аЯрЁБс>ўџ 02ўџџџ/џџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџьЅСq` јПZbjbjqPqP .::Z џџџџџџЄ*******>††††Ђ >[ ЖКааааЋЋЋк м м м м м м $ hyn *в ЋЋв в **аал & & & в "*а*ак & в к & & **& аЎ а­Э“9…Щ†є (& к + 0[ & ч єч& & Lч*r hЋ"Э& хљйЋЋЋ  ЋЋЋ[ в в в в >>>Ф„>>>>>>******џџџџ Niemann-Pick Type A and B - WHY CHANGE THE NAME? Over the last decade it has become apparent that Niemann-Pick Types A & B, rather than being two separate ‘types’ of the disease, actually represent the opposite ends of a spectrum of the same disease, both caused by a deficiency of the enzyme Acid Sphingomyelinase (ASM). Many variations exist within this spectrum, in terms of clinical symptoms and rate of progression. In the USA, scientists are beginning to refer to NP Types A & B under the name "Acid Sphingomyelinase Deficiency disease (ASMD)" rather than "Niemann-Pick disease" as this more accurately describes the condition. This is not in fact a new term of reference - Niemann-Pick diseases Types A and B were generally known as Acid Sphingomyelinase Deficiency disease (ASMD) until the early 1960’s. Often Niemann-Pick Types A and B do not get the attention they deserve, as compared to the more widely known Niemann-Pick Type C. With this in mind, scientists worldwide would prefer to give Niemann-Pick Types A and B their correct name, to avoid confusion and to assist in raising the profile of this condition. Patients at the severe end of the ASMD spectrum, Niemann-Pick Type A, have a very rapidly progressive neurodegenerative disease usually resulting in death before the age of 4. In contrast, patients at the opposite end of the spectrum, Niemann-Pick Type B, have a more variable spectrum of severity and symptom involvement. This condition is usually diagnosed in childhood, due to the presence of an enlarged liver and/or spleen. The majority of NPB patients survive well into adulthood with little or no neurological involvement, but respiratory problems can cause disease complications. The NPDG (UK)’s Clinical Nurse Specialist, Jackie Imrie, is currently working with Dr Ed Schuchman, Professor and Vice Chairman for Research at Mount Sinai School of Medicine, to produce an information booklet about ASMD Niemann-Pick Disease. The booklet will be entitled “Acid Sphingomyelinase Deficiency Niemann-Pick Disease and its potential treatment.” If you would like to contribute to the booklet, perhaps by providing a story about how the disease has affected you or your family, or if you would like to know more about the