Tehran University of Medical Sciences

Science Communicator Platform

Stay connected! Follow us on X network (Twitter):
Share this content! On (X network) By
Could Drainage Related Ultrasonography Prevent Unnecessary Interventions in Patients With Wolfram Syndrome? Publisher



Mehdizadeh M1 ; Eftekharzadeh S2 ; Shabanan SH2 ; Sobhani M3 ; Kajbafzadeh AM2
Authors
Show Affiliations
Authors Affiliations
  1. 1. Department of Radiology, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran
  2. 2. Pediatric Urology and Regenerative Medicine Research Center, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran
  3. 3. Iranian Blood Transfusion Research Center, High Institute for Research and Education in Transfusion Medicine, Tehran, Iran

Source: Iranian Journal of Pediatrics Published:2020


Abstract

Background: Upper urinary tract dilation, the most common urological manifestation of the Wolfram syndrome (WS), is mainly non-obstructive and secondary to other components of the disease such as diabetes insipidus. Misdiagnosis of the type of the hydroureter inWolfram patients and encountering them as obstructive uropathies has led to ineffectual surgeries such as ureter reimplantation. Based on previous studies drainage related ultrasonography (DRUS) is a beneficial means of distinction between obstructive and non-obstructive hydroureters. Objectives: To avoid unnecessary interventions in patients with WS by detecting hydroureters’ types using DRUS. Methods: Seven patients (14 ureters) with a mean ± SD age of 24.43 ± 4.25 months who were diagnosed with WS were included in this retrospective study. The definite diagnosis of the non-obstructive type of hydroureter was assessed by appropriate imaging modalities. The maximum diameter of these 14 ureters, before (D1) and after (D2) 3 hours of catheterization were observed by ultrasonography. Values were recorded as D ratio ([(|D1 - D2|)/D1] × 100) and the cutoff point of 22% for D ratio was set to discriminate the subtypes of the hydroureter. Results: Measurement of maximum diameter of ureter prior to catheterization indicated a mean ± SD diameter of 20.64 ± 2.73 mm; decreasing to 11.07 ± 2.64 mm after 3 hours of catheterization which indicates a significant decrease. Mean D ratio of 14 hydroureters was 45.95±13.01% which indicated significantly higher percentage than 22%, revealing that hydroureters’ type in WS is non-obstructive. Conclusions: DRUS is a useful method for the assessment of the hydroureter’s type in WS and it could prevent performing unnecessary surgeries in WS patients. © 2020, Author(s).
Related Docs
1. Drainage-Related Ultrasonography (Drus): A Novel Technique for Discriminating Obstructive and Nonobstructive Hydroureters in Children, Journal of Ultrasound (2015)
2. Drainage Related Ultra Sonography; Acute Us Technique in Categorizing Primary Mega Ureter, Acta Medica Mediterranea (2016)
3. Management of Bladder Dysfunction in Wolfram Syndrome With Mitrofanoff Appendicovesicostomy: Long-Term Follow-Up, Journal of Pediatric Surgery (2015)
4. Clinical and Molecular Assessment of 13 Iranian Families With Wolfram Syndrome, Endocrine (2019)
5. Molecular Investigation of Wfs1 Gene Exon 8 in Iranian Patients With Wolfram Syndrome, International Journal of Diabetes in Developing Countries (2016)
6. Clinical and Genetic Analysis of Two Wolfram Syndrome Families With High Occurrence of Wolfram Syndrome and Diabetes Type Ii: A Case Report, BMC Medical Genetics (2020)
Experts (# of related papers)
View all Related Experts
Abdolmohammad Kajbafzadeh (4)
Mehrzad Mehdizadeh (2)