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11/50. Diffuse chronic granulomatous mucocutaneous candidiasis.

    A 3-year-old Thai boy with diffuse chronic granulomatous mucocutaneous candidiasis, recurrent bacterial skin infection and adrenal insufficiency is reported. candida albicans was demonstrated in the dermal granuloma. He had a defect in cell-mediated immunity and was anemic. Although therapy with topical clotrimazole, oral iron, systemic antibiotic and low-dose of prednisone gave a dramatic result, he died of disseminated cryptococcosis. ( info)

12/50. Acute disseminated phycomycosis in a patient with impaired neutrophil granulocyte function.

    A 13-year-old girl with no previously known predisposing disease developed phycomycosis involving the left lung, pleura and shoulder, the left side of the neck, the left thigh, the kidneys and the brain. Prolonged amphotericin b therapy resulted in clinical improvement, but the disease was wide-spread when the patient died 5 months after debut of symptoms from a subarachnoid haemorrhage due to fungal destruction of the basilar artery. During hospitalization, a marked reduction in the bactericidal activity of circulating neutrophil granulocytes was repeatedly demonstrated and the endotoxin stimulated nitroblu tetrazolium test was negative. Together with the demonstration of granuloma formation and the accumulation of lipid-laden histiocytes in the spleen, lymph nodes, bone marrow and the thymus, these findings indicate that the patient had a less severe form of chronic granulomatous disease. ( info)

13/50. An inherited defect in granulocyte function: impaired chemotaxis, phagocytosis and intracellular killing of microorganisms.

    The neutrophil granulocytes of a 1-year-old boy with severe recurrent infections were found to have almost no chemotactic responsiveness, impaired phagocytosis and reduced intracellular killing of candida albicans "in vitro". During the course of a febrile illness of unknown etiology which was accompanied by a leukemoid reaction, phagocytic activity became normal; the chemotactic response was also increased but still remained slightly subnormal. family studies suggested that the defect was an inherited autosomal recessive one. ( info)

14/50. Multiple leukocyte abnormalities in chronic granulomatous disease: a familial study.

    A variety of leukocyte enzyme activities were studied in an 11-year-old female with chronic granulomatous disease (CGD) and several members of her family. Leukocyte glucose-6-phosphate dehydrogenase (G-6-PD) activity was 17 nmol/min/mg protein in the patient; two brothers with symptoms of recurrent bacterial infections have G-6-PD activities of 58 and 37 nmol/min/mg protein; the activites of this enzyme in both parents, maternal grandmother, and one additional brother were within normal limits. Storage at 4 degrees or heating at 37 degrees over a 120-min period revealed a marked lability of G-6-PD activity in the patient's cells which could not be stabilized by the addition of nadp and 2-mercaptoethanol; this lability was not seen in other family members tested. Activities of leukocyte glutathione reductase were reduced in both parents and the two affected male siblings with values of 18, 23, 23, and 24 nmol/min/mg protein, respectively. Activities of leukocyte glutathione peroxidase were reduced in all of the immediate family members tested, with values ranging from 11.2 to 43 nmol/min/mg protein; the activity of this enzyme in the patient was 38.5. Leukocyte nadp content in the patient, father, and two affected male siblings were 16.5, 23.4, 22.2, and 28.2 nmol/15 min/10(7) leukocytes, respectively. ( info)

15/50. Unusual expression of IgG Fc receptors on peripheral granulocytes from patients with leukocyte adhesion deficiency (CD11/CD18 deficiency).

    Leukocyte adhesion deficiency (LAD) is a hereditary disease characterized by defective expression of leukocyte adhesion glycoproteins; lymphocyte function-associated Ag-1 (CD11a/CD18), CR3 (CD11b/CD18) and p150,95 (CD11c/CD18). granulocytes, monocytes, and lymphocytes of patients with LAD show profoundly defective in vivo and in vitro adherence-dependent immune functions. We investigated the expression of FcR for IgG on polymorphonuclear cells (PMN) and monocytes from patients with LAD, and their luminol- and lucigenin-enhanced chemiluminescence production in response to SRBC sensitized with murine (m) IgG2a and IgG2b. Unstimulated patient PMN showed an enhanced chemiluminescence in response to mIgG2a-SRBC and an increased phagocytosis of mIgG2a-SRBC. The up-regulated functions were inhibited by monomeric human IgG in a dose-dependent manner, which was attributed to an increase in expression of FcRI on patient PMN, as shown by flow cytometry using monoclonal antibody, 32.2, specific for human FcRI. In contrast, neither the expression of FcR on the monocytes of LAD patients nor their FcR-mediated functions were different from those of controls. ( info)

16/50. Spinal anaesthesia in a child with Job's syndrome, pneumatoceles and empyema.

