Donough O'Brien

The treatment of maple syrup urine disease

Summary The early management of 3 infants with maple syrup urine disease is described with particular reference to variations in daily requirements for branched-chain amino acids. The use of a new dry base mix consisting of a dextrimaltose-corn oil-mineral mix, an iron-vitamin mix, and an amino acid mix as a formula base has facilitated earlier home management of these patients.

Basic Science Review : Calcium and Phosphorus Current Concepts of Metabolism:

in the right handed direction. Each of the polypeptide units (molecular weight 120,000) is composed of four subunits overlapping one another by one quarter of their length and joined by ester linking. Cross linkage between polypeptide units and between tropocollagen molecules appear as the collagen matures and. determines to a large extent its physicochemical properties 20 (see ~’i~. 1). Structurally, the bone matrix organization is such that it is calcifiable: it is believed that the unique relationship of bone mucopolysaccharides with collagen accounts for the ability of bone matrix to mineralize .:17

Childhood Diabetes A Contemporary Re-evaluation

There is still much ignorance and un certainty as to the relative merits of strict vs. free diets, oral vs. injectable drugs, the basic physiologic faults, the useful ness of the sugar tolerance and related tests, etc. Here is a straightforward ex position of the newer insights into patho genesis which are pertinent to the best care of the child with diabetes.

Inborn Errors of Metabolism

Human nutrition science and practise to the outsider, at any rate, appears to emphasize the availability, procurement, preparation, and requirement of foodstuffs in man in both health and disease. Special consideration has, of course, been given to defining needs at various ages, the Recommended Dietary Allowances (RDAs), to the biochemistry of nutrients, and to the health problems of too little and too much food. However, adequate nutrition is only achieved when nutrients complete their roles in providing energy and in intermediate metabolism. Any acquired or genetically determined aberration of these processes can thus be construed as a disorder of nutrition. For the nutritionist the special interest of these conditions undoubtedly lies in the opportunities for dietary manipulation as a means of therapy (American Academy of Pediatrics, 1976a,b).