Antley-Bixler syndrome from a prognostic perspective: report of a case and review of the literature.

The Antley-Bixler syndrome (ABS) is characterized by craniosynostosis, radiohumeral synostosis, and femoral bowing. Other findings include a trapezoid-shaped head, deformed ears, severe midface hypoplasia, choanal atresia or stenosis, and long bone fractures. Most ABS cases have died in the first months of life from respiratory complications. The poor prognosis in this condition makes counseling difficult and early termination of pregnancy a consideration. The medical and surgical management information presented here can be used as a guide for counseling parents in the future. We report on a new patient with ABS who now at age 3 yr, has been followed by the medical staff of Riley Children's Hospital since birth. She has had successful medical and surgical management. Although the multisynostoses seen in this disorder is undoubtedly related to the soft tissue malformations such as choanal stenosis and midface hypoplasia, the cause remains unknown. The literature is also reviewed in this condition.

Experimental study of the relationship between the base impedance and its time derivative in impedance plethysmography.

Transthoracic electrical impedance Z consists of a constant baseline component Z0 and the time variable component delta Z such that Z = Z0 + delta Z. The first time derivative of Z has a negative peak, the magnitude of which is (dZ/dt)min. Measurements of 170 volunteers show that (dZ/dt)min is a function of the constant baseline component Z0. This implies that at least a part of the difference of the magnitudes of (dZ/dt)min on Z0 must be taken into account in order to avoid erroneous conclusions or inaccurate calculations.

Effectiveness of two feeding methods in improving energy intake and growth of infants with cleft palate: a randomized study.

OBJECTIVE: To compare two feeding methods advocated for infants with cleft palate: (a) a squeezable plastic container with a narrow, long crosscut nipple (squeezable cleft palate nurser); and (b) a standard nipple with a crosscut (crosscut nipple). The effectiveness of a nutrition intervention protocol for these infants was also documented. DESIGN: Thirty-one infants (median age = 15 days) were randomized to one of two feeding methods (18 infants, squeezable cleft lip/palate nurser; 13 infants, crosscut nipple) within sex (21 boys, 10 girls) and palatal defect (22 cleft lip and palate, 9 isolated cleft palate) categories. The intervention included feeding technique instructions, nutrition counseling at each clinic visit, use of the same 20 kcal/oz standard formula for 12 months, and introduction of infant and soft table foods at 6 months. Four-day food records and growth data were obtained. MAIN OUTCOME MEASURES: Mean energy and protein intakes at 3 and 6 months of age and growth measurements during the first 18 months of life were obtained. STATISTICAL ANALYSES: A repeated measures analysis of variance for intakes was performed with time as the repeated measure and feeding method as the covariable. Similar analyses were completed for growth measures with sex and feeding method as covariates. RESULTS: Mean energy intake at 3 and 6 months of age (P = .24) and growth measurements during the first 18 months of life (P values: weight gain [grams per day], .73; weight, .21; length, .07; head circumference, .18; triceps and subscapular skinfolds and mid-arm circumference, .47, .48, and .69, respectively) were not significantly different. Both feeding methods were effective in supporting normal growth. APPLICATIONS: With adequate instruction related to the use of either feeding technique and close nutrition follow-up early in infancy, a dietitian or other health care practitioner may advise the use of either feeding method. These data support the need for feeding and nutrition education and early nutrition intervention.

Poloxamer 188 as vehicle for injectable diazepam.

The significant occurrence of thrombophlebitis in patients administered diazepam intravenously was described recently. This side effect has been attributed to the crystallization of diazepam and its subsequent precipitation upon contact with blood or intravenous fluids. The current study was designed to reveal whether the solubilizing capability of poloxamer 188 reduces the incidence of thrombotic and inflammatory effects of diazepam in rabbits. The incidence of early (3-hr) ear vein necrosis was 72% in the diazepam-treated ears, while the incidence of necrosis in the ears that received poloxamer 188 as a vehicle for diazepam was 25%. The occurrence of thrombosis and loss of vessel integrity also was higher in diazepam-treated ears than in those treated with diazepam plus poloxamer 188. Solubilization of diazepam with poloxamer 188 may decrease the incidence of the tested side effects.

Early surgery for craniofacial synostosis: an 8-year experience.

A prospective review is presented of 50 patients with one of the craniofacial synostosis syndromes who underwent early interventive craniofacial surgical correction (average age 7.6 months at time of surgery). The study has demonstrated the efficacy and safety of the techniques when employed in the infant. Satisfactory cranio-orbital form was achieved in the majority of the patients, although 10 patients required secondary surgery because of sutural refusion or the development of turricephaly or calvarial contour irregularities. Despite earlier hopes, this surgery did not result in the development of satisfactory occlusal relationships and midfacial form in the craniofacial dysostosis group (Crouzon's, Apert's, etc.). Based on this clinical experience, a surgical treatment plan is presented for the newborn with craniofacial synostosis.

Basic principles of electrohaemodynamics.

Electrohaemodynamics (EHD) is a method of measuring cardiac output continuously, also cardiac index, stroke volume, blood flow through limbs, vascular resistance, useful work output from the heart, elastic properties of arterial walls, and contractile and rate of energy generation properties of heart muscle. The method is based on simultaneous measurements of the time variations of arterial blood pressure and electrical conductance in a portion of human body. The measurements, in the form of analogue signals, are processed by means of a microprocessor or a mini-computer. The processing is based on equations which combine haemodynamics with measurements of electrical conductance and arterial pressure variation. Direct comparison of simultaneous cardiac output measurements using EHD and thermal dilution, as well as indirect comparisons with the Fick and dye dilution methods, show that EHD is at least as accurate as these methods. The reproducibility of EHD is within one percent when the subjects are in the steady state, which appears to be better than any other known technique.