Congenital Upper Tract Anomalies: Duplication, Cystic Renal Dysplasia, Multicystic Dysplastic Kidney

2020 ◽  
pp. 247-261
Author(s):  
David Chalmers
Keyword(s):  
Renal Dysplasia ◽  
Multicystic Dysplastic Kidney ◽  
Upper Tract ◽  
Dysplastic Kidney ◽  
Cystic Renal Dysplasia

Distal ureteral atresia – an embryological dilemma

2022 ◽  
pp. 205141582110481
Author(s):  
Subramanya Kattepura ◽  
Attibele Mahadevaiah Shubha ◽  
Kanishka Das
Keyword(s):  
Preoperative Diagnosis ◽  
Renal Dysplasia ◽  
Urogenital Sinus ◽  
Multicystic Dysplastic Kidney ◽  
Level Of Evidence ◽  
Case Presentation ◽  
Contralateral Kidney ◽  
Urinary Tract Anomaly ◽  
Dysplastic Kidney ◽  
Associated Malformations

Introduction: Distal ureteral atresia is a rare urinary tract anomaly generally associated with ipsilateral renal dysplasia and abnormalities such as multicystic dysplastic kidney, hydronephrosis and megaureter in the contralateral kidney. Despite burgeoning investigation modalities, definitive preoperative diagnosis of this condition is rarely feasible, also the embryological disarray of events that result in the development of this anomaly and the associated malformations is not clearly understood. Case presentation: We hereby report two cases of distal ureteral atresia and discuss the diversity in their presentations, diagnosis, atypical associations and management and review the possible embryological mal-development. Conclusion: Distal ureteral atresia with urogenital sinus as in Case 1 has not been documented so far and a plausible embryological explanation is deduced regarding its occurrence. The course of the affected kidney following timely and adequate relief of obstruction in Case 2 is depicted, highlighting the eventual management. Level of evidence: Not applicable

High Activation of the AKT Pathway in Human Multicystic Renal Dysplasia

Pathobiology ◽  
2020 ◽  
Vol 87 (5) ◽  
pp. 302-310
Author(s):  
Alexia Apostolou ◽  
Brice Poreau ◽  
Loris Delrieu ◽  
Julien Thévenon ◽  
Pierre-Simon Jouk ◽  
...  
Keyword(s):  
Mesangial Cells ◽  
Renal Dysplasia ◽  
Mtor Pathway ◽  
Multicystic Dysplastic Kidney ◽  
Renal Development ◽  
Glomerular Mesangial Cells ◽  
Dysplastic Kidney ◽  
Cell Proliferation And Differentiation ◽  
Identical Manner ◽  
Multicystic Renal Dysplasia

Multicystic renal dysplasia is a congenital cystic anomaly of the kidney caused by abnormal metanephric differentiation with immature tubules. It is surrounded by mesenchymal collars and islands of immature mesenchyma present between the cysts. The PI3K-AKT-mTOR signaling pathway is a key regulator involved in cell growth, proliferation, motility, survival, and apoptosis. Activation of the PI3K-AKT-mTOR pathway results in the survival and proliferation of tumor cells in many cancers. The aim of this study is to analyze the topographic expression of phospho-AKT, phospho-mTOR, and phospho-70S6K in renal development and in the multicystic dysplastic kidney (MCDK). A total of 17 fetal kidneys of development age from the first to the third trimester and 13 cases of pathological kidneys with MCDK were analyzed by immunohistochemistry in order to evaluate the expression of phospho-AKT (S473), phospho-mTOR, and phospho-70S6K. Phospho-AKT and phospho-mTOR were expressed early in renal development and in an identical manner for every structure derived from the ureteric bud, such as collecting ducts and urothelium. Phospho-p70S6K was expressed early in the urothelium and in glomerular mesangial cells. Later, their expressions differed according to the needs of cell proliferation and differentiation over time by becoming more selective. In MCDK, phospho-AKT, phospho-mTOR, and phospho-70S6K have the same profile: a high cytoplasmic expression in cystic epithelium, loose mesenchyma, and primitive tubes. This study demonstrates the essential and specific role of the PI3K-AKT-mTOR pathway in the formation of cysts in multicystic renal dysplasia.

Cytogenomic aberrations in isolated multicystic dysplastic kidney in children

2021 ◽  
Author(s):  
Tian-Jian Chen ◽  
Renfang Song ◽  
Adam Janssen ◽  
Ihor V. Yosypiv
Keyword(s):  
Multicystic Dysplastic Kidney ◽  
Dysplastic Kidney

scholarly journals Laparoscopic transposition for crossing vessels (vascular hitch) in pure extrinsic pelvic-ureteric junction obstruction: a successful case report of a 2-year-old infant with horseshoe kidney

2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Satoshi Ieiri ◽  
Kouji Nagata
Keyword(s):  
Laparoscopic Surgery ◽  
Left Kidney ◽  
Horseshoe Kidney ◽  
Renal Scintigraphy ◽  
Multicystic Dysplastic Kidney ◽  
Extrinsic Compression ◽  
Dysplastic Kidney ◽  
Renal Vessels ◽  
The Right ◽  
Crossing Vessels

