• Title/Summary/Keyword: cystic fibrosis

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The Study for Results of Complex Cystic Breast Masses by Biopsy on Ultrasound (초음파상 복합성 낭성 유방 종괴의 조직 검사 결과에 관한 연구)

  • Kang, Hye-Kyoung;Dong, Kyung-Rae
    • Journal of radiological science and technology
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    • v.31 no.2
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    • pp.129-134
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    • 2008
  • Purpose: We examined the roles of Ultrasonography conductors by analyzing the results of tissue biopsy of complex cystic masse under the guidance of breast US. Objects and methods: This study was performed to a group of 178 who showed breast US indicating complex cystic masses among 342 patients who were definitely diagnosed by tissue biopsies and operations in our hospital from June 30th, 2003 to June 30th, 2007. The evaluation of tissues around, calcification, the distribution state of blood flow were excluded from the analysis subjects and logic 200 made by GE corporation and gun for core biopsy(Kimal corp., K7/MBD23) were used in this study. Results: The biopsy results of 178 subjects showed FCC (fibrocystic change)(n=56 : 31.4%), Fibrosis (n=41 : 23.0%), Fibroadenoma (n=20 : 11.2%), Epithelial hyperplasia (n=17 : 9.6%), Carcinoma (n=15 : 8.4%), Fibroadipose (n=8 : 4.5%), Sclerosing adenosis (n=7 : 3.9%), Duct ectasia (n=5 : 2.8%), Papiloma (n=5 : 2.8%), and Fat necrosis (n=1 : 0.6%), Hemangioma (n=1 : 0.6%), Abscess (n=1 : 0.6%), Dystrophic calcification(n=1 : 0.6%). Conclusion: The US showed that the results of the tissue biopsy of complex cystic masses were mostly carcinoma(8.4%). Most of them were benign and only 9.6% of epithelial hyperplasia which has high progression rate into malignant tumors epidemically showed malignancy. Most of them were included in the spectrum of fibrous cystic nodule. Even though these results are confirmed, further studies are required. As a result, a nodule which is not certified by US should be right to take the tissue biopsy, but if it's difficult due to patients or another reasons, re-check tests in three months are required. And systemic ultrasonography evaluation should be well recognized to conduct more careful and specific tests.

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Secondary renal amyloidosis in a 13-year-old girl with bronchiectasis

  • Yang, Eun-Ae;Lee, Dong-Won;Hyun, Myung-Chul;Cho, Min-Hyun
    • Clinical and Experimental Pediatrics
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    • v.53 no.7
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    • pp.770-773
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    • 2010
  • A 13-year-old girl was diagnosed with non-cystic fibrosis (CF)-related multifocal bronchiectasis accompanied by nephrotic-range proteinuria of unknown cause. On renal biopsy, there were many segmental homogeneous deposits of amyloid tissue with positive Congo red staining in the glomeruli and interstitium. On electron microscopy, relatively straight, non-branching, randomly arranged amyloid fibrils were showed in the mesangium of the glomeruli. These fibrils were approximately 10 nm in diameter, compatible with secondary amyloidosis. Her level of serum amyloid A was remarkably elevated. To our knowledge, this girl is the first case of secondary renal amyloidosis induced by bronchiectasis in Korean children.

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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