Lefu la tsoekere la phosphate ke e 'ngoe ea maloetse a fumanehang seoelo litloaelong tsa bana.
Ona ke lefu le bakoang ke liphatsa tsa lefutso, le tšoanang le li-rickets, le hlahang khahlano le semelo sa bofokoli ba phosphate ka har'a methapo ea kutlo.
Liphetoho tse joalo li lebisa nts'etsopele ea hypophosphatemia, ho kenngoa ha calcium le phosphorus ka mpeng ea mala mme ka lebaka leo, mineralization e sa lokang.
Joalokaha u tseba, karolo ea bohlokoahali ea lisele tsa masapo ke calcium, eo motho a e amohelang ho tsoa tikolohong hammoho le lijo tse anngoeng. Ho monya ntho ena ea lik'hemik'hale ho nolofalletsoa ke metsoako ea phosphorus.
Pathogenesis ea lefu la tsoekere ea phosphate e amahanngoa le phetoho ea liphatsa tsa lefutso e lebisang ho pherekano mokhoeng oa phosphate ho tsoa methapong ea kutlo e kenang maling, e ileng ea baka mofere-fere oa mafu a amanang le ts'ebetso ea methapo a amanang le demineralization ea masapo.
Hobaneng lefu le hlaha?
Lefu la tsoekere la bana ho bana ke lebaka la phetoho ea mofuta o mong o teng ho X chromosome.
Lefu lena le futsoa ho latela mofuta o hlaheletseng, ke hore, ho tloha ho ntate ea kulang ho ea ho bara bohle ba banana, le ho tloha ho 'm'a ea kulang ho isa ho 50% ea bashanyana ba tsoetsoeng le 25% ea banana (ha feela mosali a le lefu lena).
Boikoetlisong ba bana, ho fumanoa likotsi tsa "hypophosphatemic rickets" tse hlahang ka mofetše oa liphio.
Ka moriana, lefu la tsoekere la paraneoplastic phosphate le lona lea khetholoha, le etsahalang ka lebaka la tlhahiso e eketsehang ea li-hormone tsa parathyroid ke lisele tsa tumor. Ntle le moo, bokuli bona ba lefutso e kanna ea eba karolo ea mafu a amanang le tsamaiso, haholo lefu la Fanconi.
Boloetse bo bonahala joang?
Ka bongaka ba sejoale-joale, ke moetlo ho khetholla mefuta e mene e meholo ea li-hypophosphatemic rickets, tse bontšitsoeng ka mokhoa oa bongaka ho tloha lilemong tsa pele tsa bophelo ba mokuli e monyane mme ba na le litšobotsi tsa bona.
Type I lefu la tsoekere la phosphate kapa hypophosphatemia le amanang le X chromosome ke lefu le futsitsoeng, sesosa se ka sehloohong sa ho ts'oaroa ha phosphorous ka har'a lipompo tsa renal, ho fokotseha hoa phallo ea phosphates maling, nts'etsopele ea phosphaturia le ponahalo ea liphetoho tsa mofuta o moholo oa lithane tsa masapo tse thibelang ho amohela. tekanyetso e lekaneng ea vithamine D.
Lefu lena le iponahatsa nakong ea lilemo tse peli tsa pele tsa bophelo ba lesea mme le bonahatsoa ke ponahalo ea matšoao a latelang:
- ho khutlela morao bokaholimo ba matla a mesifa e eketsehileng;
- tsoelopele e tsoetseng pele kapa ho ba sieo ka botlalo ha enamel menoana;
- tahlehelo ea moriri
- phatlalatso ea valgus ea pheletso e tlase, e hlahella hammoho le mehato ea pele ea ngoana;
- lipontšo tsa radiological tsa rickets, tse tsoelang pele le litekanyetso tse tloaelehileng tsa vithamine D;
- ho fokotseha hoa boemo ba phosphates maling le keketseho ea moroto oa bona;
- lieha ho theoa ha tsamaiso ea musculoskeletal ho latela lilemo.
Lefu la tsoekere la Mofuta oa II oa Phosphate ke mofuta o mong oa hypophosphatemia o atileng haholo, o atileng haholo 'me o sa amanngoe le chromosome ea X ea thobalano.
Lefu la tsoekere la phofo ea Type II le tšoauoa ka matšoao a kang:
- ho hlaha ha lefu lena ho tloha ho likhoeli tse 12;
- ntlafatso ea bohlokoa lipakeng tse tlase le bofokoli ba skeleton;
- ho haelloa ke mokhoa oa ho khutlisa le ho holisa 'mele o matla;
- matšoao a radiological a ricores e bonolo le osteomalacia ea lithane tsa masapo;
- litekong tsa laboratori, ho na le phokotso ea likhahla tsa phosphates maling le litekanyetso tse eketsehileng moriring o khahlano le maemo a tloaelehileng a calcium.
Phosphate lefu la tsoekere la mofuta oa III kapa hypocalcemic rickets ke lefu la lefutso le futsitsoeng ka mokhoa o ikhethileng oa ho ikamahanya le maemo 'me ho its'etleha ka ho itseng ho vithamini D. Matšoao a pele a lefu lena a hlaha leha a sa le monyane.
Ha u hlahloba mokuli e monyane, ho ka etsahala ho tseba hore na matšoao a 'mele a teng, a teng:
- ho teneha, ho lla, ho tšoha;
- molumo oa mesifa o fokotsehileng;
- conculsive syndrome;
- kholo e tlase, nts'etsopele e potlakileng ea bofokoli;
- enamel ea leino le lebe;
- ho qalella ho tsamaea;
- hypophosphatemia e ikemiselitsoe maling, le hyperphosphaturia ka har'a moroto;
- Liphetoho tse thata joalo ka li-rickets libakeng tsa kholo, hammoho le matšoao a ho fokola ha masapo a lithane tsa axial li tlalehiloe ka nepo.
