scholarly journals Anaesthetic Implications of the Mucopolysaccharidoses: A Fifteen-Year Experience in a Children's Hospital

1983 ◽  
Vol 11 (3) ◽  
pp. 198-202 ◽  
Author(s):  
David Baines ◽  
John Keneally

Patients suffering from the mucopolysaccharidoses, most commonly Hurler's syndrome, present special problems in anaesthesia. A retrospective review of the anaesthetic management of such patients over fifteen years revealed a high incidence of airway problems. Two case histories illustrating these difficulties are presented.

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 3471-3471
Author(s):  
Marissa A. Just ◽  
Joanna Robles ◽  
Karan R. Kumar ◽  
Andrew Yazman ◽  
Jennifer A. Rothman ◽  
...  

Introduction: The incidence of venous thromboembolism (VTE) in hospitalized pediatric patients is increasing secondary to the growing medical complexity of pediatric patients and the increasing use of central venous catheters. Pediatric patients diagnosed with VTE have up to 2% mortality associated directly with their thromboses. While incidence, risk factor identification and preventive strategies are well established in hospitalized adults, this information is limited in the pediatric population. There are currently no standardized VTE risk screening tools or thromboprophylaxis guidelines for children at Duke Children's Hospital. The incidence of hospital acquired VTE (HA-VTE), as well as their associated risk factors were investigated in a retrospective review. Methods: Medical records of pediatric patients hospitalized at Duke Children's Hospital during June 2018 through November 2018 were reviewed. The EPIC SlicerDicer tool was used to identify patients with ICD-10 diagnoses codes related to thrombosis or treated with anticoagulants. Included patients were diagnosed with HA-VTE during their hospitalization or within 14 days of discharge. Data collected included demographics, thrombosis characteristics, family history, mobility, and acute or chronic co-morbid conditions. The characteristics of the study population were described by median (with 25th and 75th percentiles) for continuous variables and frequencies (with percentages) for binary or categorical variables. Results: Out of 4,176 total pediatric admissions to all units of Duke Children's Hospital (ages 0-18.99 years) during the inclusion timeframe, 33 VTE events were identified. The incidence of VTE events per 1000 patient days was 0.98. The complete patient and VTE event characteristics are listed in Tables 1 and 2. The median age of patients with VTE events was 0.4 years. Of the identified cohort, 73% had an associated central venous line (CVL). Neonates with congenital cardiac disease comprised the majority of the cohort. Other common patient characteristics observed in this cohort included impaired mobility, recent major surgery, and recent mechanical ventilation. Of the 33 VTE diagnoses, 70% received therapeutic anticoagulation with enoxaparin or unfractionated heparin. Only 2 patients (8%) received prophylactic anticoagulation prior to their diagnosis of VTE. Conclusions: The retrospective review of HA-VTE events at Duke Children's Hospital identified that the majority of the events occurred in neonates with congenital cardiac disease and the presence of CVLs. It was also noted that there was no standardization among the use of anticoagulation agents that were initiated for treatment of VTE. Furthermore, few patients received VTE prophylaxis during the hospitalization. A limitation of this review was that it was retrospective and the documentation of family history of thrombosis was inconsistent. It is also possible that several VTE events were missed due to inadequate ICD-10 coding. Based on the results of this review, there is a need to implement a risk stratification tool and develop standardized recommendations of VTE prophylaxis and treatments for pediatric patients admitted to Duke Children's Hospital. There is an additional quality improvement phase of this project and the goal is to implement a risk calculator that is based on information learned from the retrospective review. Ultimately, this risk calculator will help to decrease the incidence of VTE events at Duke Children's Hospital. Disclosures Rothman: Agios: Honoraria, Research Funding; Pfizer: Consultancy, Honoraria, Research Funding; Novartis: Honoraria, Research Funding.


2011 ◽  
Vol 50 (12) ◽  
pp. 1110-1115 ◽  
Author(s):  
Jose M. Garza ◽  
Cade M. Nylund ◽  
Ajay Kaul

Objectives. Cough, pain, and desaturation episodes in infants are often ascribed to gastroesophageal reflux, and many are empirically treated with acid suppression medications. The authors hypothesize that most of these symptoms are not related to gastroesophageal reflux. Methods. Retrospective review of 186 combined pH–multichannel intraluminal impedance studies performed in infants at Cincinnati Children’s Hospital. Results. Of 4159 symptoms reported 1504 (36%) were associated with reflux events (27% nonacid and 9% acid). When total number of symptoms and reflux events were taken into consideration, nonacid reflux events were as likely to be associated with a symptom as acid reflux events ( P = .66). Conclusion. The extra-esophageal symptoms commonly attributed to gastroesophageal reflux in infants are most often not associated with a reflux event. Even though causality cannot be definitively proven, in the minority in whom a symptom association is observed, nonacid events are as likely as acid events to cause symptoms.


