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Ke ʻike nei ʻoe i nā waihona momona ma nā ʻāpana like ʻole o kou kino? E aʻo kākou e pili ana i ka Lipodystrophy!

Ke ʻike nei ʻoe i nā waihona momona ma nā ʻāpana like ʻole o kou kino? E aʻo kākou e pili ana i ka Lipodystrophy!

Ua ʻike anei ʻoe, a i ʻole ua ʻike paha, i ka nalowale koke ʻana o ka momona ma kekahi mau wahi o kou kino, a i ʻole ka piʻi ʻana o ka momona i makemake ʻole ʻia ma nā wahi ʻē aʻe? Hiki ke noʻonoʻo ʻole ʻia kēia i kekahi manawa. I kēia lā e kamaʻilio mākou e pili ana i kēia ʻano. Ma nā ʻōlelo lapaʻau, ua kapa ʻia kēia ʻo Lipodystrophy . Mai hopohopo, ʻoiai paha he mea ʻē ke kani ʻana o ka inoa, e kamaʻilio maʻalahi kākou e pili ana.

He aha ka Lipodystrophy?

I ka ʻōlelo maʻalahi, ʻo Lipodystrophy ka nalowale piha a hapa paha o ka ʻiʻo adipose, ka mea a mākou e kapa pinepine ai he "momona," ma kekahi mau wahi, a i ʻole ka hōʻiliʻili ʻana o ka momona ma nā wahi like ʻole o ke kino.

E noʻonoʻo ʻoe e emi ana kou kaumaha ma kekahi mau wahi o kou kino, e like me kou mau lima a me kou mau wāwae, a e loaʻa ana ka momona keu ma nā wahi e like me kou maka a me kou ʻāʻī. ʻO kēia ka mea e hana ʻia ma Lipodystrophy. Aia nā ʻano like ʻole o kēia ʻano, ʻo kekahi o ia mau mea he genetic (ʻo ia hoʻi, kahi mea i hānau ʻia ai mākou), aʻo kekahi e ulu aʻe ma hope ma muli o nā kūlana olakino ʻē aʻe. Hoʻopilikia kēlā me kēia ʻano i nā kānaka me ke ʻano ʻokoʻa a he ʻokoʻa nā hōʻailona.

No ke aha he mea nui ka momona (ʻiʻo adipose) i ko mākou kino?

I kēia manawa ke noʻonoʻo nei paha ʻoe, "ʻO ka liʻiliʻi o kou momona, ʻoi aku ka maikaʻi." Akā ʻo ka ʻoiaʻiʻo, hana ka ʻiʻo adipose i nā hana koʻikoʻi he nui i loko o ko mākou kino. E nānā:

  • Ka mālama ʻana i ka ikehu: Aia i loko o kēia mau ʻiʻo momona e mālama ʻia ai nā calorie keu a mākou e loaʻa ai mai ka meaʻai a mākou e ʻai ai i ikehu.
  • Ke hāʻawi nei i ka palekana: Hāʻawi ia i ka pale e like me ka uluna i nā ʻano kino like ʻole i loko o ko mākou kino.
  • Ka mālama ʻana i ka mehana: Mālama i ka wela o ke kino a pale iā mākou mai ke anu.
  • Hoʻokuʻu ʻia ka hormone: Hoʻopuka ʻia a hoʻokuʻu ʻia kekahi mau hormones koʻikoʻi, e like me Leptin, i loko o ke kahe koko.
  • Ke kāohi ʻana i ka mumū: Kōkua pū ia i ka kaohi ʻana i ka mumū i loko o ke kino.

No laila, ke nalowale a waiho ʻia paha kēia ʻiʻo momona ma muli o ka lipodystrophy, ʻaʻole wale ko mākou helehelena e loli ai, akā ua hoʻopilikia ʻia nā hana metabolic koʻikoʻi i loko o ke kino. Nui ka poʻe me ka lipodystrophy e pilikia ana i ka loaʻa ʻana o nā maʻi e like me ka maʻi diabetes mellitus a me ka loaʻa ʻana o nā pae cholesterol koko maʻamau ʻole.

Aia kekahi mau ʻano o ka lipodystrophy?

ʻAe, hiki ke hoʻokaʻawale ʻia ka lipodystrophy i ʻelua mau ʻano nui: genetic a loaʻa.

Nā ʻano o ka Lipodystrophy Genetic

He mau mea hoʻoilina kēia, ʻo ia hoʻi, nā ʻano i kū mai ma muli o nā hoʻololi genetic.

  • ʻO ka Lipodystrophy Laulā Congenital (CGL): ʻO kēia ka maʻi Berardinelli-Seip.ʻIke ʻia hoʻi ʻo. He kūlana kakaikahi loa kēia. Ma kēia, ua aneane nalowale loa a hapa nui paha ka momona o ke kino. Hoʻokumu ʻia kēia e kahi kīnā genetic i loaʻa i ka wā hānau. Hiki ke ʻike pinepine ʻia kēia kūlana i loko o ka makahiki mua o ke ola.
  • ʻO ka Familial Partial Lipodystrophy (FPLD): He maʻi genetic nō hoʻi kēia. Eia nō naʻe, ʻike pinepine ʻia ma hope o ke ola. I kēia kūlana, nalowale ka momona o ke keiki ma nā wāwae a me nā lima, a hōʻiliʻili i ka momona hou aʻe ma ka maka a me ka ʻāʻī.

Nā ʻano o ka Lipodystrophy i loaʻa

ʻO kēia nā ʻano e kū mai ana ma hope o ko kākou hānau ʻana a i ko kākou wā e ola ana no nā kumu like ʻole.

  • ʻO ka Lipodystrophy Laulā i Loaʻa (AGL): ʻIke ʻia hoʻi ʻo Lawrence syndrome , ʻo kēia ʻano ke kumu o ka pohō momona, ma ka maka, ka ʻāʻī, nā lima, a me nā wāwae. Hiki ke hana koke ʻia kēia pohō momona, i loko o kekahi mau pule, a lohi paha i nā mahina a i ʻole nā ​​​​makahiki. Hoʻomaka pinepine ia i ka wā kamaliʻi a i ʻole ka wā ʻōpio, akā hiki ke hana i kēlā me kēia makahiki. Nui nā kumu hiki o AGL.
  • ʻO ka Lipodystrophy hapa i loaʻa (APL): ʻIke ʻia hoʻi ʻo Barraquer-Simons syndrome , ʻo kēia kūlana ke kumu o ka nalowale mālie o ka momona mai ka maka, ka ʻāʻī, nā lima, a me ka umauma i ka wā kamaliʻi. Loaʻa paha i kekahi poʻe ka momona keu ma nā wahi e like me ka ʻōpū, nā wāwae, a me nā puʻupuʻu. Hoʻopili pinepine ʻia ka APL me nā kūlana autoimmune .
  • ʻO ka lipodystrophy i hoʻokomo ʻia e ka lāʻau antiretroviral ikaika loa (HAART) (LD-HIV): Loaʻa kēia ʻano lipodystrophy i nā poʻe i loaʻa i ka maʻi immunodeficiency kanaka (HIV) ma hope o ko lākou loaʻa ʻana i ka lāʻau antiretroviral ikaika loa (HAART) e komo pū ana me nā mea hoʻopaʻa protease HIV-1 . ʻO ka hanana o kēia ʻano maʻi e pili ana i ka ikaika a me ka lōʻihi o ka lāʻau. ʻIke pinepine ka poʻe me LD-HIV i ka nalowale mālie o ka momona mai ko lākou mau lima, nā wāwae, a me ka maka. Hiki i kekahi poʻe ke loaʻa ka momona keu a puni ko lākou maka, ʻāʻī, kua o luna, a me ka pūhaka.
  • ʻO ka Lipodystrophy kūloko: ʻO kēia ka wā e nalowale ai ka momona ma kahi liʻiliʻi o ke kino. No ka laʻana, hiki ke loaʻa kēia ʻano ke hoʻokomo pinepine ʻia nā lāʻau lapaʻau (e like me ka insulin) i loko o ka wahi like. Me he dimple liʻiliʻi lā, akā ʻaʻole i hoʻopilikia ʻia ka ʻili e uhi ana.

He like anei ka lipodystrophy a me ka lipoatrophy?

