A novel atypical presentation of insulin autoimmune syndrome (Hirata’s disease) in a child
-
Cresio Alves
,
Julia Constança
Abstract
Insulin autoimmune syndrome (IAS) or Hirata’s disease is a rare cause of hyperinsulinemic hypoglycemia. We report the case of a child with a mild, atypical presentation of IAS. A previously healthy girl, aged 7 years old, developed non-ketotic fasting hypoglycemia during treatment for pneumonia. Laboratory evaluation during hypoglycemia showed the following results: serum glucose, 32 mg/dL (1.8 mmol/L); insulin, 5.6 μIU/mL (38.9 pmol/L); C-peptide, 1.4 ng/mL (0.47 nmol/L); anti-insulin antibody, 6.2% (normal, <2.4%); absence of ketonuria; and positive glucagon stimulation test result. Search for mutation in genes ABCC8, KCNJ11, GLUD1 and MEN1 was negative. Human leukocyte antigen (HLA) typing was HLA-DRB1*1104. Computed tomography scan of the abdomen showed a normal result. The patient evolved with spontaneous resolution of the hypoglycemia, within 30 days, with normalization of serum anti-insulin titers. The serum levels of insulin and anti-insulin antibodies in the patient of this report were not extremely high as previously reported. This novel, mild, or forme fruste presentation of IAS expands the previously reported spectrum of this disease.
Contribution of each author for the writing of the paper
CA and JC participated in the conception, design and data acquisition; wrote the initial the draft; and revised the final version. DDL, KS and CS and SE tested the ABCC8, KCNJ11, GLUD1 and MEN-1 mutations and contributed to the final version. All authors acknowledge that they have participated sufficiently in the work to take responsibility for its content.
Conflict of interest statement
Grants or fellowships supporting the paper: None.
Potential conflict of interests: The authors state that there is no financial or other relationship which might lead to conflict of interests.
References
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©2013 by Walter de Gruyter Berlin Boston
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- Lingual thyroid
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- A levothyroxine dose recommendation for the treatment of children and adolescents with autoimmune thyroiditis induced hypothyroidism
- Fasting glucagon-like peptide-1 in patients with overt hyperthyroidism and euthyroid congenital hypothyroidism
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- Evaluation of bone mineral density in children with type 1 diabetes mellitus
- Observational study of diabetes management in type 1 diabetic school-age children during holiday versus school days
- Relationship of body mass index and GAD65 antibody status on β-cell secretion at diabetes onset in African-American children
- Serum dipeptidyl peptidase 4 activity in children with type 1 diabetes mellitus
- Concomitant autoantibodies in newly diagnosed diabetic children with transient celiac serology or proven celiac disease
- Assessment of user-friendliness of the Norditropin FlexPro for pediatric patients treated with recombinant human growth hormone: results of an open-label user survey
- Cognitive impairment and gray/white matter volume abnormalities in pediatric patients with Turner syndrome presenting with various karyotypes
- Prevalence of metabolic syndrome among urban Indian adolescents and its relation with insulin resistance (HOMA-IR)
- Prevalence of metabolic syndrome in obese Chilean children and association with gene variants of the leptin-melanocortin system
- Evaluation of serum neopterin levels and its relationship with adipokines in pediatric obesity-related nonalcoholic fatty liver disease and healthy adolescents
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- Patient reports
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- Successful subcutaneous glucagon use for persistent hypoglycaemia in congenital hyperinsulinism
- A novel atypical presentation of insulin autoimmune syndrome (Hirata’s disease) in a child
- Diagnosis of septo-optic dysplasia in a neonate with hypernatremia, hypoglycemia, and persistent hypothermia
- Speech and language delay in two children: an unusual presentation of hyperthyroidism
- Lissencephaly presenting with congenital hypothyroidism
- PHEX gene mutation in a Chinese family with six cases of X-linked hypophosphatemic rickets
- Primary hyperparathyroidism as an extremely rare cause of secondary myelofibrosis in childhood
- A family with Camurati-Engelman disease: the role of the missense p.R218C mutation in TGFbeta1 in bones and endocrine glands
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- Type 1 diabetes mellitus in a patient with homozygous sickle cell anemia
- Short communication
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