Abstract Background Pediatric hydronephrosis induced by pelvic-ureteric junction obstruction (PUJO) is treated by dismembered pyeloplasty (DP) via open and laparoscopic surgery. The etiology of PUJO involves both intrinsic stenosis and extrinsic compression of crossing vessels (CVs). PUJO owing to CVs is also treated by DP, as there is no consensus concerning this vascular condition. We encountered a 2-year-old infant with pure extrinsic PUJO combined with horseshoe kidney who successfully underwent laparoscopic transposition for CVs (vascular hitch). Case presentation A 2-year-old boy was prenatally diagnosed with left multicystic dysplastic kidney (MDCK) and right hydronephrosis and received a definitive diagnosis after birth. At 6 months old, renal scintigraphy revealed a non-functioning pattern in the left kidney and an obstructive pattern in the right, showing no response to furosemide loading. The patient also had recurrent urinary tract infection, and his right hydronephrosis gradually worsened. We decided to perform surgery for the right PUJO. Preoperative enhanced computed tomography detected three right renal vessels independently branching from the abdominal aorta. The middle renal vessels were located at the ventral side of the pelvis and coincident with the site of PUJO. These vessels were suspected of being CVs. The patient underwent laparoscopic surgery electively. A 5-mm trocar was inserted at the umbilicus for a 5-mm, 30° rigid scope. Two additional ports were then inserted under laparoscope inspection. The dilated right pelvis and CVs were detected after ascending colon mobilization. To confirm the pathogenesis of PUJO, the CVs were dissected and taped. After taping the CVs, an intraoperative diuretic test was performed using furosemide loading. Peristalsis of the right ureter was recognized, and the extrinsic PUJO owing to the CVs was definitively confirmed. We therefore performed transposition for the CVs (vascular hitch procedure). The CVs were mobilized in the cranial direction and those were wrapped by dilated pelvis. The post-operative course was uneventful. The renal scintigraphy findings improved and showed a favorable response of furosemide loading. Conclusions The laparoscopic vascular hitch procedure is minimally invasive and effective for extrinsic PUJO due to CVs. Anastomotic stricture after Anderson and Hynes DP can be prevented by appropriate patient selection.

Multicystic Dysplastic Kidney: Conservative Management and Follow-Up

Renal Failure ◽  
2005 ◽  
Vol 27 (2) ◽  
pp. 189-192 ◽  
Author(s):  
Said Al-Ghwery ◽  
Abdulrahman Al-Asmari
Keyword(s):  
Conservative Management ◽  
Multicystic Dysplastic Kidney ◽  
Dysplastic Kidney

scholarly journals A splice site variant in INPP5E causes diffuse cystic renal dysplasia and hepatic fibrosis in dogs

PLoS ONE ◽  
2018 ◽  
Vol 13 (9) ◽  
pp. e0204073 ◽  
Author(s):  
Kati J. Dillard ◽  
Marjo K. Hytönen ◽  
Daniel Fischer ◽  
Kimmo Tanhuanpää ◽  
Mari S. Lehti ◽  
...  
Keyword(s):  
Hepatic Fibrosis ◽  
Splice Site ◽  
Renal Dysplasia ◽  
Cystic Renal Dysplasia ◽  
Splice Site Variant

Multicystic dysplastic kidney

2003 ◽  
pp. 809-816
Author(s):  
David Thomas ◽  
Azad Najmaldin
Keyword(s):  
Multicystic Dysplastic Kidney ◽  
Dysplastic Kidney

Early Nephrectomy in Unilateral Multicystic Dysplastic Kidney in Children Cures Hypertension Early: An Observation

2017 ◽  
Vol 27 (06) ◽  
pp. 533-537 ◽  
Author(s):  
Vijai Upadhyaya ◽  
Manish Gupta ◽  
Laxmi Bharti ◽  
Ram Rao ◽  
Sheo Kumar ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Antihypertensive Drugs ◽  
Case Series ◽  
Multicystic Dysplastic Kidney ◽  
Time Duration ◽  
Level Of Evidence ◽  
Variable Time ◽  
Dysplastic Kidney ◽  
Diagnosis Of Hypertension

Objective Surgery is still indicated as the treatment of choice in subset of patients with unilateral multicystic dysplastic kidney (UMCDK) because of its potential complications and malignant change. The purpose of this study is to present our observation that early nephrectomy may cure hypertension early in children with UMCDK and review the literature. Materials and Methods We report here four children (two males and two females) with antenatally diagnosed UMCDK with hypertension, treated in the past 4 years. All have antenatal diagnosis of UMCDK and referred to us after their birth. Diagnosis of hypertension (blood pressure > 95th percentile) was made after 3 months of follow-up in all patients. Nephrectomy was performed in all children in variable time duration. Hypertension, urinary tract infection, and desire of parents were the main indication of surgery. Results All children showed decrease in the size of their dysplastic kidney with time, but hypertension was persistent and needed antihypertensive drugs; even increase in the doses in successive follow-up. After nephrectomy, three children who were operated early got cured having normal blood pressure within variable time duration, while a child, operated late, still had high blood pressure and needed antihypertensive drugs. Conclusion Early nephrectomy in recently diagnosed hypertension in UMCDK is advisable and can cure hypertension early. Levels of Evidence The level of evidence is IV (case series with no comparison group).

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