Search

A neonate with transposition of the great arteries developed chylothorax following ligation of the patent ductus arteriosus. At first, multiple thoracenteses and chest drainage were employed without significant diminution of pleural fluid production. Administration of a formula containing medium chain triglycerides (MCT) was associated with a marked decrease of pleural fluid accumulation within a few days. Total lipid concentration of pleural fluid prior to the diet was 890 mg/100 ml and diminished to 40 mg/100 ml after eight days of administration of the diet. Cholesterol and cholesterol esters decreased from 101 mg/100 ml to 24 mg/100 ml and from 83 mg/100 ml to 9 mg/100 ml, respectively. Total triglyceride concentration fell from 111 mg/100 ml to 2 mg/100 ml. Only negligible amounts of fatty acids with a chain length lower than capric (C10:0) were recovered from the analyses of the samples. Thereafter, x-ray films revealed no pleural effusion and at the third-month follow-up visit the chest remained clear. The marked diminution in volume of the pleural effusion in this case is thought to be associated with MCT feedings. MCT passes directly into the portal system, bypassing the lymphatic pathways and still provides adequate caloric intake. Thus, it seems ideal for the treatment of conditions which have a compromised lymphatic drainage. Both clinical data and biochemical findings in this case strongly suggest the use of MCT diet as an important adjunct of the management of neonatal chylothorax. [ABSTRACT FROM AUTHOR]

An attempt to treat a case of histidinemia using a low histidine protein hydrolysate is describe. The diet was started at the age of 7 months in a male infant with severe clinical disorder. The diagnosis was made at 24 weeks, which is the youngest recorded case. Good control of histidine metabolism was achieved. There was an initial improvement in growth rate but very little effect on development otherwise. Death from bronchopneumonia occurred in the fourth year. The brain showed cerebral maldevelopment, likely to be of prenatal origin, as well as typical postepileptic encephalopathy. The etiology of this malformation is discussed in relation to histidinemia and the family history of neurologic disorder. [ABSTRACT FROM AUTHOR]

Focuses on a study which discussed dietary treatment of an infant with isovaleric acidemia. Patient and methodology; Details of the recommended dietary treatment; Observations and treatment outcome; Analysis of the case.