• Title/Summary/Keyword: Chronic granulomatous disease

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Occupational Lung Diseases: Spectrum of Common Imaging Manifestations

  • Alexander W. Matyga;Lydia Chelala;Jonathan H. Chung
    • Korean Journal of Radiology
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    • v.24 no.8
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    • pp.795-806
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    • 2023
  • Occupational lung diseases (OLD) are a group of preventable conditions caused by noxious inhalation exposure in the workplace. Workers in various industries are at a higher risk of developing OLD. Despite regulations contributing to a decreased incidence, OLD remain among the most frequently diagnosed work-related conditions, contributing to significant morbidity and mortality. A multidisciplinary discussion (MDD) is necessary for a timely diagnosis. Imaging, particularly computed tomography, plays a central role in diagnosing OLD and excluding other inhalational lung diseases. OLD can be broadly classified into fibrotic and non-fibrotic forms. Imaging reflects variable degrees of inflammation and fibrosis involving the airways, parenchyma, and pleura. Common manifestations include classical pneumoconioses, chronic granulomatous diseases (CGD), and small and large airway diseases. Imaging is influenced by the type of inciting exposure. The findings of airway disease may be subtle or solely uncovered upon expiration. High-resolution chest CT, including expiratory-phase imaging, should be performed in all patients with suspected OLD. Radiologists should familiarize themselves with these imaging features to improve diagnostic accuracy.

Targeted busulfan and fludarabine-based conditioning for bone marrow transplantation in chronic granulomatous disease

  • Ju, Hee Young;Kang, Hyoung Jin;Hong, Che Ry;Lee, Ji Won;Kim, Hyery;Song, Sang Hoon;Yu, Kyung-Sang;Jang, In-Jin;Park, June Dong;Park, Kyung Duk;Shin, Hee Young;Kim, Joong-Gon;Ahn, Hyo Seop
    • Clinical and Experimental Pediatrics
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    • v.59 no.sup1
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    • pp.57-59
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    • 2016
  • Chronic granulomatous disease (CGD) is a primary immunodeficiency disease caused by impaired phagocytic function. Hematopoietic stem cell transplantation (HSCT) is a definitive cure for CGD; however, the use of HSCT is limited because of associated problems, including transplantation-related mortality and engraftment failure. We report a case of a patient with CGD who underwent successful HSCT following a targeted busulfan and fludarabine reduced-toxicity myeloablative conditioning. Intravenous busulfan was administered once daily for 4 consecutive days (days -8 to -5), and the target area under the curve was $75,000{\mu}g{\cdot}hr/L$. Fludarabine ($40mg/m^2$) was administered once daily for 6 consecutive days from days -8 to -3. Antithymocyte globulin (2.5 mg/kg/day) was administered from days -4 to -2. The patient underwent successful engraftment and did not have any severe toxicity related to the transplantation. Conditioning with a targeted busulfan and fludarabine regimen could provide a better outcome for HSCT in CGD, with close regulation of the busulfan dose.

Sinonasal sarcoidosis of the maxillary sinus and infraorbital nerve: a case report

  • Joseph, Benny;Vyloppilli, Suresh;Sayd, Shermil;Ummer, Niyas
    • Journal of the Korean Association of Oral and Maxillofacial Surgeons
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    • v.41 no.4
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    • pp.217-221
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    • 2015
  • Sinonasal sarcoidosis in the head and neck region is infrequent. Its occurrence can be either isolated in combination with other systems. The literature reveals that the occurrence of sinonasal sarcoidosis without lung involvement is rare. In general, sarcoidosis is a chronic non-caseating granulomatous disease of unknown origin, often identified after biopsy. In this article, we report on a benign tumor of the face that produced a diagnostic dilemma, necessitating refinement of the surgical access and in toto removal of the benign tumor.

The oriental-western literal study of Crohn's disease (크론씨병(극한성(局限性) 장염(腸炎))에 관(關)한 한의학적(韓醫學的) 고찰(考察))

