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Use of Ultra-high Field MRI in Small Rodent Models of Polycystic Kidney Disease for In Vivo Phenotyping and Drug Monitoring
Published on: June 23, 2015
Pyloric stenosis in a newborn baby with polycystic kidneys
J Tennakoon1, T H H G Koh, G Alcock
1Neonatal Intensive Care Unit, The Townsville Hospital, Douglas, Queensland, Australia. janakatennakoon@yahoo.com
Insights
Infantile hypertrophic pyloric stenosis (IHPS) and polycystic kidney disease (PKD) may be linked by nitric oxide synthetase (NOS) deficiency. This deficiency could explain pyloric stenosis, hypertension, and cyst development in PKD patients.
Area of Science:
- Nephrology
- Pediatric Surgery
- Genetics
Background:
- Infantile hypertrophic pyloric stenosis (IHPS) is associated with a higher rate of renal anomalies.
- Previous case reports suggest a potential link between IHPS and polycystic kidney disease (PKD).
Observation:
- The association between IHPS and PKD remains unclear, with possibilities including genetic factors, shared pathophysiology, or coincidence.
- A proposed hypothesis involves nitric oxide synthetase (NOS) deficiency as a potential link.
Findings:
- NOS deficiency may impair smooth muscle relaxation due to reduced local nitric oxide availability.
- This mechanism could contribute to the development of pyloric stenosis.
- NOS deficiency might also play a role in hypertension and cyst formation in autosomal-dominant PKD.
Implications:
- Understanding the role of NOS deficiency could offer new insights into the pathogenesis of both IHPS and PKD.
- This hypothesis warrants further investigation to clarify the relationship between these conditions.
- Potential therapeutic strategies targeting nitric oxide pathways could be explored for related disorders.
Abstract:
Patients with infantile hypertrophic pyloric stenosis (IHPS) have an increased incidence of renal anomalies. Association of IHPS and polycystic kidney disease (PKD) has been reported in three case histories. It is uncertain whether the association of PKD and IHPS is due to genetics, a pathophysiological link or a chance phenomenon. One hypothesis linking the two conditions is nitric oxide synthetase (NOS) deficiency. NOS deficiency leads to lack of locally available nitric oxide causing a failure of smooth muscle relaxation. It is therefore plausible that NOS deficiency plays a part in the pathogenesis of pyloric stenosis, hypertension and cyst development in autosomal-dominant PKD.
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