Ib Qhov Kev Mob Hnyav Hydronephrosis Tswj Raws Li Cystic raum Kab Mob

Cov ntsiab lus

Congenital anomalies ntawm cov kab mob genitourinary, suav nrogcongenital hydronephrosis nyob rau hauv cov me nyuam, yog cov feem ntaumorphological txawv txavkuaj mob los ntawmfetal ultrasound, tab sis muaj ntau qhov xwm txheej uas nws nyuaj rau kev sib txawv ntawm lawvkab mob cystic raum. Cov ntaub ntawv no yog ib tug tub muaj hnub nyoog 2- xyoo.Hydronephrosistau sau tseg thaum lub sij hawmlub sij hawm fetal, tab sis vim yog tsev neeg keeb kwm ntawm cystic raum kab mob, nws tau kuaj pom tias yog tib yam kab mob.

Txawm hais tias nws nyob hauv kev soj ntsuam, nws raug xa mus rau peb lub tsev kho mob raurov ntsuam xyuas. Kev soj ntsuam kaw tau pom tias muaj kev cuam tshuam ntawm lub raum sab laug pelvic ureteric junction thiabmob raum pelvic dilatation, thiab kev kuaj mob hnyav hydronephrosis tau tsim. MAG3 scintigraphy qhia lub raum ua haujlwm<40% and poor urinary drainage, and pyeloplasty was performed at the age of 1 year. One year has passed since the surgery, and although left renal pelvis enlargement remains, renal function, including urine and blood tests, is within normal range. In this case, the influence of family history and the unconfirmed diagnosis resulted in excessive anxiety about the kab mob caj ces. Nyob rau hauv rooj plaub uas qhov sib txawv yog qhov nyuaj, ceev faj soj ntsuam thiab kuaj mob, nrog rau covLub sijhawm kuaj mob, yog tsim nyog.

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Ua ntej

Kev siv dav davfetal ultrasound(Tebchaws Asmeskas) tau ua kom muaj kev tshawb pom ntxov thiab kev kuaj mob ntawm lub raum thiab cov zis (CAKUT) xws li kab mob raum thiab hydronephrosis thaum lub sijhawm cev xeeb tub. Tam sim no koj tuaj yeem ua nws 1).

Txawm li cas los xij, qee zaum, nws nyuaj rau kev sib txawv ntawm qhov hnyav hydronephrosis thiab cystic raum kab mob xws li multicystic dysplastic raum (MCDK), uas tuaj yeem ua rau muaj qhov tsis zoo hauv kev kuaj mob ua ntej yug menyuam. Lub sijhawm no, kuv tau ntsib ib kis mob hnyav hydronephrosis hauv ib tug txiv neej uas muaj keeb kwm ntawm tsev neeg muaj zogpolycystic raum kab mob(PKD), uas tau tswj hwm raws li kab mob hauv lub raum.

Qhia.

cas

Case: 2 xyoos, tus tub

Kev tsis txaus siab tseem ceeb: Kaw kev kuaj mob raum cyst

Keeb kwm ntawm qhov ncauj tshuaj thaum cev xeeb tub: Tsis muaj angiotensin-hloov enzyme inhibitors lossis angiotensin receptor antagonists. Levothyroxine tau noj vim subclinical hypothyroidism.

Cev xeeb tub: Tsis muaj qhov txawv txav tau sau tseg txog thaum lub lim tiam 29 ntawm cev xeeb tub, thiab lub sijhawm cev xeeb tub yog qhov tsis zoo. Thaum 31 lub lis piam thiab 4 hnub ntawm cev xeeb tub, fetal US qhia hydronephrosis nyob rau sab laug (Society for Fetal Urology: SFU grade 3) thiab sab xis (SFU qib 1) thawj zaug. Txawm hais tias tsev neeg keeb kwm tau qhia txog keeb kwm muaj zog ntawm PKD, nws kuj tau piav qhia tias muaj qhov ua tau ntawm MCDK hauv tsev neeg, vim tias qhov loj thiab me me cyst zoo li dilations tau pom nyob rau hauv fetal sab laug raum thaum cev xeeb tub. . . Perinatal keeb kwm: Lub cev xeeb tub. Tus me nyuam yug los ntawm lub hnub nyoog gestational ntawm 39 lub lis piam thiab 4 hnub, hnyav 2862 g, thiab tau xa ib txwm. Apgar 9/10 ntsiab lus (1 feeb / 5 feeb). Yav dhau los kev kho mob keeb kwm: Tsis muaj dab tsi noteworthy

