Renal cystic diseases during the perinatal and neonatal period

2020 ◽  
pp. 1-14
Author(s):  
R. Raina ◽  
M. DeCoy ◽  
R. Chakraborty ◽  
S. Mahajan ◽  
R. Moran ◽  
...  
Keyword(s):  
Healthcare Providers ◽  
Multicystic Dysplastic Kidney ◽  
Renal Diseases ◽  
Polycystic Kidney ◽  
Specific Group ◽  
Dysplastic Kidney ◽  
Life Threatening ◽  
Calyceal Diverticula ◽  
Renal Cystic Diseases ◽  
Multiple Cysts

Renal cystic diseases are a clinically and genetically diverse group of renal diseases that can manifest in utero, infancy, or throughout childhood and adulthood. These diseases may be unilateral or bilateral with a single cyst or multiple cysts, or with increased echogenicity of the renal cortex without macroscopic cysts. Certain cystic renal diseases are life-threatening, with many developing chronic kidney and hepatic disease if not recognized early enough. Therefore, due to the prevalence and life-altering complications of this specific group of diseases in vulnerable populations, it is crucial for clinicians and healthcare providers to have an overall understanding of cystic diseases and how to pre-emptively detect and manage these conditions. In this review, we discuss in detail the epidemiology, genetics and pathophysiology, diagnosis, presentation, and management of numerous genetic and sporadic renal cystic diseases, such as polycystic kidney disease, multicystic dysplastic kidney, and calyceal diverticula, with an emphasis on prenatal care and pregnancy counseling.

scholarly journals Localized Aortic Dissection in a Patient with Polycystic Kidney Disease: A Case Report

2021 ◽  
Vol 6 (04) ◽  
pp. 285-287
Author(s):  
Sarah A. Alkuraydis ◽  
Abdulaziz S. Allihimy ◽  
Osama Smettei ◽  
Rami M Abazid
Keyword(s):  
Chest Pain ◽  
Aortic Dissection ◽  
Acute Chest Pain ◽  
Uncontrolled Hypertension ◽  
Polycystic Kidney ◽  
Threatening Condition ◽  
Life Threatening ◽  
History Of ◽  
The Right ◽  
Multiple Cysts

Aortic dissection (AD) is the most frequent life-threatening aortic disorder. It is commonly associated with hypertension; however, aortic dissection occasionally represents a complication of more complex syndromes. In this article we aim to report. A 40-year-old male patient, with a known case of ADPKD and a strong family history of ADPKD. He presented to the emergency department with prolonged sharp retrosternal chest pain radiating to the back and uncontrolled hypertension. Computed tomography angiography showed a localized dissection flap at the aortic root and multiple cysts in the right kidney. AD is a life-threatening condition and should be suspected in patients presenting with acute chest pain with history of ADPKD.

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.

scholarly journals The relation of anthropometric measurements and insulin resistance in patients with polycystic kidney disease

2016 ◽  
Vol 4 (3) ◽  
pp. 127-134
Author(s):  
Bennur Esen ◽  
Emel Sağlam Gokmen ◽  
Mahmut Kaya ◽  
Burak Ozkan ◽  
Ahmet Engin Atay
Keyword(s):  
Insulin Resistance ◽  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Fat Distribution ◽  
Anthropometric Measurements ◽  
Renal Diseases ◽  
Model Assessment ◽  
Polycystic Kidney ◽  
Renal Replacement Therapies ◽  
Autosomal Dominant Polycystic Kidney

AbstractObjectiveTo examine the frequency of insulin resistance (IR) and its relation with anthropometric measurements in patients with autosomal dominant polycystic kidney disease (ADPKD).Material and MethodsNonobese 82 patients with ADPKD and 58 age matched healthy controls were enrolled into the study. None of participants were diabetic or receiving renal replacement therapies (RRT). IR was determined by homeostasis model assessment of insulin resistance (HOMA-IR) formula. Tanita body composition analyzer was used for anthropometric measurements. Creatinine clearance of participant were assessed by the modification of diet in renal diseases (MDRD).ResultsPatients with ADPKD had significantly higher level of urea and creatinine, microalbuminuria, and lower level of MDRD. Body fat distribution and HOMA-IR in both the groups were similar. Systolic and diastolic blood pressure of patients were higher than those of controls.ConclusionWe failed to determine a higher frequency of IR among patients with ADPKD.

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

Multicystic dysplastic kidney

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