scholarly journals P.107 Response to the Ketogenic Diet in refractory epileptic spasms at BC Children’s Hospital

Author(s):  
PM Webb ◽  
A Datta

Background: Epileptic spasms (ES) are a devastating seizure type with poor neurodevelopmental outcome; 1/3 are resistant to treatment with first line therapies. Recently attention has been drawn to the ketogenic diet (KD) as a potentially effective therapy, though data regarding optimal time of initiation, and its sustained effectiveness, are lacking. Methods: Retrospective chart review of all patients with ES treated with KD at BC Children’s Hospital between 2002 and 2020 (n=28) with comparison of spasm response based on age of initiation of KD in two groups: < 12 months (n=11) and ≥ 12 months (n=17). Results: Comparing the <12 months and ≥ 12 months groups showed: unknown etiology in 9% vs 25%; spasm freedom for 3 months on KD in 18% vs 41%; median time to spasm freedom was 2 vs 6 weeks; relapse after a period of spasm freedom occurred in 66% vs 70%. Conclusions: Although more effective in children ≥ 12 months of age in the first 3 months, spasm freedom in either group was not sustained with KD. KD is recommended as early therapy for refractory ES, but this study suggests clinicians be aware the KD has limited efficacy in long-term control of ES and must be used with other therapies.

2020 ◽  
Vol 7 (Supplement_1) ◽  
pp. S331-S332
Author(s):  
Catherine Foster ◽  
Lucila Marquez ◽  
Tjin Koy ◽  
Ila Singh ◽  
Judith Campbell

Abstract Background Accurate diagnosis of coronavirus disease 2019 (COVID-19) is key for source control and interrupting disease transmission. To better understand the length of viral shedding in children and potential infection control implications, we describe 51 children with COVID-19 who underwent repeat testing for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) at Texas Children’s Hospital (TCH). Methods We performed a retrospective chart review of all pediatric patients (&lt; 21 years of age) with ≥ 2 nasopharyngeal specimens tested for SARS-CoV-2 by reverse transcription-polymerase chain reaction (rt-PCR) and at least one positive result between 3/13/2020 and 6/7/2020 through the TCH Molecular Microbiology Laboratory. Results Fifty-one patients met inclusion criteria. The median age was 8.6 years (0.02–19.2 years). Sixteen (31%) children were hospitalized. Fourteen (27%) patients underwent testing for surveillance purposes (including 3 admitted patients). Two SARS-CoV-2 tests were performed in 25 (49%) children; while 12 (24%) children had 3 tests, 4 (8%) children had 4 tests, and 10 (20%) children had ≥ 5 tests (including 1 patient with underlying malignancy who had 9 SARS-CoV-2 PCRs performed). SARS-CoV-2 testing timeline for 9 hospitalized children is shown (Fig 1). The median time between collection of tests 1 and 2 was 14 days (n=51, range 1, 53 days). For children with conversion (first detected to first not-detected sample), the median time was 15 days (n=31, range 1, 45 days). For patients with consecutive positive SARS-CoV-2 PCRs, the median time of positivity was 10 days (n=19, range 2, 31). One patient with malignancy had 5 tests over 6 weeks in the outpatient setting and each time alternated between detected and not-detected. Following diagnosis with COVID-19, one patient with sickle cell disease likely had re-infection and had a positive test after having 2 consecutive negative tests; his last SARS-CoV-2 rt-PCR was positive 68 days after initial positive. Fig 1. Timing of Repeat SARS-CoV-2 PCRs in Select Hospitalized Children with COVID-19 Conclusion We observed variation in the duration of SARS-CoV-2 rt-PCR positivity in children with COVID-19. For children with COVID-19, a single negative molecular assay for SARS-CoV-2 may not be predictive of sustained negativity. Disclosures All Authors: No reported disclosures


2021 ◽  
pp. 019459982110151
Author(s):  
Rahul G. Baijal ◽  
Karla E. Wyatt ◽  
Teniola Shittu ◽  
Eugenia Y. Chen ◽  
Eric Z. Wei ◽  
...  

