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. 2019 Oct 9;5(10):eaax2011. doi: 10.1126/sciadv.aax2011

Table 1. Clinical PHH phenotypes recapitulated by this neonatal LPA-induced mouse model.

Sequelae reported in clinical studies following cohorts of hydrocephalic patients compared to phenotypes observed in this neonatal model of PHH. IVH was mimicked by injecting LPA directly into the lateral ventricle of postnatal day 8 (P8) mice.

Hydrocephalus
characteristics
Clinical studies Neonatal model
Bleeding/hemorrhage Yes (13) Mimicked
Ventricular dilation Yes (13) Yes
Elevated CSF pressure Yes (13) Yes
Early lethality 75% untreated (41)
and 20% treated (3)
80% by 12 weeks
Loss of ependymal
layer
Yes (15, 16) Yes
Ciliary defects Yes (42) Yes
Aqueductal occlusion Sometimes (43) No
Monocyte infiltration Implicated in shunt
failure (44) and
systemic
inflammation (45)
Yes