Abstract
Type 1 diabetes mellitus (T1DM) is an autoimmune disease characterized by insufficient insulin production of the pancreatic beta-cells. Patients with T1DM will have a higher risk of other autoimmune disorders like celiac and thyroid diseases. Hypothyroidism is the failure of the thyroid gland to secrete an adequate amount of thyroxine, which is required for physical growth, brain development, and cellular metabolism. Most studies reported that children with T1DM have a higher incidence of hypothyroidism than normal children, with 9.6% having hypothyroidism and 19% having positive anti-TPO antibodies. Hypothyroidism will aggravate the condition in a child with T1DM and vice versa. Uncontrolled diabetes for a long time might increase insulin resistance due to complete depression of the hypothalamus-pituitary thyroid axis. A rare complication of poorly controlled T1DM is Mauriac syndrome, characterized by elevated liver enzymes, hyperlipidemia, cushingoid features, growth retardation, and hepatomegaly due to glycogenic hepatopathy. Van Wyk–Grumbach syndrome is also a rare complication of long-standing, untreated hypothyroidism, manifested by breast development, multicystic ovary, uterine bleeding associated with lack of pubic and axillary hair growth, and delayed bone age. Here, we report a case with two rare complications of Mauriac syndrome and Van Wyk–Grumbach syndrome in a child with hypothyroidism and poorly controlled T1DM.
References
Nederstigt C, Uitbeijerse BS, Janssen LG, Corssmit EP, de Koning EJ, Dekkers OM. Associated auto-immune disease in type 1 diabetes patients: a systematic review and meta-analysis. Eur J Endocrinol. 2019 Feb 1;180(2):135-144. https://doi.org/10.1530/eje-18-0515
Fatourechi A, Ardakani HM, Sayarifard F, Sheikh M. Hypothyroidism among pediatric patients with type 1 diabetes mellitus, from patients’ characteristics to disease severity. Clin Pediatr Endocrinol. 2017;26(2):73-80. https://doi.org/10.1297/cpe.26.73
Giorda CB, Carnà P, Romeo F, Costa G, Tartaglino B, Gnavi R. Prevalence, incidence and associated comorbidities of treated hypothyroidism: an update from a European population. Eur J Endocrinol. 2017 May 1;176(5):533-42. https://doi.org/10.1530/eje-16-0559
Medhioub M, Ayedi H, Chelbi E, Khsiba A, Hamzaoui L, Azzouz MM. Mauriac syndrome: An unusual presentation with portal fibrosis. Presse Med. 2019 Jun;48(6):718-720. https://doi.org/10.1016/j.lpm.2019.05.013
Pant V, Baral S. Van Wyk Grumbach syndrome with precocious puberty and ovarian cysts: Value of thyroid function tests. J Pediatr Surg Case Rep. 2019 Apr 1;43:32-4. https://doi.org/10.1016/j.epsc.2019.01.010
Topaloğlu Ö, Demiral Sezer S, Demir B, Akarken D. Mauriac syndrome: Case report and review of the literature. Turk J Endoc Metab. 2017; 21(4): 131-135. https://doi.org/10.25179/tjem.2017-56496
Casto C, Pepe G, Li Pomi A, Corica D, Aversa T, Wasniewska M. Hashimoto’s thyroiditis and Graves’ disease in genetic syndromes in pediatric age. Genes. 2021 Feb;12(2):222. https://doi.org/10.3390/genes12020222
Ruggeri RM, Trimarchi F, Giuffrida G, Certo R, Cama E, Campennì A, et al. Autoimmune comorbidities in Hashimoto's thyroiditis: different patterns of association in adulthood and childhood/adolescence. Eur J Endocrinol. 2017 Feb 1;176(2):133-41. https://doi.org/10.1530/eje-16-0737
Won JH, Lee JY, Hong HS, Jeong SH. Thyroid nodules and cancer in children and adolescents affected by Hashimoto’s thyroiditis. British J Radiol. 2018 Jul;91(1087):20180014. https://doi.org/10.1259/bjr.20180014
Sahana PK, Deshmukh AS, Sengupta N, Das C, Dasgupta R. Case Report: An unusual case of incomplete isosexual precocious puberty in a young girl with juvenile hypothyroidism. J Clin Scientific Res. 2014;3(3): 188-190. https://doi.org/10.15380/2277-5706.JCSR.13.072
Marr A, Hardy K, Curtis J. A 14-year-old girl with short stature, incomplete puberty and severe menstrual bleeding. Paediatr Child Health. 2018 May;23(2):85-88. https://doi.org/10.1093%2Fpch%2Fpxx113
Ayub SS, Ruzic A, Taylor JA. Ovarian cysts, vaginal bleeding and hypothyroidism in a 4-year-old female with Down Syndrome: A case of Van Wyk-Grumbach Syndrome. J pediatr surg. 2017 Oct 1;25:5-9. https://doi.org/10.1016/j.epsc.2017.07.007
Reddy P, Tiwari K, Kulkarni A, Parikh K, Khubchandani R. Van Wyk Grumbach syndrome: a rare consequence of hypothyroidism. Indian J Pediatr. 2018 Nov;85(11):1028-30. https://doi.org/10.1007/s12098-018-2704-2
Kacem FH, Kalthoum M, Ammar M, Sefi W, Mnif M, Abid M. Van Wyk and Grumbach Syndrome: An Unusual Presentation of Acquired Hypothyroidism. Mymensingh Med J. 2017 Jul; 26(3)667-670.
Egodawaththe NS, Seneviratne SN, Gunasekara S, Amarasekara SM, Weerasekara K. Van Wyk-Grumbach syndrome and oligosyndactyly in a 6-year-old girl: a case report. J Med Case Reports. 2020 Dec;14(1):1-7. https://doi.org/10.1186/s13256-020-02472-z
Lin CH, Lee YJ, Huang CY, Kao HA, Shih BF, Wang AM, et al. Thyroid function in children with newly diagnosed type 1 diabetes mellitus. Acta Paediatr Taiwan. 2003 May-Jun;44(3):145-9.
Balsamo C, Zucchini S, Maltoni G, Rollo A, Martini AL, Mazzanti L, Pession A, et al. Relationships between thyroid function and autoimmunity with metabolic derangement at the onset of type 1 diabetes: a cross-sectional and longitudinal study. J Endocrinol Investigation. 2015 Jun;38(6):701-7. https://doi.org/10.1007/s40618-015-0248-0
This work is licensed under a Creative Commons Attribution 4.0 International License.
Copyright (c) 2023 Aida Al Jabri, Aeshah Al Johar, Mohamed Tahar Yacoubi