"Cri du Chat" due to a ring-B chromosome.
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Biomedical subjects
Publications and source records attributed to R Tompkins.
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The biliary secretion of sulindac and metabolites after a single 400 mg oral dose of the drug was studied in 3 elective gallbladder surgical patients following placement of an occludable T-tube in the common bile duct. Bile and systemic plasma were sampled at frequent intervals for up to 36 h postdose. The apparent biliary clearance (Vcl,bile) of the prodrug sulindac is about 25 times greater than that of the pharmacologically active sulfide metabolite. The total biliary flux of drug in normal man with an uninterrupted enterohepatic cycle, calculated from Vcl,bile and historic mean plasma drug AUCinfinity0 values, averages 144 and 12.2 per cent of the dose as sulindac and the sulfide metabolite, respectively. Thus, enterohepatic recycling of the drug in man is principally in the form of the prodrug which not only limits exposure of the intestine to the active moiety but also sustains systemic concentrations of active drug upon reabsorption of the prodrug.
By cooling the blood just before returning it to the body, marked reduction in the temperature of the heart, brain, liver, and kidney can be produced easily and with great economy in heat transfer, since the great bulk of the animal or patient, consisting of skin, muscle, and bone, is cooled much more slowly. This results in a marked oxygen saving, due to the direct antimetabolic effect of the cooling. Metabolic acidosis can be avoided and so far no irreversible effects of the cooling have been discoverable. Warming is so greatly facilitated by the heat stored in the nonvital areas that it is not necessary to warm the blood returning from the extracorporeal circuit. The use of safe, low, extracorporeal flows offers a number of possible advantages including great simplicity of cannulation and of the pump oxygenator design and operation.
Hypertrophic scarring is a major source of morbidity in patients with burns. The physiologic characteristics are poorly understood, but increased neovascularity is typically seen in those wounds destined to become hypertrophic. We theorize that ablation of the developing neovasculature may favorably influence the development of the hypertrophic scar. The goal of this pilot trial was to establish the practicality and safety of tunable dye laser neovessel ablation at 585 nm. Ten sites of evolving hypertrophic scar in nine children were treated with a series of 450 msec 6.75 J/cm2 pulses at 585 nm. Although all children had the expected transient posttreatment purpura, no pain, ulceration, pruritus, or worsening of the lesions was seen. The technique appears safe and is worthy of continuing investigation. Investigations with higher fluences and multiple treatments are in progress.
To facilitate effective management of pain and anxiety, and to permit more objective assessment of changes in this management, a pain and anxiety guideline was developed and has been followed uniformly for 3 years. The guideline describes four patient care categories: (1) ventilated acute, (2) nonventilated acute, (3) chronic acute, and (4) reconstructive. A small and consistent formulary was emphasized. A specific guideline for background, procedural, and transition pain and anxiety management was developed for each patient care category. All pain and anxiety medications given to all acutely burned children admitted to the Institute for 12 consecutive months were recorded, and daily pain and anxiety discomfort scores were noted using a 5-level action-based bedside scoring system. Doses of individual pain and anxiety medications were calculated as mg per kg per patient-day in each category, and all doses were found to be within guideline specifications. The efficacy of the guideline was judged by four discomfort scores: (1) background pain, (2) procedural pain, (3) background anxiety, and (4) procedural anxiety, and were adequate in all patient categories. There were no complications related to overmedication experienced during the interval. Our objective was to develop a guideline for pain and anxiety management that: (1) was safe and effective over a broad range of ages and injury acuities seen in the unit, (2) was explicit in its recommendations, (3) had a limited formulary to optimize staff familiarity with agents used, and (4) took advantage of the presence of a bedside nurse to continuously evaluate efficacy and intervene when needed through dose-ranging. Although many drugs are appropriate, our choices were based on institutional familiarity and simplicity. This process of developing a clear and consistent guideline can be duplicated in any unit.
