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The Turkish Journal of Pediatrics May 2024Given the strong genetic background of familial Mediterranean fever (FMF), the frequently reported co-existing diseases in children with FMF should also be investigated...
BACKGROUND
Given the strong genetic background of familial Mediterranean fever (FMF), the frequently reported co-existing diseases in children with FMF should also be investigated in other family members. Therefore, we aimed to examine the medical conditions of first-degree relatives (FDRs) of our pediatric patients with FMF in the present study.
METHODS
Chronic diseases of FDRs of pediatric 449 FMF, 147 juvenile idiopathic arthritis (JIA) patients and 93 healthy controls (HC) were questioned during their routine clinical visits for 9 consecutive months.
RESULTS
A total of 1975 FDRs of 449 FMF, 690 FDRs of 147 JIA patients, and 406 FDRs of 93 HC were included into the study. The most common medical conditions were non-atopic asthma (n=71, 3.6%), type 2 DM (n=14, 2%), and tonsillectomy history (n=12, 2.95%) in the FMF, JIA, and HC groups, respectively. Atopic diseases (FMF vs. JIA: p=0.013; FMF vs. HC: p=0.014), rheumatic diseases (FMF vs. JIA: p=0.030; FMF vs. HC: p=0.017), and surgical histories (FMF vs. JIA: p<0.01; FMF vs. HC: p=0.026), including adenoidectomy, tonsillectomy, and appendectomy, were significantly more common in the FMF group than in other groups.
CONCLUSIONS
Our novel findings may contribute to understanding the hereditary burden of co-existing diseases in children with FMF and encourage further studies involving genetic screenings.
Topics: Humans; Familial Mediterranean Fever; Female; Male; Child; Child, Preschool; Arthritis, Juvenile; Adolescent; Turkey; Case-Control Studies; Family; Adult; Asthma
PubMed: 38814299
DOI: 10.24953/turkjpediatr.2024.4589 -
F1000Research 2024Hunter syndrome (MPS II), an X-linked recessive lysosomal storage disorder, is a result of deficiency of the iduronate 2-sulfatase enzyme (IDS), leading to cognitive...
Hunter syndrome (MPS II), an X-linked recessive lysosomal storage disorder, is a result of deficiency of the iduronate 2-sulfatase enzyme (IDS), leading to cognitive impairment, systemic organ involvement, and increased dental problems. This case report describes the management of a child with Hunter syndrome who was referred to the Department of Paediatric and Preventive Dentistry for pain in the upper front teeth. Intraoral examination revealed severe early childhood caries, prompting planning for full-mouth rehabilitation under general anaesthesia due to the child's uncooperative behaviour. In response to recommendations from the Department of Otolaryngology and the Department of Paediatric Surgery, a comprehensive treatment plan consolidated full-mouth rehabilitation in addition to adenoidectomy and inguinal and umbilical herniotomy procedures during a single session of general anaesthesia. Successful interventions were reflected in the uneventful one-month follow-up of the patient, highlighting the efficacy of the interdisciplinary approach. The key takeaway underscores the importance of collaborative interventions, emphasising singular intubation for patients requiring recurrent hospitalisations, providing both monetary relief and reducing post operative healing time. Designed to address global developmental delay in the child, a personalised home care plan was also implemented. Evaluation of plaque and gingival indices before and after the home care regimen demonstrated a notable improvement, indicating an enhanced oral quality of life.
Topics: Humans; Mucopolysaccharidosis II; Quality of Life; Male; Child; Holistic Health; Dental Care
PubMed: 38812528
DOI: 10.12688/f1000research.146468.1 -
Frontiers in Pharmacology 2024Glycopyrrolate is commonly researched as a preoperative medication or in conjunction with cholinesterase inhibitors to counteract the lingering muscarinic effects of...
Effects of glycopyrrolate and atropine for oral secretions and perioperative hemodynamics in children undergoing tonsillectomy and adenoidectomy: a prospective, single-center, randomized, double-blind, controlled trial.
INTRODUCTION
Glycopyrrolate is commonly researched as a preoperative medication or in conjunction with cholinesterase inhibitors to counteract the lingering muscarinic effects of non-depolarizing muscarinic agents. However, studies have yielded inconsistent results regarding the superiority of glycopyrrolate over other anti-cholinergic drugs, such as atropine, particularly its effect on heart rate, blood pressure (BP), and glandular secretions. This study aimed to evaluate the differences in perioperative oral secretions, hemodynamics, and recovery quality with glycopyrrolate versus those with atropine before anesthesia induction in children undergoing tonsillectomy and adenoidectomy.
METHODS
In this prospective, single-center, randomized, double-blind, controlled trial, a total of 103 children were randomly assigned to group A (n = 51, glycopyrrolate 0.005 mg/kg) or B (n = 52, atropine 0.01 mg/kg). The follow-up anesthetic induction and maintenance protocols were the same in both groups. Vital signs, duration of surgery, extubation time, degree of wetness around the vocal cords during tracheal intubation, weight of oral secretions, and perioperative complications were recorded.
