Lipodystrophy and other severe insulin resistance (SIR) syndromes are rare causes of diabetes which are frequently associated with delayed diagnosis and severe metabolic disease. Diagnosis and management is complex and guidelines for management do not currently exist. The National Severe Insulin Resistance (NSIR) Service was commissioned by the National Specialist Commissioning Team for adults and children with lipodystrophy and/or SIR in England. It offers a multi-disciplinary clinic with consultants in adult and paediatric Diabetes and Endocrinology, specialist nurses and dietitians and support from the clinical genetics and obesity teams. The NSIR service uses a new model of cross-organisational working which includes the same team providing adult and paediatric management with several members of the same family often affected.
The rarity of severe insulin resistance and lipodystrophy leaves patients subject to poor clinical management and outcomes, but research has contributed to improvements. Since 1996, patients with severe forms of insulin resistance but not severe obesity have contributed to a unique collection (‘biobank’) of DNA and blood samples. The genetic basis for 10 previously uncharacterised syndromes have been identified to date.
Research regarding the effects of recombinant leptin therapy on severely obese children with congenital leptin deficiency provided experience for our current clinical practice. The clinical applicability of this research led to the Department of Health commissioning a national multidisciplinary severe insulin resistance NHS service based at Addenbrooke’s Hospital in Cambridge, the only such service in the world.
The service aims to support patients and carers, and to establish and disseminate evidence-based therapy recommendations. Mechanisms include: provision of a precise diagnosis, patient education, raising the profile of severe insulin resistance/lipodystrophy as a clinical problem, and provision of treatment interventions such as dietary and pharmacological therapies.Feedback has been encouraging. There may be potential to set up centres elsewhere in the UK. It is hoped the service will be commissioned for patients in Wales, Scotland and Northern Ireland, although this is subject to Department of Health funding. It is likely that diagnosis in patients with apparent type 2 diabetes will become more complex and accurate in the future.
Back to the top