Objectives The aim of this study was to determine the incidence of perioperative respiratory complications in children following tonsillectomy with cold and hot dissection surgical techniques. Study Design The study was a retrospective cohort study. Setting Retrospective chart review was performed for all children presenting for a tonsillectomy at Texas Children’s Hospital from November 2015 to December 2017. Methods Pre- and intraoperative patient factors, including surgical technique with cold or hot dissection (electrocautery or radiofrequency ablation), and perioperative anesthetic factors were collected to determine the incidence of perioperative respiratory complications. Results A total of 2437 patients underwent a tonsillectomy at Texas Children’s Hospital from November 2015 to December 2017. The incidence of perioperative respiratory complications was 20.0% (n = 487). Sickle cell disease, cardiac disease, reactive airway disease, pulmonary disease, age >2 and <3 years, and obesity, defined as a body mass index >95th percentile for age, were significant for overall perioperative respiratory complications. There was no difference in the incidence of perioperative respiratory complications in children undergoing tonsillectomy by cold or hot dissection. Conclusion Perioperative respiratory complications following tonsillectomy are more affected by patient factors than surgical technique.


2017 ◽  
Vol 06 (03) ◽  
pp. 149-155
Author(s):  
Raffaele Falsaperla ◽  
Robinson Gutierrez ◽  
Gabriela Valenzuela ◽  
Piero Pavone ◽  
Sebastian Fortini ◽  
...  

Objective We evaluated the electroclinical features, etiology, treatment, and outcome of 16 patients with single-epileptic spasms (ESs) with or without hypsarrhythmia (WoH). Methods Nine boys and seven girls had single-ESs. ESs were considered as single epileptic spasm variants when no other spasm occurred for 1 minute before and after each spasm. Age at the onset of ESs was between 2 and 84 months, with a mean age of 11 months. Results We recognized a group of 15 patients with single-ESs as the main type of seizure; 6 patients with WoH and 9 patients with hypsarrhythmia, respectively. Nine of these 15 patients had other types of seizures before the onset of single-ESs, and 12 patients had other types of seizures during the period in which the ESs occurred. Nine of 15 patients had a structural and seven had an unknown etiology. In 10 cases, the ESs were refractory to antiepileptic drugs, while 4 patients responded well to adrenocorticotropic hormone (ACTH), 1 to pyridoxine, and 2 to the ketogenic diet (KD). The remaining patient (patient.16) had single-ESs and electroclinical features of Lennox–Gastaut syndrome (LGS). Conclusion In this article, we present a series of infants who had daily single-ESs with or WoH. Those with single-ESs with hypsarrhythmia evolved to an epileptic encephalopathy. Video-electroencephalogram (EEG) and polygraphic-EEG recordings are crucial to identify the single-ESs.


2020 ◽  
Vol 36 (6) ◽  
Author(s):  
Muhammad Sohail Arshad ◽  
Hafiz Muhammad Anwar-ul-Haq ◽  
Mudasser Adnan ◽  
Arif Zulqarnain

Background & Objectives: Heart diseases in paediatric population are considered to be significant contributors to mortality and morbidity. Congenital heart disease (CHD) as well as acquired heart disease (AHD) are frequent causes of hospital admission among children. This study was aimed at finding out frequency and pattern of heart diseases in admitted patients at The Children’s Hospital, Multan. Methods: This study is a retrospective chart review of five years at Paediatric Cardiology Department of The Children’s Hospital and The Institute of Child Health, Multan, Pakistan, from January 2015 to December 2019. Children aged one month to 15 years, admitted as a diagnosed case of heart disease on the basis of echocardiography were included. Results: Out of a total of 4115 confirmed cases of heart disease admitted during the study period, 3250 (79.0%) were CHD while 865 (21.0%) were AHD. Overall, 2861 (69.5%) patients were aged less than one year. VSD followed by ASD were the commonest acyanotic heart lesion seen among 927 (28.5%) and 644 (19.8%) cases while TOF was the commonest cyanotic type heart lesion found in 396 (12.2%). Rheumatic heart disease (RHD) was the commonest type of AHD, seen in 330 (38.2%) cases followed by acute myocarditis found in 230 (26.6%) cases. Conclusion: Burden of heart diseases is rising in our region. VSD, ASD and TOF were the most common types of CHDs while RHD and acute myocarditis were the most frequent types of AHDs. doi: https://doi.org/10.12669/pjms.36.6.2312 How to cite this:Arshad MS , Anwar-ul-Haq HM, Adnan M, Zulqarnain A. Frequency and pattern of Paediatric Heart Diseases: Five years experience at The Children’s Hospital, Multan. Pak J Med Sci. 2020;36(6):---------. doi: https://doi.org/10.12669/pjms.36.6.2312 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


