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Multicystic Dysplastic Kidney (Multicystic Kidney)

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A nongenetic defect due to malformation of the KIDNEY which appears as a bunch of grapes with multiple renal cysts but lacking the normal renal bean shape, and the collection drainage system. This condition can be detected in-utero with ULTRASONOGRAPHY.

Also Known As:

Multicystic Kidney; Bilateral Multicystic Dysplastic Kidneys; Multicystic Kidney Dysplasia; Multicystic Renal Dysplasia; Unilateral Multicystic Dysplastic Kidney; Dysplasia, Multicystic Kidney; Dysplasia, Multicystic Renal; Dysplasias, Multicystic Kidney; Dysplasias, Multicystic Renal; Kidney Dysplasia, Multicystic; Kidney Dysplasias, Multicystic; Multicystic Dysplastic Kidneys; Multicystic Kidney Dysplasias; Multicystic Kidneys; Multicystic Renal Dysplasias; Renal Dysplasia, Multicystic; Renal Dysplasias, Multicystic

Networked: 180 relevant articles (1 outcomes, 9 trials/studies)

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Disease Context: Research Results

Related Diseases

1.	Kidney Diseases (Kidney Disease)
2.	Abdominal Pain (Pain, Abdominal)
3.	Kidney Neoplasms (Kidney Cancer)
4.	Hydronephrosis
5.	Polycystic Kidney Diseases (Polycystic Kidney Disease)

Experts

1.	Antignac, Corinne: 4 articles (01/2014 - 06/2010)
2.	Heidet, Laurence: 3 articles (01/2014 - 06/2010)
3.	Schreuder, Michiel F: 3 articles (10/2012 - 06/2008)
4.	Salomon, Rémi: 3 articles (12/2011 - 06/2010)
5.	Miyano, Takeshi: 3 articles (05/2005 - 01/2004)
6.	Yamataka, Atsuyuki: 3 articles (05/2005 - 01/2004)
7.	Kaneko, Kazunari: 3 articles (05/2005 - 01/2004)
8.	Loget, Philippe: 2 articles (01/2014 - 07/2011)
9.	Tonni, Gabriele: 2 articles (09/2013 - 01/2008)
10.	Martínez-Frías, María Luisa: 2 articles (09/2013 - 12/2007)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Multicystic Dysplastic Kidney:

