Publications by authors named "Porter F"

Pain and stress have been shown to induce significant physiological and behavioral reactions in newborn infants, even in those born prematurely. Infants who are born prematurely or seriously ill are commonly exposed to multiple painful and stressful events as part of their prolonged hospitalizations and required medical procedures. There is now evidence that these early events not only induce acute changes, but that permanent structural and functional changes may also result.

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Objective: Previous reports have shown that pain is managed inadequately in newborn infants. Ironically, clinicians believe that infants can experience pain much like adults, that infants are exposed daily to painful procedures, and that pain protection should be provided. In adults, a close relationship has been shown in how adults behave in response to pain, how painful they sense the stimulus to be, and physical measurements of the intensity of the stimulus.

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In order to explain the phenotype observed in Lhx2 mutant embryos, we previously proposed that an Lhx2 related gene might exist. We now have cloned a new LIM/homeobox gene called Lhx9. Lhx9 is closely related to Lhx2 and is expressed in the developing central nervous system (CNS).

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Background: Symptoms from asthma are often prominent at night. In adults significant circadian variation has been shown with reduced peak expiratory flow rates and increased bronchial reactivity to methacholine in the early morning hours. Because adolescence is the age group with the greatest increase in asthma-related deaths in the 1980s, we hypothesized that adolescents might be susceptible to circadian variation of airway reactivity.

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Background: Previous reports have suggested that healthy, full-term newborn infants who are in more aroused behavioral states tend to respond more robustly to painful events. Others have shown that acutely ill premature and full-term infants who undergo significant handling and immobilization as part of required nursery procedures respond less robustly to concurrent painful events.

Purpose: To investigate, using an experimental manipulation, the effect of arousal associated with handling and immobilization on response to acute pain in generally healthy, premature and full-term newborn infants.

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Objective: Emergency management of pediatric fractures and dislocations requires effective analgesia, yet children's pain is often undertreated. We compared the safety and efficacy of fentanyl- versus ketamine- based protocols.

Methodology: Patients 5 to 15 years of age needing emergency fracture or joint reduction (FR) were randomized to receive intravenous midazolam plus either fentanyl (F/M) or ketamine (K/M).

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The Smith-Lemli-Opitz syndrome (SLOS; also known as "RSH syndrome" [MIM 270400]) is an autosomal recessive multiple malformation syndrome due to a defect in cholesterol biosynthesis. Children with SLOS have elevated serum 7-dehydrocholesterol (7-DHC) levels and typically have low serum cholesterol levels. On the basis of this biochemical abnormality, it has been proposed that mutations in the human sterol Delta7-reductase (7-DHC reductase; E.

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Objective: This case-control study investigated the protective efficacy against pertussis of three doses of a two-component acellular pertussis vaccine (manufactured by Biken in Japan) combined with diphtheria and tetanus toxoids (manufactured by Connaught Laboratories in the US) in infants.

Methods: A case-control study was performed in 63 pediatric practices in Germany. Prospective recruitment of 16,780 infants ages 6 to 17 weeks took place between February, 1993, and July, 1994.

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Background: Despite an increased awareness among clinicians regarding pain and pain management for infants undergoing surgery, pain associated with procedures performed outside the operating room may not be adequately managed.

Purpose: To examine the beliefs and self-described behavior of physicians and nurses regarding the management of procedural pain in newborn infants.

Methods: A survey was distributed to 467 clinicians (nurses and physicians) working in 11 level II and 4 level III nurseries in a large metropolitan area.

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Inheritance of an inactivated form of the VHL tumor suppressor gene predisposes patients to develop von Hippel-Lindau disease, and somatic VHL inactivation is an early genetic event leading to the development of sporadic renal cell carcinoma. The VHL gene was disrupted by targeted homologous recombination in murine embryonic stem cells, and a mouse line containing an inactivated VHL allele was generated. While heterozygous VHL (+/-) mice appeared phenotypically normal, VHL -/- mice died in utero at 10.

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OBJECTIVE: To compare the polymerase chain reaction (PCR) results with conventional culture results for the diagnosis of Bordetella pertussis infections. METHODS: PCR and culture were performed in the course of a large vaccine efficacy trial in Germany on specimens taken from 7153 children less-than-or-equal2 years of age with cough illness lasting >6 days, and laboratory results were compared with clinical data also obtained from the patients. Calcium alginate nasopharyngeal swabs were taken for culture and clinical data were obtained from patients.

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We investigated the function of Lhx2, a LIM homeobox gene expressed in developing B-cells, forebrain and neural retina, by analyzing embryos deficient in functional Lhx2 protein. Lhx2 mutant embryos are anophthalmic, have malformations of the cerebral cortex, and die in utero due to severe anemia. In Lhx2-/- embryos specification of the optic vesicle occurs; however, development of the eye arrests prior to formation of an optic cup.

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The family of basic helix-loop-helix (bHLH) genes comprises transcription factors involved in many aspects of growth and development. We have previously described two bHLH transcription factors, Nhlh1 and Nhlh2 (originally named NSCL1 and NSCL2). The nucleotide and predicted protein sequences of Nhlh1 and Nhlh2 are homologous within their bHLH domain where there are only three conservative amino acid differences.

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To investigate the effect of dementia on response to pain, 51 community-dwelling, generally healthy, cognitively intact individuals > or = 65 years old and 44 community- or nursing home-dwelling persons > or = 65 years old with varying severity of dementia were studied. Cognitive status was assessed by standardized clinical evaluation and psychometric test performance. The following responses were measured before, during and after a standard venipuncture procedure: heart rate, the amplitude of respiratory sinus arrhythmia (RSA), self-reported anxiety and pain, and videotaped facial expressions.

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We report on a case of Klippel-Trenaunay Weber syndrome (KTWS) associated with a reciprocal translocation [46,XX,t (5;11) (q13.3;p15.1)].

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To investigate the relations among popular measures of neonatal stress and their link to subsequent temperament, 50 full-term newborns from a normal care nursery were examined responding to a heelstick blood draw. Baseline and heelstick measures of behavioral state, heart period, vagal tone, and salivary cortisol were obtained. Recovery measures of behavioral and cardiac activity were also analyzed.

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To evaluate the efficacy of subcutaneous administration of lidocaine for reducing physiologic instability in acutely ill newborns during clinically required procedures, 81 neonates who required lumbar punctures within the first month of life were stratified by birth weight and respiratory support and randomly assigned to an experimental or a control group. The experimental group received an injection of 0.1 mL/kg of 1% lidocaine prior to the lumbar puncture.

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A group of 32 patients with low vision who were considered clinically appropriate candidates for visual rehabilitation with telescopic spectacles were prospectively studied before the first attempted use of these visual aids. Laboratory measurements were made of: (1) rotational head stability in pitch and yaw during quiet standing; (2) sensitivity of visual acuity with telescopic spectacles to imposed yaw head motion; and (3) ocular stabilization reflexes during passive, whole-body rotation in the horizontal plane. Predicted likelihood of successful use of telescopic spectacles was prospectively computed for each patient using the measurement of head stability in the pitch axis and the sensitivity of visual acuity with telescopic spectacles to head motion using a previously described statistical method.

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