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"thalassaemia major"¿¡ ´ëÇÑ °Ë»ö °á°úÀÔ´Ï´Ù. °Ë»ö °á°ú º¸´Â µµÁß¿¡ Tab ۸¦ ´©¸£½Ã¸é °Ë»ö âÀÌ ¼±Åõ˴ϴÙ.
À̰ÍÀ» ¿øÇϼ̽À´Ï±î?
¾Ë±â½¬¿î ÀÇÇпë¾îÇ®ÀÌÁý, ¼­¿ïÀÇ´ë ±³¼ö ÁöÁ¦±Ù, °í·ÁÀÇÇÐ ÃâÆÇ À¯»ç °Ë»ö °á°ú : 1 ÆäÀÌÁö: 1
¿µ¹® labium major ÇÑ±Û ´ëÀ½¼ø
¼³¸í   
  ¿©¼ºÀÇ ¼º±â °¡Àå ¹Ù±ùÂÊ¿¡ ÇØ´çÇϴ ºÎºÐ.
´ëÇÑÀÇÇù ÀÇÇпë¾î »çÀü °Ë»ö À¯»ç °Ë»ö °á°ú : 15 ÆäÀÌÁö: 1
  • ¿µ¹®
    ÇѱÛ
  • fonticulus major
    ¾Õ¼ý±¸¸Û, ´ëõ¹®
  • labium major
    ´ëÀ½¼ø
  • Leishmania major
    Å«¸®½´¸¸Æí¸ðÃæ
  • major basic protein
    ÁÖ¿ä±âÃʴܹéÁú, ÁÖ±âÀú´Ü¹éÁú
  • major calyx
    Å«ÄáÆÏÀÜ, ´ë½Å¹è
  • major dense line
    ūġ¹Ð¼±
  • major depression
    ÁÖ¿ä¿ì¿ïÁõ
  • major depressive episode
    ÁÖ¿ä¿ì¿ïº´»ðÈ­, ÁÖ¿ä¿ì¿ïº´¿¡ÇǼҵå
  • major epilepsy
    ´ë°£Áú
  • major hand
    ÀÍÀº¼Õ, ¿ì¼¼¼Õ
  • major histocompatibility antigen
    ÁÖÁ¶Á÷ÀûÇÕ¼ºÇ׿ø
  • major histocompatibility complex
    ÁÖÁ¶Á÷ÀûÇÕº¹ÇÕü
  • major histocompatibility gene
    ÁÖÁ¶Á÷ÀûÇÕ¼ºÀ¯ÀüÀÚ
  • major histocompatibility system
    ÁÖÁ¶Á÷ÀûÇÕ°èÅë
  • major lymphatic drainage
    Áֿ串ÇÁ¹èÃâ
´ëÇÑÀÇÇù Çʼö ÀÇÇпë¾îÁý »çÀü °Ë»ö À¯»ç °Ë»ö °á°ú : 6 ÆäÀÌÁö: 1
  • ¿µ¹®
    ÇѱÛ
  • major depression
    ÁÖ¿ä¿ì¿ïÁõ
  • major lymphatic drainage
    Áֿ串ÇÁÀ¯Ãâ
  • pectoralis major myocutaneous flap
    Å«°¡½¿±ÙÇÇÆÇ, ´ëÈä±ÙÇÇÆÇ
  • Leishmania major
    Å«¸®½´¸¸Æí¸ðÃæ
  • pectoralis major muscle
    Å«°¡½¿±Ù, ´ëÈä±Ù
  • major tranquilizer
    °­·Â½Å°æ¾ÈÁ¤Á¦
¿¾ ´ëÇÑÀÇÇù ÀÇÇпë¾î »çÀü °Ë»ö À¯»ç °Ë»ö °á°ú : 15 ÆäÀÌÁö: 1
  • ¿µ¹®
    ÇѱÛ
  • major histocompatibility antigen
    ÁÖÁ¶Á÷ÀûÇÕÇ׿ø
  • major thymic bud
    Å«°¡½¿»ù½Ï
  • major calyx
    Å«ÄáÆÏÀÜ
  • major histocompatibility complex
    ÁÖÁ¶Á÷ÀûÇÕº¹ÇÕü
  • major affective disorder
    ÁÖ¿äÁ¤µ¿Àå¾Ö, ÁÖ¿äÁ¤µ¿º´
  • major epilepsy
    ´ë°£Áú
  • major depressive episode
    ÁÖ¿ä¿ì¿ï»ðÈ­
