PubMed · 5000841
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P Maroteaux, B Martinelli, E Campailla. 1971-10-09. [Acromesomelic dwarfism].. https://pubmed.ncbi.nlm.nih.gov/5000841/
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Intracranial MR imaging was performed in five patients with achondroplasia. All patients had narrowing of the subarachnoid space at the level of the foramen magnum that was mainly due to protrusion of the posterior aspect. Three patients had compressive deformities of the brainstem and/or upper cervical spine. Among them, two patients had deformities of the pons. Relative upward displacement of the brainstem was seen in all patients. Hydrocephalus was seen in three patients.
A pilot study was carried out to examine the safety and efficacy of recombinant human growth hormone for growth-promoting therapy of achondroplasia. The data suggest that the agent in doses used to treat non-GH-deficient forms of short stature (0.3 mg/kg/wk) modestly increases overall height velocity in some children with achondroplasia. The effect was seen mainly in children with the lowest growth velocities prior to treatment. No untoward effects were noted. Several questions were raised that require further study.
The diagnosis of growth hormone deficiency is established on the basis of auxological and endocrinological studies. Without treatment, the final stature will be reduced; by way of treatment, administration of biosynthetic human growth hormone is required. Total and partial growth hormone deficiency can be distinguished, as also neurosecretory dysfunction of growth hormone secretion. Growth hormone can also be successfully administered to girls with the Ullrich-Turner syndrome, but the doses required are somewhat higher than in the case of growth hormone deficiency. Other forms of reduced stature might possibly benefit from growth hormone treatment, but the results of ongoing studies on this point must first be obtained.