Objective: To determine the prevalence of endocrinopath-ies, neuroradiographical findings, and growth derangements in young children with optic nerve hypoplasia (ONH). Study design: A prospective observational study e...Objective: To determine the prevalence of endocrinopath-ies, neuroradiographical findings, and growth derangements in young children with optic nerve hypoplasia (ONH). Study design: A prospective observational study examined the prevalence of endocrinopathies at study enrollment and growth patterns in children with ONH. Subjects (n = 47, mean ±SD 15.2 ±10.6 months) were followed until 59.0 ±6.2 months of age. Results: The prevalence of endocrinopathies was 71.7%: 64.1%of subjects had growth hormone (GH) axis abnormalities, 48.5%hyperprolactinemia, 34.9%hypothyroidism, 17.1%adrenal insufficiency, and 4.3%diabetes insipidus (DI). Endocrinopathies were not associated with ONH laterality, absence of the septum pellucidum, or pituitary abnormalities on neuroimaging. End height standard deviation score (SDS) was similar to start length SDS independent of GH surrogate status. A significant increase in end weight SDS was found for the cohort (P < .001). A body mass index (BMI) > 85th percentile was noted in 44.4%of the cohort and in 52.1%of subjects with GH axis abnormalities. Initial hyperprolactinemia was positively associated with increased end BMI SDS (P = .004)-. Conclusions: These prospective findings confirm the high prevalence of pituitary endocrinopathies in children with ONH reported in previous retrospective studies. Our data reveal that some of these children maintain normal height velocity despite GH axis abnormalities, and, as a group, they are at high risk for increased BMI.展开更多
As the use of inhaled nitric oxide (iNO) resulted in a decline in the need for extracorporeal membrane oxygenation (ECMO) in neonates with hypoxic respiratory failure, iNO has become an accepted treatment modality eve...As the use of inhaled nitric oxide (iNO) resulted in a decline in the need for extracorporeal membrane oxygenation (ECMO) in neonates with hypoxic respiratory failure, iNO has become an accepted treatment modality even in non-ECMO centers. However, because not all neonates respond to iNO, the timely identification and transfer of nonresponders to an ECMO center are important. Abstract: The objective of this study was to identify the risk factors predictive of the need of ECMO in neonates with hypoxic respiratory failure after the first 6 hours of iNO treatment in an ECMO center. Methods and Patient Population: Forty-nine patients with hypoxic respiratory failure transferred for iNO therapy and potential ECMO during a 2- year period were identified in this retrospective study. None of the patients had received iNO before admission. Strict clinical guidelines were used to standardize lung inflation, cardiovascular support, and iNO administration and weaning and to define treatment failure. The relationship between treatment failure (ie, the need for ECMO) and a set of suspected risk factors after 6 hours of iNO administration was examined by logistic regression analysis. Results: Twenty-two neonates responded to iNO(non-ECMO group)whereas 27 neonates failed and met ECMO criteria (ECMO group). There was no difference between the 2 groups in demographic data, ventilatory support, air leak syndrome at 6 hours of iNO treatment, and survival to discharge. However, the dose and duration of iNO therapy were predictive of the need for ECMO with an adjusted odds ratio of 1.12 (95% CI, 1.01- 1.25; P = .04) and 0.45 (95% CI, 0.27- 0.65; P = .0002), respectively. Conclusions: By the end of the first 6 hours of iNO treatment and under the specific conditions established by the use of the clinical guidelines, the dose and the duration of iNO administration were predictive of the probability for the need of ECMO in this patient population. Thus, one can establish a center-specific predictability model for the need of ECMO in neonates with hypoxic respiratory failure treated with iNO if strict clinical guidelines for iNO administration and weaning and respiratory and cardiovascular support are used in the given center.展开更多
The aim of this study was to review the outcome of surgical management of various types of perineal masses encountered in patients with anorectal malformations (ARM). Retrospective review from 2 large pediatric anorec...The aim of this study was to review the outcome of surgical management of various types of perineal masses encountered in patients with anorectal malformations (ARM). Retrospective review from 2 large pediatric anorectal referral centers. Twentytwo patients with a perineal mass were identified in more than 2000 patients treated for an ARM over a 15-year period. The 22 patients (4 men) represented all levels of severity of ARMs. The lesions were of 3 types: lipomas (n = 10), vascular anomalies (n = 4), and hamartomas/ choristomas (n = 8). The lipomas were carefully removed from between the muscle fibers during the posterior sagittal