scholarly journals The use of rapamycin to treat vascular tumours and malformations: A single-centre experience

2019 ◽  
Author(s):  
Soumitra Tole ◽  
Michelle Fantauzzi ◽  
Diana Cottingham ◽  
Joao G Amaral ◽  
Philip R John ◽  
...  
Keyword(s):  
Clinical Response ◽  
Vascular Malformations ◽  
Tertiary Care ◽  
Lesion Size ◽  
Response To Therapy ◽  
Symptom Improvement ◽  
First Year ◽  
Large Tertiary Care ◽  
Patient Reported ◽  
First Year Of Life

Abstract Objectives To assess the safety and efficacy of rapamycin in treating children with vascular tumours and malformations. Study design We performed a retrospective review at a large tertiary care paediatric centre to assess the efficacy and safety of using rapamycin to treat vascular tumours and malformations. Response to therapy was defined by patient-reported symptom improvement, radiological reduction in size of lesions, and/or improvement of laboratory parameters. Results Forty-two patients (7 with vascular tumours and 35 with vascular malformations) have been treated with rapamycin. Despite 33 of 42 patients being diagnosed in the first year of life, the median age of initiating rapamycin was 11 years. Of the 38 children treated for a minimum of 4 months, 29 (76%) exhibited a clinical response. Twenty-one patients had follow-up imaging studies and of these, 16 (76%) had radiographic decrease in lesion size. Median time to demonstration of response was 49 days. All five children with vascular tumours and all three children with vascular malformations under the age of 4 years showed a clinical response. Response rate was lower for children ≥ 4 years of age (0/2, 0% for vascular tumours; 21/28, 75% for vascular malformations). No patient experienced an infection directly related to rapamycin or discontinued rapamycin due to toxicity. Conclusions Rapamycin is safe and efficacious in most children with select vascular tumours and malformations. Young children appear to respond better, suggesting that early initiation of rapamycin should be considered.

Who needs prenatal counselling with a pediatric surgeon? Experience from a large tertiary care university hospital

2022 ◽  
Vol 0 (0) ◽  
Author(s):  
Alizée Froeliger ◽  
Luke Harper ◽  
Sara Tunon de Lara ◽  
Frédéric Lavrand ◽  
Maya Loot ◽  
...  
Keyword(s):  
Tertiary Care ◽  
Fetal Mri ◽  
University Hospital ◽  
First Year ◽  
Pediatric Surgeon ◽  
Prenatal Consultation ◽  
Prenatal Counselling ◽  
Large Tertiary Care ◽  
Surgeon Experience ◽  
First Year Of Life

Abstract Objectives To describe our experience with prenatal counselling for surgical anomalies in a large volume center. The secondary aim is to suggest a list of prenatal abnormalities warranting counselling by a pediatric surgeon. Methods We reviewed all prenatal counselling consultations performed by the pediatric surgery team between January 1st, 2015 and December 31st, 2016. Results A total of 169 patients or couples had a prenatal consultation with a pediatric surgeon. Prenatal work-up included a fetal MRI in 26% of cases, mainly for digestive and thoracic pathologies (56.1% of cases). Consultation with the pediatric surgeon led mainly to recommendations concerning the place of delivery. Induction for reasons related to the fetal anomaly occurred in 22.2% of cases. Most children were surgically treated within the first year of life (63.5%). Correlation between predicted prognosis and actual status at four years of life was 96.9%. Correlation between prenatal and postnatal diagnosis was 87.4%. Conclusions Prenatal counselling by a pediatric surgeon allows couples to obtain clear information on the pathology of their unborn child, giving them greater autonomy in their decision to continue the pregnancy.

scholarly journals 13 Evaluating Potential Overuse of Intravenous Hydration in Patients Hospitalized with Bronchiolitis

2020 ◽  
Vol 25 (Supplement_2) ◽  
pp. e5-e5
Author(s):  
Vénitia Langlois ◽  
Chloé Sainte-Marie-Lestage ◽  
Anne-Claude Bernard-Bonnin ◽  
Nathalie Lucas ◽  
Pascal Bédard ◽  
...  
Keyword(s):  
Management Practice ◽  
Tertiary Care ◽  
Pediatric Emergency ◽  
Local Order ◽  
First Year ◽  
National Guidelines ◽  
Oral Hydration ◽  
Intravenous Hydration ◽  
First Year Of Life ◽  
Local Guideline

