Prune manava syndrome

Prune tiyan syndrome o se kulupu o le seasea fanau mai faaletonu e aofia ai nei tolu autu faafitauli:

• Le lelei atinaʻe o maso manava, mafua ai le paʻu o le manava vaega e maanuminumi pei o se paluga
• Vaʻaia le faʻavae
• Faʻafitauli o le misia

O mafuaʻaga tonu o le prune tiyan syndrome e le o iloa. O le tulaga e aafia tele tama.

A o i ai i le manava, o le atiina ae o le pepe manava fulafula i suavai. Tele taimi, o le mafuaʻaga o se faʻafitauli i le auala urinary. Ua mou atu le suavai ina ua uma ona fanau mai, ma tau atu ai i se manava maanuminumi e pei o se paluga. O lenei foliga mai e sili atu ona maitauina ona o le leai o maso manava.

O vaivai maso manava mafai mafua:

• Paneta
• Faatuai i le nofo ma savali
• Faigata tale

O faʻafitauli o le urinary tract e mafai ona mafua ai le faigata ona toaga.

O se fafine ua maʻito ma se pepe ua maua i le prune perut syndrome atonu e le lava le vai amniotic (le suavai e siomia le fetus). E mafua ai ona maua e le pepe ni faʻafitauli o le mama mai le oomi i totonu o le manava.

O se ultrasound faia i le taimi o maʻito e ono faʻaalia ai o le pepe ua i ai se puta fulafula pe faʻateleina fatugaʻo.

I nisi tulaga, o le maitaga ultrasound ono mafai foi ona fesoasoani e faʻamautinoa pe o le pepe ei ai:

• Faʻafitauli o le fatu
• Ivi poʻo maso e le masani ai
• Manava ma manava faʻafitauli
• Mama le lava

O suʻega ia e ono faia i le pepe pe a fanau mai e faʻamaonia le tulaga:

• Tofotofoga toto
• Intravenous pyelogram (IVP)
• Ultrasound
• Voiding cystourethrogram (VCUG)
• X-ray
• CT faʻasolosolo
  

O le taotoga vave e fautuaina e faʻalelei maso vaivai o le manava, faʻafitauli o le urinary tract, ma le le faʻasolosolo fualaʻau.

E ono avatua i le pepe ni vailaʻau faʻafomaʻi e togafitia ai pe fesoasoani ai i le puipuia o faʻamaʻi pipisi.

O faʻamatalaga nei e mafai ona maua mai ai nisi faʻamatalaga ile prune tiyan syndrome:

• Prune Belly Syndrome Network - prunebelly.org
• National Organization for Rare Disorder - rarediseases.org/rare-diseases/prune-belly-syndrome

Prune tiyan syndrome o se ogaoga ma masani ona lamatia ai le ola.

Tele pepe meamea ile tulaga lea e fananau mai pe feoti i totonu ole uluaʻi vaiaso ole olaga. O le mafuaʻaga o le oti e mafua mai iga ogaoga mama poʻo fatugaʻo, pe mai le tuʻufaʻatasia o faʻafitauli fanau.

O nisi pepe faatoa fananau mai e ola ma mafai ona tupu masani. O isi o loʻo faʻaauau pea ona maua i latou ile tele o faʻafitauli tau soifua maloloina.

Faʻalavelave e faʻamoemoe i isi faʻafitauli. O mea taatele e:

• Paneta
• Maʻi ole ponaivi (vaevaevae, vae ua gagau, vaevaega leiloa, tamatamailima, poʻo tamaivae, alamea)
• Faamai o le urinary tract (atonu e manaʻomia dialysis ma se totoga totoina)

Ole suʻega ole suʻesuʻe e mafai ona oʻo atu ai i le maʻitaga poʻo le kanesa.

O le Prune tiyan syndrome e masani ona iloa ae e leʻi fanau mai pe fanau foi le pepe.

A iai sau tamaititi ua maua ile faʻamaʻi ole manava o le manava, valaʻau lau fomaʻi i le faʻailoga muamua o le siama urinary tract poʻo isi faʻamaʻi urinary.

Afai o se maitaga ultrasound faʻaalia ai o lau pepe ua i ai se puta fulafula poʻo faʻateleina fatugaʻo, talanoa i se faʻapitoa i maʻitaga tele lamatiaga po o perinatology.

E leai se auala iloa e puipuia ai lenei tulaga. A faʻapea e maua le pepe ile faʻamaʻi pipisi aʻo leʻi fanau mai, i ni mea e seasea tupu, o le taotoga ile taimi ole maʻitaga e mafai ona fesoasoani e taofi le faʻafitauli mai le alualu i luma i le manava o le manava.

Eagle-Barrett faʻamaʻi; Triad syndrome

Caldamone AA, Denes FT. Prune-manava syndrome. I le: Wein AJ, Kavoussi LR, Partin AW, Peters CA, eds. Campbell-Walsh Urology. 11th lomiga. Filatelefia, PA: Elsevier; 2016: mata 140.

Elder JS. Faʻalavelaveina o le auala urinary. I le: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Tusitusiga o Pediatrics. 21 ed. Filatelefia, PA: Elsevier; 2020: chap 555.

Merguerian PA, Rowe CK. Eseesega atinaʻe o le genitourinary system. I: Gleason CA, Juul SE, eds. O Faʻamaʻi o Avery o le Fanau Fou. 10 ed. Filatelefia, PA: Elsevier; 2018: mata 88.