Eosinophilic esophagitis is a newly recognized disease first described about 50 years ago.The definition,diagnosis,and management have evolved with new published consensus guidelines and newly approved treatment avail...Eosinophilic esophagitis is a newly recognized disease first described about 50 years ago.The definition,diagnosis,and management have evolved with new published consensus guidelines and newly approved treatment available to pediatricians,enabling a better understanding of this disease and more targeted treatment for patients.We describe the definition,presentation,and diagnosis of eosinophilic esophagitis including management,challenges,and future directions in children.The definition,diagnosis,and management of eosinophilic esophagitis have evolved over the last 50 years.Consensus guidelines and newly approved biologic treatment have enabled pediatricians to better understand this disease and allow for more targeted treatment for patients.We describe the definition,presentation,diagnosis,management,and treatment in addition to the challenges and future directions of eosinophilic esophagitis management in children.展开更多
AIM To investigate the use of a multidisciplinary, longitudinal simulation to educate pediatric residents and nurses on management of pediatric diabetic ketoacidosis.METHODS A multidisciplinary, multiple step simulati...AIM To investigate the use of a multidisciplinary, longitudinal simulation to educate pediatric residents and nurses on management of pediatric diabetic ketoacidosis.METHODS A multidisciplinary, multiple step simulation course was developed by faculty and staff using a modified Delphi method from the Pediatric Simulation Center and pediatric endocrinology department. Effectiveness of the simulation for the residents was measured with a pre- and post-test and a reference group not exposed to simulation. A follow up post-test was completed 3-6 mo after the simulation. Nurses completed a survey regarding the education activity. RESULTS Pediatric and medicine-pediatric residents(n = 20) and pediatric nurses(n = 25) completed the simulation course. Graduating residents(n = 16) were used as reference group. Pretest results were similar in the control and intervention group(74% ± 10% vs 76% ± 15%, P = 0.658). After completing the intervention, participants improved in the immediate post-test in comparison to themselves and the control group(84% ± 12% post study; P < 0.05). The 3-6 mo follow up post-test results demonstrated knowledge decay when compared to their immediate post-test results(78% ± 14%, P = 0.761). Residents and nurses felt the interdisciplinary and longitudinal nature of the simulation helped with learning.CONCLUSION Results suggest a multidisciplinary, longitudinal simulation improves immediate post-intervention knowledge but important knowledge decay occurs, future studies are needed to determine ways to decrease this decay.展开更多
Introduction: Activated partial thromboplastin time (aPTT) is one of the most used coagulation tests in preoperative evaluation. Incidental detection of a prolonged aPTT is a problem in primary care, in which the gene...Introduction: Activated partial thromboplastin time (aPTT) is one of the most used coagulation tests in preoperative evaluation. Incidental detection of a prolonged aPTT is a problem in primary care, in which the general pediatrician should be able to attend its initial management. Objective: To describe final diagnosis of patients with prolonged aPTT in preoperative study. Materials and Methods: This is a descriptive study of patients referred from otorhinolaryngology. Results: Totally, 508 adenoidectomies and/or tonsillectomies were performed in our center, 38 of which referred patients (7.5%) with prolonged aPTT, and 30 of which met inclusion criteria. The median age was 4 years. 56.6% of patients were males. 76.6% of patients normalized aPTT at the second follow-up. Among these, 73.9% showed a normal study, 4.3% ha2d lupus anticoagulant and in 21.7% Von Willebrand disease was detected. Among patients that persisted with prolonged aPTT, 42.8% had coagulant factors deficiency, 28.5% had lupus anticoagulant and in 28.5% of patients a diagnosis could not be achieved with the tests used in the present study. Multivariate analysis did not show correlation between final diagnosis and the variables measured. Conclusion: The presence of a prolonged aPTT in children under preoperative study is due to a pre-analytic factor in the majority of cases or to the presence of lupus anticoagulant, normalizing values on follow-up. We suggest that a new aPTT be performed on these patients, and only those that persist altered or present a symptoms and family history of coagulation disorders be referred to hematology.展开更多