ʻO ka Lipodystrophy kahi huaʻōlelo maʻamau no ka hoʻolaha ʻano ʻole o ka momona. Pili pū ia me Lipoatrophy , ʻo ia hoʻi ka nalowale o ka momona. Hoʻohana kekahi poʻe ʻepekema a me nā kauka i nā huaʻōlelo ʻelua e wehewehe i ke kūlana like. I ka ʻōlelo maʻalahi, ʻo Lipoatrophy kahi ʻāpana o Lipodystrophy.

ʻO wai ka mea i hoʻopilikia nui ʻia e kēia maʻi? Pehea ka maʻamau o ia mea?

Hoʻomaka ka hapa nui o nā ʻano lipodystrophy i ka wā kamaliʻi, akā hiki ke ulu pū nā ʻano i loaʻa i nā pākeke.

Me ka ʻokoʻa o ka lipodystrophy i hoʻokomo ʻia e HAART (LD-HIV) , ʻoi aku ka nui o nā ʻano ʻē aʻe o ka lipodystrophy i loaʻa i nā wahine. Ma muli o ka maʻi HIV i nā kāne, ʻoi aku ka nui o ka LD-HIV i nā kāne.

Ma keʻano laulā, he mea kākaʻikahi loa ka lipodystrophy. Eia nō naʻe, ke lilo nei nā ʻano lipodystrophy i loaʻa (no ke ola holoʻokoʻa) i mea maʻamau ma muli o nā hopena ʻaoʻao o kekahi mau lāʻau lapaʻau.

Pehea ka hopena o ka lipodystrophy i koʻu kino?

Hoʻopilikia kēlā me kēia ʻano lipodystrophy i ke kino ma ke ʻano ʻokoʻa, akā aia ʻelua mau kumu nui i maʻamau: ka nalowale ʻana o ka ʻiʻo adipose a me ka nele o ka hormone leptin .

Ka hopena o ka nalowale ʻana o ka ʻiʻo adipose ma ke kino

Hana ʻia ko kākou ʻiʻo momona e nā pūnaewele i kapa ʻia he adipocytes . Loaʻa i kēlā me kēia pūnaewele adipocyte ma kahi o 90% o nā kulu lipid. Mālama kēia mau adipocytes i ka momona (triglycerides). Ke hōʻino ʻia kēia mau pūnaewele momona ma muli o ka lipodystrophy, ʻaʻole hiki iā lākou ke mālama pono i ka momona.

I kekahi mau hihia o Lipodystrophy, ua waiho hewa ʻia kēia momona nalowale i loko o nā ʻiʻo ʻē aʻe o ko mākou kino, no ka laʻana, i loko o ke akepaʻa, ka pancreas, a i ʻole nā ​​ʻiʻo ʻiʻo. Hiki i kēia ke alakaʻi i nā pilikia olakino e like me:

  • Maʻi akepaʻa momona / steatosis hepatic
  • Ke kū'ē'ē o ka insulin a me ke ki'eki'e o ka insulin i loko o ke koko (hyperinsulinemia)
  • Maʻi diabetes (Diabetes Mellitus)
  • Hoʻonui ʻia nā pae triglycerides i loko o ke koko (hypertriglyceridemia)
  • ʻO ka maʻi pancreatitis
  • ʻO ka maʻi metabolic
  • Maʻi ʻaʻa coronary

Akā e hoʻomanaʻo, ʻaʻole nā ​​​​kānaka āpau me ka lipodystrophy e loaʻa i kēia mau kūlana. Loaʻa paha i kekahi nā ʻano palupalu, aʻo kekahi paha he ʻoi aku ka koʻikoʻi.

Nā hopena o ka hemahema o ka Leptin ma ke kino

I ka nalowale ʻana o ka momona o ka poʻe me ka lipodystrophy, nalowale pū kekahi mau hormones, ʻoiai ka hormone leptin .

He hormone ka Leptin i hana ʻia e ko kākou ʻiʻo momona. Kōkua ia iā kākou e mālama i ke kaumaha o ke kino maʻamau i ka wā lōʻihi. Maopopo iā ʻoe pehea? Ma ka hoʻomāʻona ʻana iā kākou a me ka kaohi ʻana i ko kākou pōloli. Ke noiʻi nei nā kānaka ʻepekema i ka leptin. Manaʻo lākou e pili ana ka leptin i kā kākou metabolism , ka hana o ka ʻōnaehana endocrine , a me ka hana o ka ʻōnaehana pale .

Hiki i ka poʻe me ka lipodystrophy ke ʻike i ka emi ʻana o ka pae leptin, hiki ke alakaʻi i ka pōloli nui, ke kū'ē ʻana o ka insulin, a me nā pilikia olakino ʻē aʻe.

He aha nā hōʻailona o ka lipodystrophy?

Ma muli o ka nui o nā ʻano lipodystrophy, ʻokoʻa loa nā hōʻailona.

ʻO ka hōʻailona maʻamau ka pohō maopopo a mau o ka momona ma kekahi mau wahi o ke kino, a i ka manawa like, kahi hōʻiliʻili ʻana o ka momona maʻamau ʻole a makemake ʻole ʻia paha ma nā wahi ʻē aʻe o ke kino.

Eia kekahi laʻana, ʻo ka poʻe me ka lipodystrophy hapa i loaʻa (APL) e emi mālie ka momona ma ko lākou helehelena, ʻāʻī, lima, a me ka umauma i ka wā kamaliʻi. Hiki i kekahi poʻe me APL ke loaʻa ka momona keu ma nā wahi e like me ka ʻōpū, nā wāwae, a me nā puʻupuʻu.

Ma muli o ke ʻano o ka lipodystrophy āu e loaʻa ai a me kou mau makahiki, ʻaʻole paha ʻoe e ʻike i kekahi mau pilikia olakino nui no nā makahiki. Eia nō naʻe, nui nā ʻano lipodystrophy e hoʻonui ai i ka cholesterol koko, triglycerides, a me ke kō (glucose). Hiki i kāu kauka ke ʻike i kēia mau mea me nā hoʻāʻo koko maʻamau, e like me ka panel lipid a me kahi panel metabolic kumu .

Hiki ke paʻakikī ke ʻike i kekahi mau ʻano lipodystrophy, ʻoiai ka lipodystrophy hapa. No laila, he mea nui e ʻike i ke kauka inā loaʻa iā ʻoe a i ʻole kāu keiki nā hōʻailona hou a inā e ʻoi aku ka ʻino o kahi hōʻailona e kū nei, me ka nānā ʻole i ke kumu.

He aha nā kumu o ka lipodystrophy?

ʻOkoʻa nā kumu o ka lipodystrophy ma muli o ke ʻano genetic a i loaʻa paha.

Nā kumu o ka Lipodystrophy Genetic

ʻO nā ʻano genetic o ka lipodystrophy - ʻo ia hoʻi ka congenital generalized lipodystrophy (CGL) a me ka familial partial lipodystrophy (FPLD) - i hoʻokumu ʻia e kekahi mau mutations genetic .

ʻO ka hoʻololi ʻana o ka genetics kahi hoʻololi i kā mākou moʻo DNA. ʻO kā mākou moʻo DNA ka mea e hāʻawi i kā mākou mau cell i ka ʻike e pono ai lākou e hana i kā lākou mau hana. No laila, inā ʻaʻole piha a hōʻino ʻia paha kekahi hapa o kēia moʻo DNA, hiki iā mākou ke loaʻa nā hōʻailona o kahi maʻi genetic.