Mofuta oa lefu la tsoekere la Phosphate IV kapa khaello ea livithamini D3 ke lefu le fuoang le fetisetsoa ho bana ho tsoa ho batsoali ka tsela e tloahelehileng ea ho ikhula hape le hlaha ho tloha lilemong tse nyane haholo.Matšoao a latelang ke a tšoaetso ea lefu la tsoekere la Mofuthu oa Mofuthu oa phofo ea IV:
- valgus deformity ea likarolo tse tlase, mokokotlo oa likarolo tse fapaneng tsa skeleton;
- cramps
- baldness le li-pathologies tse rarahaneng tsa enamel ea meno;
- Li-X-ray li supa matšoao a tloaelehileng a liratsoana.
U ka tseba lefu lee joang?
Litlhahiso tsa kliniki bakeng sa lefu la tsoekere la phosphate mabapi le tlhahlobo ea ts'oaetso ea methapo ea mafu li kenyelletsa boitšoaro bo tlamang ba lithuto tse 'maloa tse reretsoeng ho khetholla mathata a phallo ea phosphate ho renal tubules.
E meng ea mehato ea ho tsebahatsa lefu lena ke:
- pokello ea lintlha tsa maiketsetso tse tsoang mantsoeng a batsoali ba ngoana ea bonang ponahalo ea bofokoli ba marapo, mokokotlo oa maoto, kholo e makatsang le tse ling tse joalo;
- tlhahlobo ea sepheo sa mokuli e monyane ka khetho ea li-syndromes tse etellang pele;
- lipatlisiso tsa lefutso bakeng sa khethollo ea chromosome ea X le sepheo sa lefutso ho nts'etsopele ea hypophosphatemia;
- urinalysis, eo ho eona ho bolokoang likhakanyo tse ngata tsa phosphate;
- Tlhahlobo ea X-ray ea masapo a masapo a bana ka tlhaloso ea libaka tsa osteomalacia, osteoporosis, mathata a ikhethang libakeng tsa kholo;
- tlhahlobo ea mali ea laboratori bakeng sa phokotso ea phosphate maemong a tloaelehileng a calcium.
Litšobotsi tsa kalafo
Lilemo tse mashome a seng makae tse fetileng, lefu la tsoekere la phosphate le ne le le thata haholo ho le phekola, ka hona ho ne ho batla ho le thata ho thibela bofokoli ba masapo a masapo. Kajeno, ka lebaka la tlhahlobo ea maikutlo ea sejoale-joale, ho bile le monyetla oa ho ntlafatsa lipalo-palo, ho latela hore na hypophosphatemia e ka sebelisoa ka mokhoa o atlehang bakeng sa khalemelo ea bongaka, ha feela lefu lena le fumanoa le sa fumanehe.
Phekolo ea lefu la tsoekere e phosphate e kenyeletsa:
- ho khethoa ha tekanyetso e phahameng ea vithamine D (ho tloha ho li-unit tse likete tse 25 ho isa ho tse 250);
- tšebeliso ea calcium, phosphorus;
- ho ja livithamini A le E, motsoako oa citrate.
Physiotherapy ea bofokoli ba lesapo la mokokotlo e lokela ho kenyelletsa lithuto tse 'maloa tsa boikoetliso ba setsebi, hammoho le ho roala nako e telele ke setlamo sa marang-rang se tla lumella skeleton ho ba maemong a nepahetseng.
Na hoa khoneha ho thibela bokuli?
Kaha lefu le futsitse, le ka thibeloa feela ka ho sheba lelapa le lecha ka tlhahlobo ea lefutso nakong ea kemiso ea bokhachane.
Kopano ea liphatsa tsa lefutso
Ka ho khetheha, bothata bo lokela ho fanoa ho banyalani moo e mong oa balekane a nang le bothata bona. Setsebi se nang le tsebo se tla hlalosa monyetla oa ho ba le ngoana ea phetseng hantle 'me se lemose ka likotsi tsa ho ima ngoana ea tla ja lefa la lefu la e mong oa batsoali ba hae.
Thibelo ea bobeli ea lefu lena e kenyelletsa ho tsebahala ha bakuli ba ka bang teng le kalafo e loketseng ea bana ba banyenyane ba seng ba ntse ba e-na le matšoao a pele a hypophosphatemia.Ka karabelo e sa lebelloang bothateng bona, ngoana o na le mathata le litlamorao tse ngata, ho kenyelletsa:
- ho tlohella lithaka holima kholo ea 'mele le ea kelello;
- ponahalo ea litšila tse tebileng tsa lesapo la mokokotlo le lipalo tse tlase;
- pathologies e kholo ea enamel ea meno;
- ho kheloha ha masapo a pelvic, ho fokotseha ha methapo ea eona;
- tlolo ea molao ea nts'etsopele ea li-ossicles tse tsebetsong ea tsebe le bohareng ba kutlo
- urolithiasis le ho hloleha ha rems ka lebaka la eona.
Livideo tse amanang
Dr. Komarovsky ho li-rickets le khaello ea vithamine D ho bana:
Ka kakaretso, tsoelo-pele ea lefu la tsoekere ea phosphate e ntle, empa bana ba joalo ba ka hloka ntlafatso ea nako e telele le ho ikamahanya le maemo a bophelo ka ho holofala ha masapo. Bakuli ba joalo ba banyenyane ba lokela ho lula ba ngolisitsoe kahare ho dispensary mme nako le nako ba buisana ka boemo ba bona le litsebi tse ikhethang (endocrinologist, nephrologist).
