Lymphocytic infundibuloneurohypophysitis with positive anti-rabphilin-3A antibodies nine years post-onset of central diabetes insipidus
| Autor: | Yoshihisa Sugimura, Yohei Kume, Hiroko Sakuma, Mitsuaki Hosoya, Makoto Sumikoshi, Hitomi Sekine |
|---|---|
| Rok vydání: | 2021 |
| Předmět: |
Pathology
medicine.medical_specialty Endocrinology Diabetes and Metabolism Lymphocyte neurohypophysis Case Report 030209 endocrinology & metabolism 03 medical and health sciences 0302 clinical medicine Endocrinology Posterior pituitary Biopsy Medicine 030212 general & internal medicine Desmopressin Pituitary stalk rabphilin-3A medicine.diagnostic_test business.industry Magnetic resonance imaging medicine.disease GH autoimmune hypophysitis medicine.anatomical_structure diabetes insipidus Pediatrics Perinatology and Child Health Diabetes insipidus Autoimmune hypophysitis business medicine.drug |
| Zdroj: | Clinical Pediatric Endocrinology |
| ISSN: | 1347-7358 0918-5739 |
| DOI: | 10.1297/cpe.30.65 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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