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

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.

2021 ◽  
Vol 36 (1) ◽  
pp. 20-24
Author(s):  
Farhana Rahat ◽  
MF Abiduzzaman ◽  
Ahmed Murtaza Choudhury

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


2020 ◽  
Vol 25 (Supplement_2) ◽  
pp. e5-e5
Author(s):  
Vénitia Langlois ◽  
Chloé Sainte-Marie-Lestage ◽  
Anne-Claude Bernard-Bonnin ◽  
Nathalie Lucas ◽  
Pascal Bédard ◽  
...  

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.


2022 ◽  
Vol 0 (0) ◽  
Author(s):  
Alizée Froeliger ◽  
Luke Harper ◽  
Sara Tunon de Lara ◽  
Frédéric Lavrand ◽  
Maya Loot ◽  
...  

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.


2007 ◽  
Vol 137 (2) ◽  
pp. 280-283 ◽  
Author(s):  
Lesley C. French ◽  
Christopher T. Wootten ◽  
Robert G. Thomas ◽  
Wallace W. Neblett ◽  
Jay A. Werkhaven ◽  
...  

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.


Author(s):  
Soumitra Tole ◽  
Michelle Fantauzzi ◽  
Diana Cottingham ◽  
Joao G Amaral ◽  
Philip R John ◽  
...  

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.


2020 ◽  
Vol 101 (4) ◽  
pp. 538-543
Author(s):  
A V Alekseeva ◽  
E N Berezkina ◽  
K E Moiseeva ◽  
Sh D Kharbediya

Aim. To assess the impact of the number of children in the family on breastfeeding duration and vaccination coverage. Methods. 1724 mothers of 1-year old children were randomly chosen from seven children's polyclinics in St. Petersburg for an anonymous survey that was conducted by a specially designed form Questionnaire of a mother of 1-year old children. The questionnaire comprised of 20 open-ended and closed-ended questions, and included questions about: (1) timing of the attachment to the breast in obstetric hospitals; (2) causes and timing of breastfeeding abandonment; (3) presence or absence of vaccinations in the first year of life according to the National preventive vaccination schedule; (4) and reasons for mothers refusing to vaccinate their children. Results. The proportion of mothers who started artificial feeding in maternity wards immediately after the birth of the baby was the smallest among women for whom this baby was the first-born (3.4%), and the largest among families with many children (11.3%). On average, mothers with one child are breastfed until 7.360.11 months, with two children until 8.290.11 months, with three or more children until 8.780.10 months. By using one-way analysis of variance (ANOVA), it was shown the effect of the number of children in the family on the duration of breastfeeding (F=3.3). Correlation analysis revealed the negative relationship of the number of children in the family with the proportion of women who continued breastfeeding until 3 and 6 months (rxy=0.82 and rxy=0.88, respectively), and positive relationship with the proportion of mothers who continued to breastfeeding the baby after reaching a year (rxy=0.89). 12.3% of children of one-child families were not vaccinated according to the National preventive vaccination schedule, 17.7% with two-child families, 28.1% in families with three or more children. It was revealed the significant cross-group effect of the number of children in the family to vaccination coverage (F=48.7). With an increase in the number of children in the family, vaccination coverage decreases, both in general (rxy=0.88) and against individual infections, including hepatitis B, diphtheria, whooping cough, tetanus, polio, measles and rubella (rxy from 0.80 to 0.90). Conclusion. The number of children in a family impacts mothers' refusals of breastfeeding and vaccination; the more children in a family, the more prolonged breastfeeding, but less vaccination coverage due to the health status of children in the first year of life.