  • Choi, Chang-Woo;Son, Chang-Gyu;Cho, Chong-kwan
    • Journal of Haehwa Medicine
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    • v.9 no.2
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    • pp.251-268
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    • 2001
  • We arrived at the following conclusion after we have studied crohn's disease through the literatures of western and oriental medicine. 1. Crohn's disease has a characteristic of granulomatous necrosis and cicatricial inflammation which is called by segmental enteritis, granulomatous enteritis. This falls under the category of "Diarrhea", "Dysentery" and is similar to "small intestinal diarrhea", "acute temesmus", "splenic diarrhea" in oriental medicine. 2. In western medicine, the cause of crohn's disease indefinites, but it is presumed immunological unbalance of alimentary canal. In oriental medicine, it is summarized as the abnormal ascending and descending circulation of stomach and splenic energies, the hepatic stagnation, being the lower part of cleaning qi by exogenous disease, dyspeptic convulsion. 3. The presenting symptoms of crohn's disease are intermittent chronic diarrhea, fever, weight loss, abdominal spastic pain or abdominal discomfort. When anyone has a abdominal mass, a rectal abcess, and a rectal constriction by physical examination, we can doubt crohn's disease. 4. The methods of western medical treatment are a suppression of intestinal toxic contents and inflammatory mediator, a supply of nutritive substanceus to intestinal epithelial cell. Oriental medical treatments of these are "inducing diuresis", "warming kidney to reinforce yang", "nourishing qi to invigorate spleen", "eliminatin dampness by cooling" according to syndrome differentiations. As mentioned above, we can confirm possibility of oriental medical treatment that induces recovery of immunologic control function and we need advanced experiment, study, and clinical approach.

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Clinical Study on 1 Case of Soyangyin Patient Diagnosed as Crohn's Disease (크론씨병으로 진단된 소양인 환자 치험 1 례)

  • Lee, Seung-Hyun;Park, Darn-Seo
    • Journal of Physiology & Pathology in Korean Medicine
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    • v.21 no.5
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    • pp.1346-1351
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    • 2007
  • Crohn' disease is an auto-immune disease characterized by intermittent chronic diarrhea, high fever, weight loss, abdominal spastic pain or abdominal discomfort which is followed by granulomatous necrosis and cicatrical inflammation. It is also called segmental enteritis or granulomatous enteritis. In western medicine the exact cause is undefined, however it is presumed as an immunological unbalance in alimentary tract commoonly occured in ileum portion of small intestitine or ascending colon and therefore immuno suppressive agents(usually steroids) and anti-inflammatory drugs are prescribed. In case of emergency such as ileus, perforation of intestinal wall surgical methods are considered. In oriental medicine this falls under the category of diarrhea(泄瀉), dysentery(痢疾), splenic diarrhea(脾泄). As to the pathological mechanism the abnormal ascending and descending circulation of stomach and splenic energy(脾不升淸, 胃不下降) the hepatic stagnation(肝鬱氣滯) and dysfunction of small intestine in expelling urine and feces(小陽淸獨不利) all together causes such condition. Main treatments are inducing diuresis(利小便), warming kindey to reinforce yang(溫賢助陽), nourishing the middle energy to invigorate spleen(補中健脾), elimination of the dampness by cooling(淸熱燥濕). In this case the patient was diagnosed as soyangyin(少陽人) constitution and herb medicine soyangyin Hyongbangjihwan-tang(少陽人 荊防地黃湯), Sa-am acupuncture Sojangjeonggyeok(小腸政格) was applied. There was an significant improve in chief complaints and general conditions.

Case report of Johne's disease in Korean black goat (Capra hircus aegagrus) (흑염소에서의 요네병 발생 증례)

  • Rhyoo, Moon-Young;Jung, Ji-Youl;Lee, Kyung-Hyun;Her, Ji-Woong;Bae, You-Chan;Lee, Hyun-Kyoung;Baek, Kang-Hyun;Lee, Bo-Ram;Jean, Young-Hwa;Kim, Jae-Myung;Lee, Myoung-Heon;Yoon, Soon-Seek
    • Korean Journal of Veterinary Research
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    • v.53 no.4
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    • pp.273-276
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    • 2013
  • Two Korean black goat (approx. 2 and 3 years old) showing diarrhea and chronic weight loss were submitted to Animal and Plant Quarantine Agency. At necropsy, there were thickening of small intestine and enlargement of mesenteric lymph nodes. Microscopically, they had granulomatous enteritis in the small and large intestine and granulomatous lymphadenitis. By polymerase chain reaction (PCR) and acid fast stain, strong positive reaction and acid-fast rod bacteria were detected. According to the result of histopathology and PCR, we confirmed this case as Johne's disease. As far as we know, this is the first report of Johne's disease in Korean black goat.

Surgical Treatment of Chronic Constrictive Pericarditis (만성 교약성 심낭염의 외과적 치료)

  • Jang, Bong-Hyeon;Kim, Gyu-Tae
    • Journal of Chest Surgery
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    • v.20 no.2
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    • pp.317-322
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    • 1987
  • Records of 15 patients who underwent operation for constrictive pericarditis at this department from 1976 through 1984 were reviewed. All had hemodynamically significant pericardial constriction preoperatively, and pericardial disease was confirmed at operation. There were 12 males and 3 females in this series. Range of age varied from 7 years to 51 years. Clinical and histological study revealed granulomatous pericarditis compatible with the diagnosis of tuberculosis in 5 patients, non-specific chronic inflammatory changes in 6 patients and pyogenic pericarditis in 2 patients. Pericardiectomy was performed through a median sternotomy [11 cases] or bilateral anterior thoracotomy [4 cases]. The postoperative complications were observed in 4 patients. Low cardiac output was the most common complication [2 patients]. In 13 cases, excluding 2 operative deaths, preoperatively all were in New York Heart Association Class III or IV. At the time of discharge II [85%] were in Class I or II. 4 Cases were reoperated after original pericardiostomy and all resulted in marked improvement. Early surgical intervention is advisable in all patients in whom cardiac constriction is caused by either a thickened pericardium or a pericardial effusion before myocardial dysfunction occurs.

