WordNet

of first rank or importance or value; direct and immediate rather than secondary; "primary goals"; "a primary effect"; "primary sources"; "a primary interest"
a preliminary election where delegates or nominees are chosen (同)primary_election
one of the main flight feathers projecting along the outer edge of a birds wing (同)primary feather, primary quill
(astronomy) a celestial body (especially a star) relative to other objects in orbit around it
not derived from or reducible to something else; basic; "a primary instinct"
a pattern of symptoms indicative of some disease
a complex of concurrent things; "every word has a syndrome of meanings"
immunological disorder in which some part of the bodys immune system is inadequate and resistance to infectious diseases is reduced

PrepTutorEJDIC

『第一の』,『主要な』 / 『初期の』,『初等の』,初級の / 『根本的な』,基本的な,本来の,直接的な / 《名詞の前にのみ用いて》(回路・コイル・巻き・電流などが)一次の / 原色(三原色の一つ) / 《米》=primary election
(疾患の徴候となる一群の)症徴候,症候群 / (事件・社会的状態などのパターンを示す)徴候形態

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1. 原発性の体液性免疫不全：概要 primary humoral immunodeficiencies an overview
2. 感染症を繰り返す小児に対するアプローチ approach to the child with recurrent infections
3. 感染症を繰り返す成人に対するアプローチ approach to the adult with recurrent infections
4. 原発性免疫不全症における免疫グロブリン療法 immune globulin therapy in primary immunodeficiency
5. 原発性免疫不全症における悪性腫瘍 malignancy in primary immunodeficiency

English Journal

Otological findings in pediatric patients with hypogammaglobulinemia.

Tavakol M1, Kouhi A2, Abolhassani H3, Ghajar A4, Afarideh M5, Shahinpour S6, Aghamohammadi A7.Author information 1Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Science, Tehran, Iran. marzieh.tavakol@yahoo.com.2Otorhinolaryngology Research Center, Amir Alam Hospital, Department of Otolaryngology, Tehran University of Medical Sciences, Tehran, Iran. kouhi@tums.ac.ir.3Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran and Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institutet at the Karolinska University Hospital Huddinge, Stockholm, Sweden. abolhassanih@yahoo.com.4Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran. ghajar.ar@gmail.com.5Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran. mhafarideh@yahoo.com.6Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran. and1_sh@yahoo.com.7Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran. aghamohammadi@tums.ac.ir.AbstractThe main clinical presentation of patients with primary antibody deficiency (PAD) incorporates upper respiratory tract infections comprising otitis media, sinusitis and pneumonia. This study was designed to investigate clinical and paraclinical otological complications in major types of PAD. A cross sectional study was conducted on 55 PAD patients with diagnosis of selective IgA deficiency, common variable immunodeficiency (CVID), X-linked agammaglobulinemia (XLA), and hyper IgM syndrome. All patients underwent otological examinations, audiometry, and auditory brain stem response. Otological complications were detected in 54.5% of PAD patients. Conductive hearing loss was the main finding amongst PID patients (73.3%) followed by sensorineural hearing loss which was present in 8 cases. Otitis media with effusion (21.8%), chronic otitis media (27.2%), tympanosclerosis with intact tympanic membrane (5.4%) and auditory neuropathy (3.6%) were most important found complications. CVID and XLA patients with prophylactic usage of antibiotics had lower rate of audiological complications (p=0.04) and otitis media with effusion (p=0.027). As our results showed, asymptomatic otological findings were not rare in PAD patients; therefore, a systematic otological investigation is recommended as an integral part of the management and follow-up of these patients.
Iranian journal of allergy, asthma, and immunology.Iran J Allergy Asthma Immunol.2014 Jun;13(3):166-73.
The main clinical presentation of patients with primary antibody deficiency (PAD) incorporates upper respiratory tract infections comprising otitis media, sinusitis and pneumonia. This study was designed to investigate clinical and paraclinical otological complications in major types of PAD. A cross
PMID 24659120

Vestibular involvement in adults with HIV/AIDS.

