ArticleEndocrinology and metabolism (Seoul, Korea)2026
Clinical Characteristics and Outcomes of Korean Patients with Lipoid Congenital Adrenal Hyperplasia: An Analysis of Data from the Multicenter Networks for Ideal Outcomes of Pediatric Rare Endocrine and Metabolic Disease (OUTSPREAD) Cohort Study.
Article in Endocrinology and metabolism (Seoul, Korea), 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
0 citing papers in PubMed.
No citing paper in PubMed yet.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
15 authors.
Funding
Abstract
backgruoundClinical characteristics and follow-up outcomes were examined in Korean patients with lipoid congenital adrenal hyperplasia (LCAH) enrolled in the Multicenter Networks for Ideal Outcomes of Pediatric Rare Endocrine and Metabolic Disease (OUTSPREAD) cohort.
methodsThirty-one patients (classic LCAH [C-LCAH], n=29; nonclassic LCAH [NC-LCAH], n=2) from 10 centers were included. NC-LCAH was defined by any of the following: 46,XY karyotype with male external genitalia, no fludrocortisone use, or diagnosis after infancy.
resultsMedian age at diagnosis was 36.5 days (interquartile range [IQR], 14.6 to 120.5); salt-wasting occurred in 58.6% of patients, and skin hyperpigmentation was the most common clinical feature (71%). All C-LCAH patients (STAR variants: p.Q258*/p.Q258* [n=27], p.Q258*/p.Arg182His [n=1], p.Q258*/p.Arg182Cys [n=1]; karyotypes: 46,XX [n=17], 46,XY [n=12]) were diagnosed in infancy with a female phenotype. Among C-LCAH patients with 46,XX, 11 had spontaneous thelarche (median, 11.0 years; IQR, 8.2 to 12.2) and five reached menarche (median, 12.4 years; IQR, 11.9 to 13.2). Among eight C-LCAH-XX patients who underwent ultrasonography, three had unilocular ovarian cysts, including one rupture. Two NC-LCAH patients (p.Q258*/p.Val187Met and p.Ala218Val/p.Gly221Ser; both 46,XY) were diagnosed at 1.6 and 3.3 years with male external genitalia; one had spontaneous puberty at 10.6 years. Eight patients reached adult height (Z-score, 0.1; IQR, -0.9 to 1.2). The hydrocortisone dose was 21.7 mg/m2 (IQR, 14.9 to 26.5) at diagnosis and 17.3 mg/m2 (IQR, 13.9 to 22.7) at last follow-up (median age, 11.6 years; IQR, 8.9 to 17.4).
conclusionThis is the first large-scale multicenter investigation in Korea reporting long-term outcomes of LCAH. Continued longitudinal follow-up focusing on pubertal development and reproductive outcomes is warranted.
Indexed as
Identifiers
What OpenQuestion holds
Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.