Craniofacial and occlusal features of children with sickle cell disease compared to normal standards: a clinical and radiographic study of 50 paediatric patients

2019 ◽  
Vol 21 (3) ◽  
pp. 303-311 ◽  
Author(s):  
A. Pashine ◽  
R. M. Shetty ◽  
S. Y. Shetty ◽  
T. Gadekar
2020 ◽  
Vol 105 (9) ◽  
pp. e11.1-e11
Author(s):  
Masuma Dhanji

AimTo assess the prescribing of analgesia to manage pain crises in children with SCD. This was to establish whether the Trust was meeting national and local standards. Prompt pain control is essential to reduce length of stay and further complications.1Standards100% of admissions will be prescribed regular paracetamol and non-steroidal anti-inflammatory drugs (NSAIDs) at the recommended frequency unless contraindicated in accordance with national guidance.2 3100% of admissions will be prescribed appropriate doses of analgesia with consideration to weight and age in accordance with local policy.4MethodThe audit was registered with the Trust’s audit committee. A list of paediatric patients with the diagnosis of SCD was sought from paediatricians with an interest in haematology. A data collection form was created. Data was collected retrospectively over a one-year period. A total of 60 admissions were reviewed to check whether analgesia was prescribed regularly at the recommended frequency, and at the correct dose. Results were analysed using descriptive statistical analysis. Exclusion criteria included patients with hospital admissions under 24 hours.ResultsA total of 55 admissions were included in the final sample. The audit showed the Trust was non-adherent to both standards assessed. A total of 45% (95% CI [31.9%, 58.1%]) of admissions were prescribed regular analgesia. A total of 78% (95% CI [67.9%, 88.9%] of admissions were prescribed appropriate doses of analgesia. Two main reasons were found as to why analgesia was prescribed at the incorrect dose. This was due to incorrect weights recorded on the electronic system (n=4) and doses based on age only (n=8).ConclusionThe results show prescribers are familiar with the correct doses of analgesia but fail to prescribe analgesia regularly. This highlights an opportunity for education and training in the management of pain crisis in SCD. One recommendation includes development of an integrated care pathway booklet for paediatric patients presenting with pain crisis due to SCD. Integrated care pathway booklets have been implemented for other conditions such as cystic fibrosis yielding positive outcomes. The results have highlighted key issues surrounding the electronic prescribing system such as out-of-date weights remaining on the system unless updated, and default treatment protocols. The electronic prescribing system requires refinement for use within paediatrics. One suggestion includes compulsory weight field on admission. Limitations of this audit included small sample size. There was a lack of data to make suggestions based on different ages.ReferencesRees D, Olujohungbe A, Parker N, et al. Guidelines for the management of the acute painful crises in sickle cell disease. Br J Haemato 2003;120:744–752.National Institute for Health and Care Excellence (2012) Sickle cell disease: managing acute painful episodes in hospital. NICE Guideline (CG143).Paediatric Formulary Committee. BNF for Children (2018–2019). London: BMJ Group, Pharmaceutical Press, and RCPCH Publications; (2018).General Hospital (2015) Management of sickle cell disease in paediatric patients (CG377).


Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 1525-1525 ◽  
Author(s):  
Courtney L. Wendel ◽  
Jon A. Anderson ◽  
Timothy J. Blackburn ◽  
Charles T. Quinn