Abstract Background Bronchiolitis is the primary cause of hospitalization in children during the first year of life. Respiratory support and hydration are the two pillars of management. Practice guidelines for bronchiolitis (NICE and CPS) recommend nasogastric (NG) hydration before intravenous (IV) fluids if oral hydration cannot be maintained. However, IV hydration remains the primary mode of hydration at our tertiary care pediatric centre. Objectives This is a resource stewardship project aiming to evaluate the baseline use of IV versus NG hydration in children 0-15 months hospitalized for bronchiolitis and requiring supplemental hydration. The secondary objective is to evaluate potential barriers to NG hydration for these children. Design/Methods Data was collected retrospectively for the 2017-2018 bronchiolitis season. A survey was sent to all the pediatric emergency (ED) physicians and nurses, pediatric hospitalists, ED fellows, pediatric residents and ward nurses. Results Among eligible patients (n=95), IV hydration was prescribed 52% of the time, compared to 48% NG hydration. Ninety-six percent (47/49) of IV hydration was initiated in the ED, while only 4% (2/49) was initiated on the ward. Among patients who began receiving hydration in the ED (80/95, or 84%), 41% (33/80) were hydrated via NG and 59% (47/80) by IV. In the survey, while 92% (57/62) of responding physicians claimed to be aware of national guidelines for bronchiolitis, only 67% (42/62) said that NG was the recommended mode of hydration. Among ED practitioners, 56% (19/34) were up-to-date on the recommendation. Two-thirds of all respondents (physicians and nurses) felt that NG hydration would be more acceptable to parents. Among nurses, 84% (38/45) felt that IV installation was a longer procedure to complete. Despite the existence of a local order set indicating NG hydration as the preferred hydration method, only 64% (40/62) of physicians were aware of its existence, and only 23% of these (9/40) actually use the guideline. Main reasons of disuse included forgetting the guideline exists and it not being easily available. In contrast, 93% of nurses were aware of the local guideline. Conclusion Intravenous hydration is overused among patients hospitalized with bronchiolitis at our centre, and the ED is the main setting where this occurs. There is a discrepancy between physicians’ theoretical knowledge of hydration guidelines and the application of these in reality. Barriers to the use of NG hydration can be amenable to a quality improvement (QI) intervention targeting improved knowledge and use of our local guideline, and this will be our focus going forward.

CONGENITAL ABSENCE OF THE PARATHYROID AND THYMUS GLANDS IN AN INFANT

PEDIATRICS ◽  
1966 ◽  
Vol 38 (3) ◽  
pp. 412-418
Author(s):  
Leonard S. Taitz ◽  
Concepcion Zarate-Salvador ◽  
Eugene Schwartz
Keyword(s):  
Vitamin D ◽  
Parathyroid Hormone ◽  
Renal Tubular Acidosis ◽  
The Other ◽  
Congenital Absence ◽  
First Year ◽  
Associated Anomalies ◽  
Patient Reported ◽  
Renal Tubular ◽  
First Year Of Life

A female infant with congenital absence of the parathyroid and thymus glands is reported. In addition, the patient developed renal tubular acidosis which was associated with nephrocalcinosis. The relation of this complication to the presence of hypomagnesemia and the administration of parathyroid hormone calcium and vitamin D is discussed. It is proposed that two forms of primary hypoparathyroidism are seen in the first year of life. One is a non-lethal sex-linked form. The other, to which the patient reported here belongs, has a high mortality which may be due to immunological disorders secondary to the absence of the thymus gland or multiple associated anomalies.

Clinical Characteristics of Measles in Infancy: A Hospital Based Study

2021 ◽  
Vol 36 (1) ◽  
pp. 20-24
Author(s):  
Farhana Rahat ◽  
MF Abiduzzaman ◽  
Ahmed Murtaza Choudhury
Keyword(s):  
Clinical Characteristics ◽  
Tertiary Care ◽  
Signs And Symptoms ◽  
Maculopapular Rash ◽  
First Year ◽  
Runny Nose ◽  
Number Of Children ◽  
Control And Prevention ◽  
First Year Of Life ◽  
Main Complication

