I taksa hmun hrang hrangah thau a awm em? Lipodystrophy chungchang hi i zir dawn teh ang!

I taksa hmun thenkhatah thau tlakchham nghal, emaw, hmun dangah thau duh loh zawnga tihpun emaw i hmu ngai em, i tawng ngai em? Hei hi a chang chuan ngaihtuah phak loh a ni thei. Vawiin hian he dinhmun ngei hi kan sawi dawn a ni. Damdawi lam hawi chuan hei hi Lipodystrophy an ti a. Ngaihtuah buai suh, a hming chu mak tak maiin lang mahse, a awlsam zawngin sawi ila.

Lipodystrophy tih hi eng nge ni?

A awlsam zawngin, Lipodystrophy chu thau tissue, hmun thenkhata "thau" kan tih tlangpui, emaw, taksa hmun hrang hranga thau awm dan pangngai lo emaw a bo vek emaw, a then emaw a ni.

I taksa hmun thenkhat, i kut leh keah i thau a tlahniam a, i hmai leh kawr ang chi hmun thenkhatah chuan thau tam lutuk i tipung tih han ngaihtuah teh. Hei hi Lipodystrophy-ah hian a thleng thin. Hetiang dinhmun hi chi hrang hrang a awm a, a then chu genetic (i.e., kan pianpui thil) a ni a, thenkhat erawh chu a hnuah damdawi lam thil dang avanga lo awm a ni thung. A chi tin hian mi a nghawng dan a inang lo va, symptom pawh a inang lo.

Engvangin nge thau (adipose tissue) hi kan taksa tan a pawimawh em em?

Tunah chuan, "I thau tlem poh leh a tha zawk" tih i ngaihtuah mai thei. Mahse a nihna takah chuan adipose tissue hian kan taksaah hna pawimawh tak tak a thawk a ni. En teh:

• Energy storage: Heng thau tissue-ah hian kan ei leh in atanga kan calorie extra kan hmuh te hi energy angin a awm thin.
• Venhimna pe: Kan taksa peng hrang hrangte chu cushion ang maia venhimna a pe a.
• In lum vawn reng: Taksa lumna a vawng reng a, boruak lum lakah min venghim bawk.
• Hormone release: Hormone pawimawh engemaw zat, Leptin ang chi te hi siam chhuakin thisenah a chhuak thin.
• Inflammation controlling: Taksa chhunga inflammation control turin a pui bawk.

Chuvangin, he thau tissue hi lipodystrophy avanga a bo emaw, a awm dan pangngai lo taka a awm chuan kan hmel a danglam mai bakah taksaa metabolic function pawimawh tak tak te pawh a tibuai bawk. Lipodystrophy vei tam tak chu natna chi hrang hrang, zunthlum vei leh thisen cholesterol level dik lo nei thei an ni.

Lipodystrophy chi hrang hrang a awm em?

Ni e, Lipodystrophy hi chi hnih ah then theih a ni a, chungte chu genetic leh acquired te an ni.

Genetic Lipodystrophy chi hrang hrang

Chungte chu inherited, chu chu genetic mutations avanga lo awm chi hrang hrang an ni.

• Congenital Generalized Lipodystrophy (CGL): Hei hi Berardinelli-Seip natna a ni.Tih pawh a ni. Hei hi thil awm lo tak a ni. Hetah hian taksa thau chu a bo vek emaw, a tam zawk emaw a bo thin. Hei hi pian tirh atanga awm thin genetic defect avanga lo awm a ni. Hetiang dinhmun hi a dam kum khatnaah hriat theih a ni tlangpui.
• Familial Partial Lipodystrophy (FPLD): Hei hi genetic condition a ni bawk. Mahse, nakin lawkah hriatchhuah a ni fo. Hetiang dinhmunah hian naupang hian a ke leh kut ah thau a hloh ber a, a hmai leh a kawngah thau a khawlkhawm tam zawk bawk.

Acquired Lipodystrophy chi hrang hrang

Heng chi hrang hrangte hi kan pian hnu leh kan dam chhungin chhan hrang hrang avanga lo chhuak chi hrang hrang an ni.

