs showed poor serosal detail indicating fluid accumulation within peritoneal cavity. No evidence of musculoskeletal abnormalities was found. Ultrasonography presented focal wedge-shaped hyperechogenecity on the both poles of left kidney, weak or absent pulse on the distal to the external iliac artery as well as ascites and irregular liver margin. Multi-organ failure was strongly supposed by blood profile including leukocytosis, anemia, hemoglobinuria bililubinemia, hypoalbuminemia, imbalance of electrolytes, and increased hepatic and renal function values. Interestingly, the glucose level is remarkably lower in pelvic limb compared to thoracic limb. Suspected pulmonary thromboembolism, renal infarction and femoral arterial embolization causing hindlimb paralysis and dermatic necrosis were confirmed by 3D reconstructed CT imaging. Prior to taking a consideration of euthanasia, interventional radiology was experimentally attempted but failed due to not recovered from general anesthesia. Early and accurate diagnosis of thromboembolism is valuable and 3D reconstructed CT images might be very useful to show the correct way to treat effectively.

Prevention of vibriosis in sea bass, Dicentrarchus labrax using ginger nanoparticles and Saccharomyces cerevisiae

  • Korni, Fatma M.M.;Sleim, Al Shimaa A.;Abdellatief, Jehan I.;Abd-elaziz, Rehab A.
    • Journal of fish pathology
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    • v.34 no.2
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    • pp.185-199
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    • 2021
  • Vibriosis is an important septicemic bacterial disease that affects a variety of commercial fish species, including cultured Dicentrarchus labrax. Nanotechnology has become an important modern tool for fish diseases prevention. Furthermore, nanomaterials have the ability to prevent and treat fish diseases. The current study was aimed to identify the causative agent of massive mortality of D. labrax commercial farm in Alexandria, Egypt. Experimental infection and the median lethal dose (LD50) of pathogenic isolate were assessed. Also, the effect of ginger nanoparticles (GNPs) and Sacchromyces cerevisiae as feed additives for prevention of vibriosis in D. labrax was carried out. Similarly, the tissue immunstimulant genes, IL-1β and TLR2 were measured in the spleen of feeding groups. The clinical signs of naturally diseased D. labrax showed corneal opacity and paleness of gills with excessive mucous secretion. The post-mortem abnormalities were severe hemorrhage and adhesion of internal organs. After bacteriological isolation and identification, the causative agent of mortality in the current study was Vibrio alginolyticus. The LD50 of V. alginolyticus was 1.5×105.4 CFU/ml. The experimentally infected D. labrax showed ulceration, exophthalmia and skin hemorrhages. The post-mortem findings of the experimentally infected D. labrax revealed internal hemorrhage, spleen darkness and paleness of liver. There is no mortality and 100% RPS in groups fed GNPs then injected with V. alginolyticus, in those fed a combination of GNPs and S. cerevisiae and a group fed normal diet then injected with physiological saline (control negative), respectively. Contrarily, there was 10% mortality and 87.5 RPS in the group fed S. cerevisae then injected with V. alginolyticus. On the other hand, the control positive group showed 79% mortality. The spleen IL-1β and TLR2 immunostimulant genes were significantly increased in groups of fish fed GNNP, S. cerevisiae and a combination of GNPs and S. cerevisiae, respectively compared to control group. The highest stimulation of those immunostimulant genes was found in the group fed a combination of GNPs and S. cerevisiae, while fish fed S. cerevisiae had the lowest level. Dietary combination of GNPs and S. cerevisiae was shown to be efficient in preventing of vibriosis, with greatest stimulation of spleen IL-1β and TLR2 immunostimulant genes.