  • Nā kumu o ka lipodystrophy generalized congenital (CGL): ʻO nā ʻano CGL 1 a 4 i hoʻokumu ʻia e nā mutations i loko o nā genes AGPAT2, BSCL2, CAV1 , a me CAVIN1 . He mea nui kēia mau genes no ka ulu ʻana a me ka hana o nā cell mālama momona (adipocytes) i loko o kā mākou ʻiʻo momona. Hoʻopilikia nā mutations i loko o kēia mau genes i ke ʻano a me ka hana o nā adipocytes. Hoʻoilina ke keiki me CGL i kēia mau mutations gene mai ko lākou mau mākua. ʻOiai inā loaʻa i nā mākua ʻelua hoʻokahi kope o ka gene mutated, ʻaʻole lākou e hōʻike pinepine i nā hōʻailona.
  • Nā Kumu o ka Familial Partial Lipodystrophy (FPLD): Hiki i nā loli ʻana i loko o kekahi mau genes ke hana i ka FPLD. ʻO ka mea maʻamau ka LMNA.He hoʻololi ʻana i loko o kahi gene i kapa ʻia ʻo LMNA. ʻO ka gene LMNA a me nā gene ʻē aʻe i pili i ka FPLD e kuhikuhi i nā adipocytes e mālama i ka momona a hana i nā protein like ʻole e pono ai no ka mālama ʻana i ka momona. Inā loaʻa kahi hoʻololi ʻana i kekahi o kēia mau genes, ua hoʻopilikia maikaʻi ʻole ʻia ka ulu ʻana, ke ʻano, a i ʻole ka hana o nā adipocytes. Hoʻoilina ʻia ka hapa nui o nā hihia o FPLD ma ke ʻano autosomal dominant . ʻO ia hoʻi, ʻo ka loaʻa ʻana o hoʻokahi kope o ka gene i hoʻololi ʻia i loko o kēlā me kēia cell ua lawa ia e hoʻoulu ai i ka maʻi. I kekahi manawa, hoʻoilina kekahi kanaka me FPLD i ka hoʻololi ʻana mai kekahi o ko lākou mau mākua i hoʻopilikia ʻia. I nā hihia ʻē aʻe, ua hoʻokumu ʻia lākou e nā hoʻololi hou, random i loko o ka gene, a hiki ke hana ʻoiai inā ʻaʻohe mea o ka ʻohana i loaʻa i ka maʻi ma mua.

Nā kumu o ka Lipodystrophy i loaʻa

Hiki ke hana ʻia ka lipodystrophy i loaʻa e nā lāʻau lapaʻau, nā hopena autoimmune , a i ʻole nā ​​​​kumu idiopathic . ʻOiai ʻaʻohe kumu genetic pololei no ka lipodystrophy i loaʻa, manaʻoʻiʻo kekahi poʻe noiʻi he predisposition genetic paha kekahi poʻe i ka hoʻomohala ʻana i ka lipodystrophy i loaʻa.

  • Nā Kumu o ka Lipodystrophy Laulā i Loaʻa (AGL): Hiki ke loaʻa ka AGL ma hope o kahi maʻi a i ʻole ma hope o kahi maʻi o ka ʻōnaehana pale. ʻO nā maʻi i pili i ka AGL:
  • ʻO ka maʻi puʻupuʻu (Chickenpox / varicella)
  • Ka maʻi ahulau
  • ʻO ka ʻū ʻana (pertussis)
  • ʻO ka diphtheria
  • Pneumonia
  • ʻOsteomyelitis
  • ʻO ka Mononucleosis (Mononucleosis / mono)

ʻAʻole maopopo e loaʻa iā ʻoe ka AGL no ka mea wale nō he mau maʻi kēia iā ʻoe.

ʻO nā kūlana Autoimmune e pili ana me AGL:

  • ʻO ka thyroiditis autoimmune
  • ʻO ka maʻi hepatitis autoimmune
  • ʻO ka dermatomyositis ʻōpio
  • ʻO ka ʻāʻī rheumatoid
  • ʻO ka maʻi Sjogren
  • ʻO ka anemia hemolytic autoimmune

Inā loaʻa iā ʻoe kēia mau maʻi ʻōnaehana pale, ʻaʻole hiki ke ʻōlelo ʻia e loaʻa maoli iā ʻoe ʻo AGL.

I ka hapanui o nā hihia, ʻaʻole hiki ke ʻike ʻia ke kumu o AGL (idiopathic).

  • Nā Kumu o ka Lipodystrophy hapa i loaʻa (APL): Manaʻo nā kānaka ʻepekema ua hana ʻia ka APL e kā mākou ʻōnaehana pale e luku hewa ana i nā hunaola momona. ʻOi aku ma mua o 80% o ka poʻe me APL he Complement 3 i loko o ko lākou koko.Aia kahi pae haʻahaʻa o kahi protein i kapa ʻia ʻo complement factor. ʻO ka maʻamau, pili kēia protein i ka pane a kā mākou ʻōnaehana pale. Loaʻa pinepine i ka poʻe me APL kahi autoantibody i kapa ʻia ʻo complement 3-nephritic factor i loko o ko lākou koko. ʻO ka autoantibody kahi protein i loko o ka ʻōnaehana pale e hoʻouka hewa a hōʻino i nā ʻiʻo olakino.
  • Nā kumu o ka lipodystrophy i hoʻokomo ʻia e ka antiretroviral therapy (HAART) (LD-HIV): ʻAʻole maopopo i nā kānaka ʻepekema ke kumu o ka HAART, nona nā mea hoʻopaʻa protease HIV-1, e hoʻoulu ai i ka lipodystrophy.

ʻO ka nūhou maikaʻi, ʻo ka hapa nui o ka poʻe e hoʻomaka ana i ka mālama ʻana iā HIV i kēia manawa ʻaʻole pono lākou e hopohopo nui e pili ana i ka lipodystrophy, ʻoiai ʻo nā lāʻau lapaʻau HIV hou e hoʻemi iki i ka hiki ʻana mai o ke ʻano.

Pehea e ʻike ai inā loaʻa iā ʻoe ka lipodystrophy?

Inā loaʻa iā ʻoe nā hōʻailona o ka lipodystrophy, e hana kāu kauka i kahi hoʻokolokolo kino, e nīnau e pili ana i kou mōʻaukala olakino a me ka mōʻaukala olakino ʻohana, a laila e kauoha i kekahi mau hoʻokolohua e hōʻoia i ka maʻi a ʻike inā he mau kumu ʻē aʻe no kāu mau hōʻailona.

He aha nā hoʻokolohua e hoʻohana ʻia e ʻike ai i ka lipodystrophy?

Hiki i kāu kauka ke kauoha i kekahi mau hoʻokolohua e ʻike ai i ka lipodystrophy a/a i ʻole e ʻike inā he mau kumu ʻē aʻe no kāu mau hōʻailona. ʻO kēia mau mea:

  • ʻO ke kiʻi MRI (Magnetic Resonance Imaging) piha-kino: Hoʻohana kahi MRI i kahi magnet nui, nā nalu lekiō, a me kahi kamepiula e hana i nā kiʻi kikoʻī o nā ʻōpū a me nā ʻano ʻē aʻe i loko o kou kino. Hiki i kāu kauka ke kauoha i kēia hoʻokolohua e loiloi i ka haku mele ʻana a me ka hoʻolaha ʻana o ka momona i loko o kou kino.
  • ʻO ka Panel Metabolic Comprehensive: He hoʻāʻo koko kēia e ana i 14 mau mea like ʻole i loko o kou koko. Hāʻawi ia i ka ʻike koʻikoʻi e pili ana i ke kaulike kemika a me ka metabolism o kou kino. E nānā pono ke kauka i kāu mau pae kō koko (glucose) a me nā enzymes akepaʻa .
  • Panel Lipid: He hoʻāʻo koko nō hoʻi kēia. Ana ia i ka nui o nā molekala momona i kapa ʻia ʻo lipids i loko o kou koko. ʻO ka maʻamau, loaʻa i kēia hoʻāʻo ʻehā mau ana cholesterol a me kahi ana triglyceride. Hoʻoulu pinepine ka Lipodystrophy i nā pae cholesterol maʻamau ʻole.
  • Hoʻāʻo Leptin: Ana kēia i ka pae o ka leptin i loko o kou koko. Inā loaʻa iā ʻoe ka lipodystrophy a ʻoi aku ka haʻahaʻa o kāu mau pae leptin ma mua o ka maʻamau, hiki i kēia ke hāʻawi iā ʻoe i kahi manaʻo pehea e pane ai kou kino i ka leptin replacement therapy.
  • Hoʻāʻo Genetic: Inā kānalua kāu kauka he ʻano hoʻoilina (genetic) kou o Lipodystrophy, hiki iā lākou ke ʻōlelo aku i ka hoʻāʻo genetic e hōʻoia i ka maʻi.
  • ʻO ka biopsy puʻupaʻa:ʻO kēia ke ʻoki ʻana i kahi hāpana o nā ʻiʻo puʻupaʻa a nānā iā ia ma lalo o kahi microscope. Hiki i kāu kauka ke kauoha i kēia hoʻāʻo e ʻike inā ua hoʻopilikia ʻia kou mau puʻupaʻa e ka lipodystrophy.

