Ultrasound findings of pubertal development in girls with cystic fibrosis and their association with clinical outcomes and Tanner staging

Lopes, Paula de Souza Dias; Machado, Sandra Helena; Lucena, Iara Regina Siqueira; Marostica, Paulo José Cauduro

doi:10.20945/2359-3997000000404

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Archives of Endocrinology and Metabolism

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Original Article • Arch. Endocrinol. Metab. 65 (5) • 2021 • https://doi.org/10.20945/2359-3997000000404 copy

Ultrasound findings of pubertal development in girls with cystic fibrosis and their association with clinical outcomes and Tanner staging

Authorship SCIMAGO INSTITUTIONS RANKINGS

ABSTRACT

Objective:

Patients with cystic fibrosis (CF) have a high incidence of pubertal and growth delay. In girls with CF, pubertal delay has an important psychological impact. Still, only a few studies have explored the occurrence of pubertal delay in girls with CF. The aims of this study were to compare the pubertal development of girls with CF compared with healthy controls regarding Tanner staging and pelvic ultrasound and, in girls with CF, correlate the findings with those of spirometry, body mass index, Shwachman-Kulczycki score (SKS), and genotyping.

Subjects and methods:

This was a cross-sectional, case-control study including 35 girls with CF aged 6-17 years and following up at the Pediatric Pulmonology Outpatient Clinic of a tertiary hospital. These patients were compared with 59 healthy controls who had undergone pelvic ultrasound as part of another study conducted by the same group. Girls with CF were consecutively enrolled in the study during their annual routine check-up visit. Data collected in the CF group included spirometry and anthropometric results, SKS values, bone age, occurrence of current cystic fibrosis-related diabetes (CFRD) and Pseudomonas aeruginosa colonization, history of meconium ileus, genotype, ultrasound parameters, and Tanner stage.

Results:

Pelvic ultrasound findings and Tanner stage reflected less pubertal development in girls with CF compared with healthy controls. Pubertal stage in patients with CF who presented CFRD (3.17 ± 1.16), had chronic colonization by Pseudomonas aeruginosa (3.10 ± 1.10), or were homozygous for the F508del mutation (1.91 ± 1.30) was more delayed than in controls (3.41 ± 1.41). Tanner stage correlated with age at menarche, bone age, and anthropometric and ultrasound data.

Conclusions:

Girls with CF presented a delay in pubertal development evaluated by Tanner stage and ultrasound parameters, which was more evident in the presence of comorbidities.

Keywords
Cystic fibrosis; menarche; puberty; ultrasonography; Doppler; Color

Variable	Diagnosis	N	Mean (SD)/Median (IR)	p
Age (years)	Controls	59	12.24 (2.92)	0.62
Age (years)	CF	35	11.89 (3.61 )	0.62
Weight (kg)	Controls	59	52.21 (17.37)	<0.01
Weight (kg)	CF	35	38.84 (12.67)	<0.01
Height/age z-score	Controls	59	0.86 (0.06-1.66)	<0.01
Height/age z-score	CF	35	-0.37 (-0.95-0.21)	<0.01
BMI z-score	Controls	59	0.86 (0.43-1.29)	<0.01
BMI z-score	CF	35	-0.11 (-0.51-0.29)	<0.01
Target height (cm)	Controls	49	161.75 (157.75-165.00)	0.23
Target height (cm)	CF	34	159.50 (156.50-163.75)	0.23
Menarche (years)	Controls	37	12 (10.95-12.00)	0.33
Menarche (years)	CF	18	12 (11.00-13.00)	0.33
Tanner staging	Controls	59	4.00 (2.00-5.00)	0.06
Tanner staging	CF	35	3.00 (1.00-4.00)	0.06

Variable	Diagnosis	N	Mean (SD)/Median (IR)	p
AP body-to-cervix ratio	Controls	58	1.35 (0.27)	<0.01
AP body-to-cervix ratio	CF	35	1.19 (0.29)	<0.01
Longitudinal body-to-cervix ratio	Controls	57	1.22 (0.31)	0.01
Longitudinal body-to-cervix ratio	CF	35	1.09 (0.19)	0.01
AP left ovary (cm)	Controls	57	1.54 (0.43)	<0.01
AP left ovary (cm)	CF	34	1.20 (0.39)	<0.01
Volume left ovary (cm³)	Controls	57	5.00 (2.95-7.10)	0.01
Volume left ovary (cm³)	CF	34	3.65 (1.27-5.90)	0.01
Longitudinal right ovary (cm)	Controls	58	3.16 (0.75)	<0.01
Longitudinal right ovary (cm)	CF	34	2.62 (0.68)	<0.01
Transverse diameter right ovary (cm)	Controls	58	1.97 (0.58)	<0.01
Transverse diameter right ovary (cm)	CF	34	1.60 (0.50)	<0.01
AP right ovary (cm)	Controls	58	1.65 (0.56)	<0.01
AP right ovary (cm)	CF	34	1.21 (0.42)	<0.01
Volume right ovary (cm³)	Controls	58	5.85 (2.97-9.05)	<0.01
Volume right ovary (cm³)	CF	34	2.70 (1.27-4.97)	<0.01
Pulsatility indices middle uterine arteries	Controls	55	2.50 (1.90-5.10)	0.13
Pulsatility indices middle uterine arteries	CF	25	3.60 (2.45-5.75)	0.13

Variable	N	R	P
Age	35	0.83	<0.01
Age at menarche	35	-0.78	<0.01
Uterine length	35	0.78	<0.01
Uterine transversal	35	0.72	<0.01
Uterine AP	35	0.71	<0.01
Uterine volume	35	0.75	<0.01
AP body-to-cervix ratio	35	0.64	<0.01
Longitudinal body-to-cervix ratio	35	0.62	<0.01
Endometrial thickness	25	0.46	0.02
Left ovary longitudinal size	34	0.72	<0.01
Transversal left ovary	34	0.82	<0.01
AP left ovary	34	0.59	<0.01
Left ovary volume	34	0.79	<0.01
Follicles number < 1 cm in left ovary	31	0.48	<0.01
Right ovary longitudinal size	34	0.56	<0.01
Transversal right ovary	34	0.59	<0.01
Right ovary AP	34	0.61	<0.01
Right ovary volume	34	0.62	<0.01
Follicles number < 1 cm right ovary	32	0.46	<0.01
Right uterine artery pulsatility index	29	-0.37	0.04
Left uterine artery pulsatility index	27	-0.54	<0.01
Mean uterine arteries pulsatility index	25	-0.56	<0.01
Bone age (years)	18	0.82	<0.01
Bone age (z-score)	35	0.40	0.01
Forced expiratory volume in 1 second (FEV1; z-score)	35	0.10	0.53
Forced vital capacity (FVC; z-score)	35	0.08	0.62
FEV₁/FVC ratio	35	0.10	0.56
Shwachman-Kulczycki’s score	35	-0.08	0.61
Height	35	0.83	<0.01
Weight	35	0.90	<0.01
Body mass index (BMI)	35	0.75	<0.01
Height/age z-score	35	0.36	0.03

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[1] Correspondence to: Paula de Souza Dias Lopes, Avenida Itaqui, 89/203, 90460-140 – Petrópolis, Porto Alegre, RS, Brasil. pauladlopes@yahoo.com.br