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[PMID]: 24460201
[Au] Autor:Koster MI; Dinella J; Chen J; O'Shea C; Koch PJ
[Ad] Address:Department of Dermatology, University of Colorado School of Medicine and Charles C Gates Center for Regenerative Medicine and Stem Cell Biology, University of Colorado School of Medicine , Aurora, CO , USA.
[Ti] Title:Integrating animal models and in vitro tissue models to elucidate the role of desmosomal proteins in diseases.
[So] Source:Cell Commun Adhes;21(1):55-63, 2014 Feb.
[Is] ISSN:1543-5180
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:Desmosomes are intercellular junctions that provide tissues with structural stability. These junctions might also act as signaling centers that transmit environmental clues to the cell, thereby affecting cell differentiation, migration, and proliferation. The importance of desmosomes is underscored by devastating skin and heart diseases caused by mutations in desmosomal genes. Recent observations suggest that abnormal desmosomal protein expression might indirectly contribute to skin disorders previously not linked to these proteins. For example, it has been postulated that reduced desmosomal protein expression occurs in patients affected by Ankyloblepharon-ectodermal defects-cleft lip/palate syndrome (AEC), a skin fragility disorder caused by mutations in the transcription factor TP63. Currently, it is not clear how these changes in desmosomal gene expression contribute to AEC. We will discuss new approaches that combine in vitro and in vivo models to elucidate the role of desmosomal gene deregulation in human skin diseases such as AEC.
[Mh] MeSH terms primary: Cleft Lip/metabolism
Cleft Palate/metabolism
Desmosomes/metabolism
Ectodermal Dysplasia/metabolism
Eye Abnormalities/metabolism
Eyelids/abnormalities
Models, Biological
[Mh] MeSH terms secundary: Animals
Cleft Lip/genetics
Cleft Lip/pathology
Cleft Palate/genetics
Cleft Palate/pathology
Desmocollins/genetics
Desmocollins/metabolism
Desmoplakins/genetics
Desmoplakins/metabolism
Ectodermal Dysplasia/genetics
Ectodermal Dysplasia/pathology
Eye Abnormalities/genetics
Eye Abnormalities/pathology
Eyelids/metabolism
Eyelids/pathology
Humans
Transcription Factors/genetics
Transcription Factors/metabolism
Tumor Suppressor Proteins/genetics
Tumor Suppressor Proteins/metabolism
[Pt] Publication type:JOURNAL ARTICLE; RESEARCH SUPPORT, N.I.H., EXTRAMURAL; REVIEW
[Nm] Name of substance:0 (Desmocollins); 0 (Desmoplakins); 0 (TP63 protein, human); 0 (Transcription Factors); 0 (Tumor Suppressor Proteins)
[Em] Entry month:1409
[Js] Journal subset:IM
[Da] Date of entry for processing:140127
[St] Status:MEDLINE
[do] DOI:10.3109/15419061.2013.876015

  2 / 5507 MEDLINE  
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[PMID]: 25230064
[Au] Autor:Miraglia E; Ambrifi M; Persechino F; Visconti B; Calvieri S; Giustini S
[Ad] Address:Emanuele Miraglia, MD, Department of Dermatology, Policlinico Umberto I, Sapienza, Viale del Policlinico 155, I-00161 Rome, Italy; emanuele.miraglia@hotmail.it.
[Ti] Title:An unusual skin manifestation in a patient with peutz-jeghers syndrome.
[So] Source:Acta Dermatovenerol Croat;22(3):213-4, 2014 Sep.