He aha nā lāʻau lapaʻau no ka lipodystrophy?

ʻO ka mālama ʻana a me ka hoʻokele ʻana i ka lipodystrophy e pili ana i ke ʻano kikoʻī āu i loaʻa ai, a inā he mau pilikia ʻē aʻe kāu, e like me ka maʻi diabetes a i ʻole nā ​​​​pae cholesterol maʻamau.

ʻO nā lāʻau lapaʻau i hoʻohana nui ʻia no ka mālama ʻana i ka lipodystrophy:

  • Lāʻau lapaʻau pani leptin: Hiki i kāu kauka ke kuhikuhi i kahi ʻano synthetic o ka hormone leptin i kapa ʻia ʻo metreleptin . Loaʻa pinepine i ka poʻe me ka lipodystrophy ka nele o ka leptin. Kōkua kēia hormone i ka hoʻoponopono ʻana i ka metabolism o ke kino a hiki ke hoʻemi i nā pae cholesterol kiʻekiʻe a me nā triglyceride.
  • Lapaʻau no ka maʻi diabetes a me ke kūʻē ʻana i ka insulin: Inā loaʻa iā ʻoe ke kūʻē ʻana i ka insulin a/a i ʻole ka maʻi diabetes ma muli o ka lipodystrophy, hiki i kāu kauka ke kuhikuhi i nā lāʻau lapaʻau waha e like me ka pioglitazone, metformin, sulfonylureas , a i ʻole thiazolidinediones e mālama ai. Pono paha kekahi poʻe me ka lipodystrophy e lawe i ka insulin synthetic e mālama ai i kā lākou maʻi diabetes. Pono paha ʻoe e nānā mau i kāu pae kō koko ma ka home me kahi glucometer .
  • Ke kāohi ʻana i nā pae triglyceride a me ka cholesterol: ʻO nā lāʻau lapaʻau waha i ʻōlelo ʻia ma mua e kōkua i ka mālama ʻana i ka maʻi diabetes hiki ke kōkua pinepine i ka kāohi ʻana i nā pae cholesterol. Hiki i kāu kauka ke kuhikuhi i nā statins , e like me rosuvastatin a me pravastatin . Pono paha ka poʻe me nā pae triglyceride kiʻekiʻe e lawe i nā mea hoʻohui o nā derivatives fibric acid a i ʻole nā ​​​​​​waikawa momona polyunsaturated n-3 , e like me nā mea i loaʻa i ka ʻaila iʻa.
  • ʻO ke ʻoki kino a me nā kaʻina hana hoʻonaninani: ʻO ka poʻe me ka lipodystrophy ma nā wahi koʻikoʻi o ka hoʻonaninani, e like me ka maka, ka umauma, a me nā wahi genital, hiki ke hana ʻia ke ʻoki kino e hoʻomaikaʻi i ko lākou helehelena a me ka hilinaʻi iā lākou iho. Hiki i nā kauka ʻoki plastik ke hana i ka hoʻoili ʻana i nā ʻiʻo adipose autologous, ke kūkulu hou ʻana o ka maka me ka hoʻohana ʻana i nā flaps manuahi, a me ka silicone a i ʻole nā ​​​​​​mea hoʻokomo ʻē aʻe. Hiki iā lākou ke hoʻohana i ka liposuction a i ʻole nā ​​​​​​ʻano hana wehe ʻoki e wehe i nā waihona momona i makemake ʻole ʻia ma nā wahi e like me ka ʻauwae a i ʻole ke kua o ka ʻāʻī (i kapa pinepine ʻia ʻo "buffalo hump" ).

He nui nā mea e pono ai i nā kānaka ʻepekema ke aʻo e pili ana i ka mālama ʻana a me ka hoʻokele ʻana i ka lipodystrophy. I ko lākou ʻike ʻana i nā mea hou aʻe e pili ana i nā paʻakikī o nā kaʻina hana metabolic, hiki ke loaʻa nā koho lapaʻau hou aʻe.

Hiki ke pale ʻia ka lipodystrophy?

I nā manawa he nui, ʻaʻole hiki ke mālama ʻia ka lipodystrophy.

ʻAʻole hiki ke pale ʻia nā ʻano genetic o ka lipodystrophy, no ka mea, ʻo ia ka hopena o kahi hoʻololi gene i hoʻoilina ʻia. Inā ʻoe e hoʻolālā ana e loaʻa nā keiki, e hoʻomaopopo i ka pilikia o ka loaʻa ʻana o kahi keiki me kahi maʻi genetic a kamaʻilio me kāu kauka e pili ana i ka hoʻāʻo genetic.

Hoʻokumu pinepine ʻia ka lipodystrophy i loaʻa e kahi maʻi a i ʻole ka maʻi ʻōnaehana pale. ʻOiai hiki ke pale ʻia kekahi mau maʻi, e like me ka chickenpox a me ka whooping cough, ma o ka lāʻau pale, ʻaʻole hiki ke pale ʻia nā maʻi ʻē aʻe a me nā maʻi ʻōnaehana pale e pili ana me ka lipodystrophy i loaʻa.

He aha ka wānana no ka lipodystrophy?

ʻO ka nānā ʻana no ka lipodystrophy e hilinaʻi nui ʻia i ke ʻano o ka lipodystrophy āu e loaʻa ai, a inā he mau pilikia a pilikia paha kāu, e like me ka maʻi diabetes, nā pilikia o ke akepaʻa a i ʻole nā ​​​​puʻupaʻa.

ʻO ka hoʻoponopono hoʻonaninani o nā wahi momona haʻahaʻa a me nā wahi momona keu e kōkua i ka hoʻomaikaʻi ʻana i ke ʻano o ke ola no ka poʻe me ka lipodystrophy.

Inā ʻike ʻia ʻoe a i ʻole kāu keiki me ka lipodystrophy, e kamaʻilio me kāu kauka e pili ana i ka mea e manaʻo ai a pehea e hoʻokele maikaʻi ai i ke kūlana.

I ka manawa hea e ʻike ai ʻoe i ke kauka e pili ana i ka lipodystrophy?

Inā ʻike ʻoe e hoʻomau nei ka emi ʻana o ka momona o kou kino a i ʻole ke kino o kāu keiki ma nā wahi kikoʻī, a i ʻole e piʻi ana ka momona ma kekahi mau wahi, e kamaʻilio me kāu kauka.

Inā ʻike ʻia ʻoe a i ʻole kāu keiki me Lipodystrophy, pono ʻoe e hālāwai me kāu hui lapaʻau i kēlā me kēia manawa e hōʻoia i ka hana pono ʻana o kāu lāʻau lapaʻau iā ʻoe.

ʻO ka mea hope loa, nā mea e hoʻomanaʻo ai

He maʻi laha ʻole ka lipodystrophy akā he maʻi koʻikoʻi. ʻOiai ʻaʻohe lāʻau lapaʻau, hiki i kekahi mau lāʻau lapaʻau ke kōkua i ka hoʻokele ʻana i kona mau hōʻailona a me nā kūlana pili, e like me ka maʻi diabetes a me nā pae cholesterol maʻamau ʻole.

ʻOkoʻa loa ke ʻano o ka mālama ʻana mai kēlā kanaka a kēia kanaka. No laila, e hana pū kāu hui lapaʻau me ʻoe e hana i kahi hoʻolālā kūpono loa iā ʻoe. Mai makaʻu e nīnau i kāu kauka. Aia lākou ma laila e kōkua iā ʻoe. E kōkua maoli kēia ʻike iā ʻoe e ola i kahi ola olakino!


` lipodystrophy, ʻiʻo adipose, leptin, genetic, loaʻa, maʻi diabetes, cholesterol, triglyceride, HIV, nā maʻi metabolic

Frequently Asked Questions (FAQ)

He aha nā hoʻokolohua e hoʻohana ʻia e ʻike ai i ka lipodystrophy?

Hiki i kāu kauka ke kauoha i kekahi mau hoʻokolohua e ʻike ai i ka lipodystrophy a/a i ʻole e ʻike inā he mau kumu ʻē aʻe no kāu mau hōʻailona. ʻO kēia mau mea:

⚠️ Important: The medical articles and information on Nirogi Lanka are for general awareness only, and are by no means a substitute for professional medical advice, diagnosis, or treatment. For any medical problem you have, consult a qualified physician immediately.

