Sandhoff disease: a prevalent form of infantile GM2 gangliosidosis in Lebanon.

All cases clinically diagnosed as Tay-Sachs disease at the American University Hospital, Beirut, during a period of 22 years (1957--1979) were reviewed. Of a total of 15 cases, seven had serum hexosaminidase tested and proved to have Sandhoff disease. In two other cases, parents were tested and found to be Sandhoff carriers. These results indicate that Sandhoff disease is relatively prevalent in Lebanon and that it may represent the more common form of infantile GM2 gangliosidosis in this country.

Single-dose trials of monovalent A/New Jersey/76 (Hsw1N1) influenza virus vaccine in children in Durham, North Carolina.

Ninety-two children received single doses of one of 13 monovalent vaccines derived from influenza virus strain A/New Jersey/8/76 (A/NJ), and 18 children received placebo. Five influenza virus vaccines were whole-virus vaccines, and eight were split-product vaccines. Samples of sera were taken once three weeks after vaccination. All of the 29 children receiving whole-virus vaccines developed a titer of antibody to A/NJ virus of greater than or equal to 1:20, and the geometric mean titers were 1:14-1:45. One of the 60 children who received split-product vaccines developed a titer of antibody to A/NJ of greater than or equal to 1:20, and geometric mean titers were all less than 1:10. There was a trend toward correlation between titers of hemagglutination-inhibiting (HAI) antibody to A/NJ and titers of HAI antibody to A/Victoria/3/75 viruses, with a correlation coefficient of 0.40 for children who received whole-virus vaccines. Three of 60 recipients of split-product vaccines and two of 20 recipients of whole virus developed a fever of greater than or equal to 38 C. One of the two febrile children who had received whole-virus vaccine had otitis media. Therefore, reactogenicity of whole-virus vaccines and split-product vaccines appeared to be similar in these children.

Oral treatment of acrodermatitis enteropathica with zinc sulfate.

In a 7-year-old boy with acrodermatitis enteropathica, oral administration of zinc sulfate brought about complete relief of symptoms and signs, with clearing of the skin lesions, arrest of diarrhea, and growth of new hair. Considering the dramatic results obtained, we believe that oral treatment with zinc sulfate is an innocuous, inexpensive, and very effective medication for acrodermatitis enteropathica, which can replace the previously used and toxic diiodohydroxyquin-like drugs.

Tuberculous meningitis in childhood. Forty-three cases.

Forty-three patients in the pediatric age group had tuberculous meningitis. Therapy included the use of adrenocorticosteroids. All four patients who were in stage 1 on admission to the hospital, seven of the 33 in stage 2, and one of the six in stage 3 (coma) recovered without gross neurological sequelas. Eight patients died, and the remaining 23 recovered, with late neurological sequelas. Early diagnosis and appropriate treatment determine the prognosis of tuberculous meningitis.

Nocardiosis in children: report of three cases and review of the literature.

The clinical illnesses observed in three adolescents with Nocardia asteroides infection are described. The organism has been classified as a bacterium and placed in the Actinomycetaceae family. Sulfonamides remain the first choice for chemotherapy but alternative drugs are available and may be essential in successful therapy. Nocardial infections in children indicate the need for evaluation for an underlying immunological deficiency. Nocardia species should be considered as possible causative agents in immunocompromised patients with infection.

Congenital generalized lipodystrophy.

Six patients with congenital generalized lipodystrophy are described. They had generalized paucity of fat tissue, acanthosis nigricans, prominent superficial veins and muscle hypertrophy. They were mentally retarded. Three had corneal opacities. They had normal external genitalia and none was tall for age. Their bone age was advanced and some had minor skeletal anomalies and nephromegaly. The muscle histology on light microscopy was normal. The majority had elevated serum aldolase and to a lesser degree serum lactic dehydrogenase and creatinine phosphokinase. Four of five examined had a myopathic electromyogram. They had normal or deranged liver function tests. The fatty liver infiltration in one seems to be progressive. Four had a normal and two an abnormal metyrapone test. They had an age-dependent abnormality of growth hormone, insulin and carbohydrate homeostasis.