Potter syndrome is a rare congenital malformation that primarily affects male fetuses;it is characterized by pulmonary hypoplasia, skeletal malformation, and kidney abnormalities. The pressure of the uterine wall due ...Potter syndrome is a rare congenital malformation that primarily affects male fetuses;it is characterized by pulmonary hypoplasia, skeletal malformation, and kidney abnormalities. The pressure of the uterine wall due to oligohydramnios leads to an unusual facial appearance, abnormal limbs in abnormal positions, or contractures. The fetus generally dies soon after birth due to respiratory insufficiency. The baby was a live preterm male, born to a 30-year-old multigravida, out of a non-consanguineous marriage via cesarean section. There was no liquor at the time of delivery. The baby did not cry immediately after birth and required resuscitation, followed by mechanical ventilation. Multiple congenital anomalies suggestive of Potter’s syndrome were noted including facial features, flattened nose, low protruding ear, retrognathism, and epicanthal folds with unilateral atresia of the choana. Chest X-ray showed small volume lung fields suggestive of pulmonary hypoplasia, and we had on ultrasonography bilateral polycystic kidney disease on ultrasonography. At 42 hours of life, the baby developed tachypnea and severe chest retractions and died due to respiratory insufficiency. Our case highlights the importance of regular prenatal checks and examinations in each pregnancy, which helps to collect suspected cases and improve knowledge of this syndrome for better management.展开更多
Congenital hernia of the diaphragmatic dome (CHDD) is an embryonic malformation in which all or part of the diaphragmatic dome fails to develop properly. In the majority of cases (80% to 90%), this malformation affect...Congenital hernia of the diaphragmatic dome (CHDD) is an embryonic malformation in which all or part of the diaphragmatic dome fails to develop properly. In the majority of cases (80% to 90%), this malformation affects the left posterolateral part of the diaphragm, while in 10% to 15% of cases it affects the right. Bilateral cases are extremely rare, accounting for less than 1% of cases. This malformation is estimated to occur at a frequency of around 1 in 3500 births, with a male predominance. The diaphragmatic defect causes the abdominal organs to rise into the thoracic cavity during critical phases of lung development. These anomalies result in bilateral pulmonary hypoplasia, a reduced number of pulmonary vessels, and pulmonary arterial hypertension (PAH). The combination of these anatomical and functional anomalies, in varying degrees, explains the wide variability of symptoms at birth. Diagnosis is usually made prenatally by ultrasound, which enables severe forms of the disease to be detected and appropriate management initiated. The prognosis remains generally grave, with a neonatal mortality rate of between 30% and 60% depending on the study, and around half of all children will have long-term sequelae.展开更多
Focal dermal hypoplasia(FDH) is a rare disorder of the mesodermal and ectodermal tissues. Here we present an eight-year-old female known to have FDH who presents with poor weight gain and dysphagia. She was diagnosed ...Focal dermal hypoplasia(FDH) is a rare disorder of the mesodermal and ectodermal tissues. Here we present an eight-year-old female known to have FDH who presents with poor weight gain and dysphagia. She was diagnosed with multiple esophageal papillomas and eosinophilic esophagitis. She was successfully treated with argon plasma coagulation and ingested fluticasone propionate, which has not been described previously in a child.展开更多
BACKGROUND： Alcohol-based hand sanitizers （ABHSs） have been widely used in homes, workplaces and schools to prevent the spread of infectious diseases. We report a young child unintentionally ingested ABHS at a scho...BACKGROUND： Alcohol-based hand sanitizers （ABHSs） have been widely used in homes, workplaces and schools to prevent the spread of infectious diseases. We report a young child unintentionally ingested ABHS at a school, resulting in intoxication. METHODS：The child was a 6-year-old girl who had been brought to the emergency department （ED） for hypothermia, altered mental status （AMS）, periods of hypoventilation, hypothermia and vomiting. Computed tomography of her head revealed nothing abnormal in intracranial pathology. Urine drug screening was negative. Alcohol level was 205 mg/dL on admission. Other abnormal values included potassium of 2.8 mEq/L, osmolality of 340 mOsm/kg and no hypoglycemia. Further investigation revealed that the patient had gone frequently to the class restroom for ingestion of unknown quantities of ABHSs during the day. The patient was admitted for one day for intravenous fluid hydration and close observation of her mental status. RESULTS：The patient was discharged from the hospital the next day without any complications. CONCLUSION： Despite the large safety margin of ABHSs, emergency physicians need to be aware of the potential risk of ingestion of a large amount of such products in children and consider it in the assessment and management of school-age children with acute AMS.展开更多
