Diabetic ketoacidosis (DKA) is a serious, potentially lethal complication of type 1 diabetes mellitus that may be present at diagnosis. The aim of this study was to determine factors associated with presentation in DKA in new-onset youth and compare the rate of DKA and risk factors to a similar study 15 years prior. This study was a retrospective chart review of newly diagnosed patients with type 1 diabetes mellitus from 2010 to 2013. Of the 276 patients, 29% presented in DKA, compared with 38% 15 years prior ( P < .002). Those with Medicaid, those misdiagnosed at initial encounter, and those not evaluated by a pediatrician initially were more likely to present in DKA ( P = .002, P = .002, P < .001, respectively). The diagnosis of diabetes was not elicited in one third of patients who ultimately presented in DKA. Pediatricians should be reeducated to ask about polyuria and polydipsia in routine encounters. Furthermore, public awareness initiatives are needed to reduce late presentation in DKA.

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BACKGROUND: This a unique case of late-onset endocrinopathies after stem cell transplant in a girl with Griscelli syndrome. Griscelli syndrome is a rare disorder characterized by partial albinism, silver hair and immune failure with alteration in genes necessary for melanin transport, which is curative by stem cell transplant. Although late endocrinopathies are quite common in other disorders after stem cell transplant, these complications have not been reported in Griscelli syndrome. CLINICAL CASE: A 7-year old female who received a stem cell transplant as a toddler and subsequently developed graft-versus-host-disease (GvHD) at 2 years of age presented for evaluation of growth failure. Patient had severe short stature along with mild hyperthyroxinemia with subsequent diagnosis of Graves’ disease which was treated with methimazole. Although hypothyroidism is more commonly seen after stem cell transplant, rare cases of hyperthyroidism have been reported. Despite normal GH and IGF-1 levels, GH therapy was commenced due to persistent growth failure. She showed a robust increase in growth parameter from -6 to -2 SD below the mean. She started spontaneous puberty, however, biochemical evaluation showed hypergonadotropic hypogonadism with undetectable anti-mullerian hormone (AMH) which is consistent with low ovarian reserve most likely related to total body irradiation prior to stem cell transplant. CONCLUSION: Our patient demonstrates that growth failure, thyroid disease and ovarian dysfunction can be complications of stem cell transplants in young children with Griscelli syndrome. This can be a result of the underlying disease leading to transplant, conditioning regimen prior to transplant or complications thereafter. GvHD may also be a risk factor for future autoimmune endocrine complications in this syndrome and in other syndromes treated with stem cell transplant. REFERENCES: (1) Griscelli C, Prunieras M. Pigment dilution and immunodeficiency: a new syndrome. Int J Dermatol. 1978;10:788–91. (2) Sağ E, Gönç N, Alikaşifoğlu A, et al. Hyperthyroidism after allogeneic hematopoietic stem cell transplantation: A Report of Four Cases. J Clin Res Pediatr Endocrinol. 2015;4:349–54.