RESULTS
No significant differences were observed in the degree of wetness around the vocal cords during tracheal intubation, as well as in the weight of oral secretions, duration of surgery, or extubation time, between the two groups. The intraoperative and postoperative heart rates were lower in group A than in group B (110.18 ± 10.58 vs. 114.94 ± 11.14, = 0.028; 96.96 ± 10.81 vs. 103.38 ± 10.09, = 0.002). The differences observed in the intraoperative and preoperative heart rates were lower in group A than in group B (23.84 ± 9.62 vs. 29.65 ± 8.75, = 0.002). The differences observed in the postoperative and preoperative heart rates were lower in group A than in group B (10.63 ± 9.97 vs. 18.09 ± 9.39, = 0.000).
CONCLUSION
Glycopyrrolate showed a smoother change in heart rate than atropine during and after tonsillectomy and adenoidectomy, with no effect on BP or recovery quality, and did not increase oral secretions. The findings indicate that glycopyrrolate can serve as an alternative to atropine to prevent secretions in anesthesia induction for tonsillectomy and adenoidectomy in children. This study was registered with the Chinese Clinical Trial Registry (Registration Number: ChiCTR2200063578; Date of Registration: 12/09/2022).
PubMed: 38783938
DOI: 10.3389/fphar.2024.1344786 -
Pediatric Quality & Safety 2024The Institute of Medicine introduced the Learning Healthcare System concept in 2006. The system emphasizes quality, safety, and value to improve patient outcomes. The...
BACKGROUND
The Institute of Medicine introduced the Learning Healthcare System concept in 2006. The system emphasizes quality, safety, and value to improve patient outcomes. The Bellevue Clinic and Surgical Center is an ambulatory surgical center that embraces continuous quality improvement to provide exceptional patient-centered care to the pediatric surgical population.
METHODS
We used statistical process control charts to study the hospital's electronic health record data. Over the past 7 years, we have focused on the following areas: efficiency (surgical block time use), effectiveness (providing adequate analgesia after transitioning to an opioid-sparing protocol), efficacy (creating a pediatric enhanced recovery program), equity (evaluating for racial disparities in surgical readmission rates), and finally, environmental safety (tracking and reducing our facility's greenhouse gas emissions from inhaled anesthetics).
RESULTS
We have seen improvement in urology surgery efficiency, resulting in a 37% increase in monthly surgical volume, continued adaptation to our opioid-sparing protocol to further reduce postanesthesia care unit opioid administration for tonsillectomy and adenoidectomy cases, successful implementation of an enhanced recovery program, continued work to ensure equitable healthcare for our patients, and more than 85% reduction in our facility's greenhouse gas emissions from inhaled anesthetics.
CONCLUSIONS
The Bellevue Clinic and Surgical Center facility is a living example of a learning health system, which has evolved over the years through continued patient-centered QI work. Our areas of emphasis, including efficiency, effectiveness, efficacy, equity, and environmental safety, will continue to impact the community we serve positively.
PubMed: 38751896
DOI: 10.1097/pq9.0000000000000724 -
Gastroenterology Research Apr 2024Inflammatory bowel disease (IBD) is a group of chronic inflammatory gastrointestinal disorders that are caused by genetic susceptibility and environmental factors and...
BACKGROUND
Inflammatory bowel disease (IBD) is a group of chronic inflammatory gastrointestinal disorders that are caused by genetic susceptibility and environmental factors and affects a significant portion of the global population. The gut-associated lymphoid tissue (GALT) is known to play a crucial role in immune modulation and maintaining gut microbiota balance. Dysbiosis in the latter has a known link to IBD. Therefore, the increasing prevalence of adenoidectomy in children should be explored for its potential association with IBD. The objective of this paper was to assess the association between adenoid tissue removal and the risk of developing Crohn's disease (CD) and ulcerative colitis (UC).
METHODS
We conducted a pooled meta-analysis to evaluate the extended clinical outcomes in patients who underwent appendicectomy and tonsillectomy compared to those who did not. Our approach involved systematically searching the PubMed database for relevant observational studies written in English. We followed the Meta-analysis of Observational Studies in Epidemiology (MOOSE) guidelines to collect data from various time periods, and to address the diversity in study results; we employed a random-effects analysis that considered heterogeneity. For outcomes, odds ratios (ORs) were pooled using a random-effects model.
