Clinical Examples Short Stature

Case 1 was 2% years old when she was diagnosed with JIA systemic onset. She was initially very unwell with associated bone marrow suppression, and she developed pericarditis during the first year of her illness. Her management included nonsteroidal anti-inflammatories, intermittent pulses of intravenous methylprednisolone, oral prednisolone in varying doses for 13 years, intra-articular steroids on six occasions, and methotrexate for 11 years.

Her statural growth during her disease course is shown in Figure 10. In addition to growth failure, her course was complicated by reduced bone

Figure 10 Growth chart for Case 1. X = bone age; bar near top of chart (dotted lines) indicates period of growth hormone treatment.

density and consequent vertebral crush fractures at 10 years of age. She was treated with three monthly cycles of pamidronate for a year and then oral alendronate for a year.

Figure 11 Interrelationship between disease activity (ESR and prednisolone dose) and height velocity as illustrated by Case 1.

Assessment of her growth failure included GH stimulation testing, which was normal, and her IGF-1 level, which was low-normal. GH treatment was commenced when she was nearly nine years old when her height SDS was -4.3. This was continued for 6% years. Figure 11 shows her height velocity response to GH during the first four years of treatment, and also nicely demonstrates the influence of ongoing disease activity (as indicated by ESR and Prednisolone dose) on this response. The first two years of treatment coincided with disease flares and vertebral crush fractures. As has been described in many of the GH treatment studies in JIA, GH treatment maintained her height SDS between —4.4 and —4.7. It wasn't until she reached puberty late at 14 years and then had her pubertal growth spurt that her height SDS increased. This reached —3.2 SDS just following menarche in stage 4 puberty.

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