2019 ◽  
Vol 24 ◽  
pp. S4
Author(s):  
Kareena Johnson ◽  
Sue Scott ◽  
Jane Tomlinson ◽  
Greta Van Kerkwijk ◽  
Andrew Daley ◽  
...  

2020 ◽  
Vol 66 (2) ◽  
pp. S43
Author(s):  
Timothy A. Roberts ◽  
Stephani L. Stancil ◽  
Tracy L. Sandritter ◽  
Jennifer A. Wagner ◽  
Benjamin Black

2016 ◽  
Vol 40 (2) ◽  
pp. 156-160 ◽  
Author(s):  
Sheikh Sohail Ahmed ◽  
Shawn R Hicks ◽  
James E Slaven ◽  
Mara E Nitu

Objective: Sedation may be needed for safe, effective completion of pediatric dental procedures. Procedural sedation is performed in a children's hospital based dental office. The three sedation approaches: a propofolonly (P-O) approach (2–3mg/kg titrated to the needed level of sedation), an approach that includes either IV ketamine (K+P) (0.25 or 0.5mg/kg) or IV fentanyl (F+P) (0.5–1mcg/kg) prior to propofol administration. We sought to determine safety and efficacy of various propofol based sedation protocols. Study Design: Retrospective review of 222 patients receiving a propofol-only (P-O), ketamine+propofol (K+P) or fentanyl+propofol (F+P) approach. Results: There were 44 patients in P-O group, 154 in K+P group and 24 in F+P group with mean age (4.8±3.4y) and mean weight (19.7±6.7kg). All the patients completed procedures successfully. Mild hypoxemia occurred in 24% of cases and resolved with nasal cannula. Mean total dose of propofol was similar in all groups (P-O 8.2mg/kg, K+P 9.5mg/kg, F+P 9.6mg/kg, p=0.15). Although procedure and recovery times were similar in all groups, discharge times in K+P group were significantly shorter than P-O group and F+P group respectively (K+P 9.35±8.93.min, P-O 13.57±10.42min, F+P 10.42±4.40 p= 0.002). Conclusion: Sedation can be accomplished safely and effectively in a children's hospital based dental office using propofol-based sedation.


Objective: To assess the efficacy and duration of propranolol therapy in pediatric patients with parotid hemangiomas, and compare the results with the efficacy and duration of propranolol therapy in patients with infantile hemangiomas in other anatomic locations. Methods: In this retrospective review, we analyzed the electronic medical records of 21 patients with parotid hemangiomas seen at the Children’s Hospital of Orange County’s Vascular Anomalies Clinic between 2009 and 2015. We compared the duration of propranolol therapy and rate of re-growth after completion of therapy with established data for these parameters in the literature for patients with other infantile hemangiomas. Results: In our cohort, 13 of the 21 patients had completed therapy, with a mean duration of 26 months of propranolol therapy. Eighteen patients (85.7%) were treated with the goal dose of propranolol (2 mg/kg/day). Three patients required a higher dose in order to achieve significant improvement in the size of the hemangioma. All patients had some response to propranolol. Eight of the 13 patients (61.5%) who completed propranolol therapy saw regrowth once initial propranolol therapy was either weaned or stopped. Conclusion: Pediatric patients with parotid hemangiomas require longer duration of propranolol therapy than patients with other infantile hemangiomas, and a greater percentage may have regrowth after completion of therapy.


1986 ◽  
Vol 95 (4) ◽  
pp. 477-481 ◽  
Author(s):  
Carlos Gonzalez ◽  
James S. Reilly ◽  
Margaret A. Kenna ◽  
Ann E. Thompson

Nasotracheal intubation has been demonstrated to be effective in supporting the airways of children with acute epiglottitis. Length of intubation and criteria used for extubation are still controversial. A 6-year retrospective review at Children's Hospital of Pittsburgh identified 100 cases of acute epiglottitis, which were initially managed with nasotracheal intubation. Extubation was based on direct laryngeal inspection performed in the operating room (1979–1981) and, more recently, in the intensive care unit (1982–1984). Length of intubation decreased from 63.8 hours in 1979 to 42.1 hours in 1984. The percent of children intubated longer than 48 hours decreased from 69% to 22% in the same time period. These data indicate that a shorter period of intubation is aided by daily laryngeal inspection in the ICU. We propose a staging system for acute epiglottitis to aid in the decision to safely extubate these children.


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