1.	Hereditary renal agenesisIBA 10/01/2011 - "As many studies suggest that renal malformations are more often found on the left side, a meta-analysis was performed on the distribution of five different unilateral anomalies: multicystic dysplastic kidney, renal agenesis/aplasia, renal ectopia, pelviureteral junction obstruction, and non-obstructive non-refluxing megaureter. " 01/01/2006 - "This study aimed to determine the frequency of associated urological abnormalities in children with unilateral renal agenesis (RA) or multicystic dysplastic kidney (MCDK). " 10/01/2013 - "Most common associated anomaly was kidney anomalies (multicystic kidney disease and renal agenesis/dysplasia) in 8 (8.2%) patients. " 12/01/2012 - "To determine the degree of compensatory enlargement, and its time of onset, of the solitary functioning kidney (SFK) in fetuses with unilateral renal agenesis (URA) or unilateral multicystic kidney dysplasia (MCKD). " 01/01/2012 - "Patients with unilateral MultiCystic Kidney Dysplasia (MCKD) or unilateral renal agenesis (URA) have a congenital solitary functioning kidney (CSFK) that is compensatory enlarged. "
2.	Transcription Factors (Transcription Factor)IBA 06/01/2001 - "Immunohistochemical studies on the younger sibling revealed hyperproliferative dysplastic kidney tubules which overexpressed PAX2, a potentially oncogenic transcription factor, and BCL2, a cell-survival factor, surrounded by metaplastic, alpha smooth-muscle actin-positive stroma: similar patterns have been observed in patients with non-syndromic multicystic dysplastic kidneys. " 01/01/2011 - "Lim1, an embryonal transcription factor, is absent in multicystic renal dysplasia, but reactivated in nephroblastomas." 06/01/2008 - "Transcription factor 2 gene mutation may be responsible for approximately one-third of children having isolated renal cysts, multicystic dysplastic kidneys, oligomeganephronia, hypo-dysplastic kidneys, horseshoe kidneys, and hyperechogenic kidneys. " 01/01/2014 - "The aim of this study was to document the association between pancreatic agenesis or hypoplasia and multicystic renal dysplasia related to transcription factor 2 (TCF2) or hepatocyte nuclear factor 1 beta mutations. "
3.	DNA (Deoxyribonucleic Acid)IBA 08/01/1990 - "These findings do not support or negate the potential for neoplasm associated with multicystic dysplastic kidney, since a diploid deoxyribonucleic acid pattern does not eliminate the possibility of the future development of malignancy." 08/01/2009 - "DNA samples from 26 unrelated children with unilateral renal agenesis (URA), 20 with renal hypodysplasia (RHD) and 13 with multicystic dysplastic kidney (MCDK) were included in the study. " 08/01/1990 - "In an attempt to determine whether a relationship exists between renal dysplasia and neoplasia in terms of abnormalities of cellular deoxyribonucleic acid content we performed flow cytometric evaluation on 30 formalin fixed, paraffin embedded archival specimens of multicystic dysplastic kidneys. "
4.	Hepatocyte Nuclear Factor 1-betaIBA 01/01/2014 - "TCF2/HNF-1beta mutations: 3 cases of fetal severe pancreatic agenesis or hypoplasia and multicystic renal dysplasia." 01/01/2014 - "The aim of this study was to document the association between pancreatic agenesis or hypoplasia and multicystic renal dysplasia related to transcription factor 2 (TCF2) or hepatocyte nuclear factor 1 beta mutations. "
5.	ReninIBA 10/01/2002 - "Reduced renin expression and altered gene transcript profiles in multicystic dysplastic kidneys." 11/01/2001 - "Our observations suggest that multicystic dysplastic kidney may have the ability to produce renin. " 11/01/2001 - "Immunostaining of renin was observed in 26 of 29 multicystic dysplastic kidneys (90%). " 11/01/2001 - "To assess renin production we examined the distribution of renin containing cells in multicystic dysplastic kidneys using immunohistochemical methods. " 11/01/2001 - "Renin containing cells are present predominantly in scarred areas but not in dysplastic regions in multicystic dysplastic kidney."
6.	Anorectal atresiaIBA 12/15/2007 - "These included: anorectal atresia/stenosis (FR = 2.81; P = 0.03), hallucal polydactyly (FR = 3.62; P = 0.002), heterotaxy (FR = 5.70; P = 0.049), hypertrophic cardiomyopathy (HCM) (FR = 61.60; P = 0.000000), multicystic dysplastic kidneys (MDK) (FR = 5.13; P = 0.0002), and thymus aplasia/hypoplasia (FR = 29.62; P = 0.000001). " 01/01/1990 - "Report of the observation of a female newborn presenting a complex malformative set: distal anorectal malformation without fistula; bladder exstrophy without epispadias, with cutaneous bridge; congenital urethrovaginal fistula; malformative sexual ambiguity with bulky genital tubercle, whole urethra, urethral meatus on the apex of the genital tubercle, associated with a mid labial genital joint and a high genital orifice; unilateral multicystic kidney; vertebral abnormalities with supernumerary thoracolumbar vertebrae; no karyotype abnormalities and no familial pathological history; no endocrine abnormalities. " 01/01/1981 - "The clinical features--including trigonocephaly, epicanthal folds, coloboma of iris, prominent occiput, dysplastic ears, carp-shaped mouth, micrognathia, pectus excavalum single umbilical artery, anorectal malformation, dysplastic fifth fingers, hypoplastic nails, simian creases, hydronephrotic multicystic kidney, neutropenia, thrombocytopenia, and congenital heart anomalies--are compatible with the majority of reported cases. " 11/01/2010 - "The 95 ultrasound reports found in our patients described 270 abnormalities, the most frequent were: abdominal/pelvic cystic/mass (39), hydronephrosis (36), oligohydramnios (23), distended bowel/bowel obstruction (19), ascites (15), 2 vessel cord (14), dilated bladder (14), dilated ureter (14), polyhydramnios (10), echogenic bowel (8), multicystic kidney (8), "ambiguous genitalia" (7), hydrops fetalis (7), hydrocolpos (4), absent kidney (3), abnormal spine (3), and anorectal atresia (3). "
7.	bilateral Multicystic renal dysplasiaIBA 11/01/2011 - "The majority of total nephrectomies were performed for multicystic dysplastic kidneys, reflux nephropathy, pelvi-ureteric junction obstruction and dysplasia. " 08/01/2007 - "Of these, five had increasing hydronephrosis and required surgery for pelvi-ureteric junction obstruction, and three had a multicystic dysplastic kidney on postnatal scanning. " 12/01/1996 - "The 65 fetuses assessed had the following underlying causes: transient in utero hydronephrosis 34 (52.3%), idiopathic hydronephrosis 9 (13.8%), isolated vesico-ureteric reflux 5 (7.6%), vesico-ureteric reflux with other urological abnormalities 2 (3.1%), pelvi-ureteric junction obstruction 2 (3.1%), equivocal pelvi-ureteric junction obstruction/idiopathic hydronephrosis 4 (6.2%), multicystic dysplastic kidney 4 (6.2%), polycystic kidneys 1 (1.5%) and patulous extrarenal pelvis 4 (6.2%). "
8.	Familial duodenal atresiaIBA 01/01/1990 - "Detailed cytogenetic and morphological information concerning fetuses affected by omphalocele, duodenal atresia, hydrocephalus, multicystic kidney, unilateral hydronephrosis and cystic hygroma is reported. " 09/01/1981 - "These include ascites (five cases), gastroschisis (four cases), omphalocele (three cases), sacrococcygeal teratoma, cystic hygroma, hydrocele, duodenal atresia, multicystic kidney (two cases each), and one each of jejunal atresia, conjoined twins, ureteropelvic junction obstruction, urethral valves, urethral agenesis, and hydronephrosis secondary to reflux. " 03/01/1989 - "Using serial ultrasound examinations, the following lesions were found: multicystic dysplastic kidney (3 cases), ureteropelvic and ureterovesical junction stenosis (1 case), Mullerian duct cyst (1 case), sarococcygeal teratoma (3 cases), adenomatoid malformation of lung (1 case), omphalocele (1 case), gastroschisis (1 case), duodenal atresia (1 case), ileal atresia (1 case), meconium peritonitis (2 cases), imperforate anus (1 case), adrenal cyst (1 case), congenital diaphragmatic hernia (1 case) and a pair of xipho-omphalopagus twin. "
9.	AntibodiesIBA 11/01/2001 - "Immunohistochemical examination of renin was performed in 29 multicystic dysplastic kidneys from 14 boys and 15 girls 1 month to 10 years old using rabbit anti-human renin antibodies. " 01/01/2010 - "Ten multicystic dysplastic kidneys (MCDKs) were stained with hematoxylin and eosin and immunohistochemistry was performed using Wnt-1, E-cad and Sdc-1 antibodies. "
10.	Meckel syndrome type 1IBA 03/01/2001 - "Meckel Gruber syndrome (MGS), an autosomal recessive disorder characterised by posterior encephalocoele, multicystic kidneys and post-axial polydactyly should be recognised by obstetricians and paediatricians to counsel parents regarding the 25% recurrence risk. " 07/01/1993 - "Two fetuses with a severe bilateral renal abnormality (multicystic dysplastic kidney, Meckel-Gruber syndrome with polycystic kidney) and one fetus with Down syndrome (no detectable structural anomaly) were sampled. "