2021 ◽  
Vol 69 (4) ◽  
pp. 932
Author(s):  
Ramesh Kekunnaya ◽  
VivekMahendrapratap Singh ◽  
Akshay Badakere ◽  
Preeti Patil-Chhablani

2013 ◽  
Vol 10 (4) ◽  
pp. 39-43
Author(s):  
M S Geneva ◽  
D B Munblit ◽  
E I Ermolaeva ◽  
A K Pampura

Background. Atopic dermatitis (AD) prevalence may be compared in cohorts evaluated with standardized questionnaires. The prevalence of AD in Russia with the UK Working Party’s Diagnostic Criteria for Atopic Dermatitis was not tested yet. Objective. To estimate AD prevalence in a population of Moscow 1-year old children with the UK Working Party’s Diagnostic Criteria for Atopic Dermatitis Methods. A population cohort of 393 children was recruited in Postnatal Department of Moscow No.1 Maternity Hospital in 2011. One year later 363 mothers (92,4%) were questioned with the UK Working Party’s Diagnostic Criteria for Atopic Dermatitis. Cheeks/body hyperemia and/or skin rash were also estimated in 365 families in connection with food intake by a child or by his mother during breastfeeding. Sensitization at the age of one was investigated in 130 children. Results. The UK Working Party’s Diagnostic Criteria for Atopic Dermatitis diagnosed AD in 2,2% children (8 of 363). One or more episode of cheeks/body hyperemia and/or skin rash during the first year of life was in 45,75%. 5,38% of children were sensitized. Conclusion. A few amount of one year old children Moscow population is diagnosed with AD according to the UK Working Party’s Diagnostic Criteria for Atopic Dermatitis, especially in compare to the number of children with skin reactions to the dietary intake.


PEDIATRICS ◽  
1986 ◽  
Vol 77 (3) ◽  
pp. 381-381
Author(s):  
Paulette Mehta

Our daughter Asha grew up among endless conversations of our research work. Her first year of life unfolded between the pages of the book we edited on Platelets, Prostaglandins and Cardiovascular Disease. Subsequently, as she grew, nighttime discussions were on platelets and prostaglandins, dinner conversations on meetings and abstracts, bedtime stories were our manuscripts, and family albums were stacked with slides relating to our research data. Vacations were squeezed in between Mommy's and Daddy's presentations at various meetings. Nevertheless, Jay and I never noticed that Asha was different from any of her school friends. Recently, however, in preparation for her seventh birthday party, I told my daughter to bring me telephone numbers of her school friends so I could call their mothers to invite them. To my surprise, Asha returned home the next day with a 12-column spread sheet. The spread sheet was neatly labeled: Invitation List for Asha Mehta's Seventh Birthday Party. Each column was neatly sublabeled: Guest Number, Name, Date of Birth, Address, Nationality, Mother's Name, Father's Name, Number of Children in Family, Telephone Number, Response of Parents, and Remarks. Each row contained complete information on each potential guest. Finally, each column was boxed off, as though ready for statistical analysis. We finally realized the impact that our lives had had on our little child.


PEDIATRICS ◽  
1980 ◽  
Vol 65 (4) ◽  
pp. 847-847
Author(s):  
Thomas G. Flynn ◽  
John H. Kennell ◽  
Robert N. McLeod ◽  
Thomas E. Shaffer ◽  
William B. Strong ◽  
...  

Children less than 3 years old are most vulnerable to drowning, and organized efforts to reduce the toll are indicated. Heretofore, swimming instruction has concentration on school-aged children, but in recent years some emphasis has been placed on teaching younger children to swim, even during the first year of life. Although it may be possible to teach young infants to propel themselves and keep their heads above water, infants cannot be expected to learn the elements of water safety or to react appropriately in emergencies. No young child, particularly those who are preschool aged, can ever be considered "water safe." Parents may develop a false sense of security if they feel their young child can "swim" a few strokes. Additional problems may be associated with admission of infants to public swimming pools. Incontinent infants pose an aesthetic problem and make it difficult to maintain the effectiveness of chlorination. The Committee recognizes the increasing populaity of swimming programs for infants and the enjoyment of the parent and child in this shared activity, and makes the following recommendations: 1. If a parent wishes to enroll his/her infant in a water adjustment and swimming program, it should be on a one-to-one basis with the parent or a responsible adult. Organized group swimming instruction should be reserved for children more than 3 years old. 2. Instruction should be carried out by trained instructors in properly maintained pools. 3. Infants with known medical problems should receive clearance from their physician. 4. Controlled studies clarifying the possible risks to infants from swimming programs should be carried out as soon as possible.


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