1990
DOI: 10.1016/0140-6736(90)92620-w
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rhG-CSF for Shwachman's syndrome

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Cited by 16 publications
(7 citation statements)
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“…Numerous therapeutic interventions directed at bone marrow dysfunction have been used in a small number of these patients with varying success. These have included cyclosporin A for associated severe aplasia (Barrios & Kirkpatrick, 1990), granulocyte-colony stimulating factor (G-CSF) for profound neutropenia (Grill et al, 1993;Adachi et al, 1990), and lithium to improve cytoskeletal-mediated neutrophil dysfunction (Azzara et al, 1991). At present the only definitive treatment for the bone marrow dysfunction seen in SDS is allogeneic bone marrow transplantation.…”
Section: Discussionmentioning
confidence: 99%
“…Numerous therapeutic interventions directed at bone marrow dysfunction have been used in a small number of these patients with varying success. These have included cyclosporin A for associated severe aplasia (Barrios & Kirkpatrick, 1990), granulocyte-colony stimulating factor (G-CSF) for profound neutropenia (Grill et al, 1993;Adachi et al, 1990), and lithium to improve cytoskeletal-mediated neutrophil dysfunction (Azzara et al, 1991). At present the only definitive treatment for the bone marrow dysfunction seen in SDS is allogeneic bone marrow transplantation.…”
Section: Discussionmentioning
confidence: 99%
“…Two small studies involving a total of 16 patients with cyclic neutropenia have indicated that G-CSF (0.8±50 lg/kg per day) can be effective in increasing and maintaining neutrophil counts for up to 6 years [9,28], and is indicated in cyclic neutropenia when patients are symptomatic. Preliminary case study evidence suggests that G-CSF may also be bene®cial in Shwachman disease [1] and glycogenosis type Ib [60].…”
Section: Other Disordersmentioning
confidence: 97%
“…As regards neutrophil motility, few reports have so far been provided about the effects of rhG-CSF. Most of these regard the treatment of neutropenia in congenital syndromes (Adachi et al, 1990;Roesler et al, 1991;Elsner et al, 1992;McCawley et al, 1994), although, as far as we are aware, only one study has been carried out in patients undergoing chemotherapy (Spiekerman et al, 1994). All those studies were performed by the agarose method (Nelson et al, 1975).…”
mentioning
confidence: 99%