Therapies and Procedures

1.	Nephrectomy 09/01/1994 - "Improved technique of laparoscopic nephrectomy for multicystic dysplastic kidney." 04/01/2010 - "The total number of nephrectomies per year significantly increased over the period of the study (4, 6 and 8 nephrectomies per year for 1996-2000 and 2001-2008, respectively; P < 0.05), as did the number of nephrectomies for Multicystic dysplastic kidney (MCDK) (zero and 5 for 1996-2000 and 2001-2008, respectively) and wilms'tumour (8.3% and 29,16% for 1996 - 2000 and 2001 - 2008, respectively). " 08/01/1998 - "A clinical study of 204 patients with unilateral multicystic renal dysplasia referred to 30 nephrology departments was undertaken to assess the frequency of complications in children who underwent nephrectomy (n = 40) versus those who were treated conservatively (n = 164). " 04/01/2012 - "Indications for unilateral nephrectomy were multicystic dysplastic kidney (MCDK) in 23 cases (85.2%), dysplastic kidney in 3 cases (11.1%), and reflux nephropathy in 1 case (3.7%). " 08/01/2011 - "Laparoscopic nephrectomy for pelvic multicystic dysplastic kidney."
2.	Decompression 01/01/1992 - "Re: Percutaneous decompression: treatment for respiratory distress secondary to multicystic dysplastic kidney." 07/01/1990 - "Percutaneous decompression: treatment for respiratory distress secondary to multicystic dysplastic kidney." 12/01/1988 - "With injection of contrast medium, definitive diagnoses were made of a multicystic dysplastic kidney on one side and an obstructed hydronephrotic kidney on the other, thereby directing decompression of the obstructed kidney to preserve native renal function. " 07/01/1990 - "We present a case of ultrasound-guided percutaneous cyst decompression used as definitive treatment of respiratory failure associated with multicystic dysplastic kidney."
3.	Surgical Instruments (Clip) 09/01/1994 - "We report removal of a multicystic dysplastic kidney with a laparoscopic technique that uses only three trocar sites. " 06/01/2012 - "The author presents the first report in performing single trocar nephrectomy for five patients with multicystic dysplastic kidney (MCDK). " 06/01/2012 - "Single trocar retroperitoneoscopic nephrectomy for unilateral multicystic dysplastic kidney in children."
4.	Catheters 04/01/2012 - "His past medical history included 2 small bowel resections, bilateral nephrectomy and kidney transplantation for multicystic renal dysplasia, and 2 peritoneal dialysis-catheter infections. "
5.	Renal Replacement Therapy (Therapies, Renal Replacement) 10/01/2012 - "However, other congenital anomalies, such as renal hypoplasia, unilateral renal agenesis or multicystic dysplastic kidney, may also lead to a reduced nephron endowment, with an increased risk for hypertension, renal dysfunction and the need for renal replacement therapy. "