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1.
Bone Marrow Transplant ; 57(5): 734-741, 2022 05.
Article in English | MEDLINE | ID: mdl-35190674

ABSTRACT

Sufficient communication between hematopoietic stem cell transplantation (HSCT) and pediatric intensive care unit (PICU) teams is pivotal for a successful advanced support in the PICU for HSCT-related complications. We evaluated perceived communication and barriers between both teams with the aim of providing recommendations for improvement. In this cross-sectional survey, a self-designed online questionnaire was distributed among ESPNIC and EBMT members. Data were analyzed using descriptive statistics. Over half of HSCT respondents employed a transfer indication protocol and patient assessment tool, but less structured checklist prior to patient transfer. Nearly all PICU respondents perceived this checklist as improvement for communication. Most HSCT and PICU physicians have daily rounds upon patient transfer while this is mostly missing between nursing teams. Half of both HSCT and PICU nurses indicated that HSCT training for PICU nurses could improve communication and patient transfer. Most respondents indicated that structured meetings between HSCT and PICU nurses could improve communication. Overall there is good communication between HSCT and PICU units, although barriers were noted between members of both teams. Based on our findings, we recommend use of a structured and specific checklist by HSCT teams, HSCT training for PICU personnel, and structured meetings between HSCT and PICU nurses.


Subject(s)
Hematopoietic Stem Cell Transplantation , Physicians , Child , Cross-Sectional Studies , Humans , Intensive Care Units, Pediatric , Risk Factors
3.
Spine Deform ; 7(1): 176-179, 2019 01.
Article in English | MEDLINE | ID: mdl-30587313

ABSTRACT

Compression of the celiac artery by a tight arcuate ligament of the diaphragm is a rare syndrome that can arise after correction of severe kyphosis. Symptoms include abdominal pain and ileus and liver dysfunctions. These symptoms can be easily attributed to more common causes like the superior mesenteric artery syndrome, and a delay in the diagnosis of celiac artery obstruction may result in severe ischemic disease of the gastrointestinal tract. We present a case of celiac artery syndrome after correction of a kyphoscoliosis with severe sequelae that has not been documented before.


Subject(s)
Kyphosis/surgery , Median Arcuate Ligament Syndrome/etiology , Postoperative Complications/etiology , Scoliosis/surgery , Spinal Fusion/adverse effects , Adolescent , Humans , Male , Spinal Fusion/methods
4.
Bone Marrow Transplant ; 49(10): 1287-92, 2014 Oct.
Article in English | MEDLINE | ID: mdl-25068426

ABSTRACT

Exact data on prognosis of children receiving invasive mechanical ventilation (IMV) after allogeneic hematopoietic SCT (HSCT) is lacking. We therefore started a prospective registry in four European university HSCT centers (Leiden, Paris, Prague and Utrecht) and their pediatric intensive care units (PICUs). The registry started in January 2009. In January 2013, the four centers together had treated a total of 83 admissions with IMV. The case fatality rate in these patients was 52%. Mortality 6 months after PICU discharge was 45%. There were significant differences between centers in the proportion of children who received IMV after HSCT (6-23%, P<0.01), in severity of disease on admission to PICU (predicted mortality 14-37%, P<0.01), in applying noninvasive ventilation before IMV (3-75% of admissions, P<0.01) and in the use of renal replacement therapy (RRT) (8-58% of admissions, P<0.01). Severe impairment in oxygenation, use of RRT and CMV viremia were independent predictors of mortality. Our study shows that mortality in children receiving IMV after HSCT remains high, but has clearly improved compared with older studies. Patient selection and treatment in PICU differed significantly between centers, which underscores the need to standardize and optimize the PICU admission criteria, ventilatory strategies and therapies applied in PICU.


Subject(s)
Hematopoietic Stem Cell Transplantation/methods , Respiration, Artificial/methods , Transplantation Conditioning/methods , Transplantation, Homologous/methods , Adolescent , Child , Child, Preschool , Female , Hematopoietic Stem Cell Transplantation/mortality , Humans , Infant , Male , Prognosis , Prospective Studies , Risk Factors , Transplantation Conditioning/mortality , Transplantation, Homologous/mortality , Treatment Outcome
5.
Am J Med Genet A ; 140(3): 284-90, 2006 Feb 01.
Article in English | MEDLINE | ID: mdl-16411219

ABSTRACT

We describe a unique case of achondroplasia with associated complications, including severe respiratory problems. Molecular analysis of the fibroblast growth factor receptor type 3 (FGFR3) gene in this patient showed the common p.G380R mutation and a second novel p.L377R mutation. An allele-specific PCR demonstrated that these mutations were on the same allele (cis). Both mutations were not present in the parents and appear to have occurred de novo. To our knowledge, this is the first report in the literature on an achondroplasia patient with two FGFR3 mutations on the same allele.