Clinical Characteristics of Pulmonary Histiocytosis X (폐조직구증식증의 임상적 특징)

  • Hwang, Yong-Ii;Park, Gun-Min;Yim, Jae-Joon;Yoo, Chul-Gyu;Lee, Choon-Taek;Kim, Young-Whan;Han, Sung-Koo;Shim, Young-Soo
    • Tuberculosis and Respiratory Diseases
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    • v.51 no.4
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    • pp.346-353
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    • 2001
  • Background : Pulmonary Langerhans cell histiocytosis forms part of a spectrum of diseases that are characterized by a monoclonal proliferation and infiltration of organs by Langerhans cells. Several organ systems may be involved in Langerhans cell histiocytosis, including the lungs, bone, skin, pituitary gland, liver, lymph nodes and thyroid. Pulmonary histiocytosis X represents 2.8% of interstitial lung disease. Here we present the clinical, radiological, therapeutic aspects of pulmonary histiocytosis X. Method : Fourteen cases of biopsy-proven pulmonary histiocytosis X patients who were diagnosed in Seoul National University Hospital during the period from January 1990 to December 1998 were analyzed retrospectively. Result : There were 12 men and 2 women in this study. The initial presenting symptoms were dyspnea, cough, chest pain, which was associated with the pneumothorax, and chest radiography abnormalities. Only 8 patients (57%) were smokers. There were 5 patients with extra-pulmonary histiocytosis (pituitary, bone, skin). Eight patients had received the chemotherapy. There were no mortalities and only one patient experienced an aggravation of symptom during the follow-up period. Conclusion : In contrast to previous reports from other countries, the patients with pulmonary histiocytosis X in this study presented with several different clinical characteristics, such as a male predominance, relatively low smoker's rate, and a better prognosis.

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Relationship between the Economical Defects of Broiler Meat Carcass and Quality Grade A with a Meat Grader (경제성 관련 계육 도체 결함과 축산물등급사에 의한 품질 A등급의 상관관계)

  • Choe, Jun-Ho;Lee, Jae-Cheong;Jo, Cheor-Un
    • Food Science of Animal Resources
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    • v.29 no.4
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    • pp.494-499
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    • 2009
  • To investigate the relationship between the carcass defects considered by a meat grader and quality grade A, a total of 59,700 broiler carcasses, which were reared from September 1, 2007 to August 31, 2008 and randomly selected from 597 broiler farms with 100 broilers in each farm, were graded by a meat grader using the Korean meat grading system. The average slaughter weight of the broilers increased at the 31- to 33-d age but decreased after the 37-d age. The average daily gain to grade A ratio of the broilers was highest in autumn, and the bruises, flesh blemishes, and abnormalities of the carcasses were found to have been most frequent in winter (p<0.05). The number of economical defects (EDs) increased with the increase in the slaughter age, and the correlation coefficients of the EDs and grade A were -0.88, -0.48, and -0.12 in terms of the conformation, exposed flesh/cuts/tears, and skin discolorations of the carcasses, respectively. The correlation coefficients of the non-economical defects (NEDs) and the grade A of the broiler carcasses were -0.16, -0.22, and -0.32 in terms of the feathers, alien substances, and carcass treatment, respectively. The total number of defects (EDs + NEDs) and the grade A were highly correlated (-0.93), and in particular, the correlation between the grade A and the EDs was -0.91. It can be deemed that the grade A from a meat grader is highly correlated with the EDs. Therefore, the results suggest that broiler carcass grade evaluation using a meat grading system can provide direct information on profits in the market. The resulting carcass grade of broilers can also provide the information needed to decide on a proper incentive for a farm that has attained a good quality grade.

Supraclavicular Brachial Plexus block with Arm-Hyperabduction (상지(上肢) 외전위(外轉位)에서 시행(施行)한 쇄골상(鎖骨上) 상완신경총차단(上腕神經叢遮斷))

