Our two patients with lysinuric protein intolerance, a 14-year-old boy and his 12-year-old sister, showed growth retardation and their bone ages were retarded. Growth hormone secretion responded to glucagon-propranolol and showed a good response to arginine. However, growth hormone showed little response to insulin. After the oral administration of arginine hydrochloride, growth hormone showed a good response to insulin and glucagon-propranolol, and gain in height and weight accelerated. This result may suggest that an adequate supply of arginine is effective in improving the growth retardation in lysinuric protein intolerance.

Electroencephalographic aspects of coma in 201 children are presented. The ages of the subjects ranged between 1 month and 14 years. The EEG picture was not specific. Diffuse slowing and convulsive activity were present in meningoencephalitis and postconvulsive coma. Diffuse slowing was also present in coma of metabolic origin. Mixed slow and fast activity was typical of coma due to benzodiazepines, barbiturates and alcohol. In patients with intracerebral hemorrhages, there was marked focal slowing associated with generalized delta and theta waves. In 12 patients with craniocerebral trauma, the EEG showed generalized slowing which was associated with focal reduction of voltage in 6 cases and with convulsive activity in 2. Extreme slowing or an isoelectric recording had a bad prognosis. The reappearance of sleep spindles was a favorable sign.

Twenty-seven patients with lysinuric protein intolerance, an autosomal recessive disorder of diamino acid transport, have been followed at our hospital since 1965. Many of the patients had recurrent mild normochromic anaemia without apparent iron deficiency, and leuko- and/or thrombocytopenia. Serum ferritin concentrations were inappropriately high. Serum ferritin concentration appears to be a valuable index of the control of this disease.

The first patient of Turkish descent with hyperdibasicaminoaciduria is described. Recurrent diarrhea was observed only during the first three months of life. The infant exhibited low plasma levels of ornithine and arginine. Intestinal absorption of lysine was decreased. Hyperammonemia was noticed only after an i.v. alanine load. It was prevented by addition of arginine.