• 제목/요약/키워드: cystic fibrosis

검색결과 71건 처리시간 0.022초

Pigmented villonodular synovitis of the temporomandibular joint - computed tomography and magnetic resonance findings: a case report

  • Kim, Il-Kyu;Cho, Hyun-Young;Cho, Hyun-Woo;Seo, Ji-Hoon;Lee, Dong-Hwan;Peng, Wang
    • Journal of the Korean Association of Oral and Maxillofacial Surgeons
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    • 제40권3호
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    • pp.140-146
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    • 2014
  • Pigmented villonodular synovitis (PVNS) is a benign but locally aggressive and destructive disease originating in the synovial membranes. It is a proliferative disorder of unknown etiology. Involvement of the temporomandibular joint (TMJ) is very rare. Computed tomography clearly reveals areas of lytic bone erosion and sclerosis, and also clearly defines the extent of the tumor which is the focal areas of hyperdensity within the soft-tissue mass. Magnetic resonance images invariably show profound hypointensity on both T1- and T2-weighted sequences due to hemosiderin pigmentation. Additionally, high signal intensity on T2-weighted images may indicate cystic loculation of the joint fluid. This case study describes a rare case of PVNS of the TMJ with bone destruction of the mandibular condyle. Complete surgical excision of the lesion was performed through a preauricular approach with temporal extension. During the 10-year follow-up, two more operations were performed due to local recurrence and the fracture of the reconstruction plate. Total joint reconstruction with Biomet was finally performed, and the absence of disease was confirmed with a biopsy report showing fibrosis with hyalinization and mild inflammation of the excised soft tissue from the old lesion.

Case of seropositive allergic bronchopulmonary aspergillosis in a 10-year-old girl without previously documented asthma

  • Shin, Jeong Eun;Shim, Jae Won;Kim, Deok Soo;Jung, Hae Lim;Park, Moon Soo;Shim, Jung Yeon
    • Clinical and Experimental Pediatrics
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    • 제58권5호
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    • pp.190-193
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    • 2015
  • Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity lung disease due to bronchial colonization of Aspergillus fumigatus that occurs in susceptible patients with asthma or cystic fibrosis. A 10-year-old girl was referred to the Department of Pediatric Pulmonology for persistent consolidations on chest radiography. Pulmonary consolidations were observed in the right upper and left lower lobes and were not resolved with a 4-week prescription of broad-spectrum antibiotics. The patient had a history of atopic dermatitis and allergic rhinitis but no history of asthma. She had no fever but produced thick and greenish sputum. Her breathing sounds were clear. On laboratory testing, her total blood eosinophil count was $1,412/mm^3$ and total serum IgE level was 2,200 kU/L. Aspergillus was isolated in the sputum culture. The A. fumigatus-specific IgE level was 15.4 kU/L, and the Aspergillus antibody test was also positive. A chest computed tomography scan demonstrated bronchial wall thickening and consolidation without bronchiectasis. An antifungal agent was added but resulted in no improvement of pulmonary consolidations after 3 weeks. Pulmonary function test was normal. Methacholine provocation test was performed, revealing bronchial hyperreactivity ($PC_{20}=5.31mg/mL$). Although the patient had no history of asthma or bronchiectasis, ABPA-seropositivity was suspected. Oral prednisolone (1 mg/kg/day) combined with antifungal therapy was started. Pulmonary consolidations began decreasing after 1 week of treatment and completely resolved after 1 month. This is the first observed and treated case of seropositive ABPA in Korean children without previously documented asthma.

노르스름한 성대점막 색변화를 보이는 편평 성대 낭종: 개방형 성대 낭종 (An Yellowish Flat Intracordal Cyst : Open Cyst)

  • 김지훈;이은정;김연희;홍현준;최홍식
    • 대한후두음성언어의학회지
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    • 제23권1호
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    • pp.52-55
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    • 2012
  • Background and Objectives : Based on histological findings, intracordal cysts are divided in two subtypes : retention cysts and epidermoid cysts. They are typically located in the superficial layer of the lamina propria and appeared as opaque ovoid buldging masses underlying the epithelium. They are characterized by unilateral diminished mucosal wave on stroboscopy. In this article, we report some cases of patients with an oval shaped-yellowish flat cyst. Materials and Method : At the clinic of the department of otorhinolaryngology in Gangnam Severance Hospital, with 3 female complained of hoarseness as subjects, using the stroboscopy we checked preoperative and postoperative vocal cord and operative findings. Surgery was performed under general anesthesia by the senior authors. All patients noted subjectively that their performing voice was improved. Results : During surgery, an oval shaped-yellowish flat cystic lesion was distinguished from normal epithelium. On palpation of this area with microforceps and cottons, the yellowish discharge was noted to move out from the opening of the cyst. In one case, the cyst was ruptured but remove the capsule completely. In other cases, sulcus was noted at the oppsite site. Conclusion : Because of the opening, the cyst was not easy to dissect and remove completely. After the debris was move out, fibrosis around the opening and invaginated epithelium extending into the deeper layers to the fold. The cyst was must removed carefully and completely for improvement of voice quality before evolving into a sulcus vocalis.

