• Title/Summary/Keyword: Cystic fibrosis

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A Case of Neurilemmoma of Intercostal Nerve (늑간신경에 발생한 신경초종 1예)

  • Lee, Du-Yeon;Gye, Gi-Sik;Song, Gye-Yong
    • Journal of Chest Surgery
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    • v.11 no.3
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    • pp.239-245
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    • 1978
  • Recently, we experienced a case of rare neurilemmoma originated from intercostal nerve [9th] in the right chest wall in a 25 year old male officer. The tumor was incidentally found in the routine chest X ray, where the round well circumscribed mass tumor the ninth rib with notching and sclerotic margin, suggesting slowly growing benign benign of chest wall was revealed and the tumor mass was easily extirpated in the exploratory thoracotomy, with uneventful recovery. Grossly, the tumor was firm, partly soft and well circumscribed, measuring 4.5X3.0X 3.0 cm with yellowish smooth outer surface, attached with intercostal nerve trunk. Cut surface exhibits partly grayish white and largely hemorrhagic areas. Microscopically, the characteristic palisading arrangement of schwann cells and Verocay bodies are seen but dominant features are cystic degeneration and hemorrhage with organization and fibrosis. The sheath of intercostal nerve and capsule of neurilemmoma were con joined. There is no evidence of malignancy. The tumor was confirmed as neurilemmoma of intercostal nerve, Antony type B.

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Pseudomonas aeruginosa Biofilm, a Programmed Bacterial Life for Fitness

  • Lee, Keehoon;Yoon, Sang Sun
    • Journal of Microbiology and Biotechnology
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    • v.27 no.6
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    • pp.1053-1064
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    • 2017
  • A biofilm is a community of microbes that typically inhabit on surfaces and are encased in an extracellular matrix. Biofilms display very dissimilar characteristics to their planktonic counterparts. Biofilms are ubiquitous in the environment and influence our lives tremendously in both positive and negative ways. Pseudomonas aeruginosa is a bacterium known to produce robust biofilms. P. aeruginosa biofilms cause severe problems in immunocompromised patients, including those with cystic fibrosis or wound infection. Moreover, the unique biofilm properties further complicate the eradication of the biofilm infection, leading to the development of chronic infections. In this review, we discuss the history of biofilm research and general characteristics of bacterial biofilms. Then, distinct features pertaining to each stage of P. aeruginosa biofilm development are highlighted. Furthermore, infections caused by biofilms on their own or in association with other bacterial species (i.e., multispecies biofilms) are discussed in detail.

Channelopathies

  • Kim, June-Bum
    • Clinical and Experimental Pediatrics
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    • v.57 no.1
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    • pp.1-18
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    • 2014
  • Channelopathies are a heterogeneous group of disorders resulting from the dysfunction of ion channels located in the membranes of all cells and many cellular organelles. These include diseases of the nervous system (e.g., generalized epilepsy with febrile seizures plus, familial hemiplegic migraine, episodic ataxia, and hyperkalemic and hypokalemic periodic paralysis), the cardiovascular system (e.g., long QT syndrome, short QT syndrome, Brugada syndrome, and catecholaminergic polymorphic ventricular tachycardia), the respiratory system (e.g., cystic fibrosis), the endocrine system (e.g., neonatal diabetes mellitus, familial hyperinsulinemic hypoglycemia, thyrotoxic hypokalemic periodic paralysis, and familial hyperaldosteronism), the urinary system (e.g., Bartter syndrome, nephrogenic diabetes insipidus, autosomal-dominant polycystic kidney disease, and hypomagnesemia with secondary hypocalcemia), and the immune system (e.g., myasthenia gravis, neuromyelitis optica, Isaac syndrome, and anti-NMDA [N-methyl-D-aspartate] receptor encephalitis). The field of channelopathies is expanding rapidly, as is the utility of molecular-genetic and electrophysiological studies. This review provides a brief overview and update of channelopathies, with a focus on recent advances in the pathophysiological mechanisms that may help clinicians better understand, diagnose, and develop treatments for these diseases.

A Case of Mucus Plug Impaction resulted in Bronchial Obstruction (광범위 기관지 폐쇄를 유발한 점액전 1례)

  • 고중화;전영명;김휘준
    • Korean Journal of Bronchoesophagology
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    • v.3 no.2
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    • pp.313-317
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    • 1997
  • Airway mucus provides the protective functions such as lubrication, barrier, disposal of trapped materials, and humidification. In the normal state, the mucus do not interfere with Bas transport and the other vital functions of lung. In diseases such as asthma, bronchitis, and cystic fibrosis, the mucus hypersecretion was physiologically developed in the response of multiple neurohumoral mechanism system. And regardless of the mechanism, many clinical sequelae result from mucus hypersecretion: atelectasis, infection, increased airway resistance, increased work of breathing, increased cough with its resultant complication. And the condensation of mucus tv mucus hypersecretion can make the mucus plug by which bronchial obstruction is developed. We have experienced a 7 Pear-old male patient with recurrent pneumonic symtom, which the bronchial obstruction was developed by the impacted mucus plug on the bronchoscopic finding. We report this case with the review of literature.