RESULTS
Seven studies, out of a total of 114,537, met our inclusion criteria. Our meta-analysis revealed a significant association between appendicectomy and CD (OR: 1.57; 95% confidence interval (CI): 1.01 - 2.43; heterogeneity I = 93%). Similarly, we found a significant association between tonsillectomy and CD (OR: 1.93; 95% CI: 0.96 - 3.89; I = 62%). However, no significant association was observed between appendicectomy and UC (OR: 0.60; 95% CI: 0.24 - 1.47; I = 96%), while a modest association was found between tonsillectomy and UC (OR: 1.24; 95% CI: 1.18 - 1.30; I = 0%).
CONCLUSIONS
In summary, we found that the trend of appendicectomy is linked to higher odds of CD, and tonsillectomy is more likely associated with increased odds for both CD and UC, with a risk of bias present.
PubMed: 38716286
DOI: 10.14740/gr1672 -
Fujita Medical Journal May 2024The aim of this study was to evaluate the safety and efficacy of microdebrider adenoidectomy on sleep-disordered breathing among pediatric patients with OSA.
OBJECTIVES
The aim of this study was to evaluate the safety and efficacy of microdebrider adenoidectomy on sleep-disordered breathing among pediatric patients with OSA.
METHODS
In the microdebrider group (Group I), there were 30 Japanese OSA patients consisting of 26 boys and 4 girls. For comparison, we had 15 children (13 boys and 2 girls) who underwent classical adenoidectomy (Group II). Patients in Group I were selected from a pool of 95 pediatric Japanese OSA patients and were matched by age, preoperative AHI, and Kaup index with those in Group II.Parameters such as the amount of residual adenoid tissue, bleeding, duration of the procedure, and sleep-related metrics were compared between the two groups.
RESULTS
A significant improvement in postoperative AHI was observed in Group I (<0.05). The prevalence of AHI <1 was significantly higher in Group I compared with Group II (<0.05). Additionally, the amount of postoperative residual adenoid was significantly less in Group I (3/30 of Grade 3 and 4 adenoid size) than in Group II (7/15, <0.05). Furthermore, a reduction in postoperative AHI was proportionally associated with a decrease in residual adenoid.
CONCLUSIONS
The newly developed microdebrider adenoidectomy technique for pediatric OSA patients with adenotonsillar hypertrophy demonstrated greater accuracy and efficacy in ameliorating sleep apnea symptoms compared with the standard adenoidectomy approach.
PubMed: 38708075
DOI: 10.20407/fmj.2023-009 -
Cureus Apr 2024Choanal atresia obstructs the nasal passage due to abnormal bony or soft tissue remnants owing to the faulty canalization of the nasal passages during fetal development....
Choanal atresia obstructs the nasal passage due to abnormal bony or soft tissue remnants owing to the faulty canalization of the nasal passages during fetal development. The clinical manifestations are more pronounced in bilateral cases, often presenting immediately after birth with cyanosis turning pink when crying, as newborns are obligatory nasal breathers. This contrasts in unilateral cases, where the condition may present with mild symptoms and be diagnosed later in life. We present a case of a five-year-old male who initially presented with a concern for nasal polyps due to nasal congestion with absent airflow out of the right nostril. On examination of the pharynx and nose, the patient was diagnosed with nasal turbinate hypertrophy, the right more than the left, and was subsequently scheduled for bilateral inferior turbinate reduction, possible adenoidectomy, and nasal endoscopy. Intraoperatively, inspection with nasal endoscopy along with the inability to pass a catheter through the nasopharynx to reach the oropharynx was our indicator of a more severe diagnosis. Here, we report an incidental finding of the right choanal atresia and seek to highlight its importance given this incidental finding.
PubMed: 38707148
DOI: 10.7759/cureus.57669 -
Anesthesiology Apr 2024Adenotonsillectomy and tonsillectomy (now referred to as tonsillectomy thereafter) are common pediatric surgeries. Postoperative complications include hemorrhage...
BACKGROUND
Adenotonsillectomy and tonsillectomy (now referred to as tonsillectomy thereafter) are common pediatric surgeries. Postoperative complications include hemorrhage requiring surgery (2-3% of cases) and pain. While non-steroidal anti-inflammatory drugs are commonly administered for post-surgical pain, controversy exists regarding bleeding risk with cyclo-oxygenase-1 inhibition and associated platelet dysfunction. Preliminary evidence suggests selective cyclo-oxygenase-2 inhibitors, for example celecoxib, effectively manage pain without adverse events including bleeding. Given the paucity of data for routine celecoxib use after tonsillectomy, we investigated the association between post-operative celecoxib prescription and post-tonsillectomy hemorrhage requiring surgery using chart-review data from the Children's Hospital of Eastern Ontario, Canada.
METHODS
After ethics approval we performed a retrospective single-center observational cohort study in children <18 yrs undergoing tonsillectomy from January 2007 to December 2017. Cases of adenoidectomy alone were excluded due to low bleed rates. The primary outcome was the proportion of patients with post-tonsillectomy hemorrhage requiring surgery. The association between a celecoxib prescription and post-tonsillectomy hemorrhage requiring surgery was estimated using inverse probability of treatment weighting based on propensity scores and using generalized estimating equations to accommodate clustering by surgeon.
