Ugo Carpentieri

Clinical experience in prevention of candidiasis by nystatin in children with acute lymphocytic leukemia

for the prevention of renal oSteodystrophy. In addition, fluid intake was restricted (he was anephric) and an anticholinergic drug was prescribed, both of which probably contributed to the constipation. It is difficult to know which factor played .the major part in the eventual bowel obstruction, but it would seem reasonable to exert due caution when any patient requires chronic usage of aluminum hydroxide. Efforts to lessen constipation, such as liberal fluid intake, use of laxatives, and, if renal function is not compromised, alternate use of magnesium containing antacids may reduce the risk of a fecal impaction. An appropriate history for constipationshould be a part of the ongoing care of these patients while they are receiving antacid therapy.

Zinc and growth in patients with sickle cell disease

tion for intensive treatment during this period. The indiscriminate use of high doses of Factor VIII for all elbow hemorrhages in early adolescence is one possibl e response to this situation. However , the problems of the multitransfused hemophiliac patient? make this approach less than ideal, and the selective application of high-dose replacement therapy to those hemorrhages likely to do badly is a more logical solution. We have shown that elbow hemarthroses which present with pain unaccompanied by stiffness, that are tender, and that have lost more ihan 50% of movement are likely to do badly on a dose of 11 to 16 units of Factor VIIl/kg. A delay of more than three hours from the onset of symptoms is also likely to be associated with lack of response. Bleeding into target joints was associated significantly with success when a one-tailed test was used. From our clinical experience we did not expect hemorrhages into target joints to be associated with better than average respons e to an average dose of Factor VIII, and therefore believe that in this situation the use of a one-tailed test is not permissible. We have shown that trauma does not worsen the out!ook for elbow hemorrhages and that delay in treatment UP tO three hours does not appear to increase the risk. This surprising observation does not necessarily mean that delay in treatment is acceptable. The spee d with which bleeding develops is likely to vary from case to case, and early presentation may delay the onset of high-risk features. The age of the patients did not appear to influence response , although a previous study has suggested that hemorrhages increase in severity through adolescence and peak at the age of 14 to 15 years? Forty percent of the hemorrhages associated with two or more of the four poor prognosis factors needed retransfusion, and this group of hemorrhages should provide a fruitful field for studying the effect of higher initial doses of Factor VIII,

Hyperglycemia associated with the therapeutic use of l-asparaginase: Possible role of insulin receptors

A study of four children, two with acute lymphocytic leukemia and two with non-Hodgkin lymphoma, treated for three days with L-asparaginase, suggests that insulin receptors may be involved in the hyperglycemia associated with the use of L-asparaginase. Comparison of insulin receptors of circulating monocytes revealed a marked decrease in their number after treatment. This might also explain the lack of response to insulin treatment in one of the patients with symptomatic hyperglycemia.

Hyperreninemia and hyperaldosteronism in sickle cell anemia.

Fourteen patients with sickle cell anemia, ages 6 to 20 years, were studied while ingesting high- and low-sodium diets. Although none of the patients had excessive urinary loss of sodium, the majority had elevated plasma renin activities and aldosterone secretion rates. The PRA was higher in patients over 10 years of age; ASR in patients receiving the high-sodium diet increased with age. Patients with sickle cell anemia appeared to compensate for urinary sodium loss between crises. The mechanism of this loss could be a defect in the function of either the distal tubule or the loop of Henle.

Effects of iron, copper, zinc, calcium, and magnesium on human lymphocytes in culture

The effects of simultaneous changes of calcium, magnesium, iron, copper, and zinc concentrations were evaluated in normal human T and B lymphocytes, cultured in cation-depleted media. Optimal concentrations for thymidine incorporation (TI) in both cell populations were Fe and Zn 15 μM and Cu 5 μM; for t cells Ca 2 mM and Mg 4 mM; for B cells Ca 4 mM and Mg 6 mM. TI decreased with increasing molarity of cations and the decrease was particularly apparent with Cu. Minimal amounts of Ca and Mg (0.5 mM) were necessary for growth, even in presence of optimal concentrations of Fe, Cu, and Zn. Fe and Cu showed synergistic stimulatory effects at low concentrations and synergistic inhibitory effects at high concentrations. Antagonism between Fe and Zn, Cu and Zn, and Ca and Zn was also demonstrated. CD4/CD8 increased with PHA stimulation in presence of Zn, and decreased with ConA stimulation in presence of Zn or Fe. The results demonstrate: (1) the relationship and interdependence of Fe, Cu, and Zn concentrations in modulating the growth of normal lymphocytes; (2) the stimulatory effects of Fe on B cells and Zn on CD8 positive cells; (3) the inhibitory effect of Cu at concentrations lower than those of Fe and Zn; (4) the requirement of Ca and Mg in certain concentration and ratio for the action of the other cations; and (5) the Ca and Mg requirement for the growth of B cells higher than T cells.

