Biomedical subjects
J Farwell
Publications and source records attributed to J Farwell.
Infantile spasms. Comparative trial of nitrazepam and corticotropin.
Fifty-two patients were enrolled in a four-week randomized multicenter study comparing nitrazepam and corticotropin in the treatment of infantile spasms. The drugs' efficacy was evaluated in 48 patients, all less than 2 years of age. Both treatments resulted in a statistically significant reduction in spasm frequency from that at baseline, but the difference between treatments was not significant. The number of patients who experienced side effects was similar in the two treatment groups, but the adverse effects encountered among the patients treated with corticotropin were qualitatively more severe and required the discontinuation of treatment in six patients.
Cancer in relatives of children with central-nervous-system neoplasms.
We compared the occurrence of cancer in parents, siblings, and offspring of 643 patients who had central-nervous-system tumors in childhood (cases), as recorded in the Connecticut Tumor Registry, with the occurrence in parents, siblings, and offspring of 360 controls selected according to birth certificate and matched for sex, birth date, and birthplace. Overall cancer incidence was comparable in the two groups. However, 11 nervous-system tumors occurred in relatives of cases, whereas none occurred in relatives of controls (P = 0.0005). Nine relatives of cases but no relatives of controls had cancer of the hematopoietic-lymphatic system (P = 0.003). Nine siblings of cases but only one sibling of a control had cancer as children. Medulloblastoma and glioblastoma multiforme were overrepresented in the group of children whose relatives had central-nervous-system tumors. We compared the actual number of cancers of the central nervous system or hematopoietic-lymphatic system in relatives of cases with the number expected on the basis of known incidence rates and found a fivefold excess. We conclude that the occurrence of a brain tumor in a child is a marker for an increased likelihood of central-nervous-system tumors, leukemia, and childhood tumors in the family.
Second primaries in children with central nervous system tumors.
Among 670 children diagnosed with central nervous system tumors before age 20 and recorded in the Connecticut Tumor Registry, nine had a second neoplasm as well. From known age-specific and year-specific cancer incidence figures for Connecticut, the expected number of second neoplasms for the series of 670 is 0.99; therefore the relative risk is 9.1 (95% confidence limits: 4.0, 17.3). Three patients developed two central nervous system tumors, while 0.16 were expected, giving a relative risk of 19 (95% confidence limits: 3.8, 55). Six patients developed two neoplasms in childhood, versus 0.66 expected; the relative risk of this event is 9.1 (95% confidence limits: 3.3, 20). In four cases of lapse in time between the diagnosis of the first and second tumor, the first tumor had been treated with radiation. Five cancers occurred in parents or siblings of these nine patients, versus 0.91 expected; the relative risk is 5.5 (95% confidence limits: 1.2, 10.0). Three of these relatives had leukemia, while only 0.04 cases were expected (relative risk = 75). We conclude that not only is a child with CNS cancer at increased risk for other cancers, but such a child with two cancers is often part of a familial cluster with increased risk of cancer.
Adrenocorticotropic hormone controls infantile spasms independently of cortisol stimulation.
Infantile spasms constitute a severe seizure disorder unresponsive to standard anticonvulsants. Both prednisone and adrenocorticotropic hormone (ACTH) have produced remission of seizures in some patients. The mechanisms of action of these hormones are not known. Eight infants with infantile spasms were treated with prednisone for 2 weeks. This controlled the seizures in two patients. In the remaining six patients, prednisone was continued and ACTH was added. This treatment produced cessation of spasms in four patients. Serum prednisone and cortisol were measured at a number of points during treatment. In infants receiving prednisone and then prednisone plus ACTH, serum cortisol was suppressed to about one-quarter of baseline levels with the initiation of prednisone, and remained suppressed during ACTH administration. We conclude that ACTH can exert its effect on infantile spasms in the setting of adrenal suppression, and can act without stimulating endogenous cortisol production. A CNS site of action is suggested and should be sought.
Tumors of the central nervous system in adolescents.
In a series of 144 central nervous system tumors found in adolescent patients, 133 occurred intracranially. Astrocytomas comprised 27 percent, glioblastoma multiforme 12 percent, medulloblastomas 11 percent and craniopharyngiomas 8 percent. The most common symptoms were headache and vomiting. Papilledema was the most frequent neurologic finding. Supratentorial and infratentorial lesions were nearly equal in occurrence. Rapidly growing tumors, such as medulloblastoma, were less frequent in these patients than in younger children.