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Pituitary Stalk Interruption Syndrome in Chinese People: Clinical Characteristic Analysis of 55 Cases
Open accessAbstract
OBJECTIVE: Pituitary stalk interruption syndrome (PSIS) is characterized by the absence of pituitary stalk, pituitary hypoplasia, and ectopic posterior pituitary. Due to the rarity of PSIS, clinical data are limited, especially in Chinese people. Herein, we analyzed the clinical characteristics of patients diagnosed with PSIS from our center over 10 years. PATIENTS AND METHODS: We retrospectively analyzed the clinical manifestations and laboratory and MRI findings in 55 patients with PSIS. RESULTS: Of the 55 patients with PSIS, 48 (87.3%) were male. The average age was 19.7±6.7 years and there was no familial case. A history of breech delivery was documented in 40 of 45 patients (88.9%) and 19 of 55 patients (34.5%) had a history of dystocia. Short stature was found in 47 of 55 patients (85.5%) and bone age delayed 7.26±5.37 years. Secondary sex characteristics were poor or undeveloped in most patients. The prevalence of deficiencies in growth hormone, gonadotropins, corticotropin, and thyrotropin were 100%, 95.8%, 81.8%, 76.3%, respectively. Hyperprolactinemia was found in 36.4% of patients. Three or more pituitary hormone deficiencies were found in 92.7% of the patients. All patients had normal posterior pituitary function and absent pituitary stalk on imaging. The average height of anterior pituitary was 28 mm, documented anterior pituitary hypoplasia. Midline abnormalities were presented in 9.1% of patients. CONCLUSIONS: The clinical features of our Chinese PSIS patients seem to be different from other reported patients in regarding to the higher degree of hypopituitarism and lower prevalence of midline defects. In addition, our patients were older at the time of case detection and the bone age was markedly delayed. We also had no cases of familial PSIS.
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Growth hormone deficiency occurs in nearly 100% of patients with CCPHD, and hypogonadotropic hypogonadism occurs in 96% of patients.
“Growth hormone deficiency (GHD) happens in nearly 100% of patients with CCPDH, and hypogonadotropic hypogonadism (HH) occurs in 96% of patients (2).”
From Growth Hormone Is Beneficial for Induction of Spermatogenesis in Adult Patients With Congenital Combined Pituitary Hormone Deficiency (2022) · cites this paper as [2]
The Discussion reports 100% growth hormone deficiency and 95.8% gonadotropin deficiency in the authors’ series, matching the quoted percentages. But that series comprised 55 patients with pituitary stalk interruption syndrome; the paper does not establish those rates for patients with CCPHD generally. (LLM, conf=0.88)
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Flagged first, then by citations
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“patients with CCPDH, and hypogonadotropic hypogonadism (HH) occurs in 96% of patients (2).”
BackgroundSelf-citation
Metadata
- Authors
- Guo, Q., Yang, Y., Mu, Y., Lu, J., Pan, C., Dou, J., Lv, Z., Ba, J., Wang, B., Zou, X., Yang, L., Ouyang, J., Yang, G., Wang, X., Du, J., Gu, W., Jin, N., Chen, K., Zang, L., & Erickson, B. J.
- Affiliations
- Chinese PLA General Hospital (CN)
- Mayo Clinic (US)
- Corresponding author
- Mu, Yiming
- ORCID
- Venue
- PLoS ONE
- Publisher
- Public Library of Science (PLoS)
- Published
- 2013-01-14
- Volume
- 8
- Issue
- 1
- Pages
- e53579
- ISSN
- 1932-6203 (ISSN-L)
- PMC
- PMC3544917
- OpenAlex
- W2023872839
- Cited by
- 57
- References
- 31
- Citations by year
- 2026: 4 · 2025: 2 · 2024: 8 · 2023: 6 · 2022: 6 · 2021: 4 · 2020: 2 · 2019: 4 · 2018: 4 · 2017: 7 · 2016: 2 · 2015: 4 · 2014: 4
- FWCI
- 2.63
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Locations
- PLoS ONE (publishedVersion, cc-by)
- PubMed Central (publishedVersion, cc-by)
- PubMed (publishedVersion)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
- Funders
- National Natural Science Foundation of China (81270866)
- General Hospital of People’s Liberation Army
- Topics
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Pituitary Gland Disorders and Treatments
- Health Sciences › Medicine › Reproductive Medicine › Hypothalamic control of reproductive hormones
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Growth Hormone and Insulin-like Growth Factors
- Keywords
- pituitary stalk interruption syndrome, pituitary hypoplasia, ectopic posterior pituitary, hypopituitarism, short stature, delayed bone age, hyperprolactinemia, growth hormone deficiency, gonadotropin deficiency, corticotropin deficiency, thyrotropin deficiency, breech delivery, dystocia, midline defects, Chinese patients
- MeSH terms
- Adolescent, Adult, Child, China, Female, Humans, Magnetic Resonance Imaging, Male, Pituitary Diseases, Pituitary Gland, Syndrome, Asian People, Young Adult
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