• Acquired Generalized Lipodystrophy (AGL): Lawrence syndrome tia hriat bawk, ​​he natna hian thau a ti tlahniam a, a tlangpuiin hmai, kawr, kut leh keah thau a ti tlahniam thin. Hetiang thau tlahniam hi a thleng thuai thei a, kar tlemte chhungin a thleng thei a, thla emaw kum tam tak chhungin zawi zawiin a thleng thei bawk. Naupan lai emaw, tleirawl lai emaw atanga intan a ni tlangpui a, mahse kum eng pawhah a thleng thei. AGL hi a chhan awm thei engemaw zat a awm.
• Acquired Partial Lipodystrophy (APL): Barraquer-Simons syndrome tia hriat bawk, ​​he natna hian naupan laiin hmai, kawr, kut leh rilru atanga thau a hloh zauh zauh thin. Mi thenkhat chuan pum, ke, leh hnute ang chi hmunah thau tam lutuk an nei thei bawk. APL hi autoimmune conditions nen a inzawm fo thin .
• Highly active antiretroviral therapy (HAART)-induced lipodystrophy (LD-HIV): Hetiang lipodystrophy hi human immunodeficiency virus (HIV) vei te hian HIV-1 protease inhibitor te pawh telna highly active antiretroviral therapy (HAART) an dawn hnuah a thleng thin Hetiang dinhmun lo awm dan hi enkawlna nasat dan leh hun rei zawngah a innghat a ni. LD-HIV vei te hian an kut, ke leh hmai atang hian thau a tlahniam zauh zauh fo thin. Mi thenkhat chuan an hmai, kawr, hnungzang chunglam leh kawr velah thau tam lutuk an nei thei bawk.
• Localized Lipodystrophy: Hei hi taksa hmun tlemte chauh a thau a bo hian a ni. Entirnan, damdawi (insulin ang chi) chu hmun khata inject nawn fo a nih chuan he natna hi a awm thei a ni. Dimple te tak te ang mai a ni a, mahse a chunga vun hi a nghawng lo tlangpui.

Lipodystrophy leh Lipoatrophy hi thil inang an ni em?

Lipodystrophy hi thau insem darh dan pangngai lo tihna a ni. Lipoatrophy , thau hloh tihna nen pawh a inzawm bawk. Scientist leh doctor thenkhat chuan he thumal pahnih hi natna inang sawina atan an hmang thin. A awlsam zawngin sawi ila, Lipoatrophy hi Lipodystrophy subset a ni.

He natna hian tute nge a nghawng ber? Engtiang chiahin nge a hluar?

Lipodystrophy chi tam zawk hi naupan lai atanga tan a ni a, mahse acquired type hi puitlingah pawh a lo awm thei bawk.

HAART-induced lipodystrophy (LD-HIV) tih loh chu acquired lipodystrophy chi dang hian hmeichhia a nghawng nasa zawk. HIV hrik kai hi mipaah ​​a tam zawk avangin LD-HIV hi mipaah ​​pawh a tam zawk.

A tlangpuiin lipodystrophy hi a tlem hle . Mahse, damdawi thenkhat side effect avang hian acquired (lifelong) forms of lipodystrophy hi a tam chho zel a ni.

Engtin nge lipodystrophy hian ka taksa a nghawng?

Lipodystrophy chi tin hian taksa a nghawng dan a inang lo hle a, mahse thil pawimawh pahnih a awm a, chungte chu: adipose tissue hloh leh hormone leptin tlakchhamna te hi a ni.

Taksa chunga adipose tissue hloh hian nghawng a neih dan

Kan thau tissue hi adipocytes an tih cell atanga siam a ni . Adipocyte cell khatah hian lipid droplet 90% vel a awm a. Heng adipocyte te hian thau (triglycerides) an khawlkhawm thin. Heng thau cell te hi lipodystrophy avanga an chhiat chuan thau an khawlkhawm tha thei lo.