2010 ◽  
Vol 24 (2) ◽  
pp. 109-112 ◽  
Author(s):  
Seema Rajani ◽  
Hien Q Huynh ◽  
Justine Turner

BACKGROUND: Celiac disease (CD) is recognized as one of the most common and important autoimmune gastrointestinal disorders affecting children. There is evidence that a diagnosis of CD during childhood improves health outcomes. The increasing prevalence of CD is due to increased awareness of the wide range of extraintestinal symptoms associated with CD.OBJECTIVE: To determine whether there has been a temporal increase in the diagnosis of CD associated with an increased diagnosis of children without typical gastrointestinal symptoms at the Stollery Children’s Hospital (Edmonton, Alberta).METHODS: Patients with biopsy-proven CD diagnosed at the Stollery Children’s Hospital from 1998 to 2007, were identified by retrospective chart review. Baseline and follow-up data, including demographics, symptoms, risk factors, anthropometrics and laboratory investigations, were collected.RESULTS: An increase in the frequency of diagnosis of CD was noted during the study period, particularly from January 2003 onward. Before January 2003, nine children were diagnosed with CD – all with typical symptoms. Between January 2003 and January 2007, inclusive, 149 children were diagnosed with CD, of whom 46% had absent or atypical symptoms. At follow-up, 96% of patients reported improved symptoms, including 53% of individuals who reported being asymptomatic before diagnosis.CONCLUSIONS: In the last four years of the period studied, the number of children diagnosed with CD at Stollery Children’s Hospital increased 11-fold. Screening children at risk for CD, and those with atypical presentations, contributed to the increased number of diagnoses. Identification of CD and establishment of lifelong, dietary gluten avoidance during childhood has important health benefits and should be encouraged.


2020 ◽  
Vol 58 (230) ◽  
Author(s):  
Bal Mukunda Basnet ◽  
Prashant Simkhada ◽  
Anupama Thapa ◽  
Rajkumar Singh

Introduction: Tracheo-oesophageal anomaly is the abnormal communication between trachea and oesophagus. The most common type of tracheo-oesophageal anomaly is oesophageal atreasia with distal tracheo oesophageal fistula. Tracheo-oesophageal anomaly is a common neonatal problem requiring an urgent surgery. Tracheo-oesophageal anomaly can be diagnosed as an isolated malformation or as part of polymalformative syndrome with possible vertebrae anomalies also known as Vacterl syndrome. The aim of the study is to find out the prevalence of tracheo-oesophageal anomaly cases among neonates undergoing surgery in a tertiary care childrens hospital in Nepal. Methods: This is a descriptive cross sectional study conducted in a tertiary care children’s hospital in Nepal taking into account the medical records from period of 01 Jan, 2018 to Dec 31, 2019. Study population included the infants undergoing operative procedure in the operation theatre of Kanti Children’s Hospital. The calculated sample size was 306. Data was collected by retrospective chart review technique and listed in performa. Consecutive sampling technique was used. Thus collected data was entered in SPSS version 20 and necessary calculations were done. Results: The prevalence of tracheo-oesophageal anomalies was found out to be 30 (9.8%) at 95% confidence interval. The most common problem, with which infants were brought to Kanti Children’s Hospital, that required urgent neonatal surgical intervention was anorectal malformation constituting 94 (30.72%) of the surgeries followed by intestinal obstruction 76 (24.84%) which included duodenal atresia, jujunal atresia and ileal atresia. Conclusions: Tracheo-oesophageal anomaly constitutes a major portion of neonatal disease condition requiring surgery in Nepal.