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Mechanisms and Control Strategies of Antibiotic Resistance in Pathological Biofilms

  • Luo, Ying;Yang, Qianqian;Zhang, Dan;Yan, Wei
    • Journal of Microbiology and Biotechnology
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    • v.31 no.1
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    • pp.1-7
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    • 2021
  • Bacterial biofilm is a community of bacteria that are embedded and structured in a self-secreted extracellular matrix. An important clinical-related characteristic of bacterial biofilms is that they are much more resistant to antimicrobial agents than the planktonic cells (up to 1,000 times), which is one of the main causes of antibiotic resistance in clinics. Therefore, infections caused by biofilms are notoriously difficult to eradicate, such as lung infection caused by Pseudomonas aeruginosa in cystic fibrosis patients. Understanding the resistance mechanisms of biofilms will provide direct insights into how we overcome such resistance. In this review, we summarize the characteristics of biofilms and chronic infections associated with bacterial biofilms. We examine the current understanding and research progress on the major mechanisms of antibiotic resistance in biofilms, including quorum sensing. We also discuss the potential strategies that may overcome biofilm-related antibiotic resistance, focusing on targeting biofilm EPSs, blocking quorum sensing signaling, and using recombinant phages.

Minority report; Diketopiperazines and Pyocyanin as Quorum Sensing Signals in Pseudomonas aeruginosa (Minority report; Pseudomonas aeruginosa의 정족수 인식(쿼럼 센싱) 신호물질로써의 Diketopiperazines과 Pyocyanin)

  • Lee, Joon-Hee
    • Korean Journal of Microbiology
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    • v.44 no.2
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    • pp.85-92
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    • 2008
  • Pseudomonas aeruginosa is an opportunistic human pathogen, causing a wide variety of infections including cystic fibrosis, microbial keratitis, and burn wound infections. The cell-to-cell signaling mechanism known as quorum sensing (QS) plays a key role in these infections and the QS systems of P. aeruginosa have been most intensively studied. While many literatures that introduce the QS systems of P. aeruginosa have mostly focused on two major acyl-homo serine lactone (acyl-HSL) QS signals, N-3-oxododecanoyl homoserine lactone (3OC12) and N-butanoyl homoserine lactone (C4), several new signal molecules have been discovered and suggested for their significant roles in signaling and virulence of P. aeruginosa. One of them is PQS (Pseudomonas quinolone signal; 2-heptyl-3-hydroxy-4-quinolone), which is now considered as a well-characterized major signal meolecule of P. aeruginosa. In addition, recent researches have also suggested some more putative signal molecules of P. aeruginosa, which are diketopiperazines (DKPs) and pyocyanin. DKPs are cyclic dipeptides and structurally diverse depending on what amino acids are involved in composition. Some DKPs from the culture supernatant of P. aeruginosa are suggested as new diffusible signal molecules, based on their ability to activate Vibrio fischeri LuxR biosensors that are previously considered specific for acyl-HSLs. Pyocyanin (1-hydroxy-5-methyl-phenazine), one of phenazine derivatives produced by P. aeruginosa is a characteristic blue-green pigment and redox-active compound. This has been recently suggested as a terminal signaling factor to upregulate some QS-controlled genes during stationary phase under the mediation of a transcription factor, SoxR. Here, details about these newly emerging signaling molecules of P. aeruginosa are discussed.

FIBROSIS THAT OCURRED AFTER CONSERVATIVE THERAPY OF LARGE ODONTOGENIC KERATOCYST (광범위한 치성각화낭종의 보존적 치료후 발생한 섬유증)

  • Kwon, Taek-Kyun;Byun, June-Ho;Kim, Yong-Deok;Shin, Sang-Hun;Kim, Uk-Kyu;Chung, In-Kyo
    • Journal of the Korean Association of Oral and Maxillofacial Surgeons
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    • v.30 no.2
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    • pp.162-164
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    • 2004
  • Odontogenic keratocysts are developmental odontogenic cysts which derived from rests of dental laminas and often found in posterior area of mandible. Because this lesion has tendency of frequent recurrence, treatment of choice is often radical removal of the lesion. Sometimes in case of large cysts, however, conservative therapy like marsupialization is often selected in treatment plan. A 39-years old woman referred to our department for evaluation of large radiolucent lesion that occupies the areas from mandible angle to upper part of ramus and condyle. In cytology, the lesion was identified as odontogenic keratocyst. Marsupialization was our treatment of choice, and the result was so favorable. 2 years later, there was small radiolucent lesion on upper part of mandibular ramus on panoramic view. It was suspected as recurred lesion, and excisional biopsy was done. On biopsy result, it was not a cystic lesion but fibrosis.