  • fonticulus major
    (¢¡anterior fontanelle) ¾Õ¼ý±¸¸Û
  • pectoralis major myocutaneous flap
    Å«°¡½¿±ÙÇÇÆÇ, ´ëÈä±ÙÇÇÆÇ
  • major histocompatibility gene
    ÁÖÁ¶Á÷ÀûÇÕ¼ºÀ¯ÀüÀÚ
  • major hand
    ÀÍÀº¼Õ
  • Leishmania major
    Å«¸®½´¸¸Æí¸ðÃæ
  • major dense line
    ūġ¹Ð¼±
  • major operation
    ´ë¼ö¼ú
  • major surgery
    ´ë¼ö¼ú
¿¾ ´ëÇÑÀÇÇù 2 ÀÇÇпë¾î »çÀü °Ë»ö À¯»ç °Ë»ö °á°ú : 15 ÆäÀÌÁö: 1
  • ¿µ¹®
    ÇѱÛ
  • rectus capitis posterior major muscle ; muscle rectus capitis posterior major
    ´ëÈĵÎÁ÷±Ù(ÓÞý­ÔéòÁÐÉ).
  • Leishmania major
    ÁÖ ¸®½´¸¶´Ï¾Æ
  • MHC => major histocompatibility complex
    ÁÖÁ¶Á÷ÀûÇÕº¹ÇÕü
  • antigen, major histocompatibility
    ÁÖÁ¶Á÷ÀûÇÕÇ׿ø, MHCÇ׿ø
  • glandula vestibularis major<³ª>
    Å«ÀüÁ¤»ù, ´ëÀüÁ¤¼±(ÓÞîñïÔàÍ).
  • glandulae vestibularis major<³ª>
    Å«ÀüÁ¤»ù, ´ë(ÓÞ)ÀüÁ¤¼±.
  • globus major<³ª>
    °í»óüµÎºÎ(ÍÂß¾ô÷ÔéÝ»).
  • great pelvis<³ª> p. major
    Å«°ñ¹Ý, ´ë°ñ¹Ý(ÓÞÍéÚï).
  • great pelvis<³ª> p. major
    Å« °ñ¹Ý, ´ë °ñ¹Ý(ÓÞÍéÚï).
  • greater curvature of ventriculus<³ª> curvatura ventriculi major
    (À§) Å«¸¸°î, ´ë¸¸(ÓÞØ¶) À§ÀÇ .
  • greater curvature of ventriculus<³ª> curvatura ventriculi major
    ´ë¸¸(´ë¸¸)(À§ÀÇ).
  • greater occipital nerve<³ª> nervus occipitalis major
    ´ëÈĵνŰæ(¡­ãêÌè).
  • greater psoas muscle<³ª> musculus p. major
    Å«Ç㸮±Ù, ´ë¿ä±Ù(ÓÞé¦ÐÉ).
  • greater psoas muscle<³ª> musculus p. major
    Å« Ç㸮 ±Ù, ´ë ¿ä ±Ù(ÓÞé¦ÐÉ).
  • greater renal calices<³ª> calix renalis major
    Å«(½ÅÀå)¼úÀÜ, ´ë½Å¹è(ÓÞãìÛÊ).
¿¾ ´ëÇÑÀÇÇù 3 ÀÇÇпë¾î »çÀü °Ë»ö À¯»ç °Ë»ö °á°ú : 15 ÆäÀÌÁö: 1
  • ¿µ¹®
    ÇѱÛ
  • major palatine canal ; canalis palatinus major
    Å«±¸°³°ü, ´ë±¸°³°ü.
  • major palatine foramen ; foramen palatinum major
    Å«±¸°³±¸¸Û, ´ë±¸°³°ø.
  • major psoas muscle ; muscle psoas major
    Å«Ç㸮±Ù, ´ë¿ä±Ù(ÓÞé¦ÐÉ).
