Testicular function and fertility outcomes in males with CF: A multi center retrospective study of men with congenital bilateral absence of the vas deferens based on CFTR mutation status

Background: Modern cystic fibrosis (CF) treatments, particularly CFTR modulators, have allowed males with CF (MwCF) to live longer, healthier lives and pursue parenthood. Approximately 98% of MwCF have congenital bilateral absence of the vas deferens (CBAVD). While research shows MwCF experience spe...

Full description

Saved in:
Bibliographic Details
Main Authors: Rossella Cannarella, Danielle Velez Leitner, Marissa Weiss, Sarah C. Vij
Format: Article
Language:English
Published: Elsevier 2025-06-01
Series:Journal of Clinical & Translational Endocrinology
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S2214623725000067
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1849699616155500544
author Rossella Cannarella
Danielle Velez Leitner
Marissa Weiss
Sarah C. Vij
author_facet Rossella Cannarella
Danielle Velez Leitner
Marissa Weiss
Sarah C. Vij
author_sort Rossella Cannarella
collection DOAJ
description Background: Modern cystic fibrosis (CF) treatments, particularly CFTR modulators, have allowed males with CF (MwCF) to live longer, healthier lives and pursue parenthood. Approximately 98% of MwCF have congenital bilateral absence of the vas deferens (CBAVD). While research shows MwCF experience spermatogenic dysfunction alongside obstructive azoospermia, understanding male reproductive health in MwCF remains limited. This study retrospectively examines testicular function and intracytoplasmic sperm injection outcomes of the partners of males with CBAVD, stratified by CF mutation status (CF, CF carriers, no known mutation). Subjects and Methods: This multicenter, retrospective study assessed sperm retrieval outcomes and testicular function in males with CBAVD. Participants were categorized into three groups: MwCF (Group 1), CFTR gene mutation carriers (CFTR carriers, Group 2), and CBAVD males without CFTR mutations (Group 3). We collected data on genetic testing, testicular hormone levels (FSH, LH, total testosterone), sperm retrieval methods, and reproductive outcomes. Statistical analysis was used to assess intergroup differences. Results: Thirty subjects were included (Group 1: 14, Group 2: 11, Group 3: 5). No significant differences in demographic, anthropometric, or reproductive characteristics were found across groups. Hormone levels (LH, FSH, and testosterone) were similar among groups. In Group 1, 42 % had elevated FSH levels. The prevalence of hypogonadism was 16.7 % in Group 1. Group 3 had a significantly lower fertilization rate (p < 0.01), but no differences were found in blastocyst formation, pregnancy, miscarriage, or live birth rates. Conclusions: Our data support the presence of primary spermatogenic dysfunction in some MwCF. However, reproductive outcomes were similar across all groups.
format Article
id doaj-art-0c4b4970ac0c4078975073d04cedcfd3
institution DOAJ
issn 2214-6237
language English
publishDate 2025-06-01
publisher Elsevier
record_format Article
series Journal of Clinical & Translational Endocrinology
spelling doaj-art-0c4b4970ac0c4078975073d04cedcfd32025-08-20T03:18:32ZengElsevierJournal of Clinical & Translational Endocrinology2214-62372025-06-014010038810.1016/j.jcte.2025.100388Testicular function and fertility outcomes in males with CF: A multi center retrospective study of men with congenital bilateral absence of the vas deferens based on CFTR mutation statusRossella Cannarella0Danielle Velez Leitner1Marissa Weiss2Sarah C. Vij3Department of Clinical and Experimental Medicine, University of Catania 95123 Catania, Italy; Department of Urology, Cleveland Clinic Foundation, Cleveland OH USA; Corresponding author at: Department of Clinical and Experimental Medicine, University of Catania, Via S. Sofia 78, 95123, Catania, Italy.Rutgers Robert Wood Johnson Medical School, 08901 New Brunswick, NJ, USADivision of Reproductive Endocrinology &amp; Infertility, University of Pennsylvania 19104 Pennsylvania, PA, USADepartment of Surgery and Perioperative Care, University of Texas at Austin Dell Medical School, Austin TX USABackground: Modern cystic fibrosis (CF) treatments, particularly CFTR modulators, have allowed males