    We present a case of acute bowel obstruction in an immunocompromised child, who also had lobar pneumonia and a giant unilateral pneumatocele. She was successfully managed with subarachnoid anaesthesia for exploratory laparotomy to relieve a colonic obstruction. This proved to be a safe alternative to general anaesthesia with tracheal intubation in this patient and should be considered in infants and children in selected cases whenever a contraindication to general anaesthesia exists. ( info)

17/50. Impaired bacterial degradation by monocytes and macrophages from a patient with treated Whipple's disease.

    A patient with Whipple's disease is described, and multiparameter flow cytometric examinations of several of the patient's phagocyte functions 3 and 9 mo after the start of oxytetracycline therapy are reported. Almost no intracellular degradation of escherichia coli or streptococcus pyogenes proteins and dna occurred after ingestion by the patient's monocytes and macrophages. In addition, only minor digestion of phagocytized zymosan particles was detected. The mononuclear intracellular degradation was equally impaired 3 and 9 mo after the start of therapy. The monocyte and macrophage phagocytosis and intracellular killing, and all granulocyte phagocyte functions tested, were normal. The impaired mononuclear degradation of ingested material that was measured is consistent with the accumulation of periodic acid-Schiff-positive bacterial degradation products seen in macrophages of affected tissues in vivo, and suggests a key role of macrophage dysfunction in the pathogenesis of Whipple's disease. ( info)

18/50. Chronic myelogenous leukemia simulating chronic granulomatous disease.

    A 5-year illness of a child, characterized by recurrent bacterial infections and abnormal results of nitroblue tetrazolium dye reduction tests, was suggestive of chronic granulomatous disease but the illness terminated in overt myeloid leukemia. During this progression studies of leukocyte structure and metabolic activity revealed abnormalities that suggested the existence of a "preleukemic" state. ( info)

19/50. Myeloperoxidase secretion during phagocytosis: a case of a patient with impaired bactericidal activity.

    We describe a case of a 5-year-old male patient with prolonged and extensive osteomyelitis of the left femur. staphylococcus aureus was grown from blood cultures taken upon admission and also from pus drained from an incised hip joint. A defect in immune function was suspected and neutrophil function was assessed. chemotaxis and phagocytosis were normal, but phagocytosed S. aureus were not killed as efficiently as in control neutrophils. No inherent defect in the ability of these neutrophils to generate reactive oxidants was observed, but an unusual luminol-dependent chemiluminescence response was obtained during phagocytosis of latex beads or opsonized S. aureus: This was characterized by an initial rapid, but transient increase occurring within 1 min of addition of phagocytic stimulus. Whereas during phagocytosis of latex beads by control neutrophils less than 1% of the total myeloperoxidase activity was detected extracellularly, up to 15% was released from the patient's neutrophils. We propose that release of myeloperoxidase from the patient's neutrophils during phagocytosis reduces the intraphagosomal concentration of this enzyme and thus impairs the efficiency of intracellular killing of S. aureus. ( info)

20/50. Abnormal peroxidase-positive granules in "specific granule" deficiency.

    "Specific granule" deficiency (SGD) has been previously associated with lactoferrin deficiency. The antimicrobial peptides termed defensins, comprising 30% of normal primary granule proteins, have also been shown to be markedly deficient in SGD. The present study was undertaken to correlate these findings with ultrastructural morphometric analysis and peroxidase cytochemistry. Peroxidase-positive, rim-stained, large, defensin-rich dense granules, previously described as a subpopulation of azurophil or primary granules in normal neutrophils, were markedly decreased in a patient with SGD. Morphometric studies of peroxidase-positive granules indicated an average peroxidase-positive granule area (all profiles) in the patient of 0.019 /- 0.017 micron 2 (mean /- SD, n = 941) compared to control values from normal neutrophils of two volunteers of 0.049 /- 0.033 micron 2 (n = 896) and 0.050 /- 0.039 micron 2 (n = 873) (P less than 0.001 between patient and control samples). Granule histograms showed a single peak of small peroxidase-positive granules, whereas control samples contained more prominent subpopulations of larger peroxidase-positive granules. The total number of peroxidase-positive granules per 100 micron 2 of cytoplasm in the patient was 255 /- 124 (mean /- SD, n = 15 cell profiles), which was similar to control values of 266 /- 63 and 212 /- 109. Thus, the defensin deficiency in SGD is associated with a decrease in size rather than number of peroxidase-positive granules; suggesting that defensins contribute to normal peroxidase-positive granule size and that SGD is a more global granule deficiency than originally thought.(ABSTRACT TRUNCATED AT 250 WORDS) ( info)
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