A Case of Secondary Sclerosing Cholangitis in Langerhans Cell Histiocytosis (랑게르한스세포 조직구증 환아에서 발생한 속발성 경화성 담관염 1례)

  • Kim, Ja-Hyung;Choi, Bo-Hwa;Kim, Kyung-Mo;Moon, Hyung-Nam
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.4 no.1
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    • pp.120-124
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    • 2001
  • Sclerosing cholangitis is a chronic cholestatic disease characterized by inflammation and obliterative fibrosis of the bile ducts, leading to biliary cirrhosis and ultimately to liver failure. In children, sclerosing cholangitis is known to be associated with Langerhans cell histiocytosis, as well as with congenital immunodeficiencies and cystic fibrosis. Secondary sclerosing cholangitis is suspected in Langerhans cell histiocytosis with chronic cholestasis, liver dysfunction and portal hypertension. Unlike primary sclerosing cholangitis, the cholangitis associated Langerhans cell histiocytosis is destructive in nature and progresses more rapidly to biliary cirrhosis, therefore uniformly the prognosis is poor. In this setting, liver transplantation should be considered early in children with sclerosing cholangitis complicating Langerhans cell histiocytosis before end-stage liver failure and variceal bleeding. We experienced a case of secondary sclerosing cholangitis in Langerhans cell histiocytosis in a 2-year-old boy. We report this case with brief review of the related literatures.

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Histopathological Study and Expression of Beta-Catenin in Congenital Choledochal Cyst in a Tertiary Care Pediatric Referral Center in South India

  • Rashmi Tresa Philpose;Abdul Aleem Mohammed;Ashrith Reddy Gowni
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.27 no.1
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    • pp.62-70
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    • 2024
  • Purpose: Choledochal cysts are congenital anomalies that occur as localized cystic or fusiform dilatations of the biliary tree. Reflux and stasis of pancreatic enzymes in the biliary duct may relate to the development of intestinal metaplasia which might be an important factor related to the carcinogenesis of choledochal cyst, thus the expression of beta-catenin in the metaplastic epithelium might be associated with malignant transformation of choledochal cyst epithelium. Methods: This study was conducted at a tertiary care pediatric center between October 2014 and March 2017. Forty patients were evaluated for epithelial lining, mural ulceration, fibrosis, inflammation, and metaplasia. Results: Out of 40, 12 cases (30.0%) were the infantile age group and 28 cases (70.0%) were in the classic pediatric group. Ulceration was classified as grade 0 (14 cases, 35.0%), grade 1 (17 cases, 42.5%), or grade 2 (nine cases, 22.5%). Inflammation was classified as grade 0 (2 cases, 5.0%), grade 1 (26 cases, 65.0%), or grade 2 (12 cases, 30.0%). Fibrosis was classified as grade 0 (five cases, 12.5%), grade 1 (11 cases, 27.5%), grade 2 (17 cases, 42.5%), or grade 3 (seven cases, 17.5%). Metaplasia was noted in five (12.5%) out of 40 cases. All choledochal cysts with metaplasia showed beta-catenin nuclear positivity on immunohistochemistry and were followed up. Conclusion: This study emphasizes the importance of detailed histopathological examination and documentation of metaplastic changes. Metaplasia was associated with beta-catenin nuclear positivity. These findings suggest a potential role for beta-catenin as a marker of metaplastic changes in choledochal cysts.

A Case of Sj$\ddot{o}$gren's Syndrome with Interstitial Lung Disease and Multiple Cystic Lung Disease (간질성 및 다낭성 폐질환이 동반된 쇼그렌 증후군 1례)

  • Jang, Dai-Yong;Shin, Byung-Chul;Jung, Ki-Young;Kim, Jong-O;Yang, Jong-Tae;Joo, Yoo-Chul;Lee, Seung-Il
    • Tuberculosis and Respiratory Diseases
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    • v.51 no.6
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    • pp.597-602
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    • 2001
  • A 47-years-old woman presented with a 2-month history of a dry mouth and dry cough. The patient had been taking medication for Sj$\ddot{o}$gren's syndrome for approximately 7 years. The chest radiography showed multiple cystic lesions and a hazy density in both lower lung fields. The HRCT showed a diffuse ground glass like appearance and multiple variable sized cystic lesions in both lung fields. After medication, the symptoms were aggravated. Bronchoscopy was preformed with a transbronchial lung biopsy. The biopsies showed an infiltration of lymphocytes, neutrophils, monocytes and histiocytes through the interstitial space of the alveola and a widening of the alveolar septa. However, the histological findings of the cysts were not obtained. Sj$\ddot{o}$gren's syndrome is a slowly progressive inflammatory autoimmune disease, which is characterized by lymphocyte mediated destruction of the exocrine glands, with pulmonary involvement in approximately 19-65%, High-resolution CT is a sensitive technique for assessing the pulmonary involvement in patients with Sj$\ddot{o}$gren's syndrome. Although a lung biopsy is not always necessary for establishing a diagnosis of an interstitial lung disease in Sj$\ddot{o}$gren's syndrome. A lung biopsy may reveal a wide spectrum of changes ranging from a mild inflammatory response to end stage fibrosis with honeycombing. Because of the predominantly peribronchiolar inflammatory infiltration and inspissated secretions the cysts were suspected to have been formed by the ball-valve phenomen. However, no definite evidence was obtained.

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