Lipodystrophy vei thenkhatah chuan he thau hloh hi kan taksa tissue dang, entirnan, liver, pancreas emaw muscle tissue-ah te a awm dik lo. Hei hian hriselna lama harsatna a thlen thei a, chungte chu:

• Thin thau natna / hepatic steatosis a awm
• Insulin resistance leh thisena insulin level sang (hyperinsulinemia) .
• Zunthlum (Diabetes Mellitus) natna a ni.
• Thisen a triglycerides level sang (hypertriglyceridemia) .
• Pancreatitis natna a ni
• Metabolic natna (Metabolic syndrome) a ni
• Coronary artery natna a ni

Mahse, lipodystrophy vei zawng zawng hian heng natna hi an nei vek dawn lo tih hre reng ang che. Ṭhenkhat chuan a chi hrang hrang (milder forms) an nei thei a, ṭhenkhat erawh chuan a nasat zawk pawh an nei thei thung.

Leptin tlakchhamnain Taksa a nghawng dan

Lipodystrophy vei te hian thau tissue an hloh ang bawkin hormone thenkhat an hloh bawk a, a bik takin hormone leptin .

Leptin hi kan thau tissue atanga hormone siam a ni. Hun rei tak chhung chu taksa rihna pangngai kan neih theih nan min pui thin. Engtin nge tih i hria em? Kan chawmna leh kan riltam thununna hmangin. Scientist te chuan leptin hi an la zirchiang mek a ni. Leptin hian kan metabolism , endocrine system hnathawh, leh immune system hnathawh a nghawng niin an ngai.

Lipodystrophy vei te hian leptin level a tlahniam thei a, hei hian riltam lutuk, insulin resistance leh hriselna lama harsatna dang a thlen thei a ni.

Lipodystrophy natna lan chhuah dan chu engte nge ni?

Lipodystrophy chi hrang hrang a awm avangin a natna lan chhuah dan pawh a inang lo hle.

A lan chhuah dan tlangpui chu taksa hmun thenkhata thau tlahniam chiang tak leh awm reng a ni a, chutih rual chuan taksa hmun dangah thau awm dan pangngai lo emaw, duh loh zawng emaw a awm bawk.

Entirnan, acquired partial lipodystrophy (APL) vei te hian an naupan laiin an hmai, kawr, kut leh rilruah thau an hloh zauh zauh thin. APL vei thenkhat chuan an pum, ke leh hnungzang ang chiah hian thau tam lutuk an nei thei bawk.

Lipodystrophy i neih chi leh i kum a zirin kum tam tak chhung chu hriselna lama harsatna lian tham i hmu lo mai thei. Mahse, lipodystrophy chi tam tak hian thisen cholesterol, triglycerides, leh sugar (glucose) level a tisang a ni. I doctor chuan hengte hi thisen test pangngai hmangin a hmu thei a, chu chu lipid panel leh basic metabolic panel te hi a ni.

Lipodystrophy chi thenkhat, a bik takin partial lipodystrophy hi hriat a harsa thei hle. Chuvangin, nangmah emaw, i fain emaw natna lanchhuahna thar i neih emaw, natna lan chhuah tawh pakhat a zual emaw a nih chuan, a chhan eng pawh ni se, doctor pan a pawimawh hle.

Lipodystrophy hi engte nge ni?

Lipodystrophy awm chhan hi genetic emaw acquired emaw a zirin a inang lo.

Genetic Lipodystrophy awm chhan te

Lipodystrophy hi genetic forms – chu chu congenital generalized lipodystrophy (CGL) leh familial partial lipodystrophy (FPLD) te hi a ni a, chu chu genetic mutations thenkhat avanga lo awm a ni

Genetic mutation chu kan DNA sequence inthlak danglamna a ni. Kan DNA sequence hi kan cell te hnathawh tur atana an mamawh information pe tu a ni. Chuvangin, he DNA sequence thenkhat hi a kim lo emaw, a chhiat emaw chuan genetic disease symptoms kan nei thei a ni.

