SCIENTIFIC LETTER TO THE EDITOR Severe Limited Joint Mobility Syndrome in a Child with Type 1 Diabetes Mellitus Vandana Jain & Richa Jain Received: 13 July 2011 /Accepted: 25 November 2011 /Published online: 13 December 2011 # Dr. K C Chaudhuri Foundation 2011 Sir, A 14-y-old boy diagnosed as a case of type 1 diabetes mellitus at 18 mo of age and celiac disease at 6 y was noted to have progressive painless restriction of move- ment involving the proximal and distal interphalangeal joints of both the hands for duration of 6 mo. The joint restriction initially began from the fifth finger with subsequent involvement of the other fingers. The boy had been taking human regular and NPH insulin in a mixed split regimen. However, his blood sugar monitoring, dietary compliance with gluten free diet, and follow-up visits had always been very erratic and diabetes control had been quite poor. On examination, the boy was stunted (height 62% of expected), underweight (weight 70% of expected) and had prepubertal sexual maturity. The injection sites had evidence of lipodys- trophy. There was no neuropathy or retinopathy on examination. There was a fixed flexion deformity of the involved joints; with maximum restriction being present in the fifth finger; however, there was no joint swelling (Fig. 1). Other joints were normal. He was diagnosed as a case of Severe Limited Joint Mobility (LJM) syndrome on the basis of the clinical picture. Investigations revealed a high HbA 1C of 14.9 g% but no microalbuminuria. LJM syndrome, which occurs due to accumulation of stable advanced glycation end products in the periartic- ular connective tissue, is a long term complication of diabetes mellitus, associated with poor glycemic control. The joint involvement begins from interphalangeal and metacarpophalangeal joints of the fifth finger and pro- gresses radially to involve other fingers, rarely involving wrist, knee and even cervical spine. LJM is classified as (a) none (equivocal or unilateral finding); (b) mild (involvement of 1–2 interphalangeal joints, the wrist joint or only metacarpophalangeal joint bilaterally); (c) moderate (3 or more interphalangeal joints or 1 interphalangeal joint and wrist bilaterally); and (d) severe (cervical spine involvement or obvious hand deformity at rest) [1]. The arthropathy is painless and therefore often goes unheeded until it leads to loss of function due to lost range of motion. The significance of detecting LJM in patients with diabetes is that it is associated with a 3–4 fold higher incidence of retinopathy, neuropathy and nephropathy [1, 2]. The incidence of LJM has declined substantially over the years with improvement in diabetes control due to availability of better insulin regimes and monitoring devices. Infante et al. reported a decline in incidence from 31% in 1978 to 7% in 1998; with incidence of moderate–severe LJM, as seen in our patient, being only 0.6% [3]. The joint symptoms may show improvement with physiotherapy and better glycemic control. V. Jain (*) : R. Jain Division of Pediatric Endocrinology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India e-mail: [email protected] Indian J Pediatr (July 2012) 79(7):959–960 DOI 10.1007/s12098-011-0622-7

Severe Limited Joint Mobility Syndrome in a Child with Type 1 Diabetes Mellitus

Download PDF Report

Upload
richa-jain
View
214
Download
0

Embed Size (px)

Citation preview

SCIENTIFIC LETTER TO THE EDITOR

Severe Limited Joint Mobility Syndrome in a Childwith Type 1 Diabetes Mellitus

Vandana Jain & Richa Jain

Received: 13 July 2011 /Accepted: 25 November 2011 /Published online: 13 December 2011# Dr. K C Chaudhuri Foundation 2011

Sir,A 14-y-old boy diagnosed as a case of type 1 diabetesmellitus at 18 mo of age and celiac disease at 6 y wasnoted to have progressive painless restriction of move-ment involving the proximal and distal interphalangealjoints of both the hands for duration of 6 mo. The jointrestriction initially began from the fifth finger withsubsequent involvement of the other fingers. The boyhad been taking human regular and NPH insulin in amixed split regimen. However, his blood sugar monitor-ing, dietary compliance with gluten free diet, andfollow-up visits had always been very erratic and dia-betes control had been quite poor. On examination, theboy was stunted (height 62% of expected), underweight(weight 70% of expected) and had prepubertal sexualmaturity. The injection sites had evidence of lipodys-trophy. There was no neuropathy or retinopathy onexamination. There was a fixed flexion deformity ofthe involved joints; with maximum restriction beingpresent in the fifth finger; however, there was no jointswelling (Fig. 1). Other joints were normal. He wasdiagnosed as a case of Severe Limited Joint Mobility(LJM) syndrome on the basis of the clinical picture.

Investigations revealed a high HbA1C of 14.9 g% butno microalbuminuria.

LJM syndrome, which occurs due to accumulation ofstable advanced glycation end products in the periartic-ular connective tissue, is a long term complication ofdiabetes mellitus, associated with poor glycemic control.The joint involvement begins from interphalangeal andmetacarpophalangeal joints of the fifth finger and pro-gresses radially to involve other fingers, rarely involvingwrist, knee and even cervical spine. LJM is classified as(a) none (equivocal or unilateral finding); (b) mild (in-volvement of 1–2 interphalangeal joints, the wrist jointor only metacarpophalangeal joint bilaterally); (c) mod-erate (3 or more interphalangeal joints or 1 interphalan-geal joint and wrist bilaterally); and (d) severe (cervicalspine involvement or obvious hand deformity at rest)[1]. The arthropathy is painless and therefore often goesunheeded until it leads to loss of function due to lostrange of motion.

The significance of detecting LJM in patients with dia-betes is that it is associated with a 3–4 fold higher incidenceof retinopathy, neuropathy and nephropathy [1, 2]. Theincidence of LJM has declined substantially over the yearswith improvement in diabetes control due to availability ofbetter insulin regimes and monitoring devices. Infante et al.reported a decline in incidence from 31% in 1978 to 7% in1998; with incidence of moderate–severe LJM, as seen inour patient, being only 0.6% [3]. The joint symptoms mayshow improvement with physiotherapy and better glycemiccontrol.

V. Jain (*) :R. JainDivision of Pediatric Endocrinology, Department of Pediatrics,All India Institute of Medical Sciences,New Delhi, Indiae-mail: [email protected]

Indian J Pediatr (July 2012) 79(7):959–960DOI 10.1007/s12098-011-0622-7

Page 2: Severe Limited Joint Mobility Syndrome in a Child with Type 1 Diabetes Mellitus

References

1. Rosenbloom AL, Silverstein JH. Connective tissue and joint disordersin diabetes mellitus. Endocrinol Metab Clin North Am. 1996;25:473–83.

2. Rosenbloom AL. Limitation of finger joint mobility in diabetesmellitus. J Diabet Complications. 1989;3:77–87.

3. Infante JR, Rosenbloom AL, Silverstein JH, Garzarella L, PollockBH. Changes in frequency and severity of Limited joint mobility inchildren with type I diabetes mellitus between 1976–1978 and 1998.J Pediatr. 2001;138:33–7.

Fig. 1 Fixed flexiondeformity of proximal anddistal interphalangeal jointsof all fingers.

960 Indian J Pediatr (July 2012) 79(7):959–960