  • Lim, Keoun;Lim, Hwa-Taek;Kim, Dong-Keoun;Park, Wook;Kim, Sung-Yell;Oh, Hung-Kun
    • The Korean Journal of Pain
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    • v.1 no.2
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    • pp.214-222
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    • 1988
  • With the arm in hyperabduction, we have carried out 525 procedures of supraclavicular brachial plexus block from Aug. 1976 to June 1980, whereas block with the arm in adduction has been customarily performed by other authors. The anesthetic procedure is as follows: 1) The patient lies in the dorsal recumbent position without a pillow under his head or shoulder. His arm is hyperabducted more than a 90 degree angle from his side, and his head is turned to the side opposite from that to be blocked. 2) An "X" is marked at a point 1 cm above the mid clavicle, immediately lateral to the edge of the anterior scalene muscle, and on the palpable portion of the subclavian artery. The area is aseptically prepared and draped. 3) A 22 gauge 3.5cm needle attached to a syringe filled with 2% lidocaine (7~8mg/kg of body weight) and epineprine(1 : 200,000) is inserted caudally toward the second portion of the artery where it crosses the first rib and parallel with the lateral border of the muscle until a paresthesia is obtained. 4) Paresthesia is usually elicited while inserting the needle tip about 1~2 em in depth. If so, the local anesthetic solution is injected after careful aspiration. 5) If no paresthesia is elicited, the needle is withdrawn and redirected in an attempt to elicit paresthesia. 6) If, after several attempts, no paresthesia is obtained, the local anesthetic solution is injected into the perivascular sheath after confirming that the artery is not punctured. 7) Immediately after starting surgery, Valium is injected for sedation by the intravenous route in almost all cases. The age distribution of the cases was from 11 to 80 years. Sex distribution was 476 males and 49 females (Table 1). Operative procedures consisted of 103 open reductions, 114 skin grafts combined with spinal anesthesia in 14, 87 debridements, 75 repairs, i.e. tendon (41), nerve(32), and artery (2), 58 corrections of abnormalities, 27 amputations above the elbow (5), below the elbow (3) and fingers (17), 20 primary closures, 18 incisions and curettages, 2 replantations of cut fingers. respectively (Table 2). Paresthesia was obtained in all cases. Onset of analgesia occured within 5 minutes, starting in the deltoid region in almost all cases. Complete anesthesia of the entire arm appeared within 10 minutes but was delayed 15 to 20 minutes in 5 cases and failed in one case. Thus, our success rate was nearly 100%. The duration of anesthesia after a single injection ranged from $3\frac{1}{2}$ to $4\frac{1}{2}$, hours in 94% of the cases. The operative time ranged from 0.5 to 4 hours in 92.4% of the cases(Table 3). Repeat blocks were carried out in 33 cases when operative times which were more than 4 hours in 22 cases and the others were completed within 4 hours (Table 4). Two patients of the 33 cases, who received microvasular surgery were injected twice with 2% lidocaine 20 ml for a total of $13\frac{1}{2}$ hours. The 157 patients who received surgery on the forearms or hands had pneumatic tourniquets (250 torrs) applied without tourniquet pain. There was no pneumothorax, hematoma or phrenic nerve paralysis in any of the unilateral and 27 bilateral blocks, but there was hoarseness in two, Horner's syndrome in 11 and shivering in 7 cases. No general seizures or other side effects were observed. By 20ml of 60% urcgratin study, we confirm ed the position of the needle tip to be in a safer position when the arm is in hyperabduction than when it is in adduction. And also that the humoral head caused some obstraction of the distal flow of the dye, indicating that less local anesthetic solution would be needed for satisfactory anesthesia. (Fig. 3,4).

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Investigation of Diseases of Thai koi, Anabas testudineus (BLOCH) from Farming Conditions in Winter (겨울철 양식장 환경에서 Tai koi, Anabas testudineus (BLOCH)의 임상병리학적 특징)