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ICSI시대에서의 남성불임 (Male Infertility in the Era of ICSI)

  • 서주태
    • 대한생식의학회:학술대회논문집
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    • 대한불임학회 2003년도 제45차 추계학술대회
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    • pp.21-30
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    • 2003
  • As a result of the technological advance provided by intracytoplasmic sperm injection (ICSI) in 1992, the evaluation and treatment of the infertile male has changed significantly. Many men who were previously thought to be irreversibly infertile have the potential to initiate their own biologic pregnancy. However, not all men having impaired semen parameter are ideal candidates for ICSI for numerous reasons including a lack of addressing the underlying problem causing the male infertility, unknown genetic consequences, and cost-effectiveness issues. In this era of ICSI, the fundamental approach to the male with suspected subfertility is unchanged and is based on a history, physical examination, and focused laboratory testing. The urologist should approach the patient with an intent to identify remediable causes of subfertility given the specific clinical situation. For instance, should a gentleman have his varicocele repaired or vasectomy reversed, or should he proceed directly with ICSI? If no factors can be improved in a timely manner, then ICSI should be considered using the available sperm. Examples of recent advances include the diagnosis and treatment of ejaculatory duct obstruction, indications and techniques for performing testis biopsy, and technique for sperm harvesting. In addition, potential genetic causes of male subfertility should be diagnosed and discussed with the patient. Cystic fibrosis gene mutation, karyotype abnormallities, and Y-chromosome microdeletions all have recently been identified as causative for male infertility in otherwise phenotypically normal men. With recently evolved diagnostic and therapeutic techniques now available for the infertile couple, even the most severe male factor problems in patients previously considered irreversibly infertile are now potentially treatable. The physician should be aware of the availability and limitations of these new and exciting reproductive technologies because they will allow him to provide timely and more effective therapy for the infertile couple. An understanding of these advances by all physicians is important as we progress into the $21^{st}$ century

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Extracellular ATP Stimulates $Na^+\;and\;Cl^-$ Transport through the Activation of Multiple Purinergic Receptors on the Apical and Basolateral Membranes in M-1 Mouse Cortical Collecting Duct Cells

  • Jung, Jin-Sup;Hwang, Sook-Mi;Lee, Ryang-Hwa;Kang, Soo-Kyung;Woo, Jae-Suk;Kim, Yong-Keun
    • The Korean Journal of Physiology and Pharmacology
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    • 제5권3호
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    • pp.231-241
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    • 2001
  • The mammalian cortical collecting duct (CCD) plays a major role in regulating renal NaCl reabsorption, which is important in $Na^+$ and $Cl^-$ homeostasis. The M-1 cell line, derived from the mouse cortical collecting duct, has been used as a mammalian model of the study on the electrolytes transport in CCD. M-1 cells were grown on collagen-coated permeable support and short circuit current $(I_{sc})$ was measured. M-1 cells developed amiloride-sensitive current $5{\sim}7$ days after seeding. Apical and basolateral addition of ATP induced increase in $I_{sc}$ in M-1 cells, which was partly retained in $Na^+-free$ or $Cl^--free$ solution, indicating that ATP increased $Na^+$ absorption and $Cl^-$ secretion in M-1 cells. $Cl^-$ secretion was mediated by the activation of apical cystic fibrosis transmembrane regulator (CFTR) chloride channels and $Ca^{2+}-activated$ chloride channels, but $Na^+$ absorption was not mediated by activation of epithelal sodium channel (ENaC). ATP increased cAMP content in M-1 cells. The RT-PCR analysis demonstrated that M-1 cells express $P2Y_2,\;P2X_3\;and\;P2Y_4$ receptors. These results showed that ATP regulates $Na^+$ and $Cl^-$ transports via multiple P2 purinoceptors on the apical and basolateral membranes in M-1 cells.

