If the bilirubin is not rapidly reduced, ABE quickly progresses to chronic bilirubin encephalopathy. Chronic bilirubin encephalopathy (CBE)
CBE is a chronic state of severe bilirubin-induced neurological lesions. Reduction of bilirubin in this state will not reverse the sequelae. Clinically, manifestations of CBE include:
movement disorders – dyskinetic CP with often spasticity. 60% have severe motor disability (unable to walk). auditory dysfunction – auditory neuropathy (ANSD)
visual/oculomotor impairments (nystagmus, strabismus, impaired upward or downward gaze, and/or cortical visual impairment). In rare cases, decreased visual acuity(blindness) can occur. dental enamel hypoplasia/dysplasia of the deciduous teeth,
gastroesophageal reflux,
impaired digestive function. slightly decreased intellectual function: Although most individuals (approximately 85%) with kernicterus fall in normal or dull-normal range. epilepsy is uncommon.These impairments are associated with lesions in the basal ganglia, auditory nuclei of the brain stem, and oculomotor nuclei of the brain stem. Cortex and white matter are subtly involved. Cerebellum may be involved. Severe cortical involvement is uncommon. Subtle bilirubin encephalopathy (SBE)
SBE is a chronic state of mild bilirubin-induced neurological dysfunction (BIND). Clinically, this may result in neurological, learning and movement disorders, isolated hearing loss and auditory dysfunction. In the past it was thought that kernicterus (KI) often cause an intellectual disability. This was assumed due to difficulty with hearing, that is typically not detected in a normal audiogram accompanied by impairments of speech, with choreoathetosis. With advances in technology, this has proven to not be the case as those living with KI have repeatedly demonstrated their intelligence using Augmentative Communication devices.
Subjects who received L-glutamine oral powder also had fewer occurrences of acute chest syndrome (a life-threatening complication of sickle cell disease) compared with patients who received a placebo (8.6 percent vs. 23.1 percent).Common side effects of L-glutamine oral powder include constipation, nausea, headache, abdominal pain, cough, pain in the extremities, back pain and chest pain.L-glutamine oral powder received orphan drug designation. The FDA granted the approval of Endari to Emmaus Medical Inc.

Medical food
Glutamine is marketed as medical food and is prescribed when a medical professional believes a person in their care needs supplementary glutamine due to metabolic demands beyond what can be met by endogenous synthesis or diet. Safety
Glutamine is safe in adults and in preterm infants. Although glutamine is metabolized to glutamate and ammonia, both of which have neurological effects, their concentrations are not increased much, and no adverse neurological effects were detected. The observed safe level for supplemental L-glutamine in normal healthy adults is 14 g/day.Adverse effects of glutamine have been prescribed for people receiving home parenteral nutrition and those with liver-function abnormalities. Although glutamine has no effect on the proliferation of tumor cells, it is still possible that glutamine supplementation may be detrimental in some cancer types.Ceasing glutamine supplementation in people adapted to very high consumption may initiate a withdrawal effect, raising the risk of health problems such as infections or impaired integrity of the intestine. Structure
Glutamine can exist in either of two enantiomeric forms, L-glutamine and D-glutamine. The L-form is found in nature.