Tsev neeg keeb kwm: saib daim duab 1

Keeb kwm ntawm tus mob tam sim no: Tom qab yug me nyuam, tus neeg mob tau raug soj ntsuam raws li nws tus MCDK ntawm tus kws kho mob nyob ze. Thaum tus neeg mob tau mus ntsib kws kho mob hauv zos kom mob khaub thuas, nws tsev neeg tau txhawj xeeb txog nws tsev neeg muaj keeb kwm muaj kab mob raum, thiab thaum muaj hnub nyoog 8 hli, nws raug xa mus rau peb lub tsev kho mob kom paub meej. Kev tshawb pom lub cev (thawj zaug): Qhov siab: 67.6 cm (-1.5 SD), hnyav: 7.6 kg (-10 SD). Ntshav siab: 90/45 mmHg. Tus neeg mob qhov kev mob dav dav yog qhov zoo, thiab tsis muaj qhov txawv txav tau pom nyob rau hauv cardiopulmonary lossis mob plab. Tsis muaj qhov txawv txav ntawm lub ntsej muag, ceg ceg, pob txha, lossis lwm qhov chaw mos. Kev kuaj pom (Table 1): Urinalysis pom lub ntiajteb txawj nqus 1.004, protein (±), occult blood (-), qe ntshav liab<1/HPF, white blood cells <1/HPF, casts (-), protein/Cr67 (67) day old kidney. Magazine Vol. 34 No. 1

● Case report●

Ib kis mob hnyav hydronephrosis uas tau tswj hwm tus kab mob raum cystic Yuichi Morimoto, Rina Oshima, Takuji Shioya, Kohei Miyazaki, Mitsuru Okada, Keisou Sugimoto (hnub txais tos: Cuaj hlis 29, 2020 Hnub Ntiav: Lub Ob Hlis 2021 (Hnub Monday 2nd) Cov lus tseem ceeb: Fetal Kev kuaj ultrasound / hydronephrosis / cystic raum kab mob /

Cov zis muaj protein tsawg thiab nce siab {{0}}MG tau pom, nrog 0.19 g/gCr thiab 2-microglobulin (2-MG)/Cr 1.{{ 15}}4 g/mgCr. Cov ntshav kuaj pom Na 141 mEq/L, K 5.0 mEq/L, tag nrho cov protein 7.4 g/dl, albumin 4.9 g/dl, urea nitrogen 7 mg/dl, creatinine (Cr) 0.13 mg/dl, thiab kwv yees glomerular filtration rate. (eGFR) yog 154.82 l / min / 1.73 m2, cystatin C yog 0.7 mg / L, thiab tsis muaj kev poob qis hauv lub raum ua haujlwm. eGFR tau suav los ntawm kev sib npaug ntawm qhov sib npaug quintic los ntawm coefficient R (1.619) raws li phau ntawv qhia kev kuaj mob menyuam yaus CKD.

Cov duab tshawb pom: Nws Asmeskas thaum nws thawj zaug mus ntsib peb lub tsev kho mob pom tau tias muaj kev nthuav dav ntawm lub raum pelvis, dilatation ntawm yuav luag tag nrho cov calyces, thiab thinning ntawm lub raum parenchyma nyob rau hauv lub raum sab laug, zoo ib yam li kev kuaj zaum kawg ntawm lub tsev kho mob maternity ( Daim duab 2c). ). Abdominal MRI qhia tau hais tias dilatation ntawm lub raum pelvis thiab dilatation ntawm yuav luag tag nrho cov calyces nyob rau hauv lub raum sab laug. Tsis tau pom ureteral dilatation (Daim duab 2d). Nws 99mTc-DMSA scintigram, uas tau ua los txiav txim siab seb nws puas tsim nyog rau kev phais, pom tias lub raum tsis ua haujlwm qis, nrog rau lub raum sab laug ntawm tus nqi ntawm 12.4% thiab lub raum sab xis uptake ntawm 21.4% (Daim duab 2e). MAG3 scintigram qhia tias lub raum sab laug yog 31.2% thiab lub raum sab xis 68.8%, thiab tom qab diuretic loading, ob qho tib si Tmax thiab T (1/2) tau ntev nyob rau hauv lub raum sab laug, uas qhia tau hais tias muaj kev cuam tshuam (Daim duab 2f). Voiding cystourethrography (VCUG) qhia tias tsis muaj vesicoureteral reflex (VUR). Kev nce qib: Raws li cov txiaj ntsig ntawm ntau yam kev sim, qhov kev kuaj mob tsis yog MCDK tab sis sab laug hnyav hydronephrosis. Tom qab piav qhia txog kev phais rau cov niam txiv thiab tau txais kev tso cai, pyeloplasty tau ua thaum muaj hnub nyoog 1 xyoos. Cov chav kawm tsis zoo, suav nrog lub sijhawm ua haujlwm. Teb Chaws Asmeskas thaum muaj hnub nyoog 1 xyoos thiab 8 lub hlis (8 lub hlis tom qab phais) pom tias lub raum pelvis o ntawm lub raum sab laug tau zoo dua me ntsis (Daim duab 2g). Tam sim no, tsis muaj qhov hnyav dua ntawm eGFR, thiab ob qho tib si urinary protein / Cr piv (0.13 g / gCr) thiab urinary MG / Cr ratio (3.04 þ g / mgCr) nyob rau hauv ib txwm muaj.