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Ke ʻike nei ʻoe i nā waihona momona ma nā ʻāpana like ʻole o kou kino? E aʻo kākou e pili ana i ka Lipodystrophy!

Ke ʻike nei ʻoe i nā waihona momona ma nā ʻāpana like ʻole o kou kino? E aʻo kākou e pili ana i ka Lipodystrophy!

Ua ʻike anei ʻoe, a i ʻole ua ʻike paha, i ka nalowale koke ʻana o ka momona ma kekahi mau wahi o kou kino, a i ʻole ka piʻi ʻana o ka momona i makemake ʻole ʻia ma nā wahi ʻē aʻe? Hiki ke noʻonoʻo ʻole ʻia kēia i kekahi manawa. I kēia lā e kamaʻilio mākou e pili ana i kēia ʻano. Ma nā ʻōlelo lapaʻau, ua kapa ʻia kēia ʻo Lipodystrophy . Mai hopohopo, ʻoiai paha he mea ʻē ke kani ʻana o ka inoa, e kamaʻilio maʻalahi kākou e pili ana.

He aha ka Lipodystrophy?

I ka ʻōlelo maʻalahi, ʻo Lipodystrophy ka nalowale piha a hapa paha o ka ʻiʻo adipose, ka mea a mākou e kapa pinepine ai he "momona," ma kekahi mau wahi, a i ʻole ka hōʻiliʻili ʻana o ka momona ma nā wahi like ʻole o ke kino.

E noʻonoʻo ʻoe e emi ana kou kaumaha ma kekahi mau wahi o kou kino, e like me kou mau lima a me kou mau wāwae, a e loaʻa ana ka momona keu ma nā wahi e like me kou maka a me kou ʻāʻī. ʻO kēia ka mea e hana ʻia ma Lipodystrophy. Aia nā ʻano like ʻole o kēia ʻano, ʻo kekahi o ia mau mea he genetic (ʻo ia hoʻi, kahi mea i hānau ʻia ai mākou), aʻo kekahi e ulu aʻe ma hope ma muli o nā kūlana olakino ʻē aʻe. Hoʻopilikia kēlā me kēia ʻano i nā kānaka me ke ʻano ʻokoʻa a he ʻokoʻa nā hōʻailona.

No ke aha he mea nui ka momona (ʻiʻo adipose) i ko mākou kino?

I kēia manawa ke noʻonoʻo nei paha ʻoe, "ʻO ka liʻiliʻi o kou momona, ʻoi aku ka maikaʻi." Akā ʻo ka ʻoiaʻiʻo, hana ka ʻiʻo adipose i nā hana koʻikoʻi he nui i loko o ko mākou kino. E nānā:

  • Ka mālama ʻana i ka ikehu: Aia i loko o kēia mau ʻiʻo momona e mālama ʻia ai nā calorie keu a mākou e loaʻa ai mai ka meaʻai a mākou e ʻai ai i ikehu.
  • Ke hāʻawi nei i ka palekana: Hāʻawi ia i ka pale e like me ka uluna i nā ʻano kino like ʻole i loko o ko mākou kino.
  • Ka mālama ʻana i ka mehana: Mālama i ka wela o ke kino a pale iā mākou mai ke anu.
  • Hoʻokuʻu ʻia ka hormone: Hoʻopuka ʻia a hoʻokuʻu ʻia kekahi mau hormones koʻikoʻi, e like me Leptin, i loko o ke kahe koko.
  • Ke kāohi ʻana i ka mumū: Kōkua pū ia i ka kaohi ʻana i ka mumū i loko o ke kino.

No laila, ke nalowale a waiho ʻia paha kēia ʻiʻo momona ma muli o ka lipodystrophy, ʻaʻole wale ko mākou helehelena e loli ai, akā ua hoʻopilikia ʻia nā hana metabolic koʻikoʻi i loko o ke kino. Nui ka poʻe me ka lipodystrophy e pilikia ana i ka loaʻa ʻana o nā maʻi e like me ka maʻi diabetes mellitus a me ka loaʻa ʻana o nā pae cholesterol koko maʻamau ʻole.

Aia kekahi mau ʻano o ka lipodystrophy?

ʻAe, hiki ke hoʻokaʻawale ʻia ka lipodystrophy i ʻelua mau ʻano nui: genetic a loaʻa.

Nā ʻano o ka Lipodystrophy Genetic

He mau mea hoʻoilina kēia, ʻo ia hoʻi, nā ʻano i kū mai ma muli o nā hoʻololi genetic.

  • ʻO ka Lipodystrophy Laulā Congenital (CGL): ʻO kēia ka maʻi Berardinelli-Seip.ʻIke ʻia hoʻi ʻo. He kūlana kakaikahi loa kēia. Ma kēia, ua aneane nalowale loa a hapa nui paha ka momona o ke kino. Hoʻokumu ʻia kēia e kahi kīnā genetic i loaʻa i ka wā hānau. Hiki ke ʻike pinepine ʻia kēia kūlana i loko o ka makahiki mua o ke ola.
  • ʻO ka Familial Partial Lipodystrophy (FPLD): He maʻi genetic nō hoʻi kēia. Eia nō naʻe, ʻike pinepine ʻia ma hope o ke ola. I kēia kūlana, nalowale ka momona o ke keiki ma nā wāwae a me nā lima, a hōʻiliʻili i ka momona hou aʻe ma ka maka a me ka ʻāʻī.

Nā ʻano o ka Lipodystrophy i loaʻa

ʻO kēia nā ʻano e kū mai ana ma hope o ko kākou hānau ʻana a i ko kākou wā e ola ana no nā kumu like ʻole.

  • ʻO ka Lipodystrophy Laulā i Loaʻa (AGL): ʻIke ʻia hoʻi ʻo Lawrence syndrome , ʻo kēia ʻano ke kumu o ka pohō momona, ma ka maka, ka ʻāʻī, nā lima, a me nā wāwae. Hiki ke hana koke ʻia kēia pohō momona, i loko o kekahi mau pule, a lohi paha i nā mahina a i ʻole nā ​​​​makahiki. Hoʻomaka pinepine ia i ka wā kamaliʻi a i ʻole ka wā ʻōpio, akā hiki ke hana i kēlā me kēia makahiki. Nui nā kumu hiki o AGL.
  • ʻO ka Lipodystrophy hapa i loaʻa (APL): ʻIke ʻia hoʻi ʻo Barraquer-Simons syndrome , ʻo kēia kūlana ke kumu o ka nalowale mālie o ka momona mai ka maka, ka ʻāʻī, nā lima, a me ka umauma i ka wā kamaliʻi. Loaʻa paha i kekahi poʻe ka momona keu ma nā wahi e like me ka ʻōpū, nā wāwae, a me nā puʻupuʻu. Hoʻopili pinepine ʻia ka APL me nā kūlana autoimmune .
  • ʻO ka lipodystrophy i hoʻokomo ʻia e ka lāʻau antiretroviral ikaika loa (HAART) (LD-HIV): Loaʻa kēia ʻano lipodystrophy i nā poʻe i loaʻa i ka maʻi immunodeficiency kanaka (HIV) ma hope o ko lākou loaʻa ʻana i ka lāʻau antiretroviral ikaika loa (HAART) e komo pū ana me nā mea hoʻopaʻa protease HIV-1 . ʻO ka hanana o kēia ʻano maʻi e pili ana i ka ikaika a me ka lōʻihi o ka lāʻau. ʻIke pinepine ka poʻe me LD-HIV i ka nalowale mālie o ka momona mai ko lākou mau lima, nā wāwae, a me ka maka. Hiki i kekahi poʻe ke loaʻa ka momona keu a puni ko lākou maka, ʻāʻī, kua o luna, a me ka pūhaka.
  • ʻO ka Lipodystrophy kūloko: ʻO kēia ka wā e nalowale ai ka momona ma kahi liʻiliʻi o ke kino. No ka laʻana, hiki ke loaʻa kēia ʻano ke hoʻokomo pinepine ʻia nā lāʻau lapaʻau (e like me ka insulin) i loko o ka wahi like. Me he dimple liʻiliʻi lā, akā ʻaʻole i hoʻopilikia ʻia ka ʻili e uhi ana.

He like anei ka lipodystrophy a me ka lipoatrophy?