2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii438-iii439
Author(s):  
Chantel Cacciotti ◽  
Christine Chordas ◽  
Kate Valentino ◽  
Rudy Allen ◽  
Peter Manley ◽  
...  

Abstract BACKGROUND Medulloblastoma is an aggressive central nervous system (CNS) tumor that occurs mostly in the pediatric population. Treatment includes surgical resection, craniospinal radiation (CSI) and chemotherapy. Children who receive standard photon radiation (RT) are at risk for cardiac toxicities. Potential late effects include coronary artery disease, left ventricular scarring and dysfunction, valvular damage and atherosclerosis. Current survivorship guidelines recommend routine ECHO surveillance for these patients but this comes at significant health care costs over a lifetime. We describe the experience of cardiac dysfunction in medulloblastoma survivors in a multi-institution study. METHODS A retrospective chart review of medulloblastoma patients treated between 1980 and 2010 with radiation at Lurie Children’s Hospital and Dana-Farber/ Boston Children’s Hospital who had an echocardiogram done following completion of therapy. RESULTS 168 patients were treated for medulloblastoma during the study time. Of whom, 80 patients had echocardiogram follow up and 76 received photon irradiation. The latter were included in the study. The mean age at CSI was 8.6 years (range 2.9- 20), and mean number of years post RT at echocardiogram 7.4 years (range 2–16). Mean ejection fraction (EF) was 60.03% and shortening fraction (SF) 33.8%. Four patients (5%) had abnormal results, all of which had EF&lt;50%. CONCLUSION Patients who received craniospinal irradiation for medulloblastoma therapy have relatively normal echocardiograms post treatment. Although RT may result in cardiac risks, echocardiograms may not be the most cost effective or efficacious mode to evaluate the risk in these survivors long term.


2019 ◽  
Vol 9 (5) ◽  
pp. 573-579 ◽  
Author(s):  
Bryan T Nycz ◽  
Kristin Pretty ◽  
Angel Gomez-Trujillo ◽  
Brenda Sanchez ◽  
Samuel R Dominguez

Abstract Background The epidemiology, demographics, clinical presentations, and outcomes associated with enteroaggregative Escherichia coli (EAEC), enteropathogenic E. coli (EPEC), and enterotoxigenic E. coli (ETEC) pathotypes in US children are not well understood. Methods This study was a retrospective chart review of all pediatric patients with a stool sample submitted to the Children’s Hospital Colorado clinical microbiology laboratory for testing with the BioFire FilmArray Gastrointestinal Pathogen Panel from October 2015 through October 2017. Results During the study period, 5692 patient stool samples were submitted; 679 (13%) were positive for EAEC, EPEC, or ETEC. Of note, 163/232 (70%) patients with EAEC, 282/493 (57%) with EPEC, and 49/58 (85%) with ETEC had detection of at least 1 other pathogen. Of all E. coli–positive stool samples, only 158/679 (23%) were from low-risk patients who were singly infected with EAEC, EPEC, or ETEC. In this cohort, most cases were associated with acute diarrhea (50%), abdominal pain (61%), and/or cramping (49%) and presented without fever (14%), emesis (28%), or lethargy (7%). Thirteen (8%) of these 158 patients received antibiotics at the time of their initial presentation to care. Of the 145 patients who did not receive antibiotics at their initial visit, 23 (16%) returned to care due to persistence of symptoms. Conclusions Our results suggest that the majority of patients singly infected with EAEC, EPEC, or ETEC present with mild, self-limited, gastrointestinal (GI) complaints. Further research is needed to determine what role these pathogens might play in children who present with chronic or inflammatory GI symptoms.


Author(s):  
Zoe Simpson ◽  
Christin Eltze ◽  
Hannah Smith ◽  
Bahee Van de Bor ◽  
Victoria Urban ◽  
...  

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