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4세 여아에서 자연 완해된 담즙 마개 증후군 1례 (A Case of Spontaneous Resolution of Bile Plug Syndrome in a 4-year-old Girl)

  • 지금봉;송준영;유기양;민기식;김덕하;이관섭
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • 제2권2호
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    • pp.262-266
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    • 1999
  • Obstruction of the extrahepatic bile ducts is the most common cause of conjugated hyperbilirubinemia in early infancy. More than 90% of such obstructive lesions are accounted for by extrahepatic biliary atresia. A rare lesion is obstruction of the common duct by impacted, thickened secretions and bile. Bile plug syndrome is defined as extrahepatic obstruction of the bile ducts by bile sludge in term infants without anatomic abnormalities, congenital chemical defects of bile, or hepatocellular lesions. Obstruction of extrahepatic ducts by plugs of biliary material apperas to be due to the inspissation and precipitation of bile and mucus within the lumen of the ducts. Cholestasis and precipitation of bile develop in association with abnormal composition of bile in cystic fibrosis, hepatocellular damage, prolonged erythroblastic jaundice, altered biliary dynamics with total parenteral nutrition, gut dysfunction, diuretic therapy, exchange transfusions and perinatal hemolysis. In those cases, the term inspissated bile syndrome is used. The clinical and laboratory findings in bile plug syndrome are identical to those observed in biliary atresia and choledochal cyst. The diagnosis can be suspected based on the findings of clinical and laboratory examinations together with hepatobiliary imaging, ultrasonography, radionuclide scan and liver biopsy. We experienced a case of spontaneous resolution of bile plug syndrome in a 4-year-old girl. We report this case with brief review related literatures.

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Gastroesophageal Reflux Affects Sleep Quality in Snoring Obese Children

  • Machado, Rodrigo Strehl;Woodley, Frederick W;Skaggs, Beth;Lorenzo, Carlo Di;Eneli, Ihuoma;Splaingard, Mark;Mousa, Hayat
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • 제19권1호
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    • pp.12-19
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    • 2016
  • Purpose: This study was performed to evaluate the quality of sleep in snoring obese children without obstructive sleep apnea (OSA); and to study the possible relationship between sleep interruption and gastroesophageal reflux (GER) in snoring obese children. Methods: Study subjects included 13 snoring obese children who were referred to our sleep lab for possible sleep-disordered breathing. Patients underwent multichannel intraluminal impedance and esophageal pH monitoring with simultaneous polysomnography. Exclusion criteria included history of fundoplication, cystic fibrosis, and infants under the age of 2 years. Significant association between arousals and awakenings with previous reflux were defined by symptom-association probability using 2-minute intervals. Results: Sleep efficiency ranged from 67-97% (median 81%). A total of 111 reflux episodes (90% acidic) were detected during sleep, but there were more episodes per hour during awake periods after sleep onset than during sleep (median 2.3 vs. 0.6, p=0.04). There were 279 total awakenings during the sleep study; 56 (20.1%) of them in 9 patients (69.2%) were preceded by reflux episodes (55 acid, 1 non-acid). In 5 patients (38.5%), awakenings were significantly associated with reflux. Conclusion: The data suggest that acid GER causes sleep interruptions in obese children who have symptoms of snoring or restless sleep and without evidence of OSA.

맥문동 열수 추출물이 Poly I:C를 처리한 폐암세포주의 사멸 및 염증성 사이토카인 발현에 미치는 영향 (Effects of Liriope muscari Water Extracts on the Cell Death and Inflammatory Cytokine Expression of Poly I:C-treated Lung Carcinoma Cells)

