Matenda a Noonan

Matenda a Noonan ndimatenda omwe amabadwa kuchokera kubadwa (kobadwa nawo) omwe amachititsa kuti ziwalo zambiri zamthupi zizikhala motere. Nthawi zina zimaperekedwa kudzera m'mabanja (obadwa nawo).

Matenda a Noonan amalumikizidwa ndi zolakwika m'matenda angapo. Kawirikawiri, mapuloteni ena omwe amakhudzidwa ndi kukula ndi chitukuko amayamba kugwira ntchito kwambiri chifukwa cha kusintha kwa majini.

Matenda a Noonan ndiwodziwika kwambiri pawokha. Izi zikutanthauza kuti kholo limodzi lokha liyenera kupatsira jini losagwira ntchito kuti mwanayo akhale ndi matendawa. Komabe, nthawi zina mwina sangatengere cholowa.

Zizindikiro zake ndi izi:

• Kuchedwa kutha msinkhu
• Maso otsetsereka kapena otseguka
• Kutaya kwakumva (kumasiyanasiyana)
• Makutu otsika kapena owoneka bwino
• Kulemala pang'ono pamalingaliro (pafupifupi 25% ya milandu)
• Kutulutsa zikope (ptosis)
• Msinkhu waufupi
• Mbolo yaying'ono
• Machende osatsitsidwa
• Mawonekedwe osadziwika acifuwa (nthawi zambiri chifuwa cholowetsedwa chotchedwa pectus excavatum)
• Khosi loluka ndi khosi lowoneka mwachidule

Wothandizira zaumoyo adzayesa. Izi zitha kuwonetsa zizindikilo zamavuto amtima wakhanda kuyambira pomwe adabadwa. Izi zingaphatikizepo pulmonary stenosis ndi atrial septal defect.

Kuyesa kumadalira zizindikilo, koma kungaphatikizepo:

• Kuwerengera kwa Platelet
• Kuyesa kwamagazi
• ECG, chifuwa x-ray, kapena echocardiogram
• Mayesero akumva
• Mahomoni okula msinkhu

Kuyezetsa magazi kumatha kuthandizira kuzindikira matendawa.

Palibe mankhwala enieni. Wothandizira anu akuuzani chithandizo kuti muchepetse kapena kusintha zizindikilo. Hormone yakukula yakhala ikugwiritsidwa ntchito bwino kuthana ndi kutalika kwakanthawi mwa anthu ena omwe ali ndi matenda a Noonan.

Noonan Syndrome Foundation ndi malo omwe anthu omwe ali ndi vutoli amatha kupeza zambiri ndi zofunikira.

Zovuta zingaphatikizepo:

• Kutuluka magazi kapena kuphwanya
• Kupanga kwamadzimadzi m'matupi amthupi (lymphedema, cystic hygroma)
• Kulephera kukula bwino mwa makanda
• Khansa ya m'magazi ndi khansa zina
• Kudziyang'anira pansi
• Kusabereka kwa amuna ngati mayeso onse awiri ndi osayenera
• Mavuto ndi kapangidwe ka mtima
• Kutalika kwakanthawi
• Mavuto azikhalidwe chifukwa cha zizindikiritso zakuthupi

Vutoli limatha kupezeka poyesa mayeso a makanda. Katswiri wa majini nthawi zambiri amafunikira kuti azindikire matenda a Noonan.

Mabanja omwe ali ndi mbiri ya banja la matenda a Noonan angafune kulingalira za upangiri wa majini asanakhale ndi ana.

• Pectus excavatum

Cooke DW, Divall SA, Radovick S. Kukula kwabwino komanso kosabereka kwa ana. Mu: Melmed S, Auchus RJ, Goldfine AB, Koenig RJ, Rosen CJ, olemba. Buku la Williams la Endocrinology. Wolemba 14th. Philadelphia, PA: Elsevier; 2020: chap 25.

Madan-Khetarpal S, Arnold G. Matenda amtundu komanso zovuta za dysmorphic. Mu: Zitelli BJ, McIntire SC, Nowalk AJ, eds. Zitelli ndi Davis ’Atlas of Pediatric Physical Diagnosis. Wachisanu ndi chiwiri. Philadelphia, PA: Elsevier; 2018: mutu 1.

Mitchell AL. Zovuta zobadwa nazo. Mu: Martin RJ, Fanaroff AA, Walsh MC, olemba., Eds. Fanaroff ndi Martin's Neonatal-Perinatal Medicine. 11th ed. Philadelphia, PA: Elsevier; 2020: chap 30.