Laron ciwo: haddasawa, ganewar asali, magani, hangen nesa

Laron ciwo - a pathological yanayin a cikin abin da jiki yake ba su iya yarda da amfani da na girma hormone. Mafi na kowa alama na cin zarafi - wada. Daga cikin sauran cututtuka za a iya gano wani rauni daga cikin tsokoki da kuma rage halin iya jurewa, yaro da qarancin ruwa da jinkiri jima'i ci gaba, reshe rage, kazalika da kiba. Mafi sau da yawa sa mahaukaci canje-canje (maye gurbi) na wani gene da alhakin girma. Yana da aka gada a wani autosomal recessive hali.

cututtuka

Laron ciwo ne mai rare cuta. A wannan yanayin, da ganewar asali na jarirai, yawanci suna da talakawan rates na ci gaba da nauyi riba, amma a nan gaba su jiki girma slows da cika fuska. Untreated adult namiji isa iyaka girma na 130 cm. Girmancin mata ne kasa da 120 cm.

Bugu da kari ga low girma na gaban Pathology wadannan cututtuka:

• tsoka rauni, rashin ƙarfin hali.
• Da qarancin ruwa da a cikin shimfiɗar jariri.
• jinkirta aure.
• disproportionately kananan al'aurar.
• bakin ciki, da rauni gashi.
• take hakkin da tsarin da hakora.
• taqaitaccen wata gabar jiki.
• kiba.
• rarrabe fuska siffofin (shahararren goshi, sunken hanci, blue sclera ciwo).

dalilai

Laron ciwo haddasa maye gurbi hada da ci gaban hormone tsoka mai amsa sigina - gina jiki samu a cikin matsanancin membranes na sel a ko'ina cikin jiki. Tsoka mai amsa sigina girma hormone ne aka yi nufi ga gane da kuma kunna somatotropin, game da shi wajen qaddamar da girma da kuma division na sel. Maye gurbi na gene impede da al'ada aiki na nazarin halittu inji da kuma kai wa ga disturbances na cell ci gaba. Mutane da yawa cututtuka na cututtuka da rashin iyawa don samar da sakandare hormone - insulin-kamar girma factor.

gadar hali

A mafi yawan lokuta, Laron ciwo a yara da aka gada a wani autosomal recessive hanya. Wannan yana nufin cewa da gazawar ne ya sa ta maye gurbi a cikin duka biyu kofe na shafa gene a kowane cell. Iyaye da marasa lafiya da cuta yawanci suna dako na daya kwafin na mutated gene, kuma da wuya sha daga hankula manifestations na ƙeta. Lokacin da biyu dako na wani recessive autosomal cuta haife yaro gado cutar hadarin shi ne 25%. iya zama wani m m na yadda kowane daga cikin iyaye - 50%. damar ba gadon wani mahaukaci ko mutated gene - 25%.

A cewar wasu rahotanni, akwai iyalai a wadda Laron ciwo da aka gada a wani autosomal rinjaye hanya. A irin haka ne, domin watsa daga cututtuka ne mai maye gurbi a daya kwafin shafa gene a kowane cell. Wani lokaci wannan siffa rungumi dabi'ar yaro daga iyaye da Pathology na haƙuri. Da aka sani da sabon lokuta, ba gaji kwayoyin maye gurbi, a lokacin da take hakkin da aka cuta a yara a iyalan da suka ba da wata guda hali na cuta. A mutumin da ya tabbatar Pathology rahotanni mutated gene wa 'ya'yansu a 50% na lokuta.

bincikowa da

Bayan na farko kima na shakka akwai ayoyi da kuma bayyanar cututtuka da wani gwani zai iya gane asali Laron ciwo (photos na mutane da Pathology cm. A cikin labarin). ƙarin gwaje-gwaje suna da za'ayi don tabbatar da wannan bincike da kuma tasiri bambantawa, ciki har da wani jini gwajin ga takamaiman ji ba gani. Mafi sau da yawa a cikin mutane tare da cuta samu mafi girma matakan da ci gaban hormone a lokaci guda tare da wani karu a insulin-kamar girma factor. A wasu lokuta, likitoci rubũta kayyade nazarin tabbatar da cewa gene maye gurbi. diagnostic matakan da sakamakon da damar domin sanin daidai Laron ciwo.

magani

Gaba daya warkar da wannan pathological jihar na zamani magani ne ba tukuna a karfi. M far da farko mayar da hankali a kan hanzari girma, wanda aka bayar da subcutaneous allura na insulin bace hormone. Abu ta da mikakke girma (a tsawo), shi accelerates da ci gaban da kwakwalwa da kuma hana abin da ya faru na rayuwa cuta lalacewa ta hanyar shafe tsawon karanci dole sunadaran. Bugu da kari, irin wannan injections ta da jini sugar, hana jari na cholesterol da muhimmanci toned tsoka nama. Jiyya na insulin-kamar girma factor kamata a da za'ayi a karkashin akai dubawa na kwararru, kamar yadda da suka wuce haddi na bukata sashi iya kai ga tsanani kiwon lafiya matsaloli.

Outlook

Dogon lokacin da hangen nesa shi ne mafi sau da yawa quite kaffa. Laron ciwo ba ya shafar rayuwar jama'a. Haka kuma, yan dako na hereditary cuta ne da yawa kasa kasadar ciwon daji da kuma wadanda ba insulin ciwon sukari mellitus. A halin yanzu muna gudanar da m bincike don gano links tsakanin Laron ciwo, kwayoyin maye gurbi, hormone girma da kuma ci gaban da m siffofin maruran. Yana yiwuwa a cikin wadannan gene canje-canje ne key zuwa fatattakar ciwon daji.