Inherited Nasal and Laryngeal Degenerative Chondropathy

Inherited degenerative chondropathy – an autosomal dominant new clinical entity: report two cases and follow-up of four cases

“…The mode of inheritance in this report appears to be autosomal dominant as the affected brothers have two other siblings who are normal (Figure 1). This was also the pattern observed in our previous report (Kurien et al 1989).…”

“…Inherited degenerative chondropathy has been described previously in four of our patients (Kurien et al, 1989). Here we report on two additional patients, who are also siblings.…”

“…A five-year follow-up of the four patients in our previous report who are on dapsone therapy (Gouet et al, 1984) shows no evidence of progression of the disease and no other additional features have been detected. A follow-up of both the brothers in this report after two years of dapsone therapy (Kurien et al. 1989) has shown no worsening of stridor.…”

“…In addition to the saddle-nose deformity and laryngeal stenosis with cartilage calcification, these two patients were found to be of short stature, had associated tracheal stenosis, loss of thoracic kyphosis and lumbar lordosis causing straight back syndrome and a sensorineural hearing loss. In our earlier report (Kurien et al, 1989) the patients had only the first two features suggesting the involvement of the hyaline cartilages. This may be because all of them presented at less than 16 years old and involvement of elastic cartilages may be a delayed feature.…”

“…Inherited degenerative chondropathy involving laryngeal and nasal cartilages has been described previously (Kurien et al, 1989). These two patients also have inherited degenerative chondropathy.…”