booklet, please do contact Jackie on 0161 922 2414 or email  HYPERLINK "mailto:Jackie.imrie@ccmc.nhs.uk" Jackie.imrie@ccmc.nhs.uk 12GR]c{›ВД A D n | ‘ ™ І Ї ­ е & | Ћ Ю э ю ў  0 6 B Q ёуйЯТЯТЕЯЋЯЁЕЯЋ—Ћ—ЋЁЋ—ЀЀs€s€s€i\—h+зhД3іOJQJ^JhД3іOJQJ^JhУhЊ)OJQJ^JhЊ)OJQJ^Jh+зh#>~OJQJ^Jh#>~OJQJ^JhЙ*nOJQJ^JhxDХOJQJ^Jh+зh~OJQJ^JhУh~OJQJ^Jh~OJQJ^JhУOJQJ^Jh~hжNЦ5OJQJ^Jh~h+з5OJQJ^J#12Ї Ј / 0 i j ИЙWXYZњњѕѕ№ыыыыыцыыыgd„+ьgd#>~gdЊ)gd~gdжNЦZўQ v   Љ А У Ф б я ћ   ' 0 g h j Œ ‘ › Ј Џ   & @ V b j q r П Щ о п і ї 8W^ƒИЙѓщпвщѓщѓпвщѓщѓщѓщѓШпвпѓШѓШпвпѓЛБѓЇБЇѓšШѓщѓБЇШƒh„+ьOJQJ^JhŽjh#>~OJQJ^JhŽjhŽjOJQJ^JhŽjOJQJ^Jh@'=OJQJ^Jh@'=h@'=OJQJ^JhЊ)OJQJ^Jh+зhД3іOJQJ^JhД3іOJQJ^Jh#>~OJQJ^JhУh#>~OJQJ^J/1HgЪ2Л  :;<TUVWXZятитиЫиСиГСŸГГСт†|h)KOJQJ^Jh#>~OJQJ^Jh1hљcZ0JOJQJ^J'jh1hљcZOJQJU^JjhљcZOJQJU^JhљcZOJQJ^JhУh„+ьOJQJ^Jh„+ьOJQJ^Jh„+ьh„+ьOJQJ^J h„+ьh„+ьCJOJQJ^JaJ,1hАа/ Ар=!А"А# $ %ААФАФ ФџDаЩъyљКЮŒ‚ЊKЉ Jackie.imrie@ccmc.nhs.ukрЩъyљКЮŒ‚ЊKЉ Xmailto:Jackie.imrie@ccmc.nhs.ukyXє;HЏ,‚]Ф…'cЅЋ†œ@@ёџ@ жNЦNormalCJ_HaJmH sH tH DA@ђџЁD Default Paragraph FontRiѓџГR  Table Normalі4ж l4жaі (kєџС(No List4U@Ђё4 Й*n Hyperlink >*phџN^@N Й*n Normal (Web)ЄdЄd[$\$ mH sH tH Z џџџџ12ЇЈ/0ijИЙW X Y \ ˜0€€˜0€€˜0€€˜0€€˜0€€˜0€€˜0€€˜0€€˜0€€˜0€€˜0€€˜0€€˜0€€˜0€€12ЇЈ/0ijИЙW \ Kˆ00 €Kˆ00€IШ00Iˆ00IШ00 €Iˆ00€Iˆ00€Iˆ00€Kˆ00 €Iˆ00 €Zб00š0€€L>jнwР^LQ Z Z Z ; T Z Xџ€№8№@ёџџџ€€€ї№’№№0№( № №№B №S №ПЫџ ?№џџЬІ| Дљ9ЭІ|tњ9ЏЏ\ ВВ\ V*€urn:schemas-microsoft-com:office:smarttags€place€http://www.5iantlavalamp.com/_*€urn:schemas-microsoft-com:office:smarttags€country-region€http://www.5iantlavalamp.com/ Ф3 U \ 0ту V \ 33&&|Ћ6BЉяћŒ‘™š›­ЎЏ&(9@Tjq­ЎПопрії|\  U \ ˜zЂFЉ ТOЉ ТO˜gfoˆN$i˜gfoˆN$iЕ(€˜zЂFхУ~Њ)@'=#?P RљcZŽjЙ*n#>~)KK^•ўxЊ7­ыpАxDХжNЦ+з„+ьД3іџ@€\[Z `@џџUnknownџџџџџџџџџџџџGя:рAxР џTimes New Roman5€Symbol3& џ:рCxР џArial"1ˆ№аhXв&Xв&eѕeѕ!№ ДД4dV V 2ƒQ№HP №џ?фџџџџџџџџџџџџџџџџџџџџџжNЦ2џџWHY CHANGE THE NAME Jackie ImrieSueўџр…ŸђљOhЋ‘+'Гй0|˜ДРиф№  8 D P\dltфWHY CHANGE THE NAMEJackie ImrieNormalSue2Microsoft Office Word@@'‚9…Щ@'‚9…ЩeѕўџеЭеœ.“—+,љЎDеЭеœ.“—+,љЎl( hpЈАИР Шаир ш ф0Central Mcr & Mcr Childrens Univ Hosp NHS TrustV ц WHY CHANGE THE NAME TitleР 8@ _PID_HLINKSфAx= mailto:Jackie.imrie@ccmc.nhs.ukC ўџџџўџџџўџџџ !"#$%&ўџџџ()*+,-.ўџџџ§џџџ1ўџџџўџџџўџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџRoot Entryџџџџџџџџ РF€я“9…Щ3€Data џџџџџџџџџџџџ1TableџџџџWordDocumentџџџџ.SummaryInformation(џџџџџџџџџџџџDocumentSummaryInformation8џџџџџџџџ'CompObjџџџџџџџџџџџџqџџџџџџџџџџџџўџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџџўџ џџџџ РFMicrosoft Office Word Document MSWordDocWord.Document.8є9Вq