[Is] ISSN:1847-6538
[Cp] Country of publication:Croatia
[La] Language:eng
[Ab] Abstract:Peutz-Jeghers Syndrome (PJS; MIM 175200) is a rare autosomal dominant syndrome with variable inheritance, characterized by hamartomatous polyps in the gastrointestinal tract, mostly in the small bowel, and pigmented muco-cutaneous lesions (1). Hereditary disorders constitute 70% of PJS cases. The other 30% of cases occur without any previous family history, as a result of spontaneous genetic mutations. The prevalence is estimated at 1/25,000 to 1/230,000 live births, with no racial or sexual predilection (2). The majority of patients that meet the clinical diagnostic criteria have a causative mutation in the serine/threonine kinase 11 (STK11) gene, which is located at 19p13.3. Melanic spots are the earliest manifestation of PJS, typically appearing in the first year of life, and represent the muco-cutaneous marker of this syndrome. They are most commonly seen on the lips and buccal mucosa, anal and intestinal mucosa, nasal and periorbital regions, hands and feet. Rarely, nail pigmentation is observed (3). Melanonychia is a brown or black pigmentation of the nail plate caused by the presence of melanin, and is extremely rare in PJS. We report on a case of a 60-year-old woman diagnosed with Peutz Jeghers syndrome who presented an unusual acral pigmentation with longitudinal melanonychia. A 60 year old woman was referred in our department in September 2012 for evaluation of hyperpigmented macules of the fingers, lips, buccal mucosa, and nails, present from the age of 15. Brown macules of 1-3 mm were found on the lower and upper lips and gingival mucosa (Figure 1). There was no ulceration inside the mouth. The patient presented brown, round, and oval macules 1-5 mm in diameter on the fingers.The nails of the first and second finger of the right hand showed 4-5 mm thick longitudinal brownish bands (Figure 2). There was no history of any drug intake (including antimalarials, minocycline, or gold therapy), exposure to radiation, PUVA, or any trauma prior to the onset of the pigmentation. The Hutchinson sign was also negative. Physical examination showed abdominal bloating. The patient reported a minor pain throughout the abdomen on palpation. Rectal examination showed no pathological mass, and there were traces of feces on the glove. Laboratory tests were also within normal ranges except for a mild anemia, with microcytosis and iron depletion, but the stool was positive for occult blood. We then decided on further endoscopic investigation. Upper digestive endoscopy discovered more than 20 polyps in the stomach, 5-10 mm in diameter. Multiple biopsies were performed from the polyps. Colonoscopy subsequently showed two 1-1.5 cm pedunculated polyps in the sigmoid colon, which were all resected endoscopically. Histologic examination of bioptic fragments from the stomach, as well as of the polyps removed from the colon, showed proliferation and ramification of myocytes from the muscularis mucosae, surrounding the glandular epithelium and spreading in the submucosa and the muscularis propria. No sign of malignancy was observed. The presence of buccal pigmentation and multiple polyps as determined by endoscopy suggested a diagnosis of PJS. The patient reported no similar manifestations in other family members; genetic testing was not performed. The patient was subjected to gastroenterological checkups with periodic gastroscopy and colonoscopy. Peutz-Jeghers syndrome is a rare familial disorder, characterized by mucocutaneous pigmentation, gastrointestinal and extragastrointestinal hamartomatous polyps, and increased risk of malignancy (2,3). Cutaneous pigmentation is present in more than 90% of patients with PJS, appearing in early childhood, usually before five years of age, in the form of flat pigmented lesions that are irregularly oval and usually measure less than 5 mm in diameter (4). They are most commonly seen around the mouth, nose, lower lip, buccal mucosa, hands, and feet. Perianal and genital regions may also be involved, whereas the nails are rarely pigmented. A rare cutaneous manifestation associated with the PJ is longitudinal melanonychia (LM) that presents as a longitudinal pigmented band on the nail (5). LM is frequently observed in other syndromes, such Laugier-Hunziker syndrome which is typically characterized by pigmentation of the oral mucosa but with no systemic manifestations. Several other syndromes must be considered in the differential diagnosis of nail and mucocutaneous pigmentary abnormalities, including such McCune-Albright syndrome, LEOPARD syndrome, Addison Disease, LAMB syndrome, Gardener syndrome, and Cronkhite-Canada syndrome (6). LM can have many causes, including genetic predisposition, trauma, drugs, pregnancy, onychomycosis, benign nail matrix nevi, melanoma, and chemotherapeutic agents. We report on this case to emphasize the peculiarity of longitudinal melanonychia in the PJS and to stress the importance of differential diagnosis of nails pigmentation with regard to other diseases, especially nail melanoma. Since the patients with PJS are at high risk for a number of malignancies, cutaneous and mucosal manifestation may be very important early signs for proper diagnosis of the syndrome.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1409
[Js] Journal subset:IM
[St] Status:In-Data-Review

  3 / 5507 MEDLINE  
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[PMID]: 24611797
[Au] Autor:Yong R; Ranjitkar S; Townsend GC; Smith RN; Evans AR; Hughes TE; Lekkas D; Brook AH
[Ad] Address:School of Dentistry, The University of Adelaide, South Australia, Australia.
[Ti] Title:Dental phenomics: advancing genotype to phenotype correlations in craniofacial research.
