PMID- 23556479
OWN - NLM
STAT- MEDLINE
DCOM- 20140310
LR  - 20130822
IS  - 1557-9077 (Electronic)
IS  - 1050-7256 (Linking)
VI  - 23
IP  - 8
DP  - 2013 Aug
TI  - Graves' immune reconstitution inflammatory syndrome in childhood.
PG  - 1010-4
LID - 10.1089/thy.2012.0618 [doi]
AB  - BACKGROUND: The use of hematopoietic stem cell transplantations (HSCTs) as a 
      curative therapy for life-threatening immunodeficiencies has had a profound 
      impact on clinical outcomes. A subset of patients may experience immune 
      reconstitution inflammatory syndrome (IRIS) post-transplant affecting the thyroid 
      gland, but this has received little attention in the pediatric literature. We 
      present the clinical, biochemical, and cytological course of patients with 
      Graves' disease after HSCT in the pediatric population. PATIENTS AND METHODS: 
      Four children (median age 1.5 years, range 2 months-9 years) underwent HSCT. The 
      conditioning regimen included chemotherapy but not radiotherapy. None of the 
      children or their donors had evidence of thyroid disease pre-HSCT or during the 
      follow-up period. Engraftment was uneventful in all, with stable donor T-cell 
      chimerism, and none had evidence of graft-versus-host disease. RESULTS: Patients 
      developed Graves' disease soon after undergoing HSCT, with a median time interval 
      between HSCT and Graves' disease of 22 months (range 16-28 months). Graves' 
      disease was diagnosed on the basis of clinical and biochemical parameters, 
      including a suppressed thyrotropin, raised free thyroxine, and raised thyrotropin 
      receptor antibodies. Three patients were hypothyroid initially (suggestive of a 
      Th1 profile) before Graves' disease (suggestive of a Th2 profile). In three 
      patients, the clinical picture changed rapidly with hypothyroidism abruptly 
      followed by profound thyroid hormone excess. The onset of Graves' IRIS coincided 
      with a rapid expansion in naive and total CD4. CONCLUSIONS: Immunological 
      dysregulation during T-cell engraftment is the most likely mechanism for 
      developing Graves' IRIS after allogenic HSTC. Clinicians need to be aware that 
      HSCT-engendered immune recovery may result in a particularly aggressive form of 
      autoimmune thyroid disease in children with implications for the developing 
      central nervous system. Careful surveillance of thyroid function post-HSCT is 
      essential.
FAU - Sinha, Akash
AU  - Sinha A
AD  - Department of Pediatric Endocrinology, Great North Children's Hospital, Newcastle 
      upon Tyne, UK.
FAU - Abinun, Mario
AU  - Abinun M
FAU - Gennery, Andrew R
AU  - Gennery AR
FAU - Barge, Dawn
AU  - Barge D
FAU - Slatter, Mary
AU  - Slatter M
FAU - Cheetham, Tim
AU  - Cheetham T
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - United States
TA  - Thyroid
JT  - Thyroid : official journal of the American Thyroid Association
JID - 9104317
SB  - IM
MH  - Child
MH  - Child, Preschool
MH  - Female
MH  - Graves Disease/*etiology/immunology
MH  - Hematopoietic Stem Cell Transplantation/*adverse effects
MH  - Humans
MH  - Immune Reconstitution Inflammatory Syndrome/*etiology/immunology
MH  - Infant
MH  - Male
MH  - Young Adult
EDAT- 2013/04/06 06:00
MHDA- 2014/03/13 06:00
CRDT- 2013/04/06 06:00
PHST- 2013/04/06 06:00 [entrez]
PHST- 2013/04/06 06:00 [pubmed]
PHST- 2014/03/13 06:00 [medline]
AID - 10.1089/thy.2012.0618 [doi]
PST - ppublish
SO  - Thyroid. 2013 Aug;23(8):1010-4. doi: 10.1089/thy.2012.0618.