Treatment
The malabsorption resulting from lack of bile acid has resulted in elemental formula being suggested, which are low in fat with < 3% of calories derived from long chain triglycerides (LCT). However, reduced very long chain fatty acids (VLCFA) has not been shown to reduce blood VLCFA levels, likely because humans can endogenously produce most VLCFA. Plasma VLCFA levels are decreased when dietary VLCFA is reduced in conjunction with supplementation of Lorenzos oil (a 4:1 mixture of glyceryl trioleate and glyceryl trierucate) in X-ALD patients. Since docosahexaenoic acid (DHA) synthesis is impaired  [59], DHA supplementation was recommended, but a placebo-controlled study has since showed no clinical efficacy. Due to the defective bile acid synthesis, fat soluble supplements of vitamins A, D, E, and K are recommended. Prognosis
Currently, no cure for Zellweger syndrome is known, nor is there a standard course of treatment. Infections should be guarded against to prevent such complications as pneumonia and respiratory distress. Other treatment is symptomatic and supportive. Patients usually do not survive beyond one year of age. References
External links
Zellweger-Syndrome at NINDS
Zellweger syndrome at NIHs Office of Rare Diseases
This connection may or may not have a central cavity; if it does, then food within the esophagus may pass into the trachea (and on to the lungs) or alternatively, air in the trachea may cross into the esophagus.TEF can also occur due to pressure necrosis by a tracheostomy tube in apposition to a nasogastric tube (NGT). Diagnosis
TEF should be suspected once the baby fails to swallow after their first feeding during the first day of life. Esophageal atresia can be diagnosed by Ryle nasogastric tube; if the Ryle fails to pass into the stomach, then this indicates esophageal atresia and loss of communication between stomach and esophagus. TEF may be diagnosed by MRI which clarifies the atretic esophagus (if presents) and TEF, as well as its location and anatomy. Gastrograffin contrast swallow should not be used if TEF is suspected, due to its high risk of allergy and severe intractable chest infection. Classification
Fistulae between the trachea and esophagus in the newborn can be of diverse morphology and anatomical location. However, various pediatric surgical publications have attempted a classification system based on the below specified types.Not all types include both esophageal agenesis and tracheoesophageal fistula, but the most common types do. The letter codes are usually associated with the system used by Gross, while number codes are usually associated with Vogt.An additional type, "blind upper segment only" has been described, but this type is not usually included in most classifications.