[So] Source:Aust Dent J;59 Suppl 1:34-47, 2014 Jun.
[Is] ISSN:1834-7819
[Cp] Country of publication:Australia
[La] Language:eng
[Ab] Abstract:The field of dental phenomics provides many opportunities to elucidate the roles of genetic, epigenetic and environmental factors in craniofacial development. To date, research findings have helped to clarify the pathogenesis of many conditions, aiding diagnosis and clinical management. This paper provides an overview of dental phenomics research in some commonly encountered oral diseases in everyday clinical practice, as well as research relating to craniofacial growth and development. Clinically, advances in cariology and periodontology have led to better diagnostic capabilities and treatment provision. In the study of growth and development, important information regarding the varying clinical presentation and pathogenesis of many disorders is now apparent through the accurate quantification of phenotypes. Improvements in two-dimensional (2D) and three-dimensional (3D) imaging and analytical techniques have allowed for accurate dental phenotyping, and efforts are ongoing to apply these in vitro techniques to the in vivo setting. The field of dental phenomics represents an exciting avenue that links research findings to practical application, and collaboration between researcher and clinicians will help advance the field further.
[Mh] MeSH terms primary: Biomedical Research
Dentition
Epigenesis, Genetic
Genotype
Mouth Diseases/genetics
Phenotype
[Mh] MeSH terms secundary: Cleft Lip/genetics
Cleft Lip/pathology
Cleft Palate/genetics
Cleft Palate/pathology
Dental Caries/genetics
Diagnosis, Computer-Assisted/methods
Forecasting
Gingiva/anatomy & histology
Humans
Imaging, Three-Dimensional/methods
Tooth/anatomy & histology
[Pt] Publication type:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T; REVIEW
[Em] Entry month:1409
[Js] Journal subset:D; IM
[Da] Date of entry for processing:140527
[St] Status:MEDLINE
[do] DOI:10.1111/adj.12156

  4 / 5507 MEDLINE  
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[PMID]: 23722973
[Au] Autor:Menni S; Marconi M; Boccardi D; Betti R
[Ad] Address:Dermatologic Clinic, Department of Health Sciences, Univeristy of Milan , A.O.San Paolo, Via A Di Rudinì 8, IT-20142 Milan, Italy. silvano.menni@unimi.it.
[Ti] Title:Venous lakes of the lips: prevalence and associated factors.
[So] Source:Acta Derm Venereol;94(1):74-5, 2014 Jan.
[Is] ISSN:1651-2057
[Cp] Country of publication:Sweden
[La] Language:eng
[Mh] MeSH terms primary: Lip Diseases/epidemiology
Lip/blood supply
Venules/pathology
[Mh] MeSH terms secundary: Aged
Dilatation, Pathologic/epidemiology
Female
Humans
Keratosis, Actinic/epidemiology
Male
Middle Aged
Prevalence
Risk Factors
Sunburn/epidemiology
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1409
[Js] Journal subset:IM
[Da] Date of entry for processing:140109
[St] Status:MEDLINE
[do] DOI:10.2340/00015555-1569

  5 / 5507 MEDLINE  
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[PMID]: 25209227
[Au] Autor:Farronato G; Kairyte L; Giannini L; Galbiati G; Maspero C
[Ad] Address:Via Commenda 10, 20122 Milan Italy. giampietro.farronato@unimi.it.
[Ti] Title:How various surgical protocols of the unilateral cleft lip and palate influence the facial growth and possible orthodontic problems? Which is the best timing of lip, palate and alveolus repair? Literature review.
[So] Source:Stomatologija;16(2):53-60, 2014.