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Pelvic Actinomycosis Mimicking Malignancy of the Uterus: a Case Report

  • Shin, Dahye;Hwang, Jiyoung;Hong, Seong Sook;Lee, Eun Ji;Kim, Yon Hee
    • Investigative Magnetic Resonance Imaging
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    • v.23 no.2
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    • pp.136-141
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    • 2019
  • Pelvic actinomycosis is an uncommon infectious disease. It induces a chronic, suppurative illness characterized by an infiltrative and granulomatous response and, thus, the clinical and radiologic findings may mimic other inflammatory and neoplastic conditions. A 56-year-old female with a long-standing intrauterine device was diagnosed with pelvic actinomycosis manifesting as a large uterine mass with locally infiltrative spread into surrounding tissue that mimicked uterine malignancy. Actinomyces israelii infection was confirmed with a surgical specimen, and the patient was treated with antibiotic medication. Pelvic actinomycosis must be included in the differential diagnoses of patients with an infiltrative pelvic mass extending across tissue planes or in patients with findings of multiple microabscesses, particularly in a patient with an intrauterine device, even the lesion primarily involves the uterus.

Atypical Presentation of Chronic Granulomatous Disease in a Neonate with a Pulmonary Granuloma Mimicking a Tumor: A Case Report (신생아에서 종양으로 오인되는 폐 육아종의 비전형적인 소견을 보인 만성 육아종성 질환: 증례 보고)

  • Young Jin Yoo;Joo Sung Sun;Jang Hoon Lee;Hyun Joo Jung;Yeong Hwa Koh;Joonho Jung;Hyun Gi Kim
    • Journal of the Korean Society of Radiology
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    • v.81 no.4
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    • pp.990-995
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    • 2020
  • Chronic granulomatous disease (CGD) is an uncommon primary immune deficiency caused by phagocytes defective in oxygen metabolite production. It results in recurrent bacterial or fungal infections. Herein, we present a case of CGD with a large pulmonary granuloma in a neonate and review the imaging findings. The patient was a 24-day-old neonate admitted to the hospital with fever. A round opacified lesion was identified on the chest radiograph. Subsequent CT and MRI revealed a round mass with heterogeneous enhancement in the right lower lobe. There were foci of diffusion restriction in the mass. Surgical biopsy of the mass revealed chronic granuloma. Finally, the neonate was diagnosed with CGD caused by mutation of the gp91phox gene. Herein, we present the clinical and imaging findings of this unusual case of CGD.

A case study of chronic traumatic pericarditis (Hardware disease) in a Holstein cattle (젖소 만성 창상성심낭염의 증례보고 및 고찰)

  • Kim, Jongho;Lee, Kyunghyun;Roh, Su Gwon;Jo, Heon-Ho;So, ByungJae;Kim, Heui-Jin
    • Korean Journal of Veterinary Service
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    • v.44 no.1
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    • pp.45-49
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    • 2021
  • A 23-month-old Holstein cattle showed excess salivation and reluctance of walking and suddenly died after forced sudden movements. Grossly, numerous fibrous adhesions were present within cranial abdominal cavity including the reticulum and diaphragm and thoracic cavity involving lungs, pericardial sac, and heart. A perforation made by a 10 cm-long sharp-ended wire was detected in the reticulum. Histopathologically, fibrous suppurative epicarditis and myocardial necrosis were observed. Fibrosis with neovascularization were found in lungs, spleen, and liver. And granulomatous reticulitis was observed. For differential diagnosis, no pathogenic bacteria were detected through microbiological tests and PCR results were also negative for bovine susceptible pathogenic antigens. Based on the gross and histopathological examination, we diagnosed this case as chronic traumatic pericarditis. Cattle are inquisitive and prone to swallow various kinds of metallic foreign bodies since they do not use their lips. Therefore, avoiding ingestion of metallic objects in animal feed and animal areas by careful environmental management of farms is required and farmers should give the adequate minerals and vitamins into the feeds not to lick or shallow foreign bodies in case of mineral deficiency. For veterinary practitioners, physical examination, blood tests, and diagnostic imaging (X-ray and Ultrasonography) are required for an exact diagnosis. Furthermore, placing the magnets in rumen would be effective for prophylactic administrations.