anorectoplasty. The vascular anomalies (3 of 4 were hemangiomas) underwent magnetic resonance imaging preoperatively, but none were found to invade deeply and all were excised at the time of the posterior sagittal anorectoplasty. The hamartomas/choristomas all occurred in women, and 50%arose as a pedunculated mass from the vulva. The lesions contained tissues such as glia, osteoid, nephrogenic rests, and endocervical-type mucosa. One was initially misinterpreted as a teratoma, prompting awider excision. This and all subsequent patients have been correctly diagnosed pathologically as having either hamartomas or choristomas, which were not widely excised. Follow-up ranges from 5 months to 12 years. Six of the 10 lipoma patients are continent. One vascular anomaly was reexcised and there was minor wound separation in another. None of the hamartoma/choristoma lesions recurred. The presence of unusual perineal masses can add to the complexity of ARMs; however, most of these lesions can be carefully excised with preservation of the muscle complex and ultimate continence. Hamartomatous lesions can be mistaken for teratomas but do not require aggressive excision with clear margins.展开更多
Background: Injuries to the head comprise 20% to 39% of all school-related injuries. Head injuries among special education students have not been adequately described. Objectives: (1)To examine the incidence and chara...Background: Injuries to the head comprise 20% to 39% of all school-related injuries. Head injuries among special education students have not been adequately described. Objectives: (1)To examine the incidence and characteristics of head injuries in children enrolled in special education and (2) to determine the factors that increase the risk of sustaining a head injury compared with an injury to another part of the body. Methods: Pupil Accident Reports for 6769 students enrolled in 17 of 18 special education schools in 1 large urban school district during the academic years 1994-1998 were reviewed, and information on the nature of injury, external cause, and activity was abstracted. Head-injured and nonhead-injured cases were identified and compared by race, sex, age, characteristics of injury, and disability category. Results: Six hundred ninety-seven injury events were reported during the 4-year study period. The overall injury rate was 4.7 injuries per 100 student-years. Two hundred five children (29.4% ) sustained injuries to the head, and the rate of head injury was 1.3 injuries per 100 student years. Falls were the leading cause of injury. Head injuries were most commonly associated with physical education and unstructured play and usually occurred on the playground. Disproportionately more head than nonhead injuries were sustained in the classroom (12% vs 8% ) and the bathroom (9% vs 3% ). Compared with children with emotional/mental disabilities, children with multiple disabilities had the highest risk of a head injury (incidence density ratio, 2.4 95% confidence interval, 1.6-3.5 ), followed by children with physical disabilities (incidence density ratio, 1.8 95% confidence interval, 1.1-3.1 ). There appeared to be no significant difference in the rate of head injury by sex and age. Conclusions: Modifications of the classroom, bathroom, and playground environments might reduce the risk of head injuries in children enrolled in special education. Special modifications and increased supervision may, in particular, reduce the risk of head injury for children with physical and multiple disabilities.展开更多
文摘Objective: To determine the prevalence of endocrinopath-ies, neuroradiographical findings, and growth derangements in young children with optic nerve hypoplasia (ONH). Study design: A prospective observational study examined the prevalence of endocrinopathies at study enrollment and growth patterns in children with ONH. Subjects (n = 47, mean ±SD 15.2 ±10.6 months) were followed until 59.0 ±6.2 months of age. Results: The prevalence of endocrinopathies was 71.7%: 64.1%of subjects had growth hormone (GH) axis abnormalities, 48.5%hyperprolactinemia, 34.9%hypothyroidism, 17.1%adrenal insufficiency, and 4.3%diabetes insipidus (DI). Endocrinopathies were not associated with ONH laterality, absence of the septum pellucidum, or pituitary abnormalities on neuroimaging. End height standard deviation score (SDS) was similar to start length SDS independent of GH surrogate status. A significant increase in end weight SDS was found for the cohort (P < .001). A body mass index (BMI) > 85th percentile was noted in 44.4%of the cohort and in 52.1%of subjects with GH axis abnormalities. Initial hyperprolactinemia was positively associated with increased end BMI SDS (P = .004)-. Conclusions: These prospective findings confirm the high prevalence of pituitary endocrinopathies in children with ONH reported in previous retrospective studies. Our data reveal that some of these children maintain normal height velocity despite GH axis abnormalities, and, as a group, they are at high risk for increased BMI.