  • 강다연;조남준;렌친핸드;이보희;김은미;남명수;김기광
    • 대한본초학회지
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    • 제36권1호
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    • pp.97-102
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    • 2021
  • Objectives : Virus infection through the respiratory tract causes various inflammatory diseases such as pneumonia, cystic fibrosis, and obstructive pulmonary disease, causing enormous social damage. Therefore, it is very important to develop a treatment and prevention of infectious diseases. In this study, we investigated the effect of water extracts of Liriope muscari (WELM), known to improve lung function, on the inflammatory response of lung carcinoma cell line A549 cells induced by the viral double stranded RNA mimetic Polyinosinic:polycytidylic acid (Poly I:C). Methods : The cell viability by WELM treatment was analyzed using MTS assay in A549 cells. After inducing an inflammatory response to WELM-treated A549 cells with Poly I:C, the degree of apoptosis was confirmed through bright field microscopy. Interferon beta (IFN-β) mRNA expression level in A549 cells was analyzed by quantitative reverse transcription PCR (qRT-PCR). Results : WELM treatment has no significant effect on cell viability of A549 cells. We confirmed that pre-treatment of WELM effectively reduces the Poly I:C-induced apoptotic cell death in A549 cells. In addition, it was confirmed that the mRNA expression level of IFN-β, a pro-inflammatory cytokine increased by Poly I:C treatment, was significantly suppressed by WELM treatment in A549 cells. Conclusions : These results provide the evidence that WELM is effective at inhibiting inflammation on respiratory viral infections and suggest that Liriope muscari might be a valuable natural substance in the prevention and treatment of infectious diseases.

호흡기질환에 대한 장내 미생물의 임상 연구 동향 (Clinical Research Trends of Gut Microbiome for Respiratory Diseases)

  • 이수원;최진관;양원경;김승형;유이란;박양춘
    • 대한한의학회지
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    • 제42권3호
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    • pp.119-138
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    • 2021
  • Objectives: This study aimed to review the clinical research of the gut microbiome for respiratory diseases to assist the design of trials for respiratory diseases by regulating the gut microbiome with herbal medicine later. Methods: We searched three international databases (PubMed, CENTRAL and EMBASE) to investigate randomized controlled trials (RCTs) of the gut microbiome for respiratory diseases. The selected trials were analyzed by study design, subject diseases, inclusion/exclusion criteria, sample size, study period, intervention group, control group, outcome measures, and study results. Results: A total of 25 studies were included and published from 1994 to 2021 mostly in Europe and Asia. Subject diseases were many in the order of respiratory tract infection, cystic fibrosis, allergy, and so on. As outcome measures, the gut microbiome in a fecal sample was analyzed by 16S rRNA sequencing analysis method, and symptom assessment tools related each disease were used. Major intervention drugs were probiotics and the results were mostly improved in the composition and diversity of the gut microbiome. Conclusion: Clinical studies of the gut microbiome for respiratory diseases have confirmed various effects and this review provides basic data for a well-designed clinical study for respiratory diseases by regulating the gut microbiome with herbal medicine.

SARS-CoV-2 Antibodies in Children with Chronic Disease from a Pediatric Gastroenterology Outpatient Clinic

  • Kaya, Gulay;Issi, Fatma;Guven, Burcu;Ozkaya, Esra;Buruk, Celal Kurtulus;Cakir, Murat
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • 제25권5호
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    • pp.422-431
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    • 2022
  • Purpose: At the beginning of the Coronavirus disease (COVID-19) epidemic, physicians paid close attention to children with chronic diseases to prevent transmission or a severe course of infection. We aimed to measure the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) antibody levels in children with chronic gastrointestinal and liver diseases to analyze the risk factors for infection and its interaction with their primary disease. Methods: This cross-sectional study analyzed SARS-CoV-2 antibody levels in patients with gastrointestinal and liver diseases (n=141) and in healthy children (n=48) between January and February 2021. Results: During the pandemic, 10 patients (7%) and 1 child (2%) had confirmed COVID-19 infection (p=0.2). The SARS-CoV-2 antibody test was positive in 36 patients (25.5%) and 11 children (22.9%) (p=0.7). SARS-CoV-2 antibody positivity was found in 20.4%, 26.6%, 33.3%, and 33.3% of patients with chronic liver diseases, chronic gastrointestinal tract diseases, cystic fibrosis, and liver transplantation recipients, respectively (p>0.05, patients vs. healthy children). Risk factors for SARS-CoV-2 antibody positivity were COVID-19-related symptoms (47.2% vs. 14.2%, p=0.00004) and close contact with SARS-CoV-2 polymerase chain reaction-positive patients (69.4% vs. 9%, p<0.00001). The use, number, and type of immunosuppressants and primary diagnosis were not associated with SARS-CoV-2 antibody positivity. The frequency of disease activation/flare was not significant in patients with (8.3%) or without (14.2%) antibody positivity (p=0.35). Conclusion: SARS-CoV-2 antibodies in children with chronic gastrointestinal and liver diseases are similar to that in healthy children. Close follow-up is important to understand the long-term effects of past COVID-19 infection in these children.