RESULTS
An initial patient cohort of 6468 was identified and 5846 children with complete data were included in analyses. Median (interquartile range) age was 6.10 (4.40, 9.00) years and 46% were female. In our cohort, 28.1% (n=1644) were prescribed celecoxib. Among the 4996 tonsillectomy patients, 1.7% (n=86) experienced post-tonsillectomy hemorrhage requiring surgery. The proportion with post-tonsillectomy hemorrhage requiring surgery among patients who had a tonsillectomy and were or were not prescribed celecoxib was 1.94% (30/1548; 95% CI: 1.36-2.75) and 1.62% (56/3448; 95% CI: 1.25-2.10), respectively. Modelling did not identify an association between celecoxib prescription and increased odds of post-tonsillectomy hemorrhage requiring surgery (OR=1.4, 95% CI: 0.85-2.31, p=0.20).
CONCLUSIONS
Celecoxib does not significantly increase the odds of post-tonsillectomy hemorrhage requiring surgery, after adjusting for covariates. This large pediatric cohort study of celecoxib administered after tonsillectomy provides compelling evidence for safety but requires confirmation with a multi-site randomized controlled trial.
PubMed: 38684054
DOI: 10.1097/ALN.0000000000005032 -
Children (Basel, Switzerland) Mar 2024(1) Background: Sleep-disordered breathing represents a growing public health concern, especially among children and adolescents. The main risk factors for pediatric...
(1) Background: Sleep-disordered breathing represents a growing public health concern, especially among children and adolescents. The main risk factors for pediatric sleep-disordered breathing in school-age children are tonsillar and adenoid hypertrophy. Adenoidectomy, often in combination with tonsillectomy, is the primary treatment modality for pediatric sleep-disordered breathing. This study aims to comprehensively investigate various risk and protective factors in children with sleep-disordered breathing undergoing adenotonsillar or adenoidal surgeries. We also aim to explore the differences in neuropsychological profiles. (2) Methods: This is an observational, retrospective cohort study. We collected information on adenoidectomy or adenotonsillectomy in children referred to our center. We reviewed the clinical history and preoperative visits and collected data through a telephone questionnaire. The Pediatric Sleep Questionnaire (PSQ) and the Pediatric Quality of Life Inventory (PedsQL) screen sleep-disordered breathing and quality of life, respectively. The data were statistically analyzed using SPSS version 22.0 for Windows (SPSS Inc., Chicago, IL, USA). (3) Results: The study involved 138 patients, but only 100 children participated. A higher percentage of patients with sleep-disordered breathing were observed to have mothers who smoked during pregnancy. A smaller proportion of patients with sleep-disordered breathing habitually used a pacifier. A rise in physical score was associated with a reduced PSQ at follow-up ( = 0.051). An increase in the overall academic score was related to a decrease in the PSQ at follow-up ( < 0.001). A more significant proportion of patients undergoing adenotonsillectomy were observed to have a history of prematurity and cesarean birth. (4) This comprehensive study delves into the intricate interplay of risk and protective factors impacting children with sleep-disordered breathing undergoing adenotonsillectomy and adenoidectomy.
PubMed: 38671605
DOI: 10.3390/children11040388 -
Cureus Mar 2024Cushing's syndrome is a rare disease that has a different primary etiology, most often due to chronic hypercortisolism. In addition to the defined causes, in contrast to...
Cushing's syndrome is a rare disease that has a different primary etiology, most often due to chronic hypercortisolism. In addition to the defined causes, in contrast to the general population, the observed etiology in pregnant women is a benign adrenocortical adenoma, less often bilateral hyperplasia of the adrenal glands of hypothalamic-pituitary origin or Cushing's disease, and malignant adrenal root adenoma. In this study, we present the case of a 41-year-old pregnant woman experiencing her first pregnancy. Her general diseases from anamnesis were chronic obstructive pyelonephritis, acute rhythm disturbance, somnolence, pituitary adenoidectomy, and adrenalectomy of both adrenal glands. The patient was obese, with a BMI of 31.25 kg/m. She sought medical help due to fatigue, anuria, vomiting, a fever of up to 38.9°C, and hypertension. In the 18th gestational week, fetal death was diagnosed. The fever persisted for several days, and the patient had a malaise and became intoxicated with evidence of sepsis. She was hospitalized at two medical facilities for clarification. In the Department of Gynecology at the Specialized Hospital for Obstetrics and Gynecology in Varna, Bulgaria, a cesarean section was performed. The patient's condition remained stable after surgery. She was referred to a central intensive care unit for follow-up.
PubMed: 38650766
DOI: 10.7759/cureus.56769