Singleton newborn with bilateral renal and ureteric agenesis, without features of potter sequence

2021 ◽  
Vol 4 (1) ◽  
pp. 40
Author(s):  
WA Lasanthi Kumari Weerasooriya ◽  
Shenal Thalgahagoda ◽  
Dinesh Rangana ◽  
Mathula Hettiarachci
Keyword(s):  
2004 ◽  
Vol 128 (3) ◽  
pp. e44-e45
Author(s):  
Julieta E. Barroeta ◽  
Gary A. Stopyra

Abstract We describe the case of a 36-week gestational-age male stillborn with bilateral renal agenesis and a 47,XXY karyotype, as well as features of Potter sequence. No other congenital abnormalities were noted. Severe oligohydramnios was diagnosed prenatally at 30 weeks, and cytogenetic analysis was performed postmortem. Urinary tract anomalies are uncommon in association with Klinefelter syndrome. Unilateral renal agenesis has been described. We describe, to our knowledge, the first case of bilateral renal agenesis in association with 47,XXY.


1997 ◽  
Vol 88 (7) ◽  
pp. 664-669
Author(s):  
Kenji Shimada ◽  
Shozo Hosokawa ◽  
Fumi Matsumoto ◽  
Seiji Matsumoto

1997 ◽  
Vol 34 (5) ◽  
pp. 423-425 ◽  
Author(s):  
K Devriendt ◽  
P Moerman ◽  
D Van Schoubroeck ◽  
K Vandenberghe ◽  
J P Fryns

2020 ◽  
Vol 58 (223) ◽  
pp. 178-180
Author(s):  
Uttara Gautam ◽  
Rishi Kesh Kafle ◽  
Vijay Chikanbanjar ◽  
Alyssa Shakya ◽  
Rydam Basnet ◽  
...  

Potter sequence is a rare congenital malformation that primarily affects male fetuses and is characterized by pulmonary hypoplasia, skeletal malformation, and kidney abnormalities. The pressure of the uterine wall due to oligohydramnios leads to an unusual facial appearance, abnormal limbsor limbs in abnormal positions or contractures. The fetus generally dies soon after birth due to respiratory insufficiency. We presented a male baby of 35 wks gestation with birth weight 1200gms delivered by primi mother. She had severe oligohydramnios and virtually there was no liquor during birth. The baby had severe perinatal depression at birth requiring resuscitation. Multiple congenital anomalies like absence of left eye, congenital cataract on the right eye, right-sided choanal atresia, micrognathia, low set ears, beaked nose, bilateral clubbed foot with hip deformity were noted. After 2 hours of life,baby developed fast breathing and cyanosis and died due to respiratory failure.


Author(s):  
Katherine Lizeth Muñoz-Murillo ◽  
Willfrant Jhonnathan Muñoz-Murillo ◽  
Urías De Jesús Hernández-López ◽  
Laura María Aponte-Ceballos ◽  
Ivan David Lozada-Martínez ◽  
...  

2016 ◽  
Vol 58 (7) ◽  
pp. 604-606
Author(s):  
Jun Miyahara ◽  
Masaki Yamamoto ◽  
Kyoko Motoshige ◽  
Naoya Fujita ◽  
Shigeru Ohki

2001 ◽  
Vol 265 (4) ◽  
pp. 219-220 ◽  
Author(s):  
M. Arai ◽  
S. Suzuki ◽  
H. Ishino ◽  
K. Igarashi ◽  
T. Araki

2021 ◽  
Vol 25 (3) ◽  
pp. 68-74
Author(s):  
E. F. Andreeva ◽  
N. D. Savenkova

THE AIM:to describe the causes, pathogenesis, clinical course and outcome of Potter sequence in children with cystic kidney disease. PATIENTS AND METHODS:the follow-up study of 23 newborns with cystic kidney disease was studied, in which renal oligohydramnios (ROH) was confirmed prenatally by ultrasound (US). RESULTS:Of the 155 children with autosomal dominant polycystic kidney disease (ADPKD), 8 (5,2 %) prenatal after 30 weeks of gestation established ROH, at 26-32 weeks of gestation – cyst in the kidney by US, in 2 of them ROH confirmed simultaneously with the detection of cysts in kidneys of a fetus, 6 – late detection of kidney cysts. Of the 8 newborns with a very early onset ADPKD, prenatal developed in ROH conditions, in 2 (25 %) in the neonatal period diagnosed the Potter phenotype. Of the 20 children with autosomal recessive polycystic kidney disease (ARPKD), 12 (60 %) prenatally revealed ROH after 18 weeks of gestation prenatally, of these, 8 (67 %) in the neonatal period diagnosed the Potter phenotype. Of the 12 newborns with ARPKD, that developed in ROH conditions, in 5 (42 %) kidney cysts were detected prenatally by US at 32-37 weeks of gestation, in 7 (58 %) in the neonatal period. ROH and the Potter phenotype are more common with ARPKD in the fetus than with ADPKD. Among children with ARPKD and ADPKD undergoing ROH, no statistically significant differences in the frequency of deaths in the neonatal and infancy. The characteristics of course and outcome of the Potter sequence in the neonatal and infant periods in a boy with deletion of 12p and cystic kidney disease are described. ROH in 2 children with cystic kidneys and coloboma of the optic nerve disc did not lead to the formation of the Potter phenotype. In 15 children with multicystic kidney prenatal US showed no ROH. CONCLUSION: the results of a follow-up study of children after ROH and the course of the Potter sequence for different cystic kidney disease in children are presented.


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