  • major psoas muscles ; musculus psoas major
    Å«Ç㸮±Ù, ´ë¿ä±Ù.
  • pectoralis major muscle ³ª musculus pectoralis major
    ´ëÈä±Ù.
  • rectus capitis posterior major muscle ; muscle rectus capitis posterior major
    ´ëÈĵÎÁ÷±Ù(ÓÞý­ÔéòÁÐÉ).
  • antigen, major histocompatibility
    ÁÖÁ¶Á÷ÀûÇÕÇ׿ø, MHCÇ׿ø
  • arteria palatina major <³ª>
    ´ë±¸°³µ¿¸Æ, Å«±¸°³µ¿¸Æ{ÇØ}
  • asynergy major
    ´ëÇùµ¿¿îµ¿ºÒ´É(Áõ).
  • canal, major palatine
    ´ë±¸°³°ü, Å«±¸°³°ü{ÇØ}
  • clavicular part of greater pectoral muscle ; pars clavicularis pectoralis major is
    ¼â°ñºÎºÐ ´ëÈä±ÙÀÇ , ¼â°ñºÎ.
  • complex, major histocompatibility
    ÁÖÁ¶Á÷ÀûÇÕº¹ÇÕü
  • curvatura ventriculi major<³ª>
    Å«¸¸°î, ´ë¸¸(ÓÞØ¶).
  • ductus pancreaticus major<³ª>
    ´ëÃé°ü(´ëÃé°ü).
  • ductus pancreaticus major<³ª>
    Å«ÃéÀå°ü, ´ëÃé°ü(ÓÞõýη).
´ëÇÑÇØºÎÇÐȸ ÀÇÇпë¾î »çÀü °Ë»ö À¯»ç °Ë»ö °á°ú : 15 ÆäÀÌÁö: 1
  • ¿µ¹®
    ÇѱÛ
  • Labium major
    ´ëÀ½¼ø
    [¿¾ ¿ë¾î] ´ëÀ½¼ø
  • Forceps major [Occipital forceps]
    µÚÅë¼öÁý°Ô [Å«Áý°Ô]
    [¿¾ ¿ë¾î] ´ë°âÀÚ
  • Major renal calices
    Å«(ÄáÆÏ)¼úÀÜ
    [¿¾ ¿ë¾î] ´ë½Å¹è
  • Pectoralis major m.
    Å«°¡½¿±Ù
    [¿¾ ¿ë¾î] ´ëÈä±Ù
  • Major thymic bud
    Å«°¡½¿»ù½Ï
    [¿¾ ¿ë¾î] ´ëÈä¼±·Ú
  • Zygomaticus major m.
    Å«±¤´ë±Ù
    [¿¾ ¿ë¾î] ´ë°ü°ñ±Ù
  • Helicis major muscle
    Å«±ÍµÑ·¹±Ù
    [¿¾ ¿ë¾î] ´ëÀÌ·û±Ù
  • Rectus capitis posterior major m.
    Å«µÚ¸Ó¸®°ðÀº±Ù
    [¿¾ ¿ë¾î] ´ëÈĵÎÁ÷±Ù
  • Rhomboid major m.
    Å«¸¶¸§±Ù
    [¿¾ ¿ë¾î] ´ë´ÉÇü±Ù
  • Teres major m.
    Å«¿ø±Ù
    [¿¾ ¿ë¾î] ´ë¿ø±Ù
  • Subtendinous bursa of teres major m.
    Å«¿ø±ÙÈûÁÙ¹ØÁÖ¸Ó´Ï
    [¿¾ ¿ë¾î] ´ë¿ø±Ù°ÇÇϳ¶
  • Major pancreatic artery
    Å«ÀÌÀÚµ¿¸Æ
    [¿¾ ¿ë¾î] ´ëÃ鵿¸Æ
  • Major vestibular gland
    Å«Áú¾î±Í»ù
    [¿¾ ¿ë¾î] ´ëÀüÁ¤¼±
  • Major salivary glands
    ūħ»ù
    [¿¾ ¿ë¾î] ´ë±¸°­¼±
  • Psoas major m.
