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视神经发育不全和视隔-视神经-垂体发育异常患儿的住院情况

Hospitalizations in Children With Optic Nerve Hypoplasia and Septo-Optic-Pituitary Dysplasia.

作者信息

Salman Michael S, Ruth Chelsea A, Yogendran Marina S, Lix Lisa M

机构信息

Section of Pediatric Neurology, Department of Pediatrics and Child Health, Max Rady College of Medicine, Rady Faculty of Health Sciences, University of Manitoba, Winnipeg, Manitoba, Canada.

Section of Neonatology, Department of Pediatrics and Child Health, Max Rady College of Medicine, Rady Faculty of Health Sciences, University of Manitoba, Winnipeg, Manitoba, Canada.

出版信息

J Paediatr Child Health. 2025 Aug;61(8):1226-1234. doi: 10.1111/jpc.70094. Epub 2025 May 31.

DOI:10.1111/jpc.70094
PMID:40448956
Abstract

BACKGROUND AND OBJECTIVES

Optic nerve hypoplasia (ONH) causes congenital impaired vision. The diagnosis of septo-optic-pituitary dysplasia (SOD) is made when ONH occurs in conjunction with hypopituitarism or absent septum pellucidum or abnormal corpus callosum. Our objectives were to describe differences in hospitalisation rates, length of hospital stay (LOS), and the primary reasons for hospitalisations among children with ONH/SOD in comparison to controls.

METHODS

A population-based retrospective study with a case-control design was conducted. Cases were 124 ONH/SOD patients (mean age: 13 years, SD: 7.2 years), matched to 620 unrelated population-based controls (mean age: 12.4 years, SD: 7.2 years) on area of residence, year of birth, and sex. Non-parametric statistics were used to test for differences between cases and controls on number of hospital admissions, crude rates of admissions, and LOS. Relative rates (RR) with 95% confidence intervals (CIs) were estimated. Primary (most responsible) diagnoses were described for hospitalisations.

RESULTS

ONH/SOD cases had the highest percentage of hospitalisations (26.6%-32.3%) in comparison to the control group (10.2%-12.6%), especially in the SOD subgroup with hypopituitarism (42.9%-54.3%) who were also at risk of multiple hospitalisations. ONH/SOD cases had a significantly higher crude hospitalisation rate in comparison to the control group (RR range:2.3-2.8, 95% CI range:1.6-4.2), and was highest in infants < 1-year-old and in the SOD subgroup with hypopituitarism. The median LOS was significantly higher in ONH/SOD cases in comparison to the controls [median (interquartile range):7-8 (3-21) versus 3-4 (2-9) days respectively, p ≤ 0.004]. Diseases of the respiratory system were the primary reasons for hospitalisation in all groups, but they were highest in the ONH/SOD group accounting for 25.3% of their hospitalisations versus 9.5% in the unrelated controls.

CONCLUSION

Children with ONH/SOD have a higher rate of hospitalisation and LOS in comparison to controls, especially in the SOD subgroup with hypopituitarism. Diseases of the respiratory system were primarily responsible for hospitalisations in all groups, but disproportionately affected the ONH/SOD group.

摘要

背景与目的

视神经发育不全(ONH)可导致先天性视力受损。当视神经发育不全与垂体功能减退、透明隔缺如或胼胝体异常同时出现时,可诊断为隔视神经垂体发育不良(SOD)。我们的目的是描述与对照组相比,患有ONH/SOD的儿童在住院率、住院时间(LOS)以及住院的主要原因方面的差异。

方法

进行了一项基于人群的回顾性病例对照研究。病例为124例ONH/SOD患者(平均年龄:13岁,标准差:7.2岁),与620例基于人群的非相关对照(平均年龄:12.4岁,标准差:7.2岁)在居住地区、出生年份和性别方面进行匹配。使用非参数统计检验病例组和对照组在住院次数、粗住院率和住院时间方面的差异。估计相对率(RR)及其95%置信区间(CI)。描述了住院的主要(最主要)诊断。

结果

与对照组(10.2%-12.6%)相比,ONH/SOD病例的住院率最高(26.6%-32.3%),尤其是在伴有垂体功能减退的SOD亚组(42.9%-54.3%)中,这些患者也有多次住院的风险。与对照组相比,ONH/SOD病例的粗住院率显著更高(RR范围:2.3-2.8,95%CI范围:1.6-4.2),在1岁以下婴儿和伴有垂体功能减退的SOD亚组中最高。与对照组相比,ONH/SOD病例的中位住院时间显著更长[中位数(四分位间距):分别为7-8(3-21)天和3-4(2-9)天,p≤0.004]。呼吸系统疾病是所有组住院的主要原因,但在ONH/SOD组中最高,占其住院的25.3%,而在非相关对照组中为9.5%。

结论

与对照组相比,患有ONH/SOD的儿童住院率和住院时间更高,尤其是在伴有垂体功能减退的SOD亚组中。呼吸系统疾病是所有组住院的主要原因,但对ONH/SOD组的影响尤为严重。

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