Prune isisu esiswini

I-Prune isisu syndrome liqela leziphene ezinqabileyo zokuzalwa ezibandakanya ezi ngxaki zintathu ziphambili:

• Ukukhula kakubi kwezihlunu zesisu, kubangela ukuba ulusu lwendawo esiswini lushwabane njenge prune
• Amatyhalarha angaphantsi
• Iingxaki zendlela yomchamo

Oonobangela ngqo besifo esisisu se-prune isisu asaziwa. Le meko ichaphazela ikakhulu amakhwenkwe.

Ngexa sisesibelekweni, isisu somntwana esikhulayo sidumba ulwelo. Rhoqo, unobangela yingxaki kwindawo yokuchama. Ulwelo luyanyamalala emva kokuzalwa, lukhokelele kwisisu esishwabeneyo esikhangeleka ngathi sithenwe. Oku kubonakala ngakumbi ngenxa yokunqongophala kwezihlunu zesisu.

Ukuba buthathaka kwezihlunu zesisu kunokubangela:

• Ukuqunjelwa
• Ukulibazisa ukuhlala nokuhamba
• Ubunzima bokukhohlela

Iingxaki zendlela yokuchama zinokubangela ubunzima ekuchameni.

Umfazi okhulelweyo nosana one-prune isisu esiswini unokungabikho ngokwaneleyo i-amniotic fluid (ulwelo olujikeleze usana olungekazalwa). Oku kunokubangela ukuba usana lube neengxaki zemiphunga ngokucinezelwa esibelekweni.

I-ultrasound eyenziwe ngexesha lokukhulelwa ingabonisa ukuba umntwana unesibindi sokuvuvukala okanye intso eyandisiweyo.

Ngamanye amaxesha, ukukhulelwa kwe-ultrasound kunokunceda ekuchongeni ukuba umntwana:

• Iingxaki zentliziyo
• Amathambo okanye izihlunu ezingaqhelekanga
• Iingxaki zesisu kunye namathumbu
• Imiphunga engaphuhliswanga

Olu vavanyo lulandelayo lunokwenziwa emntwaneni emva kokuzalwa ukufumanisa imeko:

• Uvavanyo lwegazi
• Ipyramogram efakwa ngaphakathi (IVP)
• I-Ultrasound
• Ukuhamba ngecystourethrogram (VCUG)
• X-reyi
• Ukuskena i-CT
  

Ukuhlinzwa kwangoko kuyacetyiswa ukulungisa izihlunu ezibuthathaka zesisu, iingxaki zomchamo, kunye namasende angafunekiyo.

Usana lunokunikwa i-antibiotics ukunyanga okanye ukunceda ukuthintela usulelo lomchamo.

Ezi zixhobo zilandelayo zinokubonelela ngolwazi oluthe kratya kwi-Prune belly syndrome:

• Prune Belly Syndrome Network - prunebelly.org
• Umbutho weSizwe weengxaki zoRare- rarediseases.org/rare-diseases/Prune-belly-syndrome

I-Prune esiswini sisifo esibi kwaye esihlala sisongela ubomi.

Uninzi lweentsana ezinayo le meko zizelwe zingafanga okanye zife kwiiveki zokuqala ezimbalwa zobomi. Unobangela wokufa usuka kwiingxaki ezinzima zemiphunga okanye zezintso, okanye kwindibaniselwano yeengxaki zokuzalwa.

Olunye usana olusandul 'ukuzalwa luyaphila kwaye luyakhula ngesiqhelo. Abanye bayaqhubeka ukuba neengxaki ezininzi zonyango kunye nophuhliso.

Iingxaki zixhomekeke kwiingxaki ezinxulumene noko. Ezona zixhaphakileyo zezi:

• Ukuqunjelwa
• Ukukhubazeka kwethambo (i-clubfoot, i-hip dislocated, isitho esilahlekileyo, umnwe, okanye iinzwane, isifuba se-funnel)
• Isifo sephepha lokuchama (kunokufuna i-dialysis kunye nokufakelwa kwezintso)

Amatyhalarha angahlongozelwanga angakhokelela ekungafumani bantwana okanye umhlaza.

I-Prune isisu syndrome ihlala ifunyaniswa ngaphambi kokuzalwa okanye xa umntwana ezalwa.

Ukuba unomntwana onesifo se-prune belly syndrome, tsalela umnikezeli wakho wezempilo kumqondiso wokuqala wosulelo lwe-urinary okanye ezinye iimpawu zomchamo.

Ukuba ukukhulelwa kwe-ultrasound kubonisa ukuba umntwana wakho unesibindi sokuvuvukala okanye iintso ezandisiweyo, thetha neengcali ekukhulelweni okusemngciphekweni okanye kwi-perinatology.

Akukho ndlela yaziwayo yokuthintela le meko. Ukuba umntwana ufunyenwe enengxaki yokuchama ngaphambi kokuzalwa, kwiimeko ezinqabileyo, utyando ngexesha lokukhulelwa kunokunceda ukuthintela ingxaki ekuqhubekeni ukuya kwisifo se-prune esiswini.

I-Eagle-Barrett syndrome; Isifo seTriad

ICaldamone AA, iiDenes FT. Isifo sePrune-isisu. Ku: Wein AJ, Kavoussi LR, Partin AW, Peters CA, ii-eds. UCampbell-Walsh Urology. Ngomhla we-11. IPhiladelphia, PA: Elsevier; Ngo-2016: isahluko 140.

Umdala JS. Ukuvalwa kwendlela yokuchama. Ku: Kliegman RM, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, ii-eds. Incwadi kaNelson yeNcwadi yePediatrics. Umhla wama-21. IPhiladelphia, PA: Elsevier; 2020: isahluko 555.

Imerguerian PA, iRowe CK. Ukuphuhliswa kokungaqhelekanga kwenkqubo ye-genitourinary. Ku: Gleason CA, Juul SE, ii-eds. Izifo zika-Avery Zomntwana osandul 'ukuzalwa. Ngomhla we-10. IPhiladelphia, PA: Elsevier; I-2018: isahluko 88.