Management of congenital dysfibrinogenemia in pregnancy: A challenging patient case.

Afibrinogenemia and congenital dysfibrinogenemia (CD) are rare conditions with limited information available for appropriate management. Previous case reports have demonstrated the safe and efficacious use of fibrinogen replacement therapy (FRT) as a therapeutic approach to prevent hemorrhage and fetal loss in pregnant women with CD. In this case report, we present a 28-year-old pregnant woman who sought testing for CD given her family history.

Co-targeting myelin inhibitors and CSPGs markedly enhances regeneration of GDNF-stimulated, but not conditioning-lesioned, sensory axons into the spinal cord.

A major barrier to intraspinal regeneration after dorsal root (DR) injury is the DR entry zone (DREZ), the CNS/PNS interface. DR axons stop regenerating at the DREZ, even if regenerative capacity is increased by a nerve conditioning lesion. This potent blockade has long been attributed to myelin-associated inhibitors and (CSPGs), but incomplete lesions and conflicting reports have prevented conclusive agreement.

Spinal Cord Injury in Myelomeningocele: Prospects for Therapy.

Myelomeningocele (MMC) is the most common congenital defect of the central nervous system and results in devastating and lifelong disability. In MMC, the initial failure of neural tube closure early in gestation is followed by a progressive prenatal injury to the exposed spinal cord, which contributes to the deterioration of neurological function in fetuses. Prenatal strategies to control the spinal cord injury offer an appealing therapeutic approach to improve neurological function, although the definitive pathophysiological mechanisms of injury remain to be fully elucidated.

Time-lapse in vivo imaging of dorsal root nerve regeneration in mice.

Primary sensory axon injury is common after spinal cord and root injuries and causes patients to suffer chronic pain and persistent loss of sensation and motor coordination. The devastating consequences of such injuries are due primarily to the failure of severed axons to regenerate within the damaged CNS. Our understanding of the molecular and cellular events that play key roles in preventing or promoting functional regeneration is far from complete, in part because complex and dynamic changes associated with nerve injury have had to be deduced from comparisons of static images obtained from multiple animals after their death.