Kuj tseem muaj cov kev tshawb pom uas muaj txiaj ntsig zoo hauv kev sib txawv ntawm ob, xws li cov cyst loj tshaj plaws nyob rau hauv qhov chaw hauv lub raum pelvic-ureteric junction obstruction, whereas nws nyob rau peripheral hauv MCDK8), 9). Txawm hais tias qhov ci ntsa iab ntawm fetal US nyob rau hauv cov ntaub ntawv no tsis meej, qhov chaw ntawm lub cyst loj tshaj plaws yog lub hauv paus, ib qho kev tshawb pom uas qhia tias hydronephrosis. Tus kws kho mob obstetrician thiab gynecologist uas tau kuaj xyuas nws thawj zaug kuaj mob hydronephrosis, tab sis vim nws muaj keeb kwm ntawm tsev neeg muaj mob polycystic raum, tom qab ntawd nws tau kuaj pom tias muaj kab mob raum, nrog rau MCDK. Nws piav qhia rau nws tsev neeg txog kev muaj kab mob raum. Hauv qhov no, leej txiv tsis tau lees paub tias tau tsim muaj kab mob raum (tsuas yog Asmeskas tau ua tiav; kev kuaj caj ces tsis tau ua), thiab tus menyuam qhov txhab tau nyob rau sab laug raum. Qhov muaj peev xwm ntawm caj ces txawv txav tau xav tias tsawg. Kev ntsuam xyuas caj ces tau ua rau nws siv CAKUT vaj huam sib luag (xws li PKD1, PKD2, PKHD1, thiab HNF1B) ntawm Department of Pediatrics, Kobe University School of Medicine, thiab tsis muaj kev hloov pauv caj ces hauv tus menyuam. Lub tsev kho mob uas tus neeg mob tau thov kom ua raws li kev soj ntsuam tom qab yug me nyuam tsis muaj txoj cai tshwj xeeb rau kev sim lossis kev kho mob kom sib txawv ntawm ob, uas ua rau tus neeg mob tsev neeg muaj kev ntxhov siab ntau ntxiv txog cov caj ces ntawm tus kab mob. Nws muaj peev xwm hais tias nyob rau hauv cov ntaub ntawv no, peb yuav tsum tau piav qhia hais tias qhov muaj peev xwm ntawm hereditary cystic raum kab mob yog tsawg raws li lub unilateral xwm thiab postnatal ultrasound pom, thiab ces mus rau qhov sib txawv ntawm qhov hnyav hydronephrosis thiab MCDK. Nws ua tiav. Txawm hais tias tom qab ua raws li MCDK, qhov kev cuam tshuam ntawm lub raum tso zis txawv txav tau pom nyob rau hauv kwv yees li 1/3 ntawm cov neeg mob, thiab hydronephrosis, ureterocele, hypoplastic lub raum, thiab cov kab mob hauv qhov chaw mos uas txawv txav uas tsis yog lub raum urinary kuj tshwm sim. Nws raug suav tias yog tsim nyog los npaj cov kev ntsuam xyuas thaum ntxov, ua tib zoo xav txog poj niam txiv neej ntawm tus neeg mob.