Heinze BM1, Vinck BM2, Hofmeyr LM3, Swanepoel de W4.Author information 1Department of Speech-Language Pathology and Audiology, University of Pretoria, Pretoria, South Africa. Electronic address: barbara.heinze@up.ac.za.2Department of Speech-Language Pathology and Audiology, University of Pretoria, Pretoria, South Africa; Department of Audiology, University of Gent, Gent, Belgium. Electronic address: bart.vinck@up.ac.za.3Department of Otorhinolaryngology, University of Pretoria, Pretoria, South Africa. Electronic address: lmhofmeyr@surgeon.co.za.4Department of Speech-Language Pathology and Audiology, University of Pretoria, Pretoria, South Africa; Ear Sciences Centre, School of Surgery, University of Western Australia, Nedlands, Australia; Ear Science Institute Australia, Subiaco, Australia. Electronic address: dewet.swanepoel@up.ac.za.AbstractOBJECTIVE: HIV/AIDS is responsible for widespread clinical manifestations involving the head, and neck. The prevalence and nature of vestibular involvement is still largely unknown. This study, aimed to describe and compare the occurrence and nature of vestibular involvement among a group of, adults infected with HIV compared to a control group. It also aimed to compare the vestibular function, of symptomatic and asymptomatic HIV positive adults who receive antiretroviral (ARV) therapies to, subjects not receiving ARV.
Auris, nasus, larynx.Auris Nasus Larynx.2014 Apr;41(2):160-8. doi: 10.1016/j.anl.2013.08.003. Epub 2013 Oct 19.
OBJECTIVE: HIV/AIDS is responsible for widespread clinical manifestations involving the head, and neck. The prevalence and nature of vestibular involvement is still largely unknown. This study, aimed to describe and compare the occurrence and nature of vestibular involvement among a group of, adults
PMID 24145102

Japanese Journal

Depression of Local Cell-mediated Immunity and Histological Characteristics of Disseminated AIDS-related Mycobacterium avium Infection after the Initiation of Antiretroviral Therapy

Hibiya Kenji,Tateyama Masao,Teruya Katsuji,Mochizuki Makoto,Nakamura Hideta,Tasato Daisuke,Furugen Makoto,Higa Futoshi,Endo Hisako,Kikuchi Yoshimi,Oka Shinichi,Fujita Jiro
Internal Medicine 52(16), 1793-1803, 2013
… Objective The aim of the present study was to examine the immunohistological characteristics of disseminated Mycobacterium avium infection after the initiation of antiretroviral therapy (ART) for acquired immunodeficiency syndrome (AIDS). … Conclusion These findings suggest that local cell-mediated immunity is depressed in affected tissues and that the primary histological feature is poor organization of granulomas in mycobacterial lesions, despite the administration of adequate ART. …
NAID 130003365763

Chédiak-Higashi症候群の血球貪食性リンパ組織球症としての臨床像

宮前多佳子,井崎桜子,生田孝一郎,横田俊平,山中寿
日本臨床免疫学会会誌 36(4), 226-232, 2013
Chédiak-Higashi症候群は原発性免疫不全症候群のひとつに分類され，わが国では約14例の報告があるに過ぎない．臨床的には易感染性，部分白子症（特異な白銀髪，虹彩色素の減少，乳白色で，紫外線により赤味を帯びる皮膚），出血傾向，進行性神経障害を特徴とするが，accelerated phaseと呼ばれる増悪期には，発熱，脾腫，骨髄抑制などを伴う血球貪食性リンパ組織球症を併発する．また，主に …
NAID 130003364183

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★リンクテーブル★

リンク元	「原発性免疫不全症候群」
関連記事	「primary」「immunodeficiency」「syndrome」

「原発性免疫不全症候群」

　　[★]

英: primary immunodeficiency syndrome
同: 原発性免疫不全症先天性免疫不全症 primary immunodeficiency disease
関: 免疫不全症候群