Background: There is a global resurgence of measles among children in recent years and a number of infants are being affected. Objectives: The aim of the study was to determine the frequency of measles in infancy and to describe their clinical characteristics in a tertiary care children hospital. Methods: A prospective observational study was conducted in Dr. MR Khan Shishu Hospital and Institute of Child Health from March, 2019 to February, 2020. The children who came with signs and symptoms of measles, like fever with maculopapular rash associated with cough, runny nose and conjunctivitis were recorded and frequency of measles in infants among hospitalized measles patients was noted. Infants with measles were enrolled for the study and their clinical characteristics, complications and outcome were determined. Data were analyzed by SPSS version 23. Results: A total of 64 infants were studied. The frequency of measles in infants among hospitalized measles patients was 43%. Forty seven percent infants were between 9 to 10 months. Clinical features were typical and all had fever and maculopapular rash. Pneumonia was the main complication and occurred in 50(78%) cases which was followed by oral ulcer 42(66%), diarrhea 26(41%), febrile seizure 9(14%) and croup 5(8%). Thirty four (53%) infant had normal nutritional status. Only 19.51% infant received first dose of measles vaccine. The mortality rate was 2(3%). Conclusion: A number of children are being affected by measles before completing first year of life and they develop complications which are related to morbidity and mortality. So, control and prevention of measles in infancy should give more importance. DS (Child) H J 2020; 36(1) : 20-24

Tracheotomy in the Preschool Population: Indications and Outcomes

2007 ◽  
Vol 137 (2) ◽  
pp. 280-283 ◽  
Author(s):  
Lesley C. French ◽  
Christopher T. Wootten ◽  
Robert G. Thomas ◽  
Wallace W. Neblett ◽  
Jay A. Werkhaven ◽  
...  
Keyword(s):  
Airway Obstruction ◽  
Tertiary Care Hospital ◽  
Tertiary Care ◽  
Upper Airway ◽  
Upper Airway Obstruction ◽  
Direct Result ◽  
First Year ◽  
Care Hospital ◽  
Age Group ◽  
First Year Of Life

OBJECTIVE: Although more tracheotomy procedures are performed within the first year of life than in any other age group, preschool-aged children requiring tracheotomy remain understudied. We characterize the indications and outcomes for patients between the ages of 3 and 6 years undergoing tracheotomy. METHODS: Out of 480 pediatric tracheotomy procedures performed at a tertiary-care hospital between 1988 and 2004, 15 patients underwent primary tracheotomy between 3 and 6 years of age. RESULTS: Most (60%) procedures were performed for pulmonary toilet. Upper-airway obstruction represented the second most common indication (40%), and trauma necessitated tracheotomy procedures more often than had been predicted (40%). The decannulation rate was 40%; 2 patients died. CONCLUSION: Trauma contributed to both upper-airway obstruction as well as requirements for pulmonary toilet. These procedures performed secondary to trauma will likely continue to increase. SIGNIFICANCE: Tracheotomy procedures in the preschool population remain uncommon; however, nearly half of those studied were performed as a direct result of otherwise preventable trauma.

Clinical Management Considerations in Long-Term Survivors With Trisomy 18

PEDIATRICS ◽  
1990 ◽  
Vol 85 (5) ◽  
pp. 753-759
Author(s):  
Don C. Van Dyke ◽  
Malinda Allen
Keyword(s):  
Tertiary Care ◽  
Trisomy 18 ◽  
First Year ◽  
Chromosomal Anomalies ◽  
Medical Problems ◽  
Number Of Children ◽  
Support Organization ◽  
Present Complex ◽  
First Year Of Life ◽  
Long Term Survivors

As many as 90% or more of children with trisomy 18 die within the first year of life. A review of six patients with trisomy 18 documented by karyotype surviving past 1 year of age and of the trisomy 18 files of the Support Organization for Trisomy 18 and 13 indicated that a small number of children with trisomy 18 survive beyond their first year of life; a few live into their teens and twenties. In addition to medical problems that are unique to this chromosomal syndrome, these patients present complex medical problems common to all persons with chromosomal anomalies. The primary and tertiary care consultants who are able to provide knowledge and sensitive supportive care to children with trisomy 18 and to their parents are performing a service of significant benefit, no matter how brief the life span of the child may be.

scholarly journals Profile of congenital cataract in the first year of life from a tertiary care center in South India – A modern series

2021 ◽  
Vol 69 (4) ◽  
pp. 932
Author(s):  
Ramesh Kekunnaya ◽  
VivekMahendrapratap Singh ◽  
Akshay Badakere ◽  
Preeti Patil-Chhablani
Keyword(s):  
Congenital Cataract ◽  
South India ◽  
Care Center ◽  
Tertiary Care ◽  
First Year ◽  
Tertiary Care Center ◽  
First Year Of Life

