Topical nitrogen mustard: an effective treatment for cutaneous Langerhans cell histiocytosis.

In 16 children with multisystem Langerhans cell histiocytosis (mean age 22 months, range 5 to 36 months) severe symptomatic skin involvement was treated with topical nitrogen mustard (mechlorethamine hydrochloride). In each case, rapid clinical improvement occurred within 10 days; subsequent complete healing was observed in 14 children, and partial healing in 2 others in whom treatment was a component of palliative care. Mean duration of treatment was 3.5 months (range 2 to 6 months). Systemic treatment was averted in 11 patients because response to topical therapy was so favorable, but bone marrow or respiratory failure led to a fatal outcome in 5 other patients. Adverse effects were minimal. One patient developed contact allergy to topical nitrogen mustard after 2 years of intermittent therapy, but was successfully desensitized and was then able to continue treatment. We conclude that the topical application of nitrogen mustard is an effective treatment for cutaneous Langerhans cell histiocytosis. Although adverse effects were minimal in the short term, there remains concern about the possibility of long-term cutaneous carcinogenicity.

Precocious puberty after traumatic brain injury.

After traumatic brain injuries in 33 prepubertal children, precocious puberty was observed in seven. Precocious puberty developed significantly more frequently in girls than in boys (54.5 versus 4.5%, P less than 0.01). Six children with precocious puberty were in coma for greater than or equal to 2 weeks. Follow-up computed tomography revealed cerebral atrophy or focal encephalomalacia in all children with and 69% of children without precocious puberty. There were no striking differences in incidence of motor or cognitive deficits or posttraumatic epilepsy in children with and without precocious puberty. In four of five children, basal sex steroid levels were elevated, and the response to luteinizing hormone releasing hormone stimulation revealed a pubertal pattern after the appearance of secondary sex characteristics.

Etiologic factors in rickets of very low-birth-weight infants.

The incidence of rickets was found to be 32% (39/125) in a retrospective review of consecutive survivors of very low birth weight in whom serial radiographic and biochemical data were obtained. A higher proportion of these infants were black, had a greater initial weight loss, and had a longer hospitalization; there was a prevalence of births in the spring. Soy formula, supplemented with calcium and vitamin D but not phosphorus, was used predominantly in both groups; cumulative calcium, phosphorus, vitamin D, and caloric intakes were the same. We believe that the etiology of rickets in VLBW infants is multifactorial; however, nutritional deficiency is of central importance. Soy isolate formula, as well as human milk and many other commercially available formulas, do not provide sufficient calcium and phosphorus to keep pace with rates of intrauterine accretion. Supplementation with calcium, phosphorus, and vitamin D, beginning as soon as possible after birth, is indicated.