We grafted 10 sites on 6 children with limited (< 25%) areas of body surface available for donor harvest with an acellular allogenic dermis and a thin autograft. Matched control sites were grafted with autograft alone. Study site autografts (0.0074 +/- 0.0007 in, median 0.006 in, range 0.006 to 0.012 in) were thinner than control site autografts (0.0102 +/- 0.0008 in, median 0.012 in, range 0.006 to 0.013 in), with a P value of .015. Endopoints were initial engraftment (in percent) as judged by a blinded experienced observer and Vancouver scar scores. The 6 children (3 girls and 3 boys) had an average age of 5.2 +/- 0.9 years (range 2.8 to 10 years) and an average burn size of 68.7% +/- 6.7% total body surface area (range 47% to 85% total body surface area). The 10 study and control sites were treated with 10 separate procedures; 9 of the procedures were reconstructive and 1 was performed for the excision of an acute burn. Successful initial epithelialization was noted at 7 days postburn for 83% +/- 3.4% (range 60% to 95%) at the cryopreserved acellular human dermis sites and 83.3% +/- 4.3% (range 60% to 98%) at the control sites (not significant, P = .96). At a mean follow-up interval of 43.7 +/- 3.6 weeks (median 52, range 26 to 52 weeks), the differences between the study and control sites in the total mean (pigmentation, vascularity, pliability, and height) of the patients' Vancouver scar scores were not different.
Prompt transfer of the child with acute burns can be difficult from distant or inaccessible locations, and it is believed that the outcomes of children with serious burns whose transfer to a specialized burn care facility is delayed may be compromised. A 4-year experience with 16 consecutive children with serious burns (> or =20% of the body surface area) whose transfer to a burn care facility was delayed for 5 or more days was reviewed to document the difficulties that can follow such delays. These 16 children had an average age of 8.6+/-1.6 years and an average wound size of 57.6%+/-5.8% of the body surface area, and they arrived a mean of 16.3+/-3.4 days after the injury (range, 5 to 44 days). These children had undergone an average of 1 operation, excluding escharotomies, at referring facilities. Only 4 (25%) of the children had no infectious focus at transfer, and at admission resistant bacteria were recovered from 9 (56%) of the children and fungal organisms were found in 10 (63%). Compared with a concurrently managed matched control group of patients admitted to the burn center within 24 hours of injury, the delayed-transfer group had statistically significantly more bacteremia, renal dysfunction, wound sepsis, and central venous catheter days. It was also more expensive to manage these children; the delayed-transfer group required statistically significantly longer to achieve 95% wound closure, and they had greater total lengths of hospital stay and more rehabilitation days. The early transfer of children with serious burns to a specialized burn center may truncate hospitalization and thereby reduce costs.
To develop a standardized, practical, self-administered questionnaire to monitor pediatric patients with burns and to evaluate the effectiveness of comprehensive pediatric burn management treatments, a group of experts generated a set of items to measure relevant burn outcomes. Children between the ages of 5 and 18 years were assessed in a cross-sectional study. Both parent and adolescent responses were obtained from children 11 to 18 years old. The internal reliability of final scales ranged from 0.82 to 0.93 among parents and from 0.75 to 0.92 among adolescents. Mean differences between parent and adolescent were small; the greatest difference occurred in the appearance subscale. Parental scales showed evidence of validity and potential for sensitivity to change. In an effort to support the construct validity of the new scales, they were compared with the Child Health Questionnaire and related to each other in clinically sensible ways. These burn outcomes scales reliably and validly assess function in patients with burns, and the scales have been developed in such a way that they are likely to be sensitive to change over time.
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Gynogenesis was used to map eight loci to their centromeres in Xenopus laevis. Several loci remote from their centromeres were identified. This information may be useful in distinguishing gynogenetic diploid progeny produced by suppression of second polar bodies from gynogenetic diploid progeny homozygous at all loci produced by suppression of first cleavage of gynogenetic haploids.