    Å«Ç㸮±Ù
    [¿¾ ¿ë¾î] ´ë¿ä±Ù
´ëÇÑ»ýÈ­ÇкÐÀÚ»ý¹°ÇÐȸ ¿ë¾î »çÀü °Ë»ö À¯»ç °Ë»ö °á°ú : 5 ÆäÀÌÁö: 1
  • ¿µ¹®
    ÇѱÛ
  • major gene
    ÁÖ À¯ÀüÀÚ(ñ«ë¶îîí­)
  • major groove
    ūȨ
  • major histocompatibility complex
    ÁÖ Á¶Á÷ÀûÇÕ º¹ÇÕü(ñ«ðÚòÄîêùêÜÜùêô÷)
  • major immunogene complex
    ÁÖ ¸é¿ªÀ¯ÀüÀÚ º¹ÇÕü(ñ«Øóæµë¶îîí­ÜÜùêô÷)
  • major-minor code
    ´ë.¼Ò(ÓÞá³)ÄÚµå
KI ÀÇÇпë¾î »çÀü °Ë»ö À¯»ç °Ë»ö °á°ú : 10 ÆäÀÌÁö: 1
  • ¿µ¹®
    ÇѱÛ
  • major psoas muscle;muscle psoas major
    Å«Ç㸮±Ù, ´ë¿ä±Ù
  • forceps major
    ´ë°âÀÚ
  • major
    ÁÖ¿äÀÇ, ´ë, Å«
  • major calyx
    Å«(½ÅÀå)¼úÀÜ, ´ë½Å¹è
  • major fissure
    ´ë¿­
  • major interlobar fissure
    ´ë¿±°£¿­
  • major operation
    ´ë¼ö¼ú
  • major surgery
    ´ë¼ö¼ú
  • pectoralis major muscle
    ´ëÈä±Ù
  • teres major muscle
    Å«¿ø±Ù, ´ë¿ø±Ù
KMLE ÀÇÇоà¾î »çÀü À¯»ç °Ë»ö °á°ú : 5 ÆäÀÌÁö: 1
TM technology management; tectorial membrane; temperature by mouth; temporalis muscle; temporomandibula...
GnRH Gonadotropin Releasing Hormone  [HP 1898, 2034]
  = LHRH
  = Go...
MAD Major Antigenic Determinant
MHC Major Histocompatibility Complex
EMM erythema multiforme major
KMLE ÀÚµ¿ÃßÃâ ÀÇÇоà¾î »çÀü À¯»ç °Ë»ö °á°ú : 5 ÆäÀÌÁö: 1
beta Thal beta thalassaemia
L major Leishmania major
AdMLP Adenovirus major late promoter
Ad2MLP Adenovirus-2 major late promoter
MHC Anti-major histocompatibility complex
°æºÏ´ë Ä¡°ú´ëÇÐ ±¸°­³»°ú ±³½Ç »çÀü À¯»ç °Ë»ö °á°ú : 15 ÆäÀÌÁö: 1
  • ¿µ¹®
    ÇѱÛ
    ¼³¸í
  • arteria palatina major
    Å« ±¸°³ µ¿¸Æ, ´ë±¸°³ µ¿¸Æ
    µ¿ÀǾî=greater
  • ductus pancreaticus major
    Å« ÃéÀå°ü, ´ëÃé°ü
  • fonticulus major
    ¾Õ ¼ý±¸¸Û, ´ëõ¹®
  • hysteria major
    ´ë È÷½ºÅ׸®
    ȯ¸ù »óÅÂ. È¥¹Ì ¹× ¸¶ºñ·Î µ¹¿¬ ¹ßº´ÇÏ´Â °ÍÀ» Ư¡À¸·Î ÇÏ´Â È÷½ºÅ׸®.
  • Leishmania major
    Å« ¸®½´¸¸ Æí¸ðÃæ
  • linguoplate major connector
    ¼³¸éÆÇ ÁÖ ¿¬°á ÀåÄ¡
    ¼³¸é ÆÇÀÇ ÇÑ ºÎºÐ.