[Is] ISSN:1822-301X
[Cp] Country of publication:Lithuania
[La] Language:eng
[Ab] Abstract:OBJECTIVES. Cleft lip palate is congenital growth disease with unknown etiology, probably linked to both genetically and external causes. The aim of this work consists in presenting the effects of these diseases on cranio facial growth and the surgical protocols described in literature. MATERIALS AND METHODS. The literature review articles conducted by Medline ranged from 1998 to 2011 have been selected. The key words of the research were "cleft lip palate", "cleft lip palate facial growth", "cleft lip palate surgery". The inclusion criteria were articles that analyzed surgical protocols and the growth of unilateral lip and palate clefts, the timing repair of lip, palate and alveolus. We excluded case reports, studies without control group in the sample and the other types of publication as thesis or conference presentation. 60 articles had the selection criteria of the research. RESULTS. The cleft lip and palate is one of the most common birth defects that needs long rehabilitation between birth and adulthood. Several authors have presented surgical protocols and timing. The effects of these diseases on cranio facial growth and the importance of the early intervention have been described. CONCLUSIONS. The review describes the main surgical protocols and treatment strategies of the unilateral lip and palate clefts. The review discusses how surgery effects the midfacial skeletal growth. Studies agree that the palate repair is the main cause of the maxilla growth disturbances. About the timing of palate repair in the unilateral clefts it can be concluded that most studies found no difference between one or two stages palate repair techniques for the midfacial growth. Also from the research, studies agree that delayed hard palate repair has more positive effects on maxillary growth than that of early hard palate repair. Nevertheless good results, delayed hard palate repair technique is abandoned by many hospital centres because of worse speech outcome. The best technique of palate repair is difficult to conclude, because the research results are part of a big controversy between the centers. From the studies about the alveolar repair it can be concluded that the primary bone grafting had more negative results on the skeletal growth. Gingivoperiosteoplasty and the secondary bone grafting had more positive results for intracranial relationship. The studies agree that the best timing of lip repair is during third-sixth month of life and that lip repair could have negative influence on the maxillary growth.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1409
[Js] Journal subset:D; IM
[St] Status:In-Data-Review

  6 / 5507 MEDLINE  
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[PMID]: 24481503
[Au] Autor:Rath S; Cowan BJ; Dolman PJ
[Ad] Address:*Department of Ophthalmology and Visual Sciences and †Department of Medicine, Division of Dermatology, University of British Columbia, Vancouver, British Columbia, Canada.
[Ti] Title:Cicatricial ectropion in grzybowski type of multiple eruptive keratoacanthomas.
[So] Source:Ophthal Plast Reconstr Surg;30(2):e42-3, 2014 Mar-Apr.
[Is] ISSN:1537-2677
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:A 41-year-old woman previously diagnosed with generalized eruptive keratoacanthomas of Grzybowski type presented with bilateral lower eyelid cicatrical ectropions. She had previously undergone multiple resections of syringomatous adenomas of both nipples, facial keratoacanthomas, and a lower left lip squamous cell carcinoma. Her facial and periocular skin was thickened with a cobblestone appearance. Cicatricial ectropions involved both upper and lower eyelids. Donor skin was harvested from the dorsum of the foot as this was the only disease-free area on her body, and she achieved a stable result with reduced tearing and improved appearance.
[Mh] MeSH terms primary: Cicatrix/complications
Ectropion/complications
Keratoacanthoma/complications
[Mh] MeSH terms secundary: Adult
Cicatrix/diagnosis
Cicatrix/surgery
Ectropion/diagnosis
Ectropion/surgery
Female
Foot/surgery
Humans
Keratoacanthoma/diagnosis
Keratoacanthoma/surgery
Lacrimal Apparatus Diseases/complications
Lacrimal Apparatus Diseases/diagnosis
Lacrimal Apparatus Diseases/surgery
Skin Transplantation
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T
[Em] Entry month:1409
[Js] Journal subset:IM
[Da] Date of entry for processing:140311
[St] Status:MEDLINE
[do] DOI:10.1097/IOP.0b013e318295f946

  7 / 5507 MEDLINE  
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[PMID]: 25187723
[Au] Autor:Xu KF; Lo BH
[Ad] Address:Department of Respiratory Medicine, Peking Union Medical College Hospital, Beijing, People's Republic of China.
[Ti] Title:Lymphangioleiomyomatosis: differential diagnosis and optimal management.
[So] Source:Ther Clin Risk Manag;10:691-700, 2014.