Abstract Abstract 1525 Poster Board I-548 BACKGROUND Children with sickle cell disease (SCD) present to medical attention repeatedly throughout childhood for medical complications. Chest radiographs are often obtained for fever and respiratory symptoms, and plain radiographs are often ordered because of bone pain. Computed tomography (CT) and nuclear medicine (NM) studies may also be obtained for other complications. Exposure to medical radiation may increase the risk of cancer, especially in children. Growing children are inherently radiosensitive because of their high proportion of dividing cells, and children have more remaining years of life than adults during which cancer can develop. Therefore, it is important to determine the magnitude of medical radiation exposure in children with SCD because they could be so frequently exposed. METHODS We reviewed the medical records for all members of the Dallas Newborn Cohort (Blood 2004;103:4023-7) to determine the number and type of radiographic studies each individual received from 1996 to the present. We recorded the type of radiographic study, body location, clinical indication, date of study, age at the time of study, and the number and types of views when applicable. We also recorded slice thickness and mode for CT scans as well as injection activity, radionuclide, and type of radiopharmaceutical for nuclear medicine studies. To account for different lengths of follow-up, we standardized the number of radiographic studies to yearly rates for each individual to determine the projected number of studies a SCD patient would receive by 18 years of age. RESULTS We studied 938 patients (52.8% male) with a mean follow-up of 9.4 years (median 9.2, range 0.1 – 20.6). 571 had sickle cell anemia (SS), 283 had sickle-hemoglobin C disease (SC), 63 had Sβ+-thalassemia (Sβ+), and 21 had Sβ0-thalassemia (Sβ0). We identified 9,246 radiographic studies, including 8,697 radiographs, 441 CT scans, and 108 NM studies. 711 (76%) patients had at least one radiographic study. Patients with SS or Sβ0 were more likely to have had at least one radiographic study than those with SC or Sβ+ (77% vs. 65%; P<0.0001). The mean number of studies per patient was 9.9 [95% confidence interval (C.I.) 8.9 – 10.9; range 0 – 115], corresponding to a mean rate of 1.5 per year (95% C.I. 1.3 – 1.6; range 0 – 27.3). We project that a patient with SCD will be exposed to the radiation from 26.7 (95% C.I. 24.1 – 29.3; range 0 – 492.1) radiographic studies by 18 years of age. Approximately 5% of patients with SCD will be exposed to 100 or more radiographic studies during childhood. CONCLUSIONS Children with SCD are frequently exposed to medical radiation. Some are exposed to over 100 radiographic studies. Radiographs of the painful part are frequently obtained but are infrequently indicated. Because growing children are more radiosensitive than adults and have more remaining years of life, medical radiation exposure could be clinically significant. We are now calculating the radiation effective doses for this cohort to quantify the risk of malignancy. It is prudent to limit the medical radiation exposure of this high-risk population. Disclosures No relevant conflicts of interest to declare.


2010 ◽  
Vol 95 (10) ◽  
pp. 822-825 ◽  
Author(s):  
M. Peters ◽  
K. Fijnvandraat ◽  
X. W. van den Tweel ◽  
F. G. Garre ◽  
P. C. Giordano ◽  
...  

2020 ◽  
Vol 105 (10) ◽  
pp. 981-985
Author(s):  
Linan Zeng ◽  
Chao Wang ◽  
Min Jiang ◽  
Kexin Chen ◽  
Haiqin Zhong ◽  
...  

ObjectiveTo determine the safety of ceftriaxone in paediatric patients and systematically evaluate the categories and incidences of adverse drug reactions (ADRs) of ceftriaxone in paediatric patients.MethodsWe performed a systematic search in Medline, PubMed, Cochrane Central Register of Controlled Trials, EMBASE, CINAHL, International Pharmaceutical Abstracts and bibliographies of relevant articles up to December 2018 for all types of studies that assessed the safety of ceftriaxone in paediatric patients aged ≤18 years.Results112 studies met the inclusion criteria involving 5717 paediatric patients who received ceftriaxone and reported 1136 ADRs. The most frequent ADRs reported in prospective studies were gastrointestinal (GI) disorders (37.4 %, 292/780), followed by hepatobiliary disorders (24.6%, 192/780). Serious ADRs leading to withdrawal or discontinuation of ceftriaxone were reported in 86 paediatric patients. Immune haemolytic anaemia (34.9%, 30/86) and biliary pseudolithiasis (26.7%, 23/86) were the two major causes. Haemolytic anaemia following intravenous ceftriaxone led to death in 11 children whose primary disease was sickle cell disease. Almost all biliary pseudolithiasis are reversible. However, the incidence was high affecting one in five paediatric patients (20.7%).ConclusionsGI ADRs are the most common toxicity of ceftriaxone in paediatric patients. Immune haemolytic anaemia and biliary pseudolithiasis are the most serious ADRs and the major reasons for discontinuation of ceftriaxone. Immune haemolytic anaemia is more likely in children with sickle cell disease and may cause death. Ceftriaxone should be used with caution in children with sickle cell disease.Trial registration numberCRD42017055428


2020 ◽  
Vol 189 (3) ◽  
pp. 559-565
Author(s):  
Meghan McCormick ◽  
Troy Richardson ◽  
Bradley A. Warady ◽  
Enrico M. Novelli ◽  
Ramasubramanian Kalpatthi

2020 ◽  
Vol 67 (9) ◽  
Author(s):  
Frederick W. Thielen ◽  
Maite E. Houwing ◽  
Marjon H. Cnossen ◽  
Ilona A. al Hadithy‐Irgiztseva ◽  
Jan A. Hazelzet ◽  
...  

2018 ◽  
Vol 57 (3) ◽  
pp. 347-357 ◽  
Author(s):  
Vassilis L. Tzounakas ◽  
Serena I. Valsami ◽  
Anastasios G. Kriebardis ◽  
Issidora S. Papassideri ◽  
Jerard Seghatchian ◽  
...  

Author(s):  
KJ Sullivan ◽  
J Dayan ◽  
M Reichenbach ◽  
M Irwin ◽  
A Pitkin ◽  
...  

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