Qhov taw qhia rau kev phais mob hnyav hydronephrosis (SFU kev faib tawm qib 3 txog 4) yog txiav txim siab raws li kev txiav txim siab ntawm ib feem ntawm lub raum ua haujlwm.<40%, poor urinary drainage, and rate of decrease in partial kidney function >5 mus rau 10% 10) ... Hauv qhov no, tsis muaj qhov txo qis hauv VUR, ntshav Cr, lossis eGFR tau pom, thiab tus neeg mob muaj asymptomatic hydronephrosis, tab sis kev soj ntsuam ntawm lub raum ua haujlwm tau pom tias txo qis dua 40% ntawm lub raum sab laug thiab cov kua dej tsis zoo. tau siv los ua tus txheej txheem. Cov chav kawm tom qab phais, suav nrog lub sijhawm perioperative, yog qhov tsis zoo, thiab tsis muaj lub raum tsis ua haujlwm tau tshwm sim lossis ua tiav, txawm hais tias kev nthuav dav ntawm sab laug lub raum pelvis thiab calyx tseem nyob. Nws paub tias hydronephrosis, uas tshwm sim lig rau hauv tus menyuam hauv plab los yog vim muaj kev cuam tshuam ib nrab, kuj ua rau lub raum cortex thinning, fibrosis, thiab txo tus naj npawb ntawm nephrons11), 12) thiab cov me nyuam tau kuaj pom prenatally nrog CAKUT. Hauv kev tshawb fawb ntawm 822 tus neeg, hydronephrosis tau pom tias yog ib qho kev pheej hmoo siab rau cov kab mob raum ntev (kev phom sij piv 5.20), uas qhia tias kev ua tib zoo saib xyuas yog qhov tsim nyog13).

Xaus

Qhov tseem ceeb ntawm kev kuaj mob fetal siv fetal US yog qhov tseem ceeb heev, tab sis muaj ntau yam kab mob uas tuaj yeem ua rau muaj teeb meem hauv kev kuaj mob CAKUT, suav nrog kab mob raum, thiab kev txiav txim siab ntau dua thiab kev piav qhia txaus yuav tsum tau xav txog hauv tsev neeg keeb kwm yav dhau los. Nws yog.

Kev lees paub

Peb xav ua tsaug rau Dr. Ijima Issei, Dr. Toro Nozu, thiab Dr. Naoya Morisada ntawm Department of Pediatrics, Department of Internal Medicine, Kobe University Graduate School of Medicine, rau kev ua cov tshuaj ntsuam genetic test rau rooj plaub no . "Tsis muaj kev tshaj tawm txog kev tsis sib haum xeeb ntawm kev txaus siab raws li cov qauv tsim los ntawm Japanese Society of Pediatric Nephrology."

Cov ntawv nyeem

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Pediatr Urol 2010; 6: {2}}.

3) Shokeir AA, Nijman RJ: Antenatal hydronephrosis: hloov cov ntsiab lus hauv kev kuaj mob thiab kev tswj xyuas tom ntej. BJU Int 2000;

85: 987-994.

4) Kenji Shimada, Tomi Matsumoto, Futoshi Matsui, Toshihiko Idesako: fetal theem ntawm CAKUT kuaj thiab kho. Lub raum thiab Dialysis 2010; 68: 3:ua.

5) Hiroyuki Noguchi, Hideo Takamatsu, Hiroyuki Tahara, Takahiko Fukushige, Yasuo Adachi, Kajiya Hiroshi, Ken Kaji, Ryuichi Shimono, thiab Hiroshi Akiyama. Unilateral polycystic dysplastic raum:

Kev kuaj mob ua ntej yug menyuam. Japanese Journal of Foreign Society 1993; 29: {2}}.

6) Shunichi Makino, Hiroaki Komuro, Akira Nishi, Hideo Nagai, Mayumi Nagai, Akinori Onoe, Michiru Nakamura, Mariko Momoi, Naonori Mizukami: Kev kuaj mob ua ntej ntawm lub raum: Tshwj xeeb

Kev tshawb fawb txog 2 tus neeg mob uas muaj cysts thiab lub raum o ua kom pom kev pom ntawm daim duab. hnub tsev kawm ntawv Foreign Society Journal 2000; 36: {3}}.

7) Kenji Yoneyama, Yasuji Katayama, Masayuki Takeda, Shotaro Sato, Michio Ishida, Yoshizawa Hiroshi. Kev paub txog kev kho lub raum thiab cov zis malformations kuaj pom ua ntej yug menyuam. phau ntawv journal

1993; 84: 1275-1280.

8) Sanders RC, Hartman DS: Qhov sib txawv ntawm cov tub ntxhais hluas lub raum thiab hydronephrosis. Radiology 1984;

151: 621-625.

9) Keizo Taguchi, Kenji Shimada, Fumihiko Ikoma. Lub raum thiab urinary malformations tau kuaj pom ua ntej yug menyuam. Phau ntawv Journal ntawm Nyiv 1991; 37: {3}}.

10) Pediatric congenital hydronephrosis (pelvic-ureteric transition obstruction) qhia kev kho mob. Hnub Journal ntawm Pediatric Urology 2016; 25: {4}}.