CARCINOMA OF THE CERVIX IN THE FIRST YEAR OF LIFE

PEDIATRICS ◽  
1950 ◽  
Vol 5 (6) ◽  
pp. 924-929
Author(s):  
GEORGE P. HECKEL
Keyword(s):  
Vaginal Bleeding ◽  
First Year ◽  
Total Hysterectomy ◽  
Patient Reported ◽  
First Year Of Life

Two cases of adenocarcinoma of the cervix in girls 7 and 11 months old are described. There is probably but one previously reported case under 1 year of age. The first patient reported here died of the disease on the day of admission to the hospital, having had vaginal bleeding for about a month. The second is surviving normally 21 months after total hysterectomy. Vaginal bleeding had begun eight days before operation.

scholarly journals Comparative study of intranasal hypertonic seawater saline versus intranasal normal saline in allergic rhinitis

2016 ◽  
Vol 3 (1) ◽  
pp. 104 ◽  
Author(s):  
Roohie Singh ◽  
Jeevan R. Galagali ◽  
Santosh Kumar ◽  
Yogesh Bahurupi ◽  
Mandar Chandrachood
Keyword(s):  
Allergic Rhinitis ◽  
Normal Saline ◽  
Tertiary Care ◽  
Post Treatment ◽  
Response To Therapy ◽  
Symptom Improvement ◽  
Group A ◽  
The Difference ◽  
Pre Treatment ◽  
Group B

<p class="abstract"><strong>Background:</strong> Allergic rhinitis (AR) is a chronic disease with variable response to therapy.<strong> </strong>Nasal irrigation with saline, including hypertonic saline, has been recommended for sinonasal conditions<span lang="EN-IN">. </span></p><p class="abstract"><strong>Methods:</strong> All consecutive patients reporting with AR symptoms established by ARIA at a zonal and tertiary care referral hospital from July 1 to September 30, 2015 were enrolled in the study. Patients were randomly divided into two groups. Patients in Group A were treated with hypertonic seawater saline (HSS) 2.2% Group B with normal saline (NS) 0.9% respectively. Symptoms were assessed at the start of the treatment and after 2 months using 4 point scale.<strong></strong>60 patients were included in final analysis<span lang="EN-IN">.  </span></p><p class="abstract"><strong>Results:</strong> The mean total nasal symptoms post treatment when compared to pre-treatment were low in both the groups [2.19 (pre- treatment) vs. 1.03 (post treatment) in Group A and 2.18 (pre-treatment) vs. 1.46 (post treatment) in Group B] and the difference was statistically significant for both groups (P =0.0001). On comparing post-treatment symptom scores between both groups, Group a benefitted more than Group B and it was statistically significant (P =0.002). The difference in individual symptom improvement (except sneezing) post treatment exhibited a statistical significance in Group A.No side-effects were seen with either of the sprays<span lang="EN-IN">. </span></p><p class="abstract"><strong>Conclusions:</strong> In our study, both treatments provided clinically meaningful responses, but the overall result favored HSS. Hence, HSS can be an effective and safe therapy for AR<span lang="EN-IN">.</span></p>

The Development of Swallowing Respiratory Coordination

2009 ◽  
Vol 18 (1) ◽  
pp. 19-24
Author(s):  
Maggie-Lee Huckabee
Keyword(s):  
Cortical Activation ◽  
First Year ◽  
Healthy Children ◽  
Nasal Airflow ◽  
Single Session ◽  
Primary Finding ◽  
Patterns Of Behavior ◽  
Cortical Modulation ◽  
First Year Of Life ◽  
Swallowing Apnea

Abstract Research exists that evaluates the mechanics of swallowing respiratory coordination in healthy children and adults as well and individuals with swallowing impairment. The research program summarized in this article represents a systematic examination of swallowing respiratory coordination across the lifespan as a means of behaviorally investigating mechanisms of cortical modulation. Using time-locked recordings of submental surface electromyography, nasal airflow, and thyroid acoustics, three conditions of swallowing were evaluated in 20 adults in a single session and 10 infants in 10 sessions across the first year of life. The three swallowing conditions were selected to represent a continuum of volitional through nonvolitional swallowing control on the basis of a decreasing level of cortical activation. Our primary finding is that, across the lifespan, brainstem control strongly dictates the duration of swallowing apnea and is heavily involved in organizing the integration of swallowing and respiration, even in very early infancy. However, there is evidence that cortical modulation increases across the first 12 months of life to approximate more adult-like patterns of behavior. This modulation influences primarily conditions of volitional swallowing; sleep and naïve swallows appear to not be easily adapted by cortical regulation. Thus, it is attention, not arousal that engages cortical mechanisms.

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