• Congenital generalized lipodystrophy (CGL) awm chhan: CGL type 1 atanga 4 hi genes AGPAT2, BSCL2, CAV1 , leh CAVIN1 -a mutations avanga lo awm a ni. Heng genes te hi kan thau tissue-a thau dahkhawmna cell (adipocytes) te thanlenna leh hnathawhna atan a pawimawh hle. Heng genes-a mutations hian adipocytes structure leh function a nghawng a ni. CGL vei naupang chuan heng gene mutations te hi an nu leh pa hnen atangin a rochun thin. Nu leh pa pahnih hian mutated gene copy khat nei mah se, a tlangpuiin symptom an lantir lo.
• Familial Partial Lipodystrophy (FPLD) awm chhan): Gene engemaw zat a mutation awm hian FPLD a thlen thei a ni. A tam ber chu LMNA a ni.LMNA an tih gene pakhata mutation a awm. FPLD-a inrawlh LMNA gene leh gene dangte hian adipocyte te chu thau dahkhawmna tur leh thau dahkhawmna atana pawimawh protein hrang hrang siam turin a hrilh a ni. Heng genes zinga pakhatah hian mutation a awm chuan adipocytes te thanlenna, an insiam dan leh an hnathawh dan a nghawng tha lo hle. FPLD vei tam zawk hi autosomal dominant pattern a inherit a ni . Chumi awmzia chu cell tinah mutated gene copy khat neih hi natna thlentu atan a tawk tihna a ni. A châng chuan FPLD vei chuan an nu leh pa vei zinga pakhat aṭangin mutation hi a rochun ṭhin. A dang erawh chu gene-a mutation thar, random mutations avanga lo awm an ni a, chhungkaw zingah tumahin he natna hi an la nei lo a nih pawhin an lo awm thei a ni.

Acquired Lipodystrophy awm chhan te

Acquired lipodystrophy hi damdawi, autoimmune reaction , emaw idiopathic cause vang emaw a ni thei. Acquired lipodystrophy hi direct genetic basis awm lo mah se, zirchiangtu thenkhat chuan mi thenkhat chuan acquired lipodystrophy vei theihna genetic predisposition an nei thei niin an ngai.

• Acquired Generalized Lipodystrophy (AGL) awm chhan: AGL hi natna kai hnuah emaw, taksa hriselna natna hnuah emaw a awm thei. AGL nena inzawm infection te chu:
• Vawk natna (Chickenpox / varicella) natna hrik natna (Chickenpox / varicella) .
• Measles natna a ni
• Khuh natna (pertussis) .
• Diphtheria natna a awm
• Pneumonia natna (Pneumonia) a ni
• Osteomyelitis natna a ni
• A rilru a buai em em a, a rilru a hah em em bawk a.

Heng natna hrik i neih vang mai mai hian AGL i vei dawn tih chu a chiang lo.

AGL nena inzawm autoimmune condition hrang hrangte chu:

• Autoimmune thyroiditis natna a ni
• Autoimmune hepatitis natna a ni
• Naupangte tan vun natna (dermatomyositis) a awm
• Rheumatoid arthritis natna a ni
• Sjogren-a natna (syndrome) a ni
• Autoimmune hemolytic thisen tlakchhamna (anemia) a awm

Heng immune system natna te hi i neih chuan AGL i nei ngei ang tih theih a ni lo.

A tam zawkah chuan AGL awm chhan hi hriat theih a ni lo (idiopathic).

• Acquired Partial Lipodystrophy (APL) awm chhan): Scientist-te chuan APL hi kan immune system-in thau cell a tihchhiat sual vang niin an ngai. APL vei zinga 80% aia tam chuan an thisenah Complement 3 an nei a .Protein pakhat complement factor an tih hi a hniam hle. He protein hi a tlangpuiin kan immune system chhannaah a inrawlh thin. APL vei te hian an thisenah autoantibody complement 3-nephritic factor an tih chu an nei fo thin. Autoantibody hi immune system-a protein awm, tihsualin tissue hrisel tak tak a bei a, a tichhia a ni.