  • Ahmed, Gias Uddin;Dhar, Mili;Absar Khan, Mohammed Nurul;Choi, Jae-Suk
    • Journal of Life Science
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    • v.17 no.10
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    • pp.1309-1314
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    • 2007
  • Investigation on health conditions of Thai koi (Anabas testudineus) were carried out through clinical and histopathological observations from different farms of Mymensingh district for seven months during August 2006 to February 2007. Fish sampling and water quality parameters (temperature, dissolved oxygen and pH) were monitored on a monthly basis. Clinical examination of fishes was also carried out for any kind of abnormalities at monthly intervals. Samples of skin, muscle, gill, liver and kidney were observed by histological technique. Among the water quality parameters the values of water temperature, dissolved oxygen were found to be at unfavorable level for fish during the colder months in the farms. Clinically it was observed that fishes were more affected from December to January and almost normal in appearance during August to September and February. Different clinical symptoms like scale loss, dermal lesion, ulcer and loss of caudal fin were noticed in December and January. In histopathological study, structures of fish organs were normal from August to September. In the months of October and November, minor pathologies were found to be started. Marked pathological changes like necrosis, pyknosis, inflammation, hemorrhage, hypertrophy, hyperplasia, missing of primary and secondary gill lamellae were observed in the months of December and January. Whereas, in the month of February the pathological condition of fish gradually reduced. Again when considered individual fish farm, fishes of Reliance Aqua Farm were more affected than Sotota Matshya Hatchery. The study showed that severity of clinical and pathological changes were increased in December and January. During the period EUS and protozoan diseases were noticed in A. testudineus of the investigated farms.

Clinicopathologic features of Acute Interstitial Pneumonia (급성 간질성 폐렴의 임상적 고찰)

  • Shim, Jae-Jeong;Park, Sang-Muyn;Lee, Sang-Hwa;Lee, Jin-Gu;Cho, Jae-Yun;Song, Gwan-Gyu;In, Kwang-Ho;Yoo, Se-Hwa;Kang, Kyung-Ho
    • Tuberculosis and Respiratory Diseases
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    • v.42 no.1
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    • pp.58-66
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    • 1995
  • Background: Acute interstitial pneumonia is a relatively rare form of interstitial pneumonia, since the vast majority of interstitial pneumonia have a more chronic course. It corresponds to the lesion described by Hamman and Rich, as Hamman-Rich disease in 1944. Another name in the clinical literature is accelerated interstitial pneumonia, idiopathic acute respiratory distress syndrome (idiopathic ARDS), and the organizing stage of diffuse alveolar damage. Acute interstitial pneumonia differs from chronic interstitial pneumonia by clinical and pathologic features. Clinically, this disease is characterized by a sudden onset and a rapid course, and reversible disease. Method and Purpose: Five cases of pathologically proven acute interstitial pneumonia were retrospectively studied to define the clinical, radiologic, and pathologic features. Results: 1) The five cases ranged in age from 31 to 77 years old. The onset of illness was acute in all patients, it began with viral-like prodrome 6~40 days prior to shortness of breath, and respiratory failure eventually developed in all patients. In 2 cases, generalized skin rash was accompanied with flu-like symptoms. Etiologic agent could not be identified in any case. 2) All patients had leukocytosis and severe hypoxemia. Pulmonary function test of 3 available cases shows restrictive ventilatory defect, and one survived patient(case 5) has a complete improvement of pulmonary function after dismissal. 3) Diffuse bilateral chest infiltrates were present radiologically. Theses were the ground-glass, consolidation, and reticular densities without honeycomb fibrosis in all patients. The pathologic abnormalities were the presence of increased numbers of macrophages and the formation of hyaline membranes within alveolar spaces. There was also interstitial thickening with edema, proliferation of immature fibroblast, and hyperplasia of type II pneumocyte. In the survived patient(case5), pathologic findings were relatively early stage of acute interstitial pneumonia, such as hyaline membrane with mild interstitial fibrosis. 4) Of the 5 patients, four patients died of respiratory failure 14~90 days after onset of first symptom, and one survived and recovered in symptoms, chest X ray, and pulmonary function test Conclusion: These results emphasize that acute interstitial pneumonia is clinically, radiologically, and pathologically distinct form of interstitial pneumonia and should be separated from the group of chronic interstitial pneumonia. Further studies will be needed to evaluate the pathogenesis and the treatment of acute interstitial pneumonia.

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