ArticleClinical pediatric endocrinology : case reports and clinical investigations : official journal of the Japanese Society for Pediatric Endocrinology2021
Lymphocytic infundibuloneurohypophysitis with positive anti-rabphilin-3A antibodies nine years post-onset of central diabetes insipidus.
Article in Clinical pediatric endocrinology : case reports and clinical investigations : official journal of the Japanese Society for Pediatric Endocrinology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 9 papers.
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9 citing papers in PubMed.
- A pediatric case of central diabetes insipidus and hypopituitarism after COVID-19 suspected with lymphocytic hypophysitis with positive anti-rabphilin-3A antibodies.Clinical pediatric endocrinology : case reports and clinical investigations : official journal of the Japanese Society for Pediatric Endocrinology · 2026Article
- Diagnosis of Lymphocytic Infundibuloneurohypophysitis After Positive Anti-rabphilin-3A Antibody Test in an 8-year-old Boy with Early-onset Central Diabetes InsipidusJournal of clinical research in pediatric endocrinology · 2025Article
- A 4-year-old Boy Positive for Anti-rabphilin-3A Antibody and Diagnosed With Lymphocytic Infundibuloneurohypophysitis.JCEM case reports · 2025Article
- A case of central diabetes insipidus after COVID-19 as a probable diagnosis of lymphocytic infundibulo-neurohypophysitis with positive anti-rabphilin-3A antibodies with review of literature.Endocrine journal · 2024Review
- Absence of anti-rabphilin-3A antibodies in children and young adults with idiopathic central diabetes insipidus: a potential clue to elucidating a tumor etiology.Hormones (Athens, Greece) · 2023Review
- Central diabetes insipidus with anti-rabphilin-3A antibody positivity causing hypovolemic shock after resection of tumorous lesions in the pelvic cavity.CEN case reports · 2023Article
- Unveiling the Etiopathogenic Spectrum of Hypophysitis: A Narrative Review.Journal of personalized medicine · 2023Review
- Studies on anti-rabphilin-3A antibodies in 15 consecutive patients presenting with central diabetes insipidus at a single referral center.Scientific reports · 2022Article
- Molecular and Genetic Immune Biomarkers of Primary and Immune-Therapy Induced Hypophysitis: From Laboratories to the Clinical Practice.Journal of personalized medicine · 2021Review
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6 authors.
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Abstract
Childhood-onset lymphocytic infundibuloneurohypophysitis (LINH) due to infiltration of autoimmune lymphocyte in the neurohypophysis is rarely reported. Its definitive diagnosis requires a pituitary biopsy, which is an invasive procedure. Recently, anti-rabphilin-3A antibody has been reported as a potential diagnostic marker for LINH in adults; however, only a few cases have been reported in children. Here, we present a case of childhood-onset LINH in a 10-yr-old boy identified as anti-rabphilin-3A antibody positive during chronic phase, 9 yr post-onset of central diabetes insipidus (CDI). T1-weighted magnetic resonance imaging (MRI) revealed pituitary stalk thickening and absence of posterior pituitary bright signal spot, and the hormonal responses of the adenohypophysis to GHRH, TRH, CRH, and LHRH revealed no abnormalities during the first admission. MRI at 5 mo post-onset indicated reduced stalk swelling; however, replacement treatment with intranasal desmopressin was continued to counter unimproved CDI. Additionally, GH replacement therapy was also initiated to counter its deficiency. Pituitary re-enlargement was not observed in the subsequent routine MRI, and no increase was observed in the levels of tumor markers during follow-up, which was considered clinically consistent with LINH. Our case study suggests that anti-rabphilin-3A antibody may be considered as a useful diagnostic marker for LINH in children.
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