WFRF:(Steward C. G.)
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| Fältnamn | Indikatorer | Metadata |
|---|---|---|
| 000 | 03051naa a2200505 4500 | |
| 001 | oai:gup.ub.gu.se/58812 | |
| 003 | SwePub | |
| 008 | 240528s2004 | |||||||||||000 ||eng| | |
| 024 | 7 | a https://gup.ub.gu.se/publication/588122 URI |
| 040 | a (SwePub)gu | |
| 041 | a eng | |
| 042 | 9 SwePub | |
| 072 | 7 | a ref2 swepub-contenttype |
| 072 | 7 | a art2 swepub-publicationtype |
| 100 | 1 | a Steward, C. G.4 aut |
| 245 | 1 0 | a Severe pulmonary hypertension: a frequent complication of stem cell transplantation for malignant infantile osteopetrosis |
| 264 | 1 | c 2004 |
| 520 | a This report describes eight infants who developed acute severe pulmonary arterial hypertension (PAH) at days -2 to +89 after allogeneic stem cell transplantation (SCT) for malignant infantile osteopetrosis (MIOP). They were taken from a total of 28 children (frequency 29%) transplanted for this disease at three institutions between 1996 and 2002. Typical presentations were acute dyspnoea, hypoxia and brady/tachycardia usually in the absence of fever, crepitations or other evidence of infection. Six patients (75%) required assisted ventilation and five (62%) died. There was clinical or pathological evidence of veno-occlusive disease (VOD) in three children, but absence of VOD in the remaining five suggests that a separate disease process may be responsible for the PAH. Responses to nitric oxide (NO), defibrotide (DF), nicardipine and steroids in varying combinations were disappointing. Three children showed sustained improvement after administration of epoprostenol (EP, prostacyclin) in conjunction with NO and/or DF and remain well and free of PAH 25, 31 and 32 months post-transplant. PAH must therefore be excluded in any child who becomes acutely breathless after SCT for osteopetrosis. | |
| 653 | a Antihypertensive Agents/therapeutic use | |
| 653 | a Arrhythmia/etiology | |
| 653 | a Dyspnea/etiology | |
| 653 | a Female | |
| 653 | a Humans | |
| 653 | a Hypertension | |
| 653 | a Pulmonary/*etiology/therapy | |
| 653 | a Infant | |
| 653 | a Male | |
| 653 | a Osteopetrosis/*therapy | |
| 653 | a Positive-Pressure Respiration | |
| 653 | a Stem Cell Transplantation/*adverse effects | |
| 653 | a Transplantation | |
| 653 | a Homologous | |
| 653 | a Treatment Outcome | |
| 700 | 1 | a Pellier, I.4 aut |
| 700 | 1 | a Mahajan, A.4 aut |
| 700 | 1 | a Ashworth, M. T.4 aut |
| 700 | 1 | a Stuart, A. G.4 aut |
| 700 | 1 | a Fasth, Anders,d 1945u Gothenburg University,Göteborgs universitet,Institutionen för kvinnors och barns hälsa, Avdelningen för pediatrik,Institute for the Health of Women and Children, Dept of Paediatrics4 aut0 (Swepub:gu)xfasan |
| 700 | 1 | a Lang, D.4 aut |
| 700 | 1 | a Fischer, A.4 aut |
| 700 | 1 | a Friedrich, W.4 aut |
| 700 | 1 | a Schulz, A. S.4 aut |
| 710 | 2 | a Göteborgs universitetb Institutionen för kvinnors och barns hälsa, Avdelningen för pediatrik4 org |
| 773 | 0 | t Br J Haematolg 124:1, s. 63-71q 124:1<63-71x 0007-1048 |
| 856 | 4 8 | u https://gup.ub.gu.se/publication/58812 |
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