ʻO ka Lipodystrophy kahi huaʻōlelo maʻamau no ka hoʻolaha ʻano ʻole o ka momona. Pili pū ia me Lipoatrophy , ʻo ia hoʻi ka nalowale o ka momona. Hoʻohana kekahi poʻe ʻepekema a me nā kauka i nā huaʻōlelo ʻelua e wehewehe i ke kūlana like. I ka ʻōlelo maʻalahi, ʻo Lipoatrophy kahi ʻāpana o Lipodystrophy.

ʻO wai ka mea i hoʻopilikia nui ʻia e kēia maʻi? Pehea ka maʻamau o ia mea?

Hoʻomaka ka hapa nui o nā ʻano lipodystrophy i ka wā kamaliʻi, akā hiki ke ulu pū nā ʻano i loaʻa i nā pākeke.

Me ka ʻokoʻa o ka lipodystrophy i hoʻokomo ʻia e HAART (LD-HIV) , ʻoi aku ka nui o nā ʻano ʻē aʻe o ka lipodystrophy i loaʻa i nā wahine. Ma muli o ka maʻi HIV i nā kāne, ʻoi aku ka nui o ka LD-HIV i nā kāne.

Ma keʻano laulā, he mea kākaʻikahi loa ka lipodystrophy. Eia nō naʻe, ke lilo nei nā ʻano lipodystrophy i loaʻa (no ke ola holoʻokoʻa) i mea maʻamau ma muli o nā hopena ʻaoʻao o kekahi mau lāʻau lapaʻau.

Pehea ka hopena o ka lipodystrophy i koʻu kino?

Hoʻopilikia kēlā me kēia ʻano lipodystrophy i ke kino ma ke ʻano ʻokoʻa, akā aia ʻelua mau kumu nui i maʻamau: ka nalowale ʻana o ka ʻiʻo adipose a me ka nele o ka hormone leptin .

Ka hopena o ka nalowale ʻana o ka ʻiʻo adipose ma ke kino

Hana ʻia ko kākou ʻiʻo momona e nā pūnaewele i kapa ʻia he adipocytes . Loaʻa i kēlā me kēia pūnaewele adipocyte ma kahi o 90% o nā kulu lipid. Mālama kēia mau adipocytes i ka momona (triglycerides). Ke hōʻino ʻia kēia mau pūnaewele momona ma muli o ka lipodystrophy, ʻaʻole hiki iā lākou ke mālama pono i ka momona.

I kekahi mau hihia o Lipodystrophy, ua waiho hewa ʻia kēia momona nalowale i loko o nā ʻiʻo ʻē aʻe o ko mākou kino, no ka laʻana, i loko o ke akepaʻa, ka pancreas, a i ʻole nā ​​ʻiʻo ʻiʻo. Hiki i kēia ke alakaʻi i nā pilikia olakino e like me:

  • Maʻi akepaʻa momona / steatosis hepatic
  • Ke kū'ē'ē o ka insulin a me ke ki'eki'e o ka insulin i loko o ke koko (hyperinsulinemia)
  • Maʻi diabetes (Diabetes Mellitus)
  • Hoʻonui ʻia nā pae triglycerides i loko o ke koko (hypertriglyceridemia)
  • ʻO ka maʻi pancreatitis
  • ʻO ka maʻi metabolic
  • Maʻi ʻaʻa coronary

Akā e hoʻomanaʻo, ʻaʻole nā ​​​​kānaka āpau me ka lipodystrophy e loaʻa i kēia mau kūlana. Loaʻa paha i kekahi nā ʻano palupalu, aʻo kekahi paha he ʻoi aku ka koʻikoʻi.

Nā hopena o ka hemahema o ka Leptin ma ke kino

I ka nalowale ʻana o ka momona o ka poʻe me ka lipodystrophy, nalowale pū kekahi mau hormones, ʻoiai ka hormone leptin .

He hormone ka Leptin i hana ʻia e ko kākou ʻiʻo momona. Kōkua ia iā kākou e mālama i ke kaumaha o ke kino maʻamau i ka wā lōʻihi. Maopopo iā ʻoe pehea? Ma ka hoʻomāʻona ʻana iā kākou a me ka kaohi ʻana i ko kākou pōloli. Ke noiʻi nei nā kānaka ʻepekema i ka leptin. Manaʻo lākou e pili ana ka leptin i kā kākou metabolism , ka hana o ka ʻōnaehana endocrine , a me ka hana o ka ʻōnaehana pale .

Hiki i ka poʻe me ka lipodystrophy ke ʻike i ka emi ʻana o ka pae leptin, hiki ke alakaʻi i ka pōloli nui, ke kū'ē ʻana o ka insulin, a me nā pilikia olakino ʻē aʻe.

He aha nā hōʻailona o ka lipodystrophy?

Ma muli o ka nui o nā ʻano lipodystrophy, ʻokoʻa loa nā hōʻailona.

ʻO ka hōʻailona maʻamau ka pohō maopopo a mau o ka momona ma kekahi mau wahi o ke kino, a i ka manawa like, kahi hōʻiliʻili ʻana o ka momona maʻamau ʻole a makemake ʻole ʻia paha ma nā wahi ʻē aʻe o ke kino.

Eia kekahi laʻana, ʻo ka poʻe me ka lipodystrophy hapa i loaʻa (APL) e emi mālie ka momona ma ko lākou helehelena, ʻāʻī, lima, a me ka umauma i ka wā kamaliʻi. Hiki i kekahi poʻe me APL ke loaʻa ka momona keu ma nā wahi e like me ka ʻōpū, nā wāwae, a me nā puʻupuʻu.

Ma muli o ke ʻano o ka lipodystrophy āu e loaʻa ai a me kou mau makahiki, ʻaʻole paha ʻoe e ʻike i kekahi mau pilikia olakino nui no nā makahiki. Eia nō naʻe, nui nā ʻano lipodystrophy e hoʻonui ai i ka cholesterol koko, triglycerides, a me ke kō (glucose). Hiki i kāu kauka ke ʻike i kēia mau mea me nā hoʻāʻo koko maʻamau, e like me ka panel lipid a me kahi panel metabolic kumu .

Hiki ke paʻakikī ke ʻike i kekahi mau ʻano lipodystrophy, ʻoiai ka lipodystrophy hapa. No laila, he mea nui e ʻike i ke kauka inā loaʻa iā ʻoe a i ʻole kāu keiki nā hōʻailona hou a inā e ʻoi aku ka ʻino o kahi hōʻailona e kū nei, me ka nānā ʻole i ke kumu.

He aha nā kumu o ka lipodystrophy?

ʻOkoʻa nā kumu o ka lipodystrophy ma muli o ke ʻano genetic a i loaʻa paha.

Nā kumu o ka Lipodystrophy Genetic

ʻO nā ʻano genetic o ka lipodystrophy - ʻo ia hoʻi ka congenital generalized lipodystrophy (CGL) a me ka familial partial lipodystrophy (FPLD) - i hoʻokumu ʻia e kekahi mau mutations genetic .

ʻO ka hoʻololi ʻana o ka genetics kahi hoʻololi i kā mākou moʻo DNA. ʻO kā mākou moʻo DNA ka mea e hāʻawi i kā mākou mau cell i ka ʻike e pono ai lākou e hana i kā lākou mau hana. No laila, inā ʻaʻole piha a hōʻino ʻia paha kekahi hapa o kēia moʻo DNA, hiki iā mākou ke loaʻa nā hōʻailona o kahi maʻi genetic.

