Selective neurotrophin deficiency in infantile hypertrophic pyloric stenosis*
Section snippets
Tissue specimens
Full-thickness pyloric muscle biopsy specimens were obtained at the time of pyloromyotomy from 8 patients with IHPS (age range, 23 to 41 days; mean, 34.5 days). Age-matched controls (age range, 14 days to 82 days; mean, 43.5 days) were taken at autopsy from patients who died without evidence of gastrointestinal disease. Autopsy was performed within 12 hours of death, and no autolysis was observed in the tissues. Controls were born at term, and the causes of death included sudden infant death
Enzyme-linked immunosorbent assay analysis
IHPS samples contained less neurotrophins than controls (Fig 1).The level of NGF in the muscle of IHPS was 2.09 ± 0.78 pg/ng of total soluble protein (mean ± SD), whereas in the muscle of normal pylorus it was 5.56 ± 1.17 pg/ng of total soluble protein. The difference was statistically significant (P <.01). The amount of BDNF in IHPS was
Discussion
Neurotrophins are a family of related, target-derived neuronal growth factors that support neuronal growth, survival and differentiation.18 All members are synthesized as precursor polypeptides that, through enzymatic cleavage, yield mature homodimeric neurotrophins. Neurotrophic interaction with target neuron receptors results in a variety of trophic and tropic events. It is well established that peripheral sensory and postganglionic sympathetic neurons are under the control of neurotrophins
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Address reprint requests to Prem Puri, MS, FRCS, FRCS (Ed), FACS, Director of Research, Children's Research Centre, Our Lady's Hospital for Sick Children, Crumlin, Dublin 12, Ireland.