• Highly active antiretroviral therapy (HAART)-induced lipodystrophy (LD-HIV) awm chhan): HIV-1 protease inhibitor awmna HAART hian lipodystrophy a thlen chhan hi Scientist-te chuan an la chiang lo hle.

Thu lawmawm tak chu tuna HIV enkawl tan tawh tam zawk chuan lipodystrophy chungchangah an buai lutuk a ngai tawh lo a, HIV damdawi thar zawkte hian he natna hi a thlen tam loh phah a ni.

Engtin nge lipodystrophy i vei tih i hriat theih ang?

Lipodystrophy symptoms i neih chuan i doctor chuan physical exam a ti ang a, i medical history leh family medical history te a zawt ang a, chutah chuan test thenkhat order turin a diagnosis tih finfiah tur leh i symptoms awm chhan dang a awm leh awm loh en turin a order ang.

Lipodystrophy hriat nan eng test nge hman thin?

I doctor chuan lipodystrophy hriat theihna tur leh/ emaw, i natna lan chhuah chhan dang a awm leh awm loh enfiah nan test engemaw zat a ti thei ang. Chung zingah chuan:

• Full-body MRI (Magnetic Resonance Imaging) scan: MRI hian magnet lian tak, radio wave leh computer hmangin i taksa chhunga taksa peng leh thil dangte chipchiar taka thlalak a siam a. I doctor chuan i taksaa thau awm dan leh a insem dan tehna atan he test hi a ti thei ang.
• Comprehensive Metabolic Panel: Hei hi thisen test a ni a, i thisen chhunga thil chi hrang hrang 14 tehna a ni. I taksa chemical balance leh metabolism chungchang thu pawimawh tak tak a pe a ni. Doctor chuan i thisen sugar (glucose) level leh liver enzymes te chu a bik takin a en ang .
• Lipid Panel: Hei hi thisen test a ni bawk. I thisena thau molecule lipids an tih zat a teh thin. He test-ah hian cholesterol tehna pali leh triglyceride tehna a tel tlangpui. Lipodystrophy hian cholesterol level dik lo a thlen fo thin.
• Leptin Test: Hei hian i thisena leptin awm zat a teh thin. Lipodystrophy i neih a, i leptin level chu a tlangpui aia hniam a nih chuan hei hian i taksain leptin replacement therapy a dawn dan tur ngaihtuahna engemaw zat a pe thei che a ni.
• Genetic Testing: I doctor-in Lipodystrophy inherited (genetic) form i neih a rinhlelh chuan, i natna hriat chian nan genetic testing neih a rawt thei ang.
• Kidney biopsy atanga lak chhuah:Hei hian kal tissue sample surgery hmanga lakchhuah a, microscope hmanga enfiah a ni. I doctor chuan i kal (kidney) te chu lipodystrophy in a nghawng em tih hriat nan he test hi a ti thei ang.

Lipodystrophy hi eng enkawlna nge ni?

Lipodystrophy enkawl dan leh enkawl dan chu i chi bik i neih danah a innghat a, a kaihhnawih natna dang, zunthlum emaw, cholesterol level dik lo emaw i neih leh neih lohah a innghat bawk.

Lipodystrophy enkawlna atana hman tlanglawn tak takte chu:

• Leptin thlakna damdawi: I doctor chuan hormone leptin siam chhuah, metreleptin tih chu a pe thei ang. Lipodystrophy vei te hian leptin an tlachham fo thin. He hormone hian taksa metabolism a tidanglam thei a, cholesterol sang leh triglyceride level pawh a tihniam thei bawk.
• Diabetes leh insulin resistance enkawlna: Insulin resistance leh/ emaw lipodystrophy avanga zunthlum i neih chuan i doctor chuan a enkawl nan pioglitazone, metformin, sulfonylureas , emaw thiazolidinediones ang chi damdawi ei tur a pe thei ang. Lipodystrophy vei thenkhat chuan an zunthlum enkawlna atan synthetic insulin an ei a ngai thei bawk. Tin , in lamah glucometer hmangin i thisen sugar level i enfiah fo a ngai mai thei bawk .
• Triglyceride leh cholesterol level control: A hmaa kan sawi tawh ang khan zunthlum enkawlna atana pui thei damdawi ei te hian cholesterol level control-na kawngah a pui fo thin. I doctor chuan statin , rosuvastatin leh pravastatin te pawh a pe thei bawk . Triglyceride level sang tak nei te chuan fibric acid derivatives emaw n-3 polyunsaturated fatty acids , sangha hriak a awm ang chi supplement an ei a ngai mai thei.
• Cosmetic surgery leh tih dan: Cosmetically sensitive area, hmai, rilru leh genital area-a lipodystrophy vei te chuan an hmel leh mahni inrintawkna tihchangtlun nan cosmetic surgery an nei thei a ni. Plastic surgeon-te chuan autologous adipose tissue transplantation, free flap hmanga hmai siam thar leh silicone emaw implant dang emaw an ti thei bawk. Tin, liposuction emaw surgical removal technique hmangin an hmui emaw, an hnunglam emaw ( "buffalo hump" tia koh fo) ang chi hmuna thau duh loh awmte chu an paih chhuak thei bawk.

Scientist te hian lipodystrophy enkawl dan leh enkawl dan tur zir tur tam tak an la nei a ni. Metabolic process-a thil harsa tak takte an hmuhchhuah belh zel chuan enkawlna kawng tam zawk a awm thei ang.

Lipodystrophy hi a veng thei ang em?

A tam zawkah chuan Lipodystrophy hi ven theih a ni lo.

Genetic forms of lipodystrophy hi ven theih a ni lo, inherited gene mutation atanga lo chhuak a nih avangin. Fa neih i tum a nih chuan genetic condition nei fa i neih theihna tur hlauhawmzia hrethiam la, i doctor nen genetic testing chungchang sawipui rawh.

Acquired lipodystrophy hi infection emaw immune system natna emaw avanga lo awm a ni fo thin. Infection thenkhat, chickenpox leh whooping cough te chu vaccine hmanga ven theih a nih laiin, infection dang leh immune system natna, acquired lipodystrophy nena inzawm te chu ven theih a ni lo.

Lipodystrophy hi eng prognosis nge ni?

Lipodystrophy thlirna chu eng ang lipodystrophy nge i neih tih leh a kaihhnawih natna dang emaw, harsatna dang emaw, zunthlum, liver emaw kidney lama harsatna i neih leh neih lohah a innghat nasa hle.

Thau tlem leh thau tamna hmun cosmetic correction hian lipodystrophy vei te nunphung a tisang a ni.

Nangmah emaw i fa emaw chu lipodystrophy vei nia hriat a nih chuan i doctor nen eng nge i beisei tur tih leh a natna hi engtin nge i enkawl tha ber ang tih sawipui rawh.

Lipodystrophy chungchangah engtikah nge doctor i hmuh ang?

Nangmah emaw, i fate taksa thau chu hmun bikah a tlahniam chhunzawm zel tih i hriat chuan, emaw, hmun thenkhatah thau a pung tih i hriat chuan i doctor nen inbia ang che.

Nangmah emaw i fa emaw Lipodystrophy vei nia hriat i nih chuan i enkawlnain i tan a tha em tih enfiah turin i medical team te nen in inhmu fo tur a ni.

A tawp berah chuan thil hriat reng tur

Lipodystrophy hi natna awm lo mahse na tak a ni. Damdawi a awm loh laiin, enkawlna thenkhat chuan a natna lan chhuah dan leh a kaihhnawih natna, zunthlum leh cholesterol level pangngai lo te chu a enkawl thei a ni.

Mi hrang hrangah enkawl dan a inang lo hle. Chuvangin i medical team te chuan i tana tha ber tur ruahmanna siam turin an thawk ho ang che. Doctor zawhna zawh pawh hlau suh. I tanpui turin an awm reng a ni. He hriatna hian nun hrisel tak i neih theih nan a pui ngei ang che!

` lipodystrophy, adipose tissue, leptin, genetic, acquired, zunthlum, cholesterol, triglyceride, HIV, metabolic natna te