  • Nā kumu o ka lipodystrophy generalized congenital (CGL): ʻO nā ʻano CGL 1 a 4 i hoʻokumu ʻia e nā mutations i loko o nā genes AGPAT2, BSCL2, CAV1 , a me CAVIN1 . He mea nui kēia mau genes no ka ulu ʻana a me ka hana o nā cell mālama momona (adipocytes) i loko o kā mākou ʻiʻo momona. Hoʻopilikia nā mutations i loko o kēia mau genes i ke ʻano a me ka hana o nā adipocytes. Hoʻoilina ke keiki me CGL i kēia mau mutations gene mai ko lākou mau mākua. ʻOiai inā loaʻa i nā mākua ʻelua hoʻokahi kope o ka gene mutated, ʻaʻole lākou e hōʻike pinepine i nā hōʻailona.
  • Nā Kumu o ka Familial Partial Lipodystrophy (FPLD): Hiki i nā loli ʻana i loko o kekahi mau genes ke hana i ka FPLD. ʻO ka mea maʻamau ka LMNA.He hoʻololi ʻana i loko o kahi gene i kapa ʻia ʻo LMNA. ʻO ka gene LMNA a me nā gene ʻē aʻe i pili i ka FPLD e kuhikuhi i nā adipocytes e mālama i ka momona a hana i nā protein like ʻole e pono ai no ka mālama ʻana i ka momona. Inā loaʻa kahi hoʻololi ʻana i kekahi o kēia mau genes, ua hoʻopilikia maikaʻi ʻole ʻia ka ulu ʻana, ke ʻano, a i ʻole ka hana o nā adipocytes. Hoʻoilina ʻia ka hapa nui o nā hihia o FPLD ma ke ʻano autosomal dominant . ʻO ia hoʻi, ʻo ka loaʻa ʻana o hoʻokahi kope o ka gene i hoʻololi ʻia i loko o kēlā me kēia cell ua lawa ia e hoʻoulu ai i ka maʻi. I kekahi manawa, hoʻoilina kekahi kanaka me FPLD i ka hoʻololi ʻana mai kekahi o ko lākou mau mākua i hoʻopilikia ʻia. I nā hihia ʻē aʻe, ua hoʻokumu ʻia lākou e nā hoʻololi hou, random i loko o ka gene, a hiki ke hana ʻoiai inā ʻaʻohe mea o ka ʻohana i loaʻa i ka maʻi ma mua.

Nā kumu o ka Lipodystrophy i loaʻa

Hiki ke hana ʻia ka lipodystrophy i loaʻa e nā lāʻau lapaʻau, nā hopena autoimmune , a i ʻole nā ​​​​kumu idiopathic . ʻOiai ʻaʻohe kumu genetic pololei no ka lipodystrophy i loaʻa, manaʻoʻiʻo kekahi poʻe noiʻi he predisposition genetic paha kekahi poʻe i ka hoʻomohala ʻana i ka lipodystrophy i loaʻa.

  • Nā Kumu o ka Lipodystrophy Laulā i Loaʻa (AGL): Hiki ke loaʻa ka AGL ma hope o kahi maʻi a i ʻole ma hope o kahi maʻi o ka ʻōnaehana pale. ʻO nā maʻi i pili i ka AGL:
  • ʻO ka maʻi puʻupuʻu (Chickenpox / varicella)
  • Ka maʻi ahulau
  • ʻO ka ʻū ʻana (pertussis)
  • ʻO ka diphtheria
  • Pneumonia
  • ʻOsteomyelitis
  • ʻO ka Mononucleosis (Mononucleosis / mono)

ʻAʻole maopopo e loaʻa iā ʻoe ka AGL no ka mea wale nō he mau maʻi kēia iā ʻoe.

ʻO nā kūlana Autoimmune e pili ana me AGL:

  • ʻO ka thyroiditis autoimmune
  • ʻO ka maʻi hepatitis autoimmune
  • ʻO ka dermatomyositis ʻōpio
  • ʻO ka ʻāʻī rheumatoid
  • ʻO ka maʻi Sjogren
  • ʻO ka anemia hemolytic autoimmune

Inā loaʻa iā ʻoe kēia mau maʻi ʻōnaehana pale, ʻaʻole hiki ke ʻōlelo ʻia e loaʻa maoli iā ʻoe ʻo AGL.

I ka hapanui o nā hihia, ʻaʻole hiki ke ʻike ʻia ke kumu o AGL (idiopathic).

  • Nā Kumu o ka Lipodystrophy hapa i loaʻa (APL): Manaʻo nā kānaka ʻepekema ua hana ʻia ka APL e kā mākou ʻōnaehana pale e luku hewa ana i nā hunaola momona. ʻOi aku ma mua o 80% o ka poʻe me APL he Complement 3 i loko o ko lākou koko.Aia kahi pae haʻahaʻa o kahi protein i kapa ʻia ʻo complement factor. ʻO ka maʻamau, pili kēia protein i ka pane a kā mākou ʻōnaehana pale. Loaʻa pinepine i ka poʻe me APL kahi autoantibody i kapa ʻia ʻo complement 3-nephritic factor i loko o ko lākou koko. ʻO ka autoantibody kahi protein i loko o ka ʻōnaehana pale e hoʻouka hewa a hōʻino i nā ʻiʻo olakino.
  • Nā kumu o ka lipodystrophy i hoʻokomo ʻia e ka antiretroviral therapy (HAART) (LD-HIV): ʻAʻole maopopo i nā kānaka ʻepekema ke kumu o ka HAART, nona nā mea hoʻopaʻa protease HIV-1, e hoʻoulu ai i ka lipodystrophy.

ʻO ka nūhou maikaʻi, ʻo ka hapa nui o ka poʻe e hoʻomaka ana i ka mālama ʻana iā HIV i kēia manawa ʻaʻole pono lākou e hopohopo nui e pili ana i ka lipodystrophy, ʻoiai ʻo nā lāʻau lapaʻau HIV hou e hoʻemi iki i ka hiki ʻana mai o ke ʻano.

Pehea e ʻike ai inā loaʻa iā ʻoe ka lipodystrophy?

Inā loaʻa iā ʻoe nā hōʻailona o ka lipodystrophy, e hana kāu kauka i kahi hoʻokolokolo kino, e nīnau e pili ana i kou mōʻaukala olakino a me ka mōʻaukala olakino ʻohana, a laila e kauoha i kekahi mau hoʻokolohua e hōʻoia i ka maʻi a ʻike inā he mau kumu ʻē aʻe no kāu mau hōʻailona.

He aha nā hoʻokolohua e hoʻohana ʻia e ʻike ai i ka lipodystrophy?

Hiki i kāu kauka ke kauoha i kekahi mau hoʻokolohua e ʻike ai i ka lipodystrophy a/a i ʻole e ʻike inā he mau kumu ʻē aʻe no kāu mau hōʻailona. ʻO kēia mau mea:

  • ʻO ke kiʻi MRI (Magnetic Resonance Imaging) piha-kino: Hoʻohana kahi MRI i kahi magnet nui, nā nalu lekiō, a me kahi kamepiula e hana i nā kiʻi kikoʻī o nā ʻōpū a me nā ʻano ʻē aʻe i loko o kou kino. Hiki i kāu kauka ke kauoha i kēia hoʻokolohua e loiloi i ka haku mele ʻana a me ka hoʻolaha ʻana o ka momona i loko o kou kino.
  • ʻO ka Panel Metabolic Comprehensive: He hoʻāʻo koko kēia e ana i 14 mau mea like ʻole i loko o kou koko. Hāʻawi ia i ka ʻike koʻikoʻi e pili ana i ke kaulike kemika a me ka metabolism o kou kino. E nānā pono ke kauka i kāu mau pae kō koko (glucose) a me nā enzymes akepaʻa .
  • Panel Lipid: He hoʻāʻo koko nō hoʻi kēia. Ana ia i ka nui o nā molekala momona i kapa ʻia ʻo lipids i loko o kou koko. ʻO ka maʻamau, loaʻa i kēia hoʻāʻo ʻehā mau ana cholesterol a me kahi ana triglyceride. Hoʻoulu pinepine ka Lipodystrophy i nā pae cholesterol maʻamau ʻole.
  • Hoʻāʻo Leptin: Ana kēia i ka pae o ka leptin i loko o kou koko. Inā loaʻa iā ʻoe ka lipodystrophy a ʻoi aku ka haʻahaʻa o kāu mau pae leptin ma mua o ka maʻamau, hiki i kēia ke hāʻawi iā ʻoe i kahi manaʻo pehea e pane ai kou kino i ka leptin replacement therapy.
  • Hoʻāʻo Genetic: Inā kānalua kāu kauka he ʻano hoʻoilina (genetic) kou o Lipodystrophy, hiki iā lākou ke ʻōlelo aku i ka hoʻāʻo genetic e hōʻoia i ka maʻi.
  • ʻO ka biopsy puʻupaʻa:ʻO kēia ke ʻoki ʻana i kahi hāpana o nā ʻiʻo puʻupaʻa a nānā iā ia ma lalo o kahi microscope. Hiki i kāu kauka ke kauoha i kēia hoʻāʻo e ʻike inā ua hoʻopilikia ʻia kou mau puʻupaʻa e ka lipodystrophy.