  • major
    ´ë, ÁÖ, ÁÖ¿äÇÑ, ÁÖ¿äÀÇ, Å«
  • major amblyoscope
    ´ëÇü ¾à½Ã°æ, ´ëÇü ¾à½Ã°è
    ¾à½ÃÀÇ ´«À» ½Ã°¢¿¡ Âü¿©½Ã۵µ·Ï ÈÆ·Ã½ÃŰ°Å³ª ¶Ç´Â ¾ç¾ÈÀÇ À¶»ó·ÂÀ» ÃøÁ¤Çϰųª Áõ»ó½Ã۱â ÀÇÇÑ ±â°è.
  • major aphthae
    ´ë¾ÆÇÁŸ
  • major connector
    ÁÖ ¿¬°áÀÚ, ÁÖ ¿¬°á ÀåÄ¡
    1. ±¹¼Ò ÀÇÄ¡¿¡¼­ ¾Ç±Ã ÇÑÂÊ¿¡ ³õ¿©Áø º¸Ã¶¹°ÀÇ ÇÑ ºÎºÐ°ú ¹Ý´ëÂÊ¿¡ ³õ¿©Áø º¸Ã¶¹°ÀÇ ÇÑ ºÎºÐÀ» ¿¬°áÇÏ´Â ÀåÄ¡. 2. Ä¡±ÃÀÇ ÇÑÂÊ ±¸¼º ¿ä¼Òµé°ú ¹Ý´ëÆí ±¸¼º ¿ä¼ÒµéÀ» ¿¬°áÇØ ÁÖ´Â °¡Ã¶¼º ±¹¼Ò ÀÇÄ¡ÀÇ ÇÑ ºÎºÐÀÌ´Ù.
  • major depression
    ´ë ¿ì¿ïÁõ, ÁÖ¿ä ¿ì¿ïÁõ
    ¿À·¡ Áö¼ÓµÇ´Â ¿ì¿ïÁõÀÌ Æ¯Â¡ÀÎ Á¤½Å Àå¾Ö·Î¼­ ½Ä¿å °áÇÌ, °ú½Ä, ºÒ¸éÁõ, °ú¸éÁõ, ±â·Â »ó½Ç, ÇÇ·Î, ÀÚÁ¸½É ºÎÁ·, ÁýÁß·Â °á¿©, ³«´ã°¨ µî°ú °°Àº Áõ»óÀÌ Á¾Á¾ µ¿¹ÝµÈ´Ù.
  • major depressive disorder
    ÁÖ¿ä ¿ì¿ïÁõ
  • major fit
    ´ë¹ßÀÛ, ÁÖ ¹ßÀÛ
    ºÎÀûÀýÇϰųª ºÒ¼öÀÇÀûÀÎ ¿îµ¿¼º ¶Ç´Â Á¤½ÅÀû Ȱµ¿¼ºÀ» Ư¡À¸·Î ÇÏ´Â °¡Àå ºó¹øÇÑ ¹ßÀÛ »óÅÂ.
  • major histocompatibility complex
    ÁÖ Á¶Á÷ ÀûÇÕü, ÁÖ Á¶Á÷ ÀûÇÕ º¹ÇÕü
    ÀÎü Á¶Á÷ÇüÀ» °áÁ¤ÇÏ´Â À¯ÀüÀÚ°¡ 6¹ø ¿°»öüÀÇ ÀÛÀº ºÐÀý¿¡ º¹ÇÕü¸¦ Çü¼ºÇϰí À־ ÀÌ À¯ÀüÀÚ ¿µ¿ªÀ» À̸£´Â ¿ë¾î.
