Disorders of Sex Development: Lessons to be Learned from Studies of Spina Bifida and Craniofacial Conditions.

The purpose of this review is to discuss research methods and clinical management strategies employed with other conditions (i. e., spina bifida and craniofacial conditions) and how these methods and strategies could be applied to youth with disorders of sex development (DSD). The review focuses specifically on the potential overlap between DSD and these other conditions across the following 3 areas: (1) developmentally-oriented theories that underlie the research base for chronic physical conditions; (2) research designs and methodological features that have proved fruitful in these areas; and (3) the potential applicability to DSD of clinical management practices for youth with craniofacial conditions.

Initial experience with intravenous pentobarbital sedation for children undergoing MRI at a tertiary care pediatric hospital: the learning curve.

OBJECTIVE: Our purpose is to describe the initial experience with intravenous pentobarbital sedation in children undergoing MRI at a tertiary pediatric hospital to identify errors associated with inexperience. SUBJECTS AND METHODS: The study included the first 100 children sedated with intravenous pentobarbital prior to magnetic resonance examination at a tertiary pediatric hospital. The protocol included a maximum dose of 6 mg/kg administered in three divided doses with the total dose not to exceed 200 mg. Flow sheets documenting vital signs, administered drug doses, and adverse reactions were maintained contemporaneous to sedation. RESULTS: Sedation was successful in 92 children. Of the eight children who failed sedation, three were at least 12 years old and three weighed more than 50 kg. chi2 tests identified significantly greater failure rates in children older than 11 years or weight greater than 50 kg. Two children had prolonged sedation after the maximum suggested dose was exceeded. CONCLUSIONS: The success rate was good, but could have been improved by restricting the use of pentobarbital to children less than 12 years of age and weighing less than 50 kg. Radiologists inexperienced with intravenous sedation should strictly observe the maximum suggested dose of pentobarbital to prevent prolonged sedation.

Family focused ethics.

It is my intent to explore the family, parent, patient, social work relationships as a focus central to the solution of ethical dilemmas. In today's environment, patient selection continues to reflect persistent patterns of biased allocation of services. The ability of a family to make a decision about medical treatment begins with an understanding of how choices regarding that treatment are shared. Without taking appropriate precautions, an increased risk arises of providing surgical procedures involving real medical risk, yet unresolved psychological trauma remains unassessed. The unrelenting questions of, When?, How?, and Why?, a family should be involved in the process of ethical decision-making, begs the inherent prejudice involved.

Sedation of difficult-to-sedate children undergoing MR imaging: value of thioridazine as an adjunct to chloral hydrate.

OBJECTIVE: The purpose of this prospective study was to evaluate the safety and efficacy of thioridazine as an adjunct to chloral hydrate sedation when children undergoing MR imaging are difficult to sedate. SUBJECTS AND METHODS: All 87 children in the study either could not be sedated with chloral hydrate alone or were mentally retarded. Thioridazine (2-4 mg/kg) was administered orally 2 hr before and chloral hydrate (50-100 mg/kg) was administered orally 30 min before the 104 MR examinations. All children were monitored by continuous pulse oximetry. All images were individually evaluated by pediatric radiologists and were graded acceptable if they contained only minimal motion artifact or no motion artifact. Studies were considered successful only when 95% or more of the images were acceptable. RESULTS: MR imaging was successful in 93 (89%) of 104 examinations. The success rate for children entered into the study because of prior failure of chloral hydrate sedation was not significantly different from the success rate for children with mental retardation. A tendency for increasing failure rate with age was not significant. No serious complications occurred during the study. The most common adverse reaction, transient reduced oxygen saturation, was seen in five children. Other adverse effects encountered were vomiting in four children, hyperactivity in two children, transient tachycardia in one child, and prolonged sedation in one child. No child required hospitalization because of an adverse reaction to sedation. CONCLUSION: The study indicates that thioridazine is a safe and effective adjunct to chloral hydrate when a child undergoing MR imaging is difficult to sedate.

High-dose chloral hydrate sedation for children undergoing MR imaging: safety and efficacy in relation to age.

OBJECTIVE: Sedation is frequently essential for successful MR imaging, and chloral hydrate is the most commonly used drug for this purpose in infants and children. Our experience with these patients suggested that this sedative is less effective in older children, even when administered in high doses. However, no prospective study comparing the efficacy of chloral hydrate sedation for children of different ages undergoing MR imaging has been reported. Accordingly, we performed a study to evaluate the effectiveness and safety of chloral hydrate sedation in children of various ages. SUBJECTS AND METHODS: The study included 300 infants and children, 1 month to 11 years old (mean, 3 years), who were given oral chloral hydrate, 100 mg/kg, for sedation before MR imaging. The maximum total dose administered was 2.5 g, which limited the study to children who weighed 25 kg or less. Sedation was considered successful when MR studies were completed and at least 95% of the images had little or no motion artifact. RESULTS: Sedation was successful in 273 (91%) of 300 children. It was unsuccessful in nine of the 203 children who were 48 months old or younger (96% success rate) and in 18 of the 97 children who were more than 48 months old (81% success rate). A single-tailed t-test showed that the children in whom sedation was unsuccessful were significantly older than those in whom it was successful to the .0005 level of significance. The failure rate increased steadily for children more than 48 months old. Several failures may also have resulted from lengthy examination times. Adverse reactions to chloral hydrate sedation included hyperactivity (6%), vomiting (4%), and mild respiratory depression (4%). No adverse reaction was severe enough to require hospitalization. CONCLUSION: The higher failure rate for chloral hydrate sedation in children more than 48 months old suggests that the patient's age is an important limitation to the usefulness of chloral hydrate sedation for children undergoing MR imaging. However, the low rate of adverse reactions makes chloral hydrate a safe drug for sedation of children undergoing MR imaging.

High dose chloral hydrate sedation for children undergoing CT.

Chloral hydrate is commonly used to sedate children before CT. However, no prospective study has been published of the safety and efficacy of chloral hydrate at high dose levels for children undergoing CT. We define high dose levels of oral chloral hydrate to be 80-100 mg/kg, with a maximum total dose of 2 g. High dose chloral hydrate sedation was administered orally to 295 children for 326 CT examinations. Adverse reactions occurred in 7% of the children, with vomiting being the most common (4.3% of children). Hyperactivity and respiratory symptoms each occurred in less than 2% of children. Prolonged sedation ( greater than 2 h) was not encountered in our series. Sedation was successful in producing motion free CT examinations, so that in 303 (93%) of the cases, no repeat CT scans were needed. We conclude that high dose oral chloral hydrate provides safe and effective sedation for children undergoing CT.