[Is] ISSN:1176-6336
[Cp] Country of publication:New Zealand
[La] Language:eng
[Ab] Abstract:Lymphangioleiomyomatosis (LAM) is an uncommon disease presented as diffuse thin-walled cystic changes in the lung. The main differential diagnoses include pulmonary Langerhans' histiocytosis (PLCH), Birt-Hogg-Dubé syndrome (BHD), lymphoid interstitial pneumonia (LIP), and amyloidosis. A combination of clinical, radiological, and pathological approaches as well as genetic testing will clarify the diagnosis in most cases. LAM is a disease almost exclusively in women. Dyspnea, pneumothorax, and hemoptysis are common presentations in LAM patients. LAM is also a lymphatic disorder affecting lymphatic vessels and lymph nodes. Chylothorax, chylous ascites, and lymphangiomyomas are frequently seen. LAM can present sporadically as a single entity or as part of tuberous sclerosis complex (TSC). Angiomyolipoma (AML) is a characteristic extra-pulmonary lesion, either found in association with sporadic or TSC-related LAM. High-risk populations should be screened for LAM, including adult women with TSC and female patients with spontaneous pneumothorax, AMLs in the kidney, and diffuse cystic lung diseases. Definitive diagnosis of LAM is based on a high level of clinical suspicion on presentation supported by pathological findings or by a distinct feature, such as a history of TSC, AMLs in the kidney, chylothorax, or chylous ascites. Vascular endothelial growth factor-D (VEGF-D) in serum is a noninvasive and reliable diagnostic biomarker. In experienced centers, trans-bronchial lung biopsy (TBLB) provides a convenient and safe way to obtain lung specimens for diagnostic purposes. An effective treatment for LAM is now available, namely using a mechanistic target of rapamycin (mTOR) inhibitor such as sirolimus. Efficacy of sirolimus has been confirmed in clinical trials. Research in other molecular-targeted therapies is under investigation. A previously little-known rare disease with no cure is now better understood with regards to its pathogenesis, diagnosis, and management. In this review, current knowledge in diagnosis and differential diagnosis of LAM will be discussed, followed by the discussion of therapy with mTOR inhibitors.
[Pt] Publication type:JOURNAL ARTICLE; REVIEW
[Em] Entry month:1409
[Cu] Class update date: 140908
[Lr] Last revision date:140908
[Da] Date of entry for processing:140904
[St] Status:PubMed-not-MEDLINE
[do] DOI:10.2147/TCRM.S50784

  8 / 5507 MEDLINE  
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[PMID]: 24983770
[Au] Autor:Fan Y; Zhang J; Cai L; Wang S; Liu C; Zhang Y; You L; Fu Y; Shi Z; Yin Z; Luo L; Chang Y; Duan X
[Ad] Address:Laboratory of Molecular Iron Metabolism, College of Life Science, Hebei Normal University, Shijiazhuang 050024, PR China; Key Laboratory of Animal Physiology, Biochemistry and Molecular Biology of Hebei Province, College of Life Science, Hebei Normal University, Shijiazhuang 050024, PR China; Key La...
[Ti] Title:The effect of anti-inflammatory properties of ferritin light chain on lipopolysaccharide-induced inflammatory response in murine macrophages.
[So] Source:Biochim Biophys Acta;1843(11):2775-83, 2014 Nov.
[Is] ISSN:0006-3002
[Cp] Country of publication:Netherlands
[La] Language:eng
[Ab] Abstract:Ferritin light chain (FTL) reduces the free iron concentration by forming ferritin complexes with ferritin heavy chain (FTH). Thus, FTL competes with the Fenton reaction by acting as an antioxidant. In the present study, we determined that FTL influences the lipopolysaccharide (LPS)-induced inflammatory response. FTL protein expression was regulated by LPS stimulation in RAW264.7 cells. To investigate the role of FTL in LPS-activated murine macrophages, we established stable FTL-expressing cells and used shRNA to silence FTL expression in RAW264.7 cells. Overexpression of FTL significantly decreased the LPS-induced production of tumor necrosis factor alpha (TNF-α), interleukin 1ß (IL-1ß), nitric oxide (NO) and prostaglandin E2 (PGE2). Additionally, overexpression of FTL decreased the LPS-induced increase of the intracellular labile iron pool (LIP) and reactive oxygen species (ROS). Moreover, FTL overexpression suppressed the LPS-induced activation of MAPKs and nuclear factor-κB (NF-κB). In contrast, knockdown of FTL by shRNA showed the reverse effects. Therefore, our results indicate that FTL plays an anti-inflammatory role in response to LPS in murine macrophages and may have therapeutic potential for treating inflammatory diseases.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1409
[Js] Journal subset:IM
[St] Status:In-Data-Review

  9 / 5507 MEDLINE  
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[PMID]: 24296165
[Au] Autor:Bodner L; Manor E; Friger MD; van der Waal I
[Ad] Address:Department of Oral and Maxillofacial Surgery, Soroka Medical Center, Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer-Sheva, Israel. Electronic address: lbodner@bgu.ac.il....
