LARON SYNDROME: A CASE REPORT

Year 2017, Volume: 3 Issue: 2, 45 - 48, 02.10.2017

Nil Ceren Mungan

Abstract

Aim: The purpose of this case report is to define oral-dental findings of Laron Syndrome. Laron syndrome, is a very rare familiar disorder with autosomal recessive transition. Case Report: A 11-year-old patient applied to Marmara University Faculty of Dentistry for dental treatment. She had growth inadequacy, small face and high pitched voice which are among the symptoms of the syndrome. Materials and Methods: In the intraoral examination, decays and crowded teeth on the upper jaw were observed due to small sized maxilla. The patient also had gingival inflammation due to the poor oral hygiene. The restorations were made and the patient was informed about the oral hygiene behaviors. Clinical Findings: Patients with Laron Syndrome have severe postnatal growth failure and a low level insulin like growth factor- 1(IGF-1). Children have very high-pitched voice, saddle nose, delayed puberty and delayed onset of teething. 

Keywords

Laron syndrome, growth hormone insensitivity

References

• [1] Bhatia K, Cockerham R. Anaesthetic management of a parturient with Laron syndrome. International journal of obstetric anesthesia. 2011; 20: 344-46.
• [2] Park K. A cell therapy-based cure of the Laron Syndrome. Journal of controlled release: official journal of the Controlled Release Society. 2013; 165: 90.
• [3] Goswami S, Ghosh S, Chowdhury S. Growth hormone insensitivity syndrome: A sensitive approach. Indian journal of endocrinology and metabolism. 2012; 16: 817.
• [4] Laron Z, Kauli R, Lapkina L, et al. IGF-I deficiency, longevity and cancer protection of patients with Laron syndrome. Mutation Research/Reviews in Mutation Research. 2017; 772: 123-33.
• [5] Brooks AJ, Waters MJ. The growth hormone receptor: mechanism of activation and clinical implications. Nature Reviews Endocrinology. 2010; 6: 515-25.
• [6] Cotta OR, Santarpia L, Curtò L, et al. Primary growth hormone insensitivity (Laron syndrome) and acquired hypothyroidism: a case report. Journal of medical case reports. 2011; 5: 301.
• [7] Kornreich L, Konen O, Lilos P, et al. The globe and orbit in Laron syndrome. American Journal of Neuroradiology. 2011; 32: 1560-62.
• [8] Laron Z. Laron syndrome (primary growth hormone resistance or insensitivity): the personal experience 1958–2003. The Journal of Clinical Endocrinology & Metabolism. 2004; 89: 1031-44.
• [9] Diniz ET, Jorge AA, Arnhold IJ, et al. Novel nonsense mutation (p. Y113X) in the human growth hormone receptor gene in a Brazilian patient with Laron syndrome. Arquivos Brasileiros de Endocrinologia & Metabologia. 2008; 52: 1264-71.
• [10] Atreja G, Atreja SH, Jain N, et al. Oral manifestations in growth hormone disorders. Indian journal of endocrinology and metabolism. 2012; 16: 381