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 studiesNeonatal model
Bleeding/hemorrhageYes (13)Mimicked
Ventricular dilationYes (13)Yes
Elevated CSF pressureYes (13)Yes
Early lethality75% untreated (41)
and 20% treated (3)
80% by 12 weeks
Loss of ependymal
layer
Yes (15, 16)Yes
Ciliary defectsYes (42)Yes
Aqueductal occlusionSometimes (43)No
Monocyte infiltrationImplicated in shunt
failure (44) and
systemic
inflammation (45)
Yes