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The soft agar technique was employed to investigate factors involved in the regulation of granulopoiesis in ten children with aplastic anemia. Children with AA had greatly reduced numbers of granulocytic colony-forming cells in their bone marrow and in their peripheral blood when compared to "control" children. Colony-stimulating activity was decreased in five of the ten children tested. Serum from eight children with AA did not inhibit colony formation when added to normal adult bone marrow cells in culture. The defect in AA residues in the stem cell, with involvement of the CSA-producing cells in some cases. The serum of these patients does not contain an inhibitor of granulopoiesis.

As CrlILDRBN with acute lYmphocytic leukem!a survive longer, leukemic involvement of the testes is being recognized more frequently 1-3 and testicular biopsy, even in asymptomatic patients, is being performed on a routine basis. '-5 However, leukemic involvement of the ovaries has received little attention, and the significance of ovarian-associated relapse in relation to the eventual outcome of the patient has not been investigated. We describe two female patients with ALL who completed three years of chemotherapy. Both developed ovarian-related relapses after cessation of therapy.

evaluation method for cerebral palsy. Am J Occup Ther 9:105, 1955. 4. Levine MA, Downs RW, Moses AM, Breslau NA, Marx S J, Lasker RD, Rizzoli RE, Aurbach GD, Spiegel AM: Resistance to multiple hormones in patients with pseudohypoparathyroidism: Association with deficient activity of guanine nucleotide regulatory protein. Am J Med 74:545, 1982. 5. Glinder EM, Heth DA: Colorimetric determination with bound "calmagite" of magnesium in human blood [abstract] Clin Chem 17:662, 1975. 6. Marx S J, Sharp ME, Krudy A, Rosenblatt M, Mallette LE: Radioimmunoassay for the middle region of human parathyroid hormone: Studies with a radioiodinated synthetic peptide. J Clin Endocrinol Metab 53:76, 1981. 7. Chase LR, Melsor GL, Aurbach GD: Pseudohypoparathyroidism: Defective excretion of 3 ' 5 ' -AMP in response to parathyroid hormone. J Clin Invest 48:1832, 1969. 8. Eisman JA, Hamstra A J, Kream BE, DeLuca HF: A sensitive, precise and convenient method for determination of 1-25-(OH)2 D in human plasma. Arch Biochem Biophys 176:235, 1976. 9. Albright F: Hypoparathyroidism as a cause of osteomalacia. J Clin Endocrinol Metab 16:419, 1956. 10. Drezrter MR, Neelon FA, Jowsey J, Lebovitz HE: Hypoparathyroidism: A possible cause of osteomalacia. J Clin Endocrinol Metab 45:114, 1977. 11. Omel'chenko LI, Rodionov VP, Skrypnychencko LS: Pseudo vitamin D deficient rachitis in a child with hypoparathyroidism. Pediatr Akush Ginekol 6:28, 1978. 12. Marx S J: Hereditary resistance to 1,25-dihydroxyvitamin D. Rec Prog Horm Res 40:589, 1984. 13. Liberman VA, Samuel R, Halabe A, Kauli R, Edelstein S, Weisman Y, Papapoulos SE, Clemens TL, Fraher L J, O'Riordan JLH: End organ resistance to 1,25-dihydroxycholecaciferol. Lancet 1:504, 1980. 14. Markowitz ME, Rosen JF, Smith C, De Luca HF: 1,25Dihydroxyvitamin D3-treated hypoparathyroidism: 35 patient years in 10 children. J Clin Endocrinal Metab 55:727, 1982. 15. Pettifor JM, Ross FP, Travers R, Glorieux FH, DeLuca HF: Dietary calcium deficiency: A syndrome associated with bone deformities and elevated serum 1-25 dihydroxy vitamin D concentrations. Metab Bone Dis Rel Res 2:301, 1981. 16. Liebross BA, Coburn JW: Renal osteodystrophy. In Heath D, Marx S J, editors: Clinical endocrinology, vol 2. Calcium disorders. London, 1982, Butterworth Scientific.

Chronic benign neutropenia of childhood is a heterogenenous disorder. This study describes two severely neutropenic children with a benign clinical course and the unique bone marrow finding of macrophage engulfment of band and segmented neutrophils. Phagocytic vacuoles in the majority of macrophages contained neutrophils in various stages of digestion at both the light and electron microscope level (with as many as four neutrophils observed in single macrophages). Ultrastructural studies demonstrated that the neutrophils were morphologically normal, and apparently viable at the time of phagocytosis. This type of neutrophil phagocytosis was not observed in five other consecutively studied children with CBN. All of these children with CBN had ultrastructurally normal neutrophils, lacked demonstrable antineutrophil antibodies or serum inhibitors to in vitro myelopoiesis, and had normal colony-forming cells and colony-stimulating activity in vitro. Thus despite many similar clinical and laboratory features in children with CBN, only a unique subgroup of these patients demonstrates abnormal neutrophilmacrophage interaction.

Le priapisme est une complication occasionnelle des affections hematologiques chez l'adulte. L'erection du clitoris semble tres rare dans la leucemie de l'enfant. Un cas est decrit ici chez une fille de 11 ans

In 1956, Kostmann 1 described a condition of agranulocytosis in a Swedish family. The patients had a severe disease characterized by chronic and recurrent pyogenic infections with onset during the first year of life, associated with severe neutropenia, monocytosis, and eosinophilia. Bone marrow morpfiology demonstrated normal myeloid precursors with the absence of mature neutrophiis. CASE REPORT Patient S.H. (age 11) had had repeated life-threatening pyogenic infections since age 6 months involving her skin, umbilicus, lung, and brain. Peripheral blood and bone marrow aspiration showed persistent neutropenia with maturation arrest of the myeloid precursors in her bone marrow. (The marrow differential count showed no neutrophils or bands: promyelocytes 24%, myelocytes I0%, metamyelocytes 1%, !ymphocytes 2%, plasma cells 8%, eosinophils 28%, and normoblasts 27%.) Her neutrophil counts ranged from 0 to900/m a with no tendency to cycle while eosinophil counts were elevated (440 to 1,665/m3). Her older sister developed acute lymphocytic leukemia when she Was 6 years of age and died two years later.

recurrent pulmonary infections. The facial features included coarse eyebrows, upturned nose, and micrognathia. At first, we assumed that the child had the MarshallSmith syndrome, but the patient had no phalangeal abnormalities at 19 months. In addition to these features, the patient had mild camptodactyly, limited motion of the knees, and wide distal ends of the long bones. The facial features also included a large bifrontal diameter, fiat occiput, ocular hypertelorism, and a small fish-like mouth, features found in the Weaver-Smith syndrome. Furthermore, the patient had behavioral characteristics similar to those of patients reported by Weaver and his associates, z The child was shy toward strangers, and tilted her head downward in viewing her environment. The serum amino acid studies showed a moderate decrease in the levels of glycine, alanine, and lysine. She did not have excessive postnatal growth. Since this patient has features thought to discriminate between the Marshall-Smith syndrome and the Weaver-Smith syndrome, we assume that these syndrome are morphologic variants of a single entity.