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Megabladder, congenital(MGBL)

MedGen UID:
1684806
Concept ID:
C5231472
Congenital Abnormality
Synonyms: MEGABLADDER, CONGENITAL; MGBL
 
Gene (location): MYOCD (17p12)
 
Monarch Initiative: MONDO:0032879
OMIM®: 618719

Definition

Congenital megabladder (MGBL) is characterized by a massively dilated bladder with disrupted smooth muscle in the bladder wall. MGBL is a sex-limited trait with 95% male predominance, likely the result of differences in urethra and bladder development and length differences in urethra between males and females (Houweling et al., 2019). [from OMIM]

Clinical features

From HPO
Stage 5 chronic kidney disease
MedGen UID:
384526
Concept ID:
C2316810
Disease or Syndrome
A degree of kidney failure severe enough to require dialysis or kidney transplantation for survival characterized by a severe reduction in glomerular filtration rate (less than 15 ml/min/1.73 m2) and other manifestations including increased serum creatinine.
Fetal megacystis
MedGen UID:
419338
Concept ID:
C2931117
Disease or Syndrome
Fetal megacystis is an abnormally enlarged bladder identified at any gestational age.
Hyperechogenic kidneys
MedGen UID:
477530
Concept ID:
C3275899
Finding
An increase in amplitude of waves returned in ultrasonography of the kidney, which is generally displayed as increased brightness of the signal.
Multiple glomerular cysts
MedGen UID:
893156
Concept ID:
C4022013
Pathologic Function
The presence of many cysts in the glomerulus of the kidney related to dilatation of the Bowman's capsule.
Patent ductus arteriosus
MedGen UID:
4415
Concept ID:
C0013274
Congenital Abnormality
In utero, the ductus arteriosus (DA) serves to divert ventricular output away from the lungs and toward the placenta by connecting the main pulmonary artery to the descending aorta. A patent ductus arteriosus (PDA) in the first 3 days of life is a physiologic shunt in healthy term and preterm newborn infants, and normally is substantially closed within about 24 hours after bith and completely closed after about three weeks. Failure of physiologcal closure is referred to a persistent or patent ductus arteriosus (PDA). Depending on the degree of left-to-right shunting, PDA can have clinical consequences.
Atrial septal defect
MedGen UID:
6753
Concept ID:
C0018817
Congenital Abnormality
Atrial septal defect (ASD) is a congenital abnormality of the interatrial septum that enables blood flow between the left and right atria via the interatrial septum.
Ventricular septal defect
MedGen UID:
42366
Concept ID:
C0018818
Congenital Abnormality
A hole between the two bottom chambers (ventricles) of the heart. The defect is centered around the most superior aspect of the ventricular septum.
Bicuspid aortic valve
MedGen UID:
57436
Concept ID:
C0149630
Congenital Abnormality
Aortic valve disease-2 (AOVD2) is characterized by bicuspid aortic valve (BAV) and dilation of the ascending aorta. Calcification of the valve and the aorta has been observed, and some patients exhibit coarctation of the aorta (Tan et al., 2012; Luyckx et al., 2019; Park et al., 2019). For a general phenotypic description and a discussion of genetic heterogeneity of aortic valve disease, see AOVD1 (109730).
Left ventricular noncompaction cardiomyopathy
MedGen UID:
866782
Concept ID:
C4021133
Disease or Syndrome
Left ventricular non-compaction (LVNC) is characterized by prominent left ventricular trabeculae and deep inter-trabecular recesses. The myocardial wall is often thickened with a thin, compacted epicardial layer and a thickened endocardial layer. In some patients, LVNC is associated with left ventricular dilatation and systolic dysfunction, which can be transient in neonates.

Recent clinical studies

Etiology

Xin G, Chen R, Zhang X
Mol Med Rep 2018 Sep;18(3):2651-2660. Epub 2018 Jul 16 doi: 10.3892/mmr.2018.9284. PMID: 30015886Free PMC Article
Xin G, Chen R, Zhang X
Gene 2018 Apr 15;650:77-85. Epub 2018 Feb 2 doi: 10.1016/j.gene.2018.01.063. PMID: 29410288

Diagnosis

Jordan P, Dorval G, Arrondel C, Morinière V, Tournant C, Audrezet MP, Michel-Calemard L, Putoux A, Lesca G, Labalme A, Whalen S, Loeuillet L, Martinovic J, Attie-Bitach T, Bessières B, Schaefer E, Scheidecker S, Lambert L, Beneteau C, Patat O, Boute-Benejean O, Molin A, Guimiot F, Fontanarosa N, Nizon M, Lefebvre M, Jeanpierre C, Saunier S, Heidet L
Hum Mutat 2022 Mar;43(3):347-361. Epub 2022 Jan 10 doi: 10.1002/humu.24324. PMID: 35005812
Ruano R, Yoshizaki CT, Giron AM, Srougi M, Zugaib M
Ultrasound Obstet Gynecol 2014 Aug;44(2):238-40. Epub 2014 Jun 26 doi: 10.1002/uog.13293. PMID: 24375864
Guez S, Assael BM, Melzi ML, Tassis B, Nicolini U
J Pediatr Surg 1996 Oct;31(10):1401-4. doi: 10.1016/s0022-3468(96)90838-6. PMID: 8906671

Prognosis

Carpenter AR, McHugh KM
Pediatr Nephrol 2017 Apr;32(4):557-564. Epub 2016 Apr 26 doi: 10.1007/s00467-016-3385-6. PMID: 27115886Free PMC Article
Guez S, Assael BM, Melzi ML, Tassis B, Nicolini U
J Pediatr Surg 1996 Oct;31(10):1401-4. doi: 10.1016/s0022-3468(96)90838-6. PMID: 8906671

Clinical prediction guides

Jordan P, Dorval G, Arrondel C, Morinière V, Tournant C, Audrezet MP, Michel-Calemard L, Putoux A, Lesca G, Labalme A, Whalen S, Loeuillet L, Martinovic J, Attie-Bitach T, Bessières B, Schaefer E, Scheidecker S, Lambert L, Beneteau C, Patat O, Boute-Benejean O, Molin A, Guimiot F, Fontanarosa N, Nizon M, Lefebvre M, Jeanpierre C, Saunier S, Heidet L
Hum Mutat 2022 Mar;43(3):347-361. Epub 2022 Jan 10 doi: 10.1002/humu.24324. PMID: 35005812
Carpenter AR, McHugh KM
Pediatr Nephrol 2017 Apr;32(4):557-564. Epub 2016 Apr 26 doi: 10.1007/s00467-016-3385-6. PMID: 27115886Free PMC Article
Ingraham SE, Saha M, Carpenter AR, Robinson M, Ismail I, Singh S, Hains D, Robinson ML, Hirselj DA, Koff SA, Bates CM, McHugh KM
Pediatr Res 2010 Dec;68(6):500-7. doi: 10.1203/PDR.0b013e3181f82f15. PMID: 20736884Free PMC Article
Guez S, Assael BM, Melzi ML, Tassis B, Nicolini U
J Pediatr Surg 1996 Oct;31(10):1401-4. doi: 10.1016/s0022-3468(96)90838-6. PMID: 8906671

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