Subject(s)
Achondroplasia/genetics , Mutation , Receptor, Fibroblast Growth Factor, Type 3/genetics , Achondroplasia/complications , Alleles , Base Sequence , DNA Mutational Analysis , Fatal Outcome , Humans , Infant , Infant, Newborn , Male , Mutation, Missense , Respiratory Distress Syndrome, Newborn/complications
6.
Neurology ; 65(4): 591-2, 2005 Aug 23.
Article in English | MEDLINE | ID: mdl-16116121

ABSTRACT

To assess safety and efficacy of propofol and thiopental for refractory status epilepticus (RSE) in children, the authors reviewed 34 episodes of RSE. Thiopental was effective in most patients, but there were serious side effects. Propofol was used according to a strict protocol. It was effective in most patients, so that thiopental was not needed. Side effects were infrequent, of minor severity, and fully reversible. The authors suggest the use of propofol before thiopental.


Subject(s)
Anticonvulsants/administration & dosage , Propofol/administration & dosage , Status Epilepticus/drug therapy , Thiopental/administration & dosage , Anticonvulsants/adverse effects , Child , Clinical Protocols/standards , Dose-Response Relationship, Drug , Drug Administration Schedule , Drug Resistance/physiology , Humans , Hypertriglyceridemia/chemically induced , Liver Failure/chemically induced , Lung Diseases/chemically induced , Multiple Organ Failure/chemically induced , Propofol/adverse effects , Retrospective Studies , Rhabdomyolysis/chemically induced , Thiopental/adverse effects , Treatment Outcome
7.
Thorax ; 58(5): 383-7, 2003 May.
Article in English | MEDLINE | ID: mdl-12728156

ABSTRACT

BACKGROUND: A study was undertaken to evaluate the efficacy of dexamethasone in patients mechanically ventilated for lower respiratory infection caused by respiratory syncytial virus (RSV-LRTI). METHODS: In a multicentre randomised controlled trial patients were randomised to receive either intravenous dexamethasone (0.15 mg/kg 6 hourly for 48 hours) or placebo. End points were the duration of mechanical ventilation, length of stay (LOS) in the pediatric intensive care unit (PICU) and in hospital, and the duration of supplemental oxygen administration. RESULTS: Thirty seven patients received dexamethasone and 45 received placebo. There was no significant difference in any of the end points between the two groups. In a post hoc analysis patients were stratified into those with mild gas exchange anomalies (PaO(2)/FiO(2) >200 mm Hg and/or mean airway pressure 10 cm H(2)O, pneumonia group). In the 39 patients with bronchiolitis the duration of mechanical ventilation was 4.3 days shorter in the dexamethasone group than in the placebo group (4.9 v 9.2 days, 95% CI -7.8 to -0.8, p=0.02) and the duration of supplemental oxygen was 3.6 days shorter (7.7 v 11.3 days, 95% CI -8.0 to -0.1, p=0.048). No differences in end points were found in the pneumonia group. CONCLUSIONS: Dexamethasone had no beneficial effect in patients mechanically ventilated for RSV-LRTI but was found to have a beneficial effect in patients with bronchiolitis.


Subject(s)
Dexamethasone/administration & dosage , Glucocorticoids/administration & dosage , Respiratory Syncytial Virus Infections/drug therapy , Blood Pressure/drug effects , Bronchiolitis/drug therapy , Female , Humans , Infant , Infusions, Intravenous , Intensive Care, Neonatal , Length of Stay , Male , Oxygen/administration & dosage , Respiration, Artificial , Respiratory Syncytial Virus Infections/physiopathology , Treatment Outcome
8.
Ned Tijdschr Geneeskd ; 146(30): 1420-3, 2002 Jul 27.
Article in Dutch | MEDLINE | ID: mdl-12174438

ABSTRACT

A one-year-and-seven-months-old boy was hospitalised because of fever, cough and general malaise. A diagnosed tonsillitis and pneumonia were treated with intravenous antibiotics. His clinical condition worsened despite antibiotic therapy. After immunologic investigations revealed both a cellular and a humoral immune disorder, a broncho-alveolar lavage was performed. The culture revealed Legionella pneumophila. Antibiotic treatment was then changed to erythromycin in combination with rifampicin, with a good response. Although rarely described in childhood, one should consider L. pneumophila as a possible pathogen in immunocompromised children presenting with pneumonia.


Subject(s)
Immunocompromised Host , Legionnaires' Disease/diagnosis , Pneumonia, Bacterial/diagnosis , Anti-Bacterial Agents/therapeutic use , Anti-Infective Agents/therapeutic use , Antibiotics, Antitubercular/therapeutic use , Bronchoalveolar Lavage Fluid/microbiology , Drug Therapy, Combination/therapeutic use , Erythromycin/therapeutic use , Fluoroquinolones , Humans , Infant , Legionella pneumophila/isolation & purification , Legionnaires' Disease/drug therapy , Male , Pneumonia, Bacterial/drug therapy , Rifampin/therapeutic use , Treatment Outcome
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