[Ti] Title:Oral squamous cell carcinoma in patients twenty years of age or younger--review and analysis of 186 reported cases.
[So] Source:Oral Oncol;50(2):84-9, 2014 Feb.
[Is] ISSN:1879-0593
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:To review the literature on reported cases of squamous cell carcinoma (SCC) of the oral cavity in patients twenty-years-of-age or younger. All well-documented cases of oral SCC in patients twenty-years-of-age or less, published between 1936 and 2012, were collected and the clinicopathologic features were evaluated. Primary cases of oral SCC were selected. Age, gender, intra-oral subsite were recorded. A total of 88 articles describing 186 cases were included. The group of otherwise healthy patients had a mean age of 14.08years (range newborn-20years), the m:f ratio was 1.36; the oral subsites were the tongue, gingiva, and lower lip. A second group of patients who have disorders that predispose to cancer development, such as xeroderma pigmentosum, Fanconi's anemia, and a history of bone marrow transplant, had a mean age of 13.17years (range 5-20years); the m:f ratio was 1.23; the oral subsite was mainly the tongue. There was a slight difference between otherwise healthy patients and patients with predisposing systemic factors, but this difference was not statistically significant. It contrast to adults, there is only a weak predilection for males (m:f ratio of 1.23-1.36). In the young population SCC occurs most frequently in the tongue, followed by gingiva and lips. Unlike in adults, SCC is very uncommon in the floor of mouth. Oral SCC may, indeed, occur in patients younger than 20years and clinicians should take cognizance of this. Periodical examination of the oral cavity of young patients is recommended in cases of systemic diseases that predispose to cancer development such as xeroderma pigmentosum, Fanconi's anemia, and a history of bone marrow transplant.
[Mh] MeSH terms primary: Carcinoma, Squamous Cell/epidemiology
Mouth Neoplasms/epidemiology
[Mh] MeSH terms secundary: Adolescent
Age Factors
Carcinoma, Squamous Cell/therapy
Child
Child, Preschool
Female
Humans
Infant
Infant, Newborn
Male
Mouth Neoplasms/therapy
Prognosis
Young Adult
[Pt] Publication type:JOURNAL ARTICLE; META-ANALYSIS; REVIEW
[Em] Entry month:1409
[Js] Journal subset:IM
[Da] Date of entry for processing:140113
[St] Status:MEDLINE

  10 / 5507 MEDLINE  
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[PMID]: 25188152
[Au] Autor:Aubry MC
[Ad] Address:Mayo Clinic, Rochester, MN, USA.
[Ti] Title:Pulmonary Pathology: LC22-1 NON-NEOPLASTIC PULMONARY LYMPHOID PROLIFERATIONS.
[So] Source:Pathology;46 Suppl 2:S36-7, 2014 Oct.
[Is] ISSN:1465-3931
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:Non-neoplastic pulmonary lymphoid proliferations encompass a spectrum of disease from small airway bronchiolar disease to diffuse interstitial disease. They include follicular bronchiolitis (FB), nodular lymphoid hyperplasia (NLH), and lymphocytic interstitial pneumonia (LIP). FB is characterized by the presence of hyperplastic lymphoid follicles with germinal centers, exquisitely bronchiolocentric. Patients with FB not uncommonly suffer from connective tissue disease such as rheumatoid arthritis or immunodeficiency. The differential diagnosis of FB includes other small airway diseases such as chronic bronchiolitis or constrictive bronchiolitis. In contrast, LIP is an interstitial lung disease with diffuse marked septal thickening by small lymphocytes, with admixed plasma cells and histiocytes. Patients with LIP often present with autoimmune disorders such as Sjögrens syndrome. Chronic hypersensitivity pneumonia and non-specific interstitial pneumonia enter the differential diagnosis of LIP. Patients with NLH are typically asymptomatic and present with solitary or multiple nodular infiltrates. The lung parenchyma is focally obliterated by a mass comprised of fibrosis admixed with a dense polymorphous lymphoid infiltrate, often with numerous germinal centers. By immunohistochemistry, the lymphoid infiltrate is predominantly CD3 + T-cells with CD20 + B-cells forming the germinal centers. The plasma cells and lymphocytes are polyclonal. NLH needs to be distinguished from low-grade lymphoma, mainly MALT lymphoma.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1409
[Js] Journal subset:IM
[St] Status:In-Data-Review
[do] DOI:10.1097/01.PAT.0000454210.12598.39


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