複合およびT細胞免疫不全症候群

IMD.1132改変

疾患名	血清Ig	B細胞数	T細胞数	病因	遺伝形式	随伴症
SCID X連鎖型	↓	正常～↑	↓↓	IL-2Rγ鎖欠損	XR
SCID 常染色体劣性型	↓	↓↓～正常	↓↓	T, B細胞の成熟障害	AR
ADA欠損症	↓	↓	↓	毒性代謝物蓄積によるT,B細胞障害	AR
プリンヌクレオチドホスホリラ-ゼ欠損症	正常～↓	正常	↓(進行性)	毒性代謝物によるT細胞障害	AR	AIHA神経症状
MHCクラスII欠損症	正常～↓	正常	正常	MHCクラスII分子の転写調節障害	AR	. l ～ L
細網系形成障害	↓(母親由来)	↓↓	↓↓	リンパ球、骨髄細胞成熟障害	AR	顆粒球減少
CD3γ欠損症/CD3ε欠損症	正常	正常	正常	CD3γまたはCD3ε鎖の転写	AR
CD8欠損症	正常	正常	正常～↓	CD8陽性T細胞の成熟障害	AR

抗体不全を主とする免疫不全症候群の分類

IMD.1132改変

疾患名	血清Ig	B細胞数	病因	遺伝形式	随伴症
X連鎖無γ-グロブリン血症	↓↓	↓↓	preB→B細胞への分化障害	XR
IgM増加を伴う免疫グロブリン欠乏症(高IgM症候群)	IgM↑ IgG,IgA,lgEは↓	IgM, IgD陽性細胞は正常, 他は↓↓	XRではCD40L遺伝子変異によりクラススイッチ障害	XRその他多様	好中球減少症血小板減少症溶血性貧血
免疫グロブリン重鎖遺伝子欠失	IgG2, IgG4欠損, ときにIgE, IgA2欠損	正常	染色体14q32欠損	AR
κ鎖欠乏症.	κ鎖↓ 抗体反応は正常または↓	κ鎖陽性細胞は正常～↓	一部で染色体2p11に点変異	AR
選択的IgGサブクラス欠乏症(ときにIgA欠乏症を伴う)	1つ以上のIgGサブクラス↓	正常	アイソタイプ分化の障害	不明
common variabe immunodeficiency (分類不能型免疫不全症)	多くのアイソタイプで↓(多様)	正常～↓	IgA陽性B細胞の最終分化障害	多様	リンパ増殖性疾患悪性腫瘍自己免疫疾患
IgA欠乏症	IgA1.IgA2↓	正常	IgA陽性B細胞の最終分化障害	多様	自己免疫疾患アレルギー疾患
乳児一過性低γ-グロブリン血症	IgG.IgA↓	正常	B細胞分化障害、一部ではTヘルパー機能の成熟遅延	不明

その他の主な原発性免疫不全症候群

IMD.1133改変

疾患名	血清Ig	B細胞数	T細胞数	病因	遺伝形式	随伴症
Wiskott-Aldrich症候群	IgM↓ IgA, IgE↑	正常	↓	CD43の発現異常	XR	血小板減少アトピー様湿疹
血管拡張性運動失調症(ataxia telangiectasia)	IgA IgG2↓ IgE↓	正常	↓	DNA修復障害	AR	毛細血管拡張、小脳運動失調、AFP高値、.リンパ網内系腫瘍
DiGeorge症候群	正常	正常	正常～↓	胎生期の発生学的異常、胸腺低形成、T細胞機能不全	不明	副甲状腺機能低下先天奇形
慢性皮膚粘膜カンジダ症	正常～↑	正常	正常	力ンジダに対する特異的細胞性免疫能不全	不明	副甲状腺機能低下症甲状腺機能低下症 Addison病、糖尿病
Chediak-Higashi症候群	正常	正常	正常	cGMPの生成不全に伴う好中球の減少と機能不全	AR	部分白子症日光過敏症
補体欠損症	正常	正常	正常	おのおのの補体蛋白欠損	AR	SLE様症状ナイセリア感染症
慢性肉芽腫症	正常	正常	正常	NADPHオキシダーゼの活性低下による食細胞機能不全	XR、AR