Application of a clinical and laboratory protocol for the investigation of inborn errors of metabolism among critically ill children

2000 ◽  
Vol 76 (5) ◽  
pp. 375-82 ◽  
Author(s):  
Maria T.V. Sanseverino ◽  
Moacir Wajner ◽  
Roberto Giugliani
2021 ◽  
Vol 37 (7) ◽  
pp. e422-e423
Author(s):  
Kam Lun Ellis Hon ◽  
Karen Ka Yan Leung ◽  
Anne Mei-Kwun Kwok ◽  
Kiran M. Belaramani

2017 ◽  
Vol 34 (11-12) ◽  
pp. 985-989 ◽  
Author(s):  
Ayse Filiz Yetimakman ◽  
Selman Kesici ◽  
Murat Tanyildiz ◽  
Umut Selda Bayrakci ◽  
Benan Bayrakci

Background: Continuous renal replacement therapies (CRRTs) either as continuous venovenous hemofiltration (CVVH) or hemodiafiltration (CVVHD) are used frequently in critically ill children. Many clinical variables and technical issues are known to affect the result. The factors that could be modified to increase the survival of renal replacement are sought. As a contribution, we present the data on 104 patients who underwent CRRT within a 7-year period. Materials and Method: A total of 104 patients admitted between 2009 and 2016 were included in the study. The demographic information, admittance pediatric risk of mortality (PRISM) scores, indication for CRRT, presence of fluid overload, CRRT modality, durations of CRRT, and pediatric intensive care unit (PICU) stay were compared between survivors and nonsurvivors. Results: The overall rate of survival was 51%. Patients with fluid overload had significantly increased rate of death, CRRT duration, and PICU stay. Multiorgan dysfunction syndrome as the indication for CRRT was significantly related to decreased survival when compared to acute renal failure and acute attacks of metabolic diseases. The CRRT modality was not different between survivors and nonsurvivors. Standardized mortality ratio of the group was calculated to be 0.8. Conclusion: The CRRT in critically ill patients is successful in achieving fluid removal and correction of metabolic imbalances caused by organ failures or attacks of inborn errors of metabolism. It has a positive effect on expected mortality in high-risk PICU patients. To affect the outcome, follow-up should be focused on starting therapy in early stages of fluid overload. Prospective studies defining relative importance of risk factors causing mortality can assist in building up guidelines to affect the outcome.


2021 ◽  
Vol 69 (1) ◽  
Author(s):  
HebatAllah Fadel Algebaly ◽  
Mona Mohsen ◽  
Maggie Louis Naguib ◽  
Hafez Bazaraa ◽  
Noran Hazem ◽  
...  

Abstract Background The larynx in children is unique compared to adults. This makes the larynx more prone to trauma during intubation. Under sedation and frequent repositioning of the tube are recorded as risk factors for laryngeal injury. We examined the larynx of 40 critically ill children in the first 24 h after extubation to estimate the frequency and analyze the risk factors for laryngeal trauma using the classification system for acute laryngeal injury (CALI). Results The post-extubation stridor patients had a higher frequency of diagnosis of inborn errors of metabolism, longer duration of ventilation, longer hospital stay, moderate to severe involvement of glottic and subglottic area, frequent intubation attempts, and more than 60 s to intubate Regression analysis of the risk factors of severity of the injury has shown that development of ventilator-associated pneumonia carried the highest risk (OR 32.111 95% CI 5.660 to 182.176), followed by time elapsed till intubation in seconds (OR 11.836, 95% CI 2.889 to 48.490), number of intubation attempts (OR 10.8, CI 2.433 to 47.847), and development of pneumothorax (OR 10.231, 95% CI 1.12 to 93.3). Conclusion The incidence of intubation-related laryngeal trauma in pediatric ICU is high and varies widely from mild, non-symptomatic to moderate, and severe and could be predicted by any of the following: prolonged days of ventilation, pneumothorax, multiple tube changes, or difficult intubation.


2008 ◽  
Author(s):  
Christine Rini ◽  
Sharon Manne ◽  
Katherine Duhamel ◽  
Jane Austin ◽  
Jamie Ostroff ◽  
...  

2013 ◽  
Vol 38 (03) ◽  
Author(s):  
C Jotterand ◽  
J Depeyre ◽  
C Moullet ◽  
MH Perez ◽  
J Cotting

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