with CF (MwCF) to live longer, healthier lives and pursue parenthood. Approximately 98% of MwCF have congenital bilateral absence of the vas deferens (CBAVD). While research shows MwCF experience spermatogenic dysfunction alongside obstructive azoospermia, understanding male reproductive health in MwCF remains limited. This study retrospectively examines testicular function and intracytoplasmic sperm injection outcomes of the partners of males with CBAVD, stratified by CF mutation status (CF, CF carriers, no known mutation). Subjects and Methods: This multicenter, retrospective study assessed sperm retrieval outcomes and testicular function in males with CBAVD. Participants were categorized into three groups: MwCF (Group 1), CFTR gene mutation carriers (CFTR carriers, Group 2), and CBAVD males without CFTR mutations (Group 3). We collected data on genetic testing, testicular hormone levels (FSH, LH, total testosterone), sperm retrieval methods, and reproductive outcomes. Statistical analysis was used to assess intergroup differences. Results: Thirty subjects were included (Group 1: 14, Group 2: 11, Group 3: 5). No significant differences in demographic, anthropometric, or reproductive characteristics were found across groups. Hormone levels (LH, FSH, and testosterone) were similar among groups. In Group 1, 42 % had elevated FSH levels. The prevalence of hypogonadism was 16.7 % in Group 1. Group 3 had a significantly lower fertilization rate (p < 0.01), but no differences were found in blastocyst formation, pregnancy, miscarriage, or live birth rates. Conclusions: Our data support the presence of primary spermatogenic dysfunction in some MwCF. However, reproductive outcomes were similar across all groups.http://www.sciencedirect.com/science/article/pii/S2214623725000067TestosteroneFSHHypogonadismSperm retrievalCystic FibrosisCBAVD
spellingShingle Rossella Cannarella
Danielle Velez Leitner
Marissa Weiss
Sarah C. Vij
Testicular function and fertility outcomes in males with CF: A multi center retrospective study of men with congenital bilateral absence of the vas deferens based on CFTR mutation status
Journal of Clinical & Translational Endocrinology
Testosterone
FSH
Hypogonadism
Sperm retrieval
Cystic Fibrosis
CBAVD
title Testicular function and fertility outcomes in males with CF: A multi center retrospective study of men with congenital bilateral absence of the vas deferens based on CFTR mutation status
title_full Testicular function and fertility outcomes in males with CF: A multi center retrospective study of men with congenital bilateral absence of the vas deferens based on CFTR mutation status
title_fullStr Testicular function and fertility outcomes in males with CF: A multi center retrospective study of men with congenital bilateral absence of the vas deferens based on CFTR mutation status
title_full_unstemmed Testicular function and fertility outcomes in males with CF: A multi center retrospective study of men with congenital bilateral absence of the vas deferens based on CFTR mutation status
title_short Testicular function and fertility outcomes in males with CF: A multi center retrospective study of men with congenital bilateral absence of the vas deferens based on CFTR mutation status
title_sort testicular function and fertility outcomes in males with cf a multi center retrospective study of men with congenital bilateral absence of the vas deferens based on cftr mutation status
topic Testosterone
FSH
Hypogonadism
Sperm retrieval
Cystic Fibrosis
CBAVD
url http://www.sciencedirect.com/science/article/pii/S2214623725000067
work_keys_str_mv AT rossellacannarella testicularfunctionandfertilityoutcomesinmaleswithcfamulticenterretrospectivestudyofmenwithcongenitalbilateralabsenceofthevasdeferensbasedoncftrmutationstatus
AT daniellevelezleitner testicularfunctionandfertilityoutcomesinmaleswithcfamulticenterretrospectivestudyofmenwithcongenitalbilateralabsenceofthevasdeferensbasedoncftrmutationstatus
AT marissaweiss testicularfunctionandfertilityoutcomesinmaleswithcfamulticenterretrospectivestudyofmenwithcongenitalbilateralabsenceofthevasdeferensbasedoncftrmutationstatus
AT sarahcvij testicularfunctionandfertilityoutcomesinmaleswithcfamulticenterretrospectivestudyofmenwithcongenitalbilateralabsenceofthevasdeferensbasedoncftrmutationstatus