  • major operation
    ´ë ¼ö¼ú
CancerWEB ¿µ¿µ ÀÇÇлçÀü ¸ÂÃã °Ë»ö °á°ú : 1 ÆäÀÌÁö: 1
thalassaemia major The dire disease also known as beta thalassaemia. The clinical picture of this form of anaemia was first described in 1925 by the paediatrician thomas benton cooley. Other names for the disease are cooley's anaemia and mediterranean anaemia. The term thalassaemia was coined by the nobel prise winning pathologist george whipple and the professor of paediatrics william bradford at u. Of rochester because thalassa in greek means the sea (like the mediterrranean sea) + -aemia means in the blood so thalassaemia means sea in the blood. Thalassaemia is not just one disease. It is a complex contingent of genetic (inherited) disorders all of which involve underproduction of haemoglobin, the indispensable molecule in red blood cells that carries oxygen. The globin part of normal adult haemoglobin is made up of 2 alpha and 2 beta polypeptide chains. In beta thalassaemia, there is a mutation (change) in both beta globin chains leading to underproduction (or absence) of beta chains, underproduction of haemoglobin, and profound anaemia. The gene for beta thalassaemia is relatively frequent in people of mediterranean origin (for example, from italy and greece). Children with this disease inherit one gene for it from each parent (and so are said to be homozygous for beta thalassaemia). The parents are carriers (heterozygotes) with just one thalassaemia gene, are said to have thalassaemia minor, and are essentially normal. Their children affected with beta thalassaemia seem entirely normal at birth (because at birth we still have predominantly foetal haemoglobin which does not contain beta chains) but the anaemia emerges in the first few months of life and becomes progressively more severe leading to pallor and easy fatiguability, failure to thrive (grow), bouts of fever (due to infections) and diarrhoea. Treatment based on blood transfusions is helpful but not curative. Gene therapy will, it is hoped, be applicable to this disease.
(12 Dec 1998)
CancerWEB ¿µ¿µ ÀÇÇлçÀü À¯»ç °Ë»ö °á°ú : 15 ÆäÀÌÁö: 1
A2 thalassaemia Beta thalassaemia, heterozygous state.
(05 Mar 2000)
alpha thalassaemia <haematology> A condition characterised by the reduced synthesis of the alpha chain of haemoglobin due to abnormality in one of two or more genes that code for the synthesis of alpha-globin chains. The severity of this condition can vary from mild anaemia to death, depending on the number of genes deleted.
Heterozygous state: severe type, thalassaemia minor with 5 to 15% of Hb Barts at birth, only traces of Hb Barts in adult; mild type, 1 to 2% of Hb Barts at birth, not detectable in adult.
Homozygous state: severe type, erythroblastosis foetalis and foetal death, only Hb Barts and Hb H present; mild type not clinically defined.
See: haemoglobin H.
(05 Mar 2000)
a thalassaemia intermedia See: haemoglobin H.
(05 Mar 2000)
beta-d thalassaemia Thalassaemia due to a gene that depresses synthesis of both beta-and d-globin chains by the chromosome bearing the abnormal gene. Heterozygous state: thalassaemia minor with Hb F comprising 5 to 30% of total haemoglobin but distributed unevenly among cells, Hb A2 reduced or normal. Homozygous state: moderate anaemia with only Hb F present, no Hb A or Hb A2.
Synonym: F thalassaemia.
(05 Mar 2000)
beta thalassaemia <haematology> Thalassaemia due to one of two or more genes that depress (partially or completely) synthesis of beta-globin chains by the chromosome bearing the abnormal gene. Heterozygous state (A2 t.): thalassaemia minor with Hb A2 increased, Hb F normal or variably increased, Hb A normal or slightly reduced. Homozygous state: thalassaemia major with Hb A reduced to very low but variable levels, Hb F very high level.
A disorder characterised by reduced synthesis of the beta chains of haemoglobin. There is retardation of haemoglobin a synthesis in the heterozygous form (thalassaemia minor), which is asymptomatic, while in the homozygous form (thalassaemia major, cooley's anaemia, mediterranean anaemia, erythroblastic anaemia), which can result in severe complications and even death, haemoglobin a synthesis is absent.
(05 Mar 2000)
sickle cell-thalassaemia disease Anaemia, clinically resembling sickle cell anaemia, in which individuals are compound heterozygous for the sickle cell gene and a thalassaemia gene; about 60 to 80% of haemoglobin is Hb S, up to 20% Hb F, and the remainder Hb anaemia.
Synonym: sickle cell-thalassaemia disease.
(05 Mar 2000)
thalassaemia <haematology> A genetic form of anaemia in which there is abnormality of the globin portion of haemoglobin.
Affected individuals cannot synthesise haemoglobin properly, and they produce small, pale, short-lived red blood cells.
Widespread in Mediterranean countries.