文摘Eosinophilic esophagitis is a newly recognized disease first described about 50 years ago.The definition,diagnosis,and management have evolved with new published consensus guidelines and newly approved treatment available to pediatricians,enabling a better understanding of this disease and more targeted treatment for patients.We describe the definition,presentation,and diagnosis of eosinophilic esophagitis including management,challenges,and future directions in children.The definition,diagnosis,and management of eosinophilic esophagitis have evolved over the last 50 years.Consensus guidelines and newly approved biologic treatment have enabled pediatricians to better understand this disease and allow for more targeted treatment for patients.We describe the definition,presentation,diagnosis,management,and treatment in addition to the challenges and future directions of eosinophilic esophagitis management in children.
基金Supported by A University of Alabama at Birmingham Department of Pediatrics Founders Fund Grant
文摘AIM To investigate the use of a multidisciplinary, longitudinal simulation to educate pediatric residents and nurses on management of pediatric diabetic ketoacidosis.METHODS A multidisciplinary, multiple step simulation course was developed by faculty and staff using a modified Delphi method from the Pediatric Simulation Center and pediatric endocrinology department. Effectiveness of the simulation for the residents was measured with a pre- and post-test and a reference group not exposed to simulation. A follow up post-test was completed 3-6 mo after the simulation. Nurses completed a survey regarding the education activity. RESULTS Pediatric and medicine-pediatric residents(n = 20) and pediatric nurses(n = 25) completed the simulation course. Graduating residents(n = 16) were used as reference group. Pretest results were similar in the control and intervention group(74% ± 10% vs 76% ± 15%, P = 0.658). After completing the intervention, participants improved in the immediate post-test in comparison to themselves and the control group(84% ± 12% post study; P < 0.05). The 3-6 mo follow up post-test results demonstrated knowledge decay when compared to their immediate post-test results(78% ± 14%, P = 0.761). Residents and nurses felt the interdisciplinary and longitudinal nature of the simulation helped with learning.CONCLUSION Results suggest a multidisciplinary, longitudinal simulation improves immediate post-intervention knowledge but important knowledge decay occurs, future studies are needed to determine ways to decrease this decay.
文摘Introduction: Activated partial thromboplastin time (aPTT) is one of the most used coagulation tests in preoperative evaluation. Incidental detection of a prolonged aPTT is a problem in primary care, in which the general pediatrician should be able to attend its initial management. Objective: To describe final diagnosis of patients with prolonged aPTT in preoperative study. Materials and Methods: This is a descriptive study of patients referred from otorhinolaryngology. Results: Totally, 508 adenoidectomies and/or tonsillectomies were performed in our center, 38 of which referred patients (7.5%) with prolonged aPTT, and 30 of which met inclusion criteria. The median age was 4 years. 56.6% of patients were males. 76.6% of patients normalized aPTT at the second follow-up. Among these, 73.9% showed a normal study, 4.3% ha2d lupus anticoagulant and in 21.7% Von Willebrand disease was detected. Among patients that persisted with prolonged aPTT, 42.8% had coagulant factors deficiency, 28.5% had lupus anticoagulant and in 28.5% of patients a diagnosis could not be achieved with the tests used in the present study. Multivariate analysis did not show correlation between final diagnosis and the variables measured. Conclusion: The presence of a prolonged aPTT in children under preoperative study is due to a pre-analytic factor in the majority of cases or to the presence of lupus anticoagulant, normalizing values on follow-up. We suggest that a new aPTT be performed on these patients, and only those that persist altered or present a symptoms and family history of coagulation disorders be referred to hematology.
