"Potter sequence"

Beyond Potter's sequence: a systematic review of prenatally diagnosed prognostic factors in CAKUT PROSPEROInternational prospective register of systematic reviews Print | PDFBeyond Potter's sequence: a systematic review of prenatally diagnosed prognostic factors in CAKUTGiovanni Viggiano, Ricardo Teles Filho, Waldemar AmaralTo enable PROSPERO to focus on COVID-19 submissions, this registration . Beyond Potter's sequence: a systematic review of prenatally diagnosed prognostic factors in CAKUT. PROSPERO 2023 CRD42023395108 Available from: https://www.crd.york.ac.uk/prospero/display_record.php?ID=CRD42023395108Review questionWhat are the the prenatally diagnosed signs that correlate to perinatal death in CAKUT, other than pulmonary hypoplasia?Are there any sonographic or biochemical markers

by pressure in utero due to oligohydramnios, classically due to bilateral renal agenesis (BRA) but it can occur with other conditions, including infantile polycystic kidney disease, renal hypoplasia and obstructive uropathy. Some feel that Potter's sequence is more appropriate terminology since not every individual with the syndrome has exactly the same set of symptoms and signs but rather they share -stage renal failure, dialysis or transplantation will be needed.PrognosisPotter's syndrome with BRA is incompatible with life[12]. Babies with Potter's sequence due to other causes have a better chance of survival.Oligohydramnios of renal origin has tended to be associated with a very poor outcome but this is not invariably so and the outlook may be improving[13]. In one study, looking at long-term

J; Renal dysplasia in the neonate. Curr Opin Pediatr. 2016 Apr28(2):209-15. doi: 10.1097/MOP.0000000000000324.Shastry SM, Kolte SS, Sanagapati PR; Potter's Sequence. J Clin Neonatol. 2012 Jul1(3):157-9. doi: 10.4103/2249-4847.101705.Dicker D, Samuel N, Feldberg D, et al; The antenatal diagnosis of Potter syndrome (Potter sequence). A lethal and not-so-rare malformation. Eur J Obstet Gynecol Reprod

of membranes lead to the same clinical findings. Hence, the terms Potter sequence or oligohydramnios sequence emerged. Regardless of the root cause for oligohydramnios, the terms Potter syndrome, Potter sequence, and oligohydramnios sequence are used interchangeably in the published literature.Previous Next: PathophysiologyPrior to 16 weeks' gestation, the amount of amniotic fluid is dependent J, Kramer A, Hall JG, Pauli RM, Schimke RN, et al. Dominantly inherited renal adysplasia. Am J Med Genet. Apr/1987. 26(4):863-72. [QxMD MEDLINE Link]. 30. Curry CJ, Jensen K, Holland J, Miller L, Hall BD. The Potter sequence: a clinical analysis of 80 cases. Am J Med Genet. 1984 Dec. 19(4):679-702. [QxMD MEDLINE Link]. 31. McPherson E. Renal anomalies in families

in CEP55, present at low frequency in the Amish community, in two siblings presenting with a lethal foetal disorder. The features of the condition are reminiscent of a Meckel-like syndrome comprising of Potter sequence, hydranencephaly, and cystic dysplastic kidneys. These findings, considered alongside two recent studies of single families reporting loss of function candidate variants in CEP55, confirm

Fetal renin-angiotensin-system blockade syndrome: renal lesions. Fetuses exposed to angiotensin-converting enzyme inhibitors or angiotensin receptor antagonists during the second and/or third trimesters of gestation are at high risk of developing severe complications. They consist in fetal hypotension, and anuria/oligohydramnios leading to Potter sequence, frequently associated with hypocalvaria

Renal tubular dysgenesis. Renal tubular dysgenesis (RTD) is a severe foetal disorder characterised by the absence or poor development of proximal tubules, early onset and persistent anuria (leading to oligohydramnios and the Potter sequence) and ossification defects of the skull. In most cases, early death occurs from pulmonary hypoplasia, anuria and refractory arterial hypotension. RTD may

of membranes lead to the same clinical findings. Hence, the terms Potter sequence or oligohydramnios sequence emerged. Regardless of the root cause for oligohydramnios, the terms Potter syndrome, Potter sequence, and oligohydramnios sequence are used interchangeably in the published literature.Previous Next: PathophysiologyPrior to 16 weeks' gestation, the amount of amniotic fluid is dependent J, Kramer A, Hall JG, Pauli RM, Schimke RN, et al. Dominantly inherited renal adysplasia. Am J Med Genet. Apr/1987. 26(4):863-72. [QxMD MEDLINE Link]. 30. Curry CJ, Jensen K, Holland J, Miller L, Hall BD. The Potter sequence: a clinical analysis of 80 cases. Am J Med Genet. 1984 Dec. 19(4):679-702. [QxMD MEDLINE Link]. 31. McPherson E. Renal anomalies in families

