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Sir ARCHIBALD GARROD, who was an active member of the staff of The Hospital for Sick Children, Great Ormond Street, from 1892 to 1913, described certain "inborn errors of metabolism". This was the start of an interest in physiological as distinct from anatomical "deformities" in infants and young children. Modern developments in chemical methods such as paper chroniatography, flame photometry and spectroscopic analysis have greatly added to the knowledge of the subject. It is now known that not all such deviations from the normal in metabolism are necessarily inborn; Some are thought to be transient, the result possibly of a slow maturing of enzyme systems; some can be ameliorated if not cured by certain lines of treatment. It is now the common practice to regard any severely wasted infant who is not making normal progress as possibly suffering from some metabolic error, and undoubtedly as time progresses the elucidation of many hitherto obscure causes of "marasmus" will prove possible. A case of galactosuria had occurred under the care of one of us (A. M.) and as a result it became customary to examine, among other things, for abnormal sugars in the urine in certain babies and young children with obscure wasting. I n this rather chance way it was found that a patient was passing sucrose (saccharose, cane sugar) in the urine persistently. Two further examples of this have also been discovered and this communication deals with the clinical findings and some of the difficulties which have been encountered in studying the metabolism of sucrose in young children. The fact that all three children were mentally retarded and had an abnormality a t the lower end of the oesophagus associated with hiatus hernia suggests that this may be a hitherto undescribed syndrome. MethodsThe three patients were given diets containing various sugars t o study their effects on the urinary sugar excretion. The urine sugars were identified by one-dimensional paper chromatography by Dr. L. I. WOOLP of this hospital (WOOLF 1951), and their approximate concentrations measured. Blood sucrose was estimated as the difference between the reducing sugar levels before and 496 A. MONCRIEFF AND R. 13. WILKINSON after treatment of the whole blood with invertase (see also E L M E R KRASOWSKA and PTASZAK 1939). The reducing sugars were estimated by the micro-method of HAGEDOILN and JENSEN (1933). Case RecordsCase 1.-M.F., a female, the first and only child of Jewish parents was admitted to The, Hospital for Sick Children, Great Ormond Street, on August 11, 1952, at t>he age of 24 years. Birth was normal and the baby weighed 5 lb 3 oz (2.35 kg); she was not thought t o be premature. At two weeks of age she "fainted" and was put into an oxygen tent. There was some difficulty over breathing and head retraction was present but the cerebrospinal fluid was normal. Feeding was difficult and she was admitted to another hospital for some weeks. Weight was gained slowly and there il-ere occasional vomits which led t o a further admission when a barium swallow wa...