Intrathecal baclofen therapy after liver transplant in a patient with Crigler-Najjar syndrome.

Crigler-Najjar syndrome (CNS) is described as severe infantile, nonhemolytic, unconjugated hyperbilirubinemia and is divided into type I and type II according to the patient's response to phenobarbital treatment. Patients with type I CNS usually require a liver transplant. These patients often have spasticity and dystonia, both of which can be treated with intrathecal baclofen therapy.

Treatment of classic pantothenate kinase-associated neurodegeneration with deferiprone and intrathecal baclofen.

Neurodegeneration with brain iron accumulation (NBIA) describes a heterogeneous family of diseases characterized by high brain iron, particularly in the basal ganglia. The most common manifestation of childhood NBIA is classic pantothenate kinase-associated neurodegeneration (PKAN), a severe, progressive type of autosomal recessive neuroaxonal dystrophy characterized by early onset of symptoms (as opposed to atypical PKAN, with an average age of onset of 14 yrs). There is currently no established therapy for the disease.

The rehabilitation of children with anti-N-methyl-D-aspartate-receptor encephalitis: a case series.

Anti-N-methyl-D-aspartate (NMDA)-receptor encephalitis is a serious, complex, and potentially fatal disease in children. Children with this condition frequently present with altered mental status, rapid functional deterioration, and seizures. Despite aggressive treatment with immune therapy such as corticosteroids, intravenous immunoglobin, and plasmapheresis, children often need extensive rehabilitative services and can be left with lasting deficits.

Pediatric brain tumor rehabilitation.

Children with brain tumors experience significant functional deficits related to the primary disease process and also as a consequence of its treatment. As in adults, childhood brain tumors represent a heterogeneous group of tumors, which vary in pathologic characteristics, tumor biology, response to therapy, anatomic location, and age at diagnosis. With the advances in diagnostic strategies, neurosurgical techniques and therapeutic trials over the last 30 years, a greater proportion of these children are surviving into adulthood.

Clinical assessment and treatment of carpal tunnel syndrome in the mucopolysaccharidoses.

The mucopolysaccharidoses (MPS) are a common cause of carpal tunnel syndrome (CTS) in children and adolescents. As the MPS diseases are progressive in nature, it is essential that CTS in these children is readily diagnosed and treated, before damage to the median nerve becomes irreversible. Currently, no standards for diagnosing and treating CTS associated with MPS exist.

Outcomes of post-acute hospital episodes for young children requiring airway support.

In this descriptive study, we examined changes in invasive and non-invasive airway support; studied the rates of home discharge vs. long-term care or acute hospitalization; and examined the relationship between the level of airway support and discharge to home for 92 children (<3 years of age) with 104 admission-discharge episodes to a consortium of pediatric rehabilitation hospitals over a one-year period. We found a significant reduction (p < 0.

Ventilator weaning outcomes in chronic respiratory failure in children.

The purpose of this study was to describe mechanical ventilation weaning outcomes for children with chronic respiratory failure discharged from one of six post-acute rehabilitation facilities. Demographic, clinical and outcome data were collected from the medical record. Forty-four children were included in this prospective series; 20 (45%) were weaned off the ventilator at discharge.

Adolescent sexuality and disability.

Regardless of what our beliefs about sex and disability may be, as health care providers we can promote the health and well being of our patients with disabilities in several ways. First and perhaps foremost, physical and programmatic barriers to accessing general health care including routine gynecologic care must be dramatically reduced. The promise of Title III of the Americans with Disabilities Act must be aggressively extended to our health care system to ensure equal access to routine health care for all.