Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2021 Jan-Apr;12(1):100-105.
doi: 10.4103/njms.NJMS_55_20. Epub 2021 Mar 16.

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

Sourabh Ramesh Joshi  1 , Gowri Swaminatham Pendyala  2 , Pratima Shah  1 , Bhushan Pustake  3 , Viddyasagar Mopagar  1 , Neeta Padmawar  1
Affiliations
Case Reports

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

Sourabh Ramesh Joshi et al. Natl J Maxillofac Surg. 2021 Jan-Apr.

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.

PubMed Disclaimer

Conflict of interest statement

There are no conflicts of interest.

Figures

Figure 1
Figure 1
Macroglossia and elfin features
Figure 2
Figure 2
Marked acanthosis nigricans and hirsutism, distended abdomen
Figure 3
Figure 3
Rough skin (upper extermities)
Figure 4
Figure 4
Rough skin (lower extremities)
Figure 5
Figure 5
Crowding of teeth, hyperplastic gingiva
Figure 6
Figure 6
Postoperative picture, extraction of the offending teeth
See this image and copyright information in PMC

References

    1. Summitt RL, Favara BE. Leprechaunism (Donohue's syndrome): A case report. J Pediatr. 1969;74:601–10. - PubMed
    1. Clark DR, Edwards HE. Dysendocrinism. J Pediatr. 1948;32:739–48. - PubMed
    1. Donohue WL, Uchida I. Leprechaunism: A euphemism for a rare familial disorder. J Pediatr. 1954;45:505–19. - PubMed
    1. Hassan I, Altaf H, Yaseen A. Rabson-mendenhall syndrome. Indian J Dermatol. 2014;59:633. - PMC - PubMed
    1. Mohanan S, Chandrashekar L, Semple RK, Thappa DM, Parameswaran N, Negi VS, et al. Rabson-mendenhall syndrome with recurrent cerebral infarcts caused by a novel INSR mutation. Int J Dermatol. 2013;52:182–5. - PubMed

Publication types