文摘Potter syndrome is a rare congenital malformation that primarily affects male fetuses;it is characterized by pulmonary hypoplasia, skeletal malformation, and kidney abnormalities. The pressure of the uterine wall due to oligohydramnios leads to an unusual facial appearance, abnormal limbs in abnormal positions, or contractures. The fetus generally dies soon after birth due to respiratory insufficiency. The baby was a live preterm male, born to a 30-year-old multigravida, out of a non-consanguineous marriage via cesarean section. There was no liquor at the time of delivery. The baby did not cry immediately after birth and required resuscitation, followed by mechanical ventilation. Multiple congenital anomalies suggestive of Potter’s syndrome were noted including facial features, flattened nose, low protruding ear, retrognathism, and epicanthal folds with unilateral atresia of the choana. Chest X-ray showed small volume lung fields suggestive of pulmonary hypoplasia, and we had on ultrasonography bilateral polycystic kidney disease on ultrasonography. At 42 hours of life, the baby developed tachypnea and severe chest retractions and died due to respiratory insufficiency. Our case highlights the importance of regular prenatal checks and examinations in each pregnancy, which helps to collect suspected cases and improve knowledge of this syndrome for better management.
文摘Congenital hernia of the diaphragmatic dome (CHDD) is an embryonic malformation in which all or part of the diaphragmatic dome fails to develop properly. In the majority of cases (80% to 90%), this malformation affects the left posterolateral part of the diaphragm, while in 10% to 15% of cases it affects the right. Bilateral cases are extremely rare, accounting for less than 1% of cases. This malformation is estimated to occur at a frequency of around 1 in 3500 births, with a male predominance. The diaphragmatic defect causes the abdominal organs to rise into the thoracic cavity during critical phases of lung development. These anomalies result in bilateral pulmonary hypoplasia, a reduced number of pulmonary vessels, and pulmonary arterial hypertension (PAH). The combination of these anatomical and functional anomalies, in varying degrees, explains the wide variability of symptoms at birth. Diagnosis is usually made prenatally by ultrasound, which enables severe forms of the disease to be detected and appropriate management initiated. The prognosis remains generally grave, with a neonatal mortality rate of between 30% and 60% depending on the study, and around half of all children will have long-term sequelae.
文摘Focal dermal hypoplasia(FDH) is a rare disorder of the mesodermal and ectodermal tissues. Here we present an eight-year-old female known to have FDH who presents with poor weight gain and dysphagia. She was diagnosed with multiple esophageal papillomas and eosinophilic esophagitis. She was successfully treated with argon plasma coagulation and ingested fluticasone propionate, which has not been described previously in a child.
文摘BACKGROUND： Alcohol-based hand sanitizers （ABHSs） have been widely used in homes, workplaces and schools to prevent the spread of infectious diseases. We report a young child unintentionally ingested ABHS at a school, resulting in intoxication. METHODS：The child was a 6-year-old girl who had been brought to the emergency department （ED） for hypothermia, altered mental status （AMS）, periods of hypoventilation, hypothermia and vomiting. Computed tomography of her head revealed nothing abnormal in intracranial pathology. Urine drug screening was negative. Alcohol level was 205 mg/dL on admission. Other abnormal values included potassium of 2.8 mEq/L, osmolality of 340 mOsm/kg and no hypoglycemia. Further investigation revealed that the patient had gone frequently to the class restroom for ingestion of unknown quantities of ABHSs during the day. The patient was admitted for one day for intravenous fluid hydration and close observation of her mental status. RESULTS：The patient was discharged from the hospital the next day without any complications. CONCLUSION： Despite the large safety margin of ABHSs, emergency physicians need to be aware of the potential risk of ingestion of a large amount of such products in children and consider it in the assessment and management of school-age children with acute AMS.
