Severe insulin resistance syndrome - A rare case report and review of literature

Abstract

Insulin Resistance syndromes (IR's), are a group of genetic disorders caused due a functional defect in chromosome 19p13. It is an autosomal recessive condition. Donohue Syndrome was initially described by Donohue and Uchida in 1948 and 1954, a case of sisters born to parents with a first-degree consanguineous marriage. Infants presented with typical facial features that resembled the Leprechaun elves of Irish fairy tales. The following is a report of a rare case of dental complications of Severe Insulin Resistance Syndrome. An eight year old female child, with characteristic features of severe insulin resistance syndrome, reported to the Department of Pediatric and Preventive Dentistry, Pravara Institute of Medical Sciences, Loni, presenting with cariously destructed molars and a previous history of dental treatment under local anaesthesia. Given her condition, it was decided to reduce the multiple appointments, to one appointment with all procedures done under general anaesthesia. The following case report discusses the advantages, disadvantages and post operative complications faced when forming a treatment strategy for Severe Insulin Resistance Syndrome.

Keywords: Anesthetic considerations; HAIR-AN syndrome; Rabson–Mendenhall syndrome; leprecaunism; severe insulin resistance syndromes.

Figure 1

Macroglossia and elfin features

Figure 2

Marked acanthosis nigricans and hirsutism, distended abdomen

Figure 3

Rough skin (upper extermities)

Figure 4

Rough skin (lower extremities)

Figure 5

Crowding of teeth, hyperplastic gingiva

Figure 6

Postoperative picture, extraction of the offending teeth