of membranes lead to the same clinical findings. Hence, the terms Potter sequence or oligohydramnios sequence emerged. Regardless of the root cause for oligohydramnios, the terms Potter syndrome, Potter sequence, and oligohydramnios sequence are used interchangeably in the published literature.Previous Next: PathophysiologyPrior to 16 weeks' gestation, the amount of amniotic fluid is dependent J, Kramer A, Hall JG, Pauli RM, Schimke RN, et al. Dominantly inherited renal adysplasia. Am J Med Genet. Apr/1987. 26(4):863-72. [QxMD MEDLINE Link]. 30. Curry CJ, Jensen K, Holland J, Miller L, Hall BD. The Potter sequence: a clinical analysis of 80 cases. Am J Med Genet. 1984 Dec. 19(4):679-702. [QxMD MEDLINE Link]. 31. McPherson E. Renal anomalies in families

Genet. Apr/1987. 26(4):863-72. [QxMD MEDLINE Link]. 30. Curry CJ, Jensen K, Holland J, Miller L, Hall BD. The Potter sequence: a clinical analysis of 80 cases. Am J Med Genet. 1984 Dec. 19(4):679-702. [QxMD MEDLINE Link]. 31. McPherson E. Renal anomalies in families of individuals with congenital solitary kidney. Genet Med. 2007 May. 9(5):298-302. [QxMD MEDLINE Link

or at birth, is variable and in the most severe cases includes Potter-sequence, oligohydramnios, pulmonary hypoplasia, and massively enlarged echogenic kidneys.ORPHA:731Classification level: Disorder * Synonym(s): * AR-PKD * Prevalence: Unknown * Inheritance: Autosomal recessive * Age of onset: All ages * * ICD-10: Q61.1 * OMIM: 263200 617610 * UMLS: C0085548 * MeSH: D017044 * GARD: 8378 * MedDRA

Renin-angiotensin system in kidney development: renal tubular dysgenesis. Autosomal recessive renal tubular dysgenesis (RTD) is a severe disorder of renal tubular development characterized by early onset and persistent fetal anuria leading to oligohydramnios and the Potter sequence. At birth, blood pressure is dramatically low and perinatal death occurs in most cases. Skull ossification defects

Renal tubular dysgenesis, a not uncommon autosomal recessive disorder leading to oligohydramnios: Role of the Renin-Angiotensin system. Renal tubular dysgenesis is a clinical disorder that is observed in fetuses and characterized by the absence or poor development of proximal tubules, early onset and persistent oligohydramnios that leads to the Potter sequence, and skull ossification defects

* v * t * e Congenital malformations and deformations of urinary system Abdominal Kidney * Renal agenesis/Potter sequence, Papillorenal syndrome

resources * eMedicine: ped/2357 radio/572 * v * t * e Congenital malformations and deformations of urinary system Abdominal Kidney * Renal agenesis/Potter sequence

Chromosome 22q11 deletion presenting as the Potter sequence. A female fetus with the Potter sequence, caused by unilateral renal agenesis and contralateral multicystic renal dysplasia, was found to have a submicroscopic deletion in chromosome 22q11. The only associated anomaly was agenesis of the uterus and oviducts (Von Mayer-Rokitansky-Küster anomaly). The deletion was inherited from the father

Bilateral Optic Disc Anomalies Associated with PAX2 Mutation in a Case of Potter Sequence PURPOSE: To describe the ophthalmic findings in the fundus of a Japanese infant with Potter sequence having a mutation in the PAX2 gene. METHODS: A 1-month-old infant diagnosed with Potter sequence who had bilateral renal hypoplasia and a mutation in the PAX2 gene was subjected to detailed ophthalmic with a PAX2 mutation. CONCLUSIONS: This report shows ophthalmic findings in the youngest patient with PRS and PAX2-associated Potter sequence. Optic disc anomalies may be involved in some infants with Potter sequence. We anticipate an increase in opportunities for ophthalmic examinations in infants with diseases such as Potter sequence with previously high mortality rates.

Congenital malformations and deformations of urinary system Abdominal Kidney * Renal agenesis/Potter sequence, Papillorenal syndrome * cystic * Polycystic kidney disease * Meckel syndrome

Potter sequence Potter sequence - WikipediaPotter sequenceFrom Wikipedia, the free encyclopediaJump to navigationJump to searchNot to be confused with Doege–Potter syndrome or Pott's disease.Medical conditionPotter sequence Other names Potter's syndrome, Potter's sequence, oligohydramnios sequence Specialty Medical genetics Potter sequence is the atypical physical appearance of a baby due to oligohydramnios experienced when in the uterus.[1] It includes clubbed feet, pulmonary hypoplasia and cranial anomalies related to the oligohydramnios.[clarification needed] Oligohydramnios is the decrease in amniotic fluid volume sufficient to cause deformations in morphogenesis of the baby.Oligohydramnios is the cause of Potter sequence