J Pediatr Endocr Met 2011;24(7-8):581–583 © 2011 by Walter de Gruyter • Berlin • Boston. DOI 10.1515/JPEM.2011.253 Diabetes in young – unusual case presentation K.V.S. Hari Kumar 1, *, Sai Priya 1 , Altamash Shaikh 2 and Pitambar Prusty 1 1 Department of Endocrinology, Command Hospital, Lucknow 226002, U.P., India 2 Department of Endocrinology, PD Hinduja National Hospital and Medical Research Centre, Mumbai 400016, India Abstract Diabetes in young is increasing in prevalence with each decade. Phenotypic features like obesity and acanthosis nig- ricans characterize type 2 diabetes, whereas autoimmune diseases like vitiligo and hypothyroidism suggest type 1 dia- betes. We recently encountered a young boy with vitiligo who presented with hyperglycemia, but not associated with keto- nuria and has underlying chronic pancreatitis with secondary diabetes. We report the case for its unusual presentation and etiology of the diabetes. Keywords: adolescent diabetes; autoimmunity; chronic pancreatitis. Introduction The incidence of diabetes has increased spectacularly in the last two decades in children and adolescents. This increase is seen in both type 1 and type 2 diabetes and is parallel with increasing rates of obesity in children (1, 2). In this changing scenario, reliance on phenotypic characteristics to determine the type of diabetes is no longer feasible. We recently encountered a non-obese adolescent with evidence of autoimmune disease and presented with ketosis resistant hyperglycemia. Case report A 13-year-old boy, a product of a consanguineous marriage, presented with osmotic symptoms and weight loss of three weeks duration. He was delivered at 36 weeks of gestation with normal birth weight and had normal developmental milestones. The parents denied any similar illness in siblings or family members. There was no history of candidiasis, leg cramps, seizures, carpopedal spasm and recurrent infections in the past. He denied past history of pain abdomen, diarrhea and steatorrhea. Examination revealed a height of 150 cm (50th centile), a weight of 35 kg (25th centile), normal anthropometric proportions, depressed nasal bridge, vitiligo (Figure 1), ectodermal dystrophy (Figure 2) and prepubertal genitalia with absent axillary hair. Trousseaus test was nega- tive and he had no evidence of mucocutaneous candidiasis. His random blood sugar was 455 mg/dL without ketonuria and HbA1c was 10.4%. Other investigations revealed Hb 13.6 g/dL, normal blood counts, lipid profile (total choles- terol 146 mg/dL, HDL 44 mg/dL, LDL 97 mg/dL, triglycer- ides 122 mg/dL) and metabolic parameters. Serum calcium (9.2 mg/dL), phosphorus (3.8 mg/dL) and alkaline phos- phatase (95 IU/L) were normal. Stool examination did not reveal any fat globules. His fasting blood sugar (304 mg/ dL) and post meal blood sugar (554 mg/dL) were high with- out ketonuria. GAD antibody was negative and post meal C peptide (1.1 ng/mL) was low. His thyroid profile showed total triiodothyronine 124 ng/dL (normal 60–175), total thy- roxine 7.9 μg/dL (normal 4–11.5), thyroid stimulating hor- mone 2.2 mIU/mL (normal 0.3–4.5), thyroglobulin antibody 53.6 IU/mL (normal 0–20) and thyroid peroxidase antibody 128.7 IU/mL (normal 0–15). Ultrasonography revealed a cys- tic lesion in the region of the pancreas and plain X-ray abdo- men revealed no evidence of pancreatic calculi. CT scan of the abdomen showed atrophic pancreas with peripancreatic cyst of 4.3×3.2×2.2 cm size, splenic vein thrombosis with collateral formation (Figure 3). He was diagnosed to have diabetes secondary to chronic pancreatitis and associated autoimmune diseases like vitiligo and autoimmune thyroidi- tis. He was managed with split mix insulin regime along with advice about diabetic diet and life-style measures. He was not given any pancreatic enzyme supplements and was not investigated further for chronic pancreatitis. The pancreatic pseudocyst was left in situ in view of asymptomatic nature of illness. Detailed family history did not reveal any other case of pancreatitis and none of the parents and siblings had a similar illness. Discussion Our patient had all the features to suggest autoimmune dia- betes with coexisting vitiligo and ectodermal dystrophy which are features of Autoimmune PolyEndocrinopathy, Candidiasis, Ectodermal Dystrophy (APECED) syndrome (3). However, the absence of ketonuria, despite hyperglyce- mia, was a clue we missed initially in looking for secondary *Corresponding author: K.V.S. Hari Kumar, Department of Endocrinology, Command Hospital, Lucknow 226002, U.P., India Phone: +91-945-4674679, Fax: +91-522-2851058, E-mail: hariendo@rediffmail.com Authenticated | hariendo@gmail.com Download Date | 5/23/12 6:48 PM