Hence, there is no response to treatment with phenobarbital, which causes CYP450 enzyme induction. Most patients (type IA) have a mutation in one of the common exons (2 to 5), and have difficulties conjugating several additional substrates (several drugs and xenobiotics). A smaller percentage of patients (type IB) have mutations limited to the bilirubin-specific A1 exon; their conjugation defect is mostly restricted to bilirubin itself. Before the availability of phototherapy, these children died of kernicterus (bilirubin encephalopathy) or survived until early adulthood with clear neurological impairment. Today, therapy includes

exchange transfusions in the immediate neonatal period
12 hours/day phototherapy
heme oxygenase inhibitors to reduce transient worsening of hyperbilirubinemia (although the effect of the drug decreases over time)
oral calcium phosphate and carbonate to form complexes with bilirubin in the gut
liver transplantation before the onset of brain damage and before phototherapy becomes ineffective at later age

Type II
The inheritance patterns of both Crigler–Najjar syndrome types I and II are autosomal recessive.However, type II differs from type I in a number of different aspects:

Bilirubin levels are generally below 345 μmol/L [20 mg/dL] (range 100–430 μmol/L [6–24 mg/dL]; thus, overlap may sometimes occur), and some cases are only detected later in life. Because of lower serum bilirubin, kernicterus is rare in type II. Bile is pigmented, instead of pale in type I or dark as normal, and monoconjugates constitute the largest fraction of bile conjugates. UGT1A1 is present at reduced but detectable levels (typically <10% of normal), because of single base pair mutations.
The problem with the assessment is that patients may only report the consequences of sleepiness: loss of energy, fatigue, weariness, difficulty remembering or concentrating, etc. It is crucial to aim for objective measures to quantify the sleepiness. A good measurement tool is the multiple sleep latency test (MSLT). It assesses the sleep onset latency during the course of one day—often from 8:00 to 16:00. An average sleep onset latency of less than 5 minutes is an indication of pathological sleepiness. Severity
A number of diagnostic tests, including the Epworth Sleepiness Scale, are available to help ascertain the seriousness and likely causes of abnormal somnolence. Treatment
Somnolence is a symptom, so the treatment will depend on its cause. If the cause is the behavior and life choices of the patient (like working long hours, smoking, mental state), it may help to get plenty of rest and get rid of distractions. Its also important to investigate whats causing the problem, such as stress or anxiety, and take steps to reduce the feeling. See also
References


== External links ==