He aha nā lāʻau lapaʻau no ka lipodystrophy?

ʻO ka mālama ʻana a me ka hoʻokele ʻana i ka lipodystrophy e pili ana i ke ʻano kikoʻī āu i loaʻa ai, a inā he mau pilikia ʻē aʻe kāu, e like me ka maʻi diabetes a i ʻole nā ​​​​pae cholesterol maʻamau.

ʻO nā lāʻau lapaʻau i hoʻohana nui ʻia no ka mālama ʻana i ka lipodystrophy:

  • Lāʻau lapaʻau pani leptin: Hiki i kāu kauka ke kuhikuhi i kahi ʻano synthetic o ka hormone leptin i kapa ʻia ʻo metreleptin . Loaʻa pinepine i ka poʻe me ka lipodystrophy ka nele o ka leptin. Kōkua kēia hormone i ka hoʻoponopono ʻana i ka metabolism o ke kino a hiki ke hoʻemi i nā pae cholesterol kiʻekiʻe a me nā triglyceride.
  • Lapaʻau no ka maʻi diabetes a me ke kūʻē ʻana i ka insulin: Inā loaʻa iā ʻoe ke kūʻē ʻana i ka insulin a/a i ʻole ka maʻi diabetes ma muli o ka lipodystrophy, hiki i kāu kauka ke kuhikuhi i nā lāʻau lapaʻau waha e like me ka pioglitazone, metformin, sulfonylureas , a i ʻole thiazolidinediones e mālama ai. Pono paha kekahi poʻe me ka lipodystrophy e lawe i ka insulin synthetic e mālama ai i kā lākou maʻi diabetes. Pono paha ʻoe e nānā mau i kāu pae kō koko ma ka home me kahi glucometer .
  • Ke kāohi ʻana i nā pae triglyceride a me ka cholesterol: ʻO nā lāʻau lapaʻau waha i ʻōlelo ʻia ma mua e kōkua i ka mālama ʻana i ka maʻi diabetes hiki ke kōkua pinepine i ka kāohi ʻana i nā pae cholesterol. Hiki i kāu kauka ke kuhikuhi i nā statins , e like me rosuvastatin a me pravastatin . Pono paha ka poʻe me nā pae triglyceride kiʻekiʻe e lawe i nā mea hoʻohui o nā derivatives fibric acid a i ʻole nā ​​​​​​waikawa momona polyunsaturated n-3 , e like me nā mea i loaʻa i ka ʻaila iʻa.
  • ʻO ke ʻoki kino a me nā kaʻina hana hoʻonaninani: ʻO ka poʻe me ka lipodystrophy ma nā wahi koʻikoʻi o ka hoʻonaninani, e like me ka maka, ka umauma, a me nā wahi genital, hiki ke hana ʻia ke ʻoki kino e hoʻomaikaʻi i ko lākou helehelena a me ka hilinaʻi iā lākou iho. Hiki i nā kauka ʻoki plastik ke hana i ka hoʻoili ʻana i nā ʻiʻo adipose autologous, ke kūkulu hou ʻana o ka maka me ka hoʻohana ʻana i nā flaps manuahi, a me ka silicone a i ʻole nā ​​​​​​mea hoʻokomo ʻē aʻe. Hiki iā lākou ke hoʻohana i ka liposuction a i ʻole nā ​​​​​​ʻano hana wehe ʻoki e wehe i nā waihona momona i makemake ʻole ʻia ma nā wahi e like me ka ʻauwae a i ʻole ke kua o ka ʻāʻī (i kapa pinepine ʻia ʻo "buffalo hump" ).

He nui nā mea e pono ai i nā kānaka ʻepekema ke aʻo e pili ana i ka mālama ʻana a me ka hoʻokele ʻana i ka lipodystrophy. I ko lākou ʻike ʻana i nā mea hou aʻe e pili ana i nā paʻakikī o nā kaʻina hana metabolic, hiki ke loaʻa nā koho lapaʻau hou aʻe.

Hiki ke pale ʻia ka lipodystrophy?

I nā manawa he nui, ʻaʻole hiki ke mālama ʻia ka lipodystrophy.

ʻAʻole hiki ke pale ʻia nā ʻano genetic o ka lipodystrophy, no ka mea, ʻo ia ka hopena o kahi hoʻololi gene i hoʻoilina ʻia. Inā ʻoe e hoʻolālā ana e loaʻa nā keiki, e hoʻomaopopo i ka pilikia o ka loaʻa ʻana o kahi keiki me kahi maʻi genetic a kamaʻilio me kāu kauka e pili ana i ka hoʻāʻo genetic.

Hoʻokumu pinepine ʻia ka lipodystrophy i loaʻa e kahi maʻi a i ʻole ka maʻi ʻōnaehana pale. ʻOiai hiki ke pale ʻia kekahi mau maʻi, e like me ka chickenpox a me ka whooping cough, ma o ka lāʻau pale, ʻaʻole hiki ke pale ʻia nā maʻi ʻē aʻe a me nā maʻi ʻōnaehana pale e pili ana me ka lipodystrophy i loaʻa.

He aha ka wānana no ka lipodystrophy?

ʻO ka nānā ʻana no ka lipodystrophy e hilinaʻi nui ʻia i ke ʻano o ka lipodystrophy āu e loaʻa ai, a inā he mau pilikia a pilikia paha kāu, e like me ka maʻi diabetes, nā pilikia o ke akepaʻa a i ʻole nā ​​​​puʻupaʻa.

ʻO ka hoʻoponopono hoʻonaninani o nā wahi momona haʻahaʻa a me nā wahi momona keu e kōkua i ka hoʻomaikaʻi ʻana i ke ʻano o ke ola no ka poʻe me ka lipodystrophy.

Inā ʻike ʻia ʻoe a i ʻole kāu keiki me ka lipodystrophy, e kamaʻilio me kāu kauka e pili ana i ka mea e manaʻo ai a pehea e hoʻokele maikaʻi ai i ke kūlana.

I ka manawa hea e ʻike ai ʻoe i ke kauka e pili ana i ka lipodystrophy?

Inā ʻike ʻoe e hoʻomau nei ka emi ʻana o ka momona o kou kino a i ʻole ke kino o kāu keiki ma nā wahi kikoʻī, a i ʻole e piʻi ana ka momona ma kekahi mau wahi, e kamaʻilio me kāu kauka.

Inā ʻike ʻia ʻoe a i ʻole kāu keiki me Lipodystrophy, pono ʻoe e hālāwai me kāu hui lapaʻau i kēlā me kēia manawa e hōʻoia i ka hana pono ʻana o kāu lāʻau lapaʻau iā ʻoe.

ʻO ka mea hope loa, nā mea e hoʻomanaʻo ai

He maʻi laha ʻole ka lipodystrophy akā he maʻi koʻikoʻi. ʻOiai ʻaʻohe lāʻau lapaʻau, hiki i kekahi mau lāʻau lapaʻau ke kōkua i ka hoʻokele ʻana i kona mau hōʻailona a me nā kūlana pili, e like me ka maʻi diabetes a me nā pae cholesterol maʻamau ʻole.

ʻOkoʻa loa ke ʻano o ka mālama ʻana mai kēlā kanaka a kēia kanaka. No laila, e hana pū kāu hui lapaʻau me ʻoe e hana i kahi hoʻolālā kūpono loa iā ʻoe. Mai makaʻu e nīnau i kāu kauka. Aia lākou ma laila e kōkua iā ʻoe. E kōkua maoli kēia ʻike iā ʻoe e ola i kahi ola olakino!


` lipodystrophy, ʻiʻo adipose, leptin, genetic, loaʻa, maʻi diabetes, cholesterol, triglyceride, HIV, nā maʻi metabolic

Frequently Asked Questions (FAQ)

He aha nā hoʻokolohua e hoʻohana ʻia e ʻike ai i ka lipodystrophy?

Hiki i kāu kauka ke kauoha i kekahi mau hoʻokolohua e ʻike ai i ka lipodystrophy a/a i ʻole e ʻike inā he mau kumu ʻē aʻe no kāu mau hōʻailona. ʻO kēia mau mea:

⚠️ Important: The medical articles and information on Nirogi Lanka are for general awareness only, and are by no means a substitute for professional medical advice, diagnosis, or treatment. For any medical problem you have, consult a qualified physician immediately.

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