Leukapheresis in a 3-year-old child with lymphoma in leukemic transformation

1. Fox JP, et al: The Seattle Virus Watch VII. Observations of adenovirus infections, Am J Epidemiol 105:362, 1977. 2. Brandt CO, Kim HW, Vargusko AJ, et al: Infections in 18,000 infants and children in a controlled study of respiratory disease. I. Adenovirus pathogenicity in relation to serologic type and illness syndrome, Am J Epidemiol 90:484, 1969. 3. Herbert FA, et al: Adenovirus type 3 pneumonia causing lung damage in children, Can Med Assoc J 116:274, 1977. 4. Connor JD: Evidence of an etiological role of adenoviral infection in the pertussis syndrome, N Engl J Med 283:390, 1970. 5. Darougar S, et al: Epidemic. keratoconjunctivitis and chronic papillary conjunctivitis in London due to adenovirus 19, Br J Ophthalmol 61:73, 1977. 6. Guyer B, ODay DM, Hierholzer JC, and Schaffner W: Epidemic keratoconjunctivitis: a community outbreak of mixed adenovirus type 8 and 19 infection, J Infect Dis 132:142, 1975.

Growth, ribonucleotide reductase and metals in murine leukemic lymphocytes.

SummaryTrace metals are essential for the growth and several other properties of human lymphocytes. We studied the effects of media with variable concentrations of three metals (Fe2+, Cu2+, Zn2+), a metal chelator (deferoxamine, DFX) and a cell-growth inhibitor (hydroxy-urea) on the growth, intracellular metal concentration and activity of the enzyme ribonucleotide reductase in murine leukemic lymphocytes (L1210). Intracellular concentrations of Fe and Cu fluctuated within narrow limits in normal media, but decreased to very low concentrations in metal-poor media. The intracellular Zn concentration did not vary appreciably. Growth in intact cells decreased by 50%–70% when normal media were replaced by metal-poor media, but returned to control values when media were supplemented with gradually increasing concentrations of Fe and Cu. Fe and Cu had synergistic effects, while Zn had no stimulatory action. Hydroxyurea and DFX both inhibited cell growth, but only DFX inhibition was reversed by addition of metals. The addition of the above metals and inhibitors to the cell extracts produced effects on ribonucleotide reductase activity similar to those observed on the growth of whole cell preparations (stimulation by Fe and Cu, inhibition by Zn, DFX and hydroxyurea). These findings show that (a) the intracellular metal concentration is maintained in a narrow range during cell growth; (b) ribonucleotide reductase activity varies with cell growth; (c) ribonucleotide reductase activity and cell growth increase with Fe and Cu and decrease with Zn and DFX. Our data suggest that (a) Fe, Cu and Zn may have some effect on the growth and ribonucleotide reductase activity of L1210 cells, that (b) Fe, Cu and Zn may operate in a related and interdependent way and that (c) DFX inhibits cell growth probably through inhibition of the reductase activity and chelation of the Fe of its Fe-containing subunit. We conclude that any study on one of these metals should always include the other two and that manipulation of intracellular metals should be investigated as a potential therapeutic modulator of growth in leukemic lymphocytes.

Pyknocytosis in a neonate: an unusual presentation of hereditary elliptocytosis.

left costal margin. Serum bilirubin was 4.8 m~°le, with 2.2 mg% as glucuronide. Blood hemoglobin level was 12.8 gm/l; hematocrit 41.7%; reticulocytes 9.9%; white blood cells (WBC) 17,000 with 64% neutrophils; 7 nucleated red blood cells/100 WBC. Fifteen per cent of the erythrocytes were pyknocytes {Fi~. la). Spherocytosis, anisopoikilocytosis, and polychromasia were also present. Direct Coombs’ test, assorted cultures, and a variety of such tests as glLicose-6-phosphate dehydrogenase (G6PD), clc>ttin~ studies, platelets,

Primitive Neuroectodermal Tumor Presenting as Meningitis

PRIMITIVE NEUROECTODERMAL TUMOR (PNET) is a very rare and highly malignant neoplasm of the cerebrum with frequent dissemination to the spinal cord and extraneural metastases. It occurs primarily in children and young adults; the mean age for presentation is 6 years.’ The presence of PNET is frequently diflicult to demonstrate, and the average duration of the symptoms before diagnosis ranges from 3 weeks to 6 nionths.~.&dquo; We report a case of PNET that was remarlzable because of the initial

Leukemia with a novel 4q11q rearrangement

Translocation (4;11)(q21;q23) is characteristic of a distinct acute leukemic syndrome. We report an 8-wk-old male patient with the clinical features ascribed to t(4;11), but with an unusual chromosome rearrangement consisting of an insertion of the 11q23 band into the q21 region of chromosome #4.