M. Eugene Lahey

Histiocytosis x--an analysis of prognostic factors.

A retrospective analysis of data garnered from the study of 83 patients with histiocytosis X suggests that evaluation of the function of certain organs and the histologic appearance of the lesions may be quite valuable in determining the prognosis and treatment of affected patients.

Histiocytosis X—comparison of three treatment regimens*

The three treatment regimens evaluated in this study (vinblastine alone versus prednisone and vinblastine versus prednisone and 6-mercaptopurine) proved to be about equally efficacious in children with histiocytosis x. Inasmuch as many physicians regard this disorder as one with a high mortality rate, it is worth emphasizing that 59 of the 83 patients (71% are living).

The coincidence of neuroblastoma and acute cerebellar encephalopathy

Three patients with neuroblastoma and acute cerebellar encephalopathy are reported. This coincidence of disease occurs more commonly than one would expect on the basis of chance. This observation is supported by the finding of six other cases cited in the literature. The pathogenesis which underlies the relationship is obscure. The authors suggest that all patients with acute cerebellar encephalopathy be surveyed for an inapparent neuroblastoma. The search should include chest and skeletal roentgenograms, intravenous urogram, bone marrow examination for malignant cells, and determination of the urinary excretion of catecholamines and possibly cystathionine.

Cutaneous lesions of Letterer-Siwe disease. Electron microscopic study.

The fine structure of the cells in the cutaneous lesions of Letterer‐Siwe disease is presented. The occurrence of peculiar bodies is documented and these structures are compared with those described in other forms of the disorder and in the Langerhans cell of the epidermis. The authors suggest that these granules originate from the cytoplasmic membrane and the membranous channels of the endoplasmic reticulum or Golgi system.

Studies on iron metabolism

Summary A preliminary study of a group of infantswith iron deficiency anemia has revealed evidence of occult gastrointestinal bleeding, precipitins to cows milk, and the passage of serum proteins into gastric juice. These findings are strong evidence of gastrointestinal dysfunction. Guaiac-positive stools were found in 40per cent of the iron-deficient infants tested. Precipitins to cows milk were found in 75 to 80 per cent of the iron-deficient subjects who were taking this food prior to diagnosis. This is regarded as an abnormal finding. Serum proteins in the fasting gastric juice were demonstrated in 12/13 of anemic subjects and in 2/12 of control subjects by means of agar diffusion techniques. Semiquantitative estimates of serum albumin, and gamma globulin in the gastric juice suggest a pathologic loss of these proteins in at least some anemic subjects. These findings suggest the existence of at least 2 forms of iron deficiency anemia in infants; two forms, that is, which are etiologically distinct. The first of these has been termed “primary iron deficiency”; the second is believed to depend on primary sensitization to cows milk. Mechanisms have been proposed to explain the possible interrelationships of the causative factors in each group.

Treatment of reticuloendotheliosis with vinblastine sulfate: Preliminary report**

Three patients with proved reticuloendotheliosis (Letterer-Siwe syndrome) were treated with vinblastine sulfate in doses of 0.2 to 0.3 mg. per kilogram weekly for 8 to 15 weeks. The response in all three patients was so gratifying as to encourage further trials with this agent.

Spinal subdural hematoma in a leukemic child

Summary A 15-year-old girl with acute lymphoblasticleukemia developed signs and symptoms of cord compression following a lumbar puncture. At autopsy a large subdural hematoma was found compressing the cauda equina and extending upward around the lumbar cord. The cause of subdural hematoma of thecord are briefly discussed. The need to be aware of this potential problem and its presenting neurologic signs and symptoms is emphasized.

Sarcoidosis among children in Utah and Idaho

Eight instances of sarcoidosis proved by biopsy in children from the Utah-Idaho area are reported. All of the children were of the Caucasian race, mostly of northern European ancestry, and resided in rural or semirural communities. The disease began prior to the age of 8 years in 5 of the 8 children. Careful collection of historical, environmental, and laboratory data failed to delineate any “common denominator” pertaining to all patients. Attention is drawn to the unusual features presented in one of these children, whose clinical course and laboratory data simulated those of Stills disease, but who did not have the usual joint involvement. It is suggested that sarcoidosis of this type may be confused with “atypical Stills diseas”.

Peripheral mononeuropathy complicating anaphylactoid purpura

5. Centers for Disease Control: Pneumocystis pneumonia among persons with Hemophilia A. MMWR 31:365, 1982. 6. Marx JL: New disease baffles medical community. Science 217:618, 1982. 7. Kornfeld H, Vande Stouwe RA, Lange M, Reddy MM, et al: T-lymphocyte subpopulations in homosexual men. N Engl J Med 307:729, 1982. 8. Ziegler JL, Miner RC, Rosenbaum E, Lennette ET, et al: Outbreak of Burkitts-like lymphoma in homosexual men. Lancet 2:631, 1982. 9. Lederman MM, Ratnoff OD, Scillian J J, Jones PK, et al: Impaired cell-mediated immunity in patients with classic hemophilia. N Engl J Med 308:79, 1983. 10. Menitove JE, Aster RH, Casper JT, Lauer SJ, et al: Tlymphocyte subpopulations in patients with classic hemophilia treated with cryoprecipitate and lyophilized concentrates. N Engl J Med 308:83, 1983.

The carcinoembryonic antigen assay: Prognostic value in neural crest tumors

Summary Plasma carcinoembryonic antigen levels were measured before and after treatment in 14 children with histologically proved neural crest tumors. All eight children with a pretreatment CEA value below 4.0 ng/ml had long-term survival without evidence of tumor recurrence. Six patients had values of 4.0 ng/ml or above-the three who appear cured showed follow-up values in the normal range and the three who died had persistently elevated levels. We conclude from these data that: (1) slight but definite elevations in plasma CEA can be expected in most infants and children with neural crest tumors-an interesting biologic note; (2) the test adds little to currently available methods of early diagnosis; and (3) the assay may have value in monitoring response to therapy; our data suggest that values below 4.0 ng/ml are associated with a good outcome whereas values above this level call for a guarded prognosis.