Split hand/foot malformation (SHFM), formerly known as ectrodactyly is a rare congénital anomaly, its incidence varies from 1/8.500 to 1/25.000 live birth. It mainly affects the development of the limbs, its clin...Split hand/foot malformation (SHFM), formerly known as ectrodactyly is a rare congénital anomaly, its incidence varies from 1/8.500 to 1/25.000 live birth. It mainly affects the development of the limbs, its clinical variability is standard, can present as an isolated feature or as a syndrome associated with other congenital anomalies. Our objective was to present the two cases of SHFM, and to review the literature on the clinical aspects and discuss a probable origin. The father went to school and is a driver because the malformations concerned only the fingers, were less severe, and did not prevent the realization of certain simple gestures of the daily life. On the other hand, the malformations of the fingers of the newborn were severe and the absence of the thumbs compromised the later prehension function. Also the association of a microglossia and a cleft palate contributed to a weight loss that justified hospitalization. The clinical presentation of split hands and feet is variable and the prognosis depends on the type of anomaly. Familial cases suggest a probable genetic origin. Genetic testing is necessary to establish genetic counseling.展开更多
Background: Severe hypoglycemia is a lifethreatening iatrogenic complication in the management of diabetic ketoacidosis, however, few studies have described how often this complication occurs. Objective: To identify t...Background: Severe hypoglycemia is a lifethreatening iatrogenic complication in the management of diabetic ketoacidosis, however, few studies have described how often this complication occurs. Objective: To identify the risk factors for severe hypoglycemia in children treated for diabetic ketoacidosis and to describe the sociodemographic, clinical characteristics and outcome of these children. Methods: From January 2013 to December 2017, 69 children admitted to pediatric intensive care unit with a diagnosis of diabetic ketoacidosis were enrolled in a retrospective and prospective study. Data of children with and without severe hypoglycemia were compared. Data were analyzed using Epi Info 7.1.5.0. Results: Out of 69 children enrolled, 10 (14.5%) had severe hypoglycemia (girls: 60%, median age 144 months (interquartile range, 108?-?168 months)). Episodes of severe hypoglycemia occurred during the first 48 hours of hospitalization in 8 (80%) children and during the late night shift in 6 (60%) children. Causes of severe hypoglycemia were: excessive insulin dose (60%), inappropriate infusion solution (70%), failure to properly monitor blood glucose levels (40%). Severe hypoglycemia risk factors were: age p?= 0.00004;OR = 42.0 [95% CI: 6.3?-?279]) and severe malnutrition (p?= 0.002;OR?=?10.9 [95% CI: 2.4 -?48.6]). Of 8 (11.6%) children died, 4 had severe hypoglycemia (p?= 0.01;OR = 8.8 [95% CI, 1.7 -?44.8]). Conclusion: The importance and severity of severe hypoglycemia require preventive measures to control risk factors.展开更多
文摘Split hand/foot malformation (SHFM), formerly known as ectrodactyly is a rare congénital anomaly, its incidence varies from 1/8.500 to 1/25.000 live birth. It mainly affects the development of the limbs, its clinical variability is standard, can present as an isolated feature or as a syndrome associated with other congenital anomalies. Our objective was to present the two cases of SHFM, and to review the literature on the clinical aspects and discuss a probable origin. The father went to school and is a driver because the malformations concerned only the fingers, were less severe, and did not prevent the realization of certain simple gestures of the daily life. On the other hand, the malformations of the fingers of the newborn were severe and the absence of the thumbs compromised the later prehension function. Also the association of a microglossia and a cleft palate contributed to a weight loss that justified hospitalization. The clinical presentation of split hands and feet is variable and the prognosis depends on the type of anomaly. Familial cases suggest a probable genetic origin. Genetic testing is necessary to establish genetic counseling.
文摘Background: Severe hypoglycemia is a lifethreatening iatrogenic complication in the management of diabetic ketoacidosis, however, few studies have described how often this complication occurs. Objective: To identify the risk factors for severe hypoglycemia in children treated for diabetic ketoacidosis and to describe the sociodemographic, clinical characteristics and outcome of these children. Methods: From January 2013 to December 2017, 69 children admitted to pediatric intensive care unit with a diagnosis of diabetic ketoacidosis were enrolled in a retrospective and prospective study. Data of children with and without severe hypoglycemia were compared. Data were analyzed using Epi Info 7.1.5.0. Results: Out of 69 children enrolled, 10 (14.5%) had severe hypoglycemia (girls: 60%, median age 144 months (interquartile range, 108?-?168 months)). Episodes of severe hypoglycemia occurred during the first 48 hours of hospitalization in 8 (80%) children and during the late night shift in 6 (60%) children. Causes of severe hypoglycemia were: excessive insulin dose (60%), inappropriate infusion solution (70%), failure to properly monitor blood glucose levels (40%). Severe hypoglycemia risk factors were: age p?= 0.00004;OR = 42.0 [95% CI: 6.3?-?279]) and severe malnutrition (p?= 0.002;OR?=?10.9 [95% CI: 2.4 -?48.6]). Of 8 (11.6%) children died, 4 had severe hypoglycemia (p?= 0.01;OR = 8.8 [95% CI, 1.7 -?44.8]). Conclusion: The importance and severity of severe hypoglycemia require preventive measures to control risk factors.