Origin: Gr. Haima = blood
(11 Jan 1998)
thalassaemia, beta Also known as thalassaemia major.The clinical picture of this important type of anaemia was first described in 1925 by the paediatrician thomas benton cooley. Other names for the disease are cooley's anaemia and mediterranean anaemia. The name thalassaemia was coined by the nobel prise winning pathologist george whipple and the professor of paediatrics wm bradford at u. Of rochester because thalassa in greek means the sea (like the mediterrranean sea) + -aemia means in the blood so thalassaemia means sea in the blood. Thalassaemia is not just one disease. It is a complex contingent of genetic (inherited) disorders all of which involve underproduction of haemoglobin, the indispensable molecule in red blood cells that carries oxygen. The globin part of normal adult haemoglobin is made up of 2 alpha and 2 beta polypeptide chains. In beta thalassaemia, there is a mutation (change) in both beta globin chains leading to underproduction (or absence) of beta chains, underproduction of haemoglobin, and profound anaemia. The gene for beta thalassaemia is relatively frequent in people of mediterranean origin (for example, from italy and greece). Children with this disease inherit one gene for it from each parent. The parents are carriers (heterozygotes) with just one thalassaemia gene, are said to have thalassaemia minor, and are essentially normal. Their children affected with beta thalassaemia seem entirely normal at birth because at birth we still have predominantly foetal haemoglobin which does not contain beta chains. The anaemia surfaces in the first few months after birth and becomes progressively more severe leading to pallor and easy fatiguability, failure to thrive (grow), bouts of fever (due to infections) and diarrhoea. Treatment based on blood transfusions is helpful but not curative. Gene therapy will, it is hoped, be applicable to this disease.
(12 Dec 1998)
thalassaemia intermedia A clinical variant of thalassaemia characterised by an intermediate degree of severity. These patients have severe anaemia but usually do not require regular blood transfusions. Intermedia disorders represented a heterogeneous group of genetic disorders and may include cases with homozygous or heterozygous abnormalities in the beta-globin chain gene.
(05 Mar 2000)
thalassaemia minor Also called thalassaemia trait, thalassaemia minor is the carrier state for beta thalassaemia. People who are carriers (heterozygotes) have just one thalassaemia gene, are said to have thalassaemia minor, and are essentially normal.
(12 Dec 1998)
F thalassaemia Thalassaemia due to a gene that depresses synthesis of both beta-and d-globin chains by the chromosome bearing the abnormal gene. Heterozygous state: thalassaemia minor with Hb F comprising 5 to 30% of total haemoglobin but distributed unevenly among cells, Hb A2 reduced or normal. Homozygous state: moderate anaemia with only Hb F present, no Hb A or Hb A2.
Synonym: F thalassaemia.
(05 Mar 2000)
Lepore thalassaemia Thalassaemia syndrome due to production of abnormally structured Lepore haemoglobin. Heterozygous state: thalassaemia minor with about 10% of Hb Lepore, Hb F moderately increased, Hb A2 normal. Homozygous state: thalassaemia major with only Hb F and Hb Lepore produced, no Hb A or Hb A2.
(05 Mar 2000)
ala major ossis sphenoidalis Strong squamous processes extending in a broad superolateral curve from the body of the sphenoid bone. The greater wing presents these suraces (facies): 1) cerebral surface: forms anterior third of the floor of the lateral portions of the middle cranial fossa; 2) temporal surface: forms the deepest portion of the temporal fossa; 3) infratemporal surface, forms the "roof" of the infratemporal fossa; 4) orbital surface: forms posterolateral wall of orbit. The greater wing forms the inferior border of the supraorbital fissure, and is perforated at its root by foramina rotundum ovale, and spinosum and the pterygoid canal.
Synonym: ala major ossis sphenoidalis, ala temporalis.
(05 Mar 2000)
annulus iridis major The outer, broader of the two zones of the iris.
Synonym: annulus iridis major.
(05 Mar 2000)
aphthae major A severe form of aphthae characterised by unusually numerous, large, deep, and frequent ulcers; healing may take as long as six weeks and results in scarring.
Synonym: Mikulicz' aphthae, periadenitis mucosa necrotica recurrens, recurrent scarring aphthae, Sutton's disease.
(05 Mar 2000)
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thalassaemia major a fatal form of homozygous thalassemia (inherited from both parents) in which there is no hemoglobin
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