文摘As the use of inhaled nitric oxide (iNO) resulted in a decline in the need for extracorporeal membrane oxygenation (ECMO) in neonates with hypoxic respiratory failure, iNO has become an accepted treatment modality even in non-ECMO centers. However, because not all neonates respond to iNO, the timely identification and transfer of nonresponders to an ECMO center are important. Abstract: The objective of this study was to identify the risk factors predictive of the need of ECMO in neonates with hypoxic respiratory failure after the first 6 hours of iNO treatment in an ECMO center. Methods and Patient Population: Forty-nine patients with hypoxic respiratory failure transferred for iNO therapy and potential ECMO during a 2- year period were identified in this retrospective study. None of the patients had received iNO before admission. Strict clinical guidelines were used to standardize lung inflation, cardiovascular support, and iNO administration and weaning and to define treatment failure. The relationship between treatment failure (ie, the need for ECMO) and a set of suspected risk factors after 6 hours of iNO administration was examined by logistic regression analysis. Results: Twenty-two neonates responded to iNO(non-ECMO group)whereas 27 neonates failed and met ECMO criteria (ECMO group). There was no difference between the 2 groups in demographic data, ventilatory support, air leak syndrome at 6 hours of iNO treatment, and survival to discharge. However, the dose and duration of iNO therapy were predictive of the need for ECMO with an adjusted odds ratio of 1.12 (95% CI, 1.01- 1.25; P = .04) and 0.45 (95% CI, 0.27- 0.65; P = .0002), respectively. Conclusions: By the end of the first 6 hours of iNO treatment and under the specific conditions established by the use of the clinical guidelines, the dose and the duration of iNO administration were predictive of the probability for the need of ECMO in this patient population. Thus, one can establish a center-specific predictability model for the need of ECMO in neonates with hypoxic respiratory failure treated with iNO if strict clinical guidelines for iNO administration and weaning and respiratory and cardiovascular support are used in the given center.
文摘The aim of this study was to review the outcome of surgical management of various types of perineal masses encountered in patients with anorectal malformations (ARM). Retrospective review from 2 large pediatric anorectal referral centers. Twentytwo patients with a perineal mass were identified in more than 2000 patients treated for an ARM over a 15-year period. The 22 patients (4 men) represented all levels of severity of ARMs. The lesions were of 3 types: lipomas (n = 10), vascular anomalies (n = 4), and hamartomas/ choristomas (n = 8). The lipomas were carefully removed from between the muscle fibers during the posterior sagittal anorectoplasty. The vascular anomalies (3 of 4 were hemangiomas) underwent magnetic resonance imaging preoperatively, but none were found to invade deeply and all were excised at the time of the posterior sagittal anorectoplasty. The hamartomas/choristomas all occurred in women, and 50%arose as a pedunculated mass from the vulva. The lesions contained tissues such as glia, osteoid, nephrogenic rests, and endocervical-type mucosa. One was initially misinterpreted as a teratoma, prompting awider excision. This and all subsequent patients have been correctly diagnosed pathologically as having either hamartomas or choristomas, which were not widely excised. Follow-up ranges from 5 months to 12 years. Six of the 10 lipoma patients are continent. One vascular anomaly was reexcised and there was minor wound separation in another. None of the hamartoma/choristoma lesions recurred. The presence of unusual perineal masses can add to the complexity of ARMs; however, most of these lesions can be carefully excised with preservation of the muscle complex and ultimate continence. Hamartomatous lesions can be mistaken for teratomas but do not require aggressive excision with clear margins.
文摘Background: Injuries to the head comprise 20% to 39% of all school-related injuries. Head injuries among special education students have not been adequately described. Objectives: (1)To examine the incidence and characteristics of head injuries in children enrolled in special education and (2) to determine the factors that increase the risk of sustaining a head injury compared with an injury to another part of the body. Methods: Pupil Accident Reports for 6769 students enrolled in 17 of 18 special education schools in 1 large urban school district during the academic years 1994-1998 were reviewed, and information on the nature of injury, external cause, and activity was abstracted. Head-injured and nonhead-injured cases were identified and compared by race, sex, age, characteristics of injury, and disability category. Results: Six hundred ninety-seven injury events were reported during the 4-year study period. The overall injury rate was 4.7 injuries per 100 student-years. Two hundred five children (29.4% ) sustained injuries to the head, and the rate of head injury was 1.3 injuries per 100 student years. Falls were the leading cause of injury. Head injuries were most commonly associated with physical education and unstructured play and usually occurred on the playground. Disproportionately more head than nonhead injuries were sustained in the classroom (12% vs 8% ) and the bathroom (9% vs 3% ). Compared with children with emotional/mental disabilities, children with multiple disabilities had the highest risk of a head injury (incidence density ratio, 2.4 95% confidence interval, 1.6-3.5 ), followed by children with physical disabilities (incidence density ratio, 1.8 95% confidence interval, 1.1-3.1 ). There appeared to be no significant difference in the rate of head injury by sex and age. Conclusions: Modifications of the classroom, bathroom, and playground environments might reduce the risk of head injuries in children enrolled in special education. Special modifications and increased supervision may, in particular, reduce the risk of head injury for children with physical and multiple disabilities.
