Biomedical subjects
Publications and source records attributed to S R Cohen.
Severe morbidity and mortality may be associated with erythrodermic psoriasis, especially when complicated by septicemia. We describe five patients with erythrodermic psoriasis complicated by staphylococcal septicemia. In two, concurrent infection with HIV increased vulnerability to bacteremia.
Explore the source record for details and available documents.
OBJECTIVE: To test the acceptability, validity, and internal consistency reliability of the McGill quality of life questionnaire (MQOL) for persons living with HIV/AIDS. DESIGN: The validity of MQOL was tested by having HIV-seropositive outpatients complete the 16-item MQOL, a single-item scale (SIS) measuring overall quality of life (QOL), and a physical symptom questionnaire. METHODS: Factor analysis was used as a guide for construction of MQOL subscales. Validity was studied by determining the correlation between MQOL and SIS, and between MQOL physical measures, CD4 counts and the physical symptoms questionnaire. Multiple regression was employed to determine how best to combine MQOL subscales to predict SIS. RESULTS: MQOL was acceptable to this patient population. Factor analysis suggests that MQOL can be represented by live measures: a single item measuring physical well-being and four subscales representing physical symptoms, psychological symptoms, existential well-being, and support. Multiple regression analyses suggest that the existential domain contributes greatly to QOL for people with advanced HIV disease (CD4 counts < 100 x 10(6)/l). CONCLUSION: MQOL is an acceptable and valid measure of QOL for people living with HIV/AIDS, with meaningful and reliable subscales as well as a summary score. The inclusion of a measure of existential well-being in MQOL may make it a more valid measure of QOL, especially for people with advanced disease, than QOL instruments which do not include this domain.
Besipirdine hydrochloride (HP 749) is an indole-substituted analog of 4-aminopyridine. Besipirdine enhances both cholinergic and adrenergic neurotransmission in the central nervous system. The present study examined the efficacy and tolerability of two doses of besipirdine (5 and 20 mg b.i.d.) in 275 patients with Alzheimer disease during 3 months of treatment and for 3 months after withdrawal of treatment. Assessment after withdrawal of treatment was used in an effort to distinguish persistent efficacy attributable to a neuroprotective mechanism from reversible symptomatic efficacy. Besipirdine was generally well tolerated. The level of performance on the cognitive subscale of the Alzheimer Disease Assessment Scale (ADAS-Cog) was sustained during 3 months of treatment with besipirdine, whereas some deterioration in the performance of patients treated with placebo was observed over the same period. The small difference between active and placebo treatment groups approached, but did not reach statistical significance in the primary intent-to-treat analysis (p = 0.067); analysis of patients who completed all assessments was supportive (p = 0.031). Global ratings using the Clinician Interview-Based Impression of Change did not detect a besipirdine treatment benefit, possibly because of an adverse effect on mood and behavior in some patients. A high ratio of adrenergic to cholinergic potency may have resulted in the adverse effects of besipirdine and hence its failure to support the hypothesis that multiple neurotransmitter treatment may be more efficacious than monotherapy. The efficacy apparent on the ADAS-Cog after 3 months of treatment did not persist 3 months after withdrawal of treatment, suggesting that the benefit was symptomatic. This study provides a practical example of the use of treatment withdrawal assessment to distinguish neuroprotective from symptomatic efficacy.
An implant-supported prosthesis is often secured to the underlying gold cylinders by retaining screws. There must be access to these screws from the oral surface of the prosthesis. Conventional preparation of this access is from the oral surface. This approach is difficult because the underlying gold cylinder is obscured. It may be damaged by a rotary cutting instrument as the access is prepared above it, because the cylinder cannot be seen during the preparation. Alternatively, the access hole may be unnecessarily enlarged in an attempt to find the cylinder. This loss of structure may weaken the prosthesis. A new approach, using 18-gauge stainless steel wire as a rotary cutting instrument, permits preparation of the access chamber from the intaglio surface of the prosthesis. This reduces risk of damage to the gold cylinder and is less destructive to the overlying material.
BACKGROUND: Hypopigmented macules have been described infrequently as a presenting form of mycosis fungoides (MF). OBJECTIVE: This study was designed to clarify general characteristics of a hypopigmented MF variant. METHODS: Seven new cases were investigated with the use of descriptive epidemiology techniques. Demographic parameters, histopathology, and treatment outcomes were analyzed. These data were combined with those from prior reports to develop a broad composite view of this disease process. RESULTS: The median ages in our series were 36 years for disease onset and 39 years at biopsy diagnosis. All patients had brown or black skin. Histologic findings consistently showed a lack of epidermal atrophy and moderate to profound exocytosis. Treatment with PUVA induced rapid and complete repigmentation in six of seven patients. CONCLUSION: On the basis of our experience and a literature review, the hypopigmented variant of MF occurs in a younger population than typical forms of the disease and affects persons with dark skin almost exclusively. Microscopic features include lack of epidermal atrophy and moderate to extreme epidermotropism of infiltrating mononuclear cells. The treatment of choice appears to be PUVA.
This paper reviews several recent publications concerning the use of opioids to control cancer pain and highlights the wide variation in mean daily dose. Present methods of reporting do not provide an explanation for these widely different doses used. It is essential that we understand the circumstances in which high doses are required, as higher doses are associated with greater toxicity and higher cost. Several factors that may influence the dose of opioid required are discussed. It is suggested that reporting of cancer pain, patient population, pharmacological and nonpharmacological interventions, and toxicity be standardized to allow for rational guidelines to be established for opioid use in pain due to advanced cancer.
A 5-year review of 43 consecutive patients presenting to Scottish Rite Children's Medical Center with an open myelomeningocele defect was undertaken. The aim of the present study was to analyze the myelomeningocele defects, dimensions, and area to better define those factors that dictate the need for plastic surgical consultation for wound closure. Of the 43 patients identified, two were excluded because they first presented as older children; the remaining 41 all had their myelomeningoceles repaired within the first 36 hours of life. Of these, 31 underwent repair by the Neurosurgical Service, whereas for 10 patients (24.4%), the Plastic Surgery Service was asked to assist with closure. Comparison showed the mean (+/- standard deviation) area in the referred patients was 27.4 (7.6) cm2 versus 17.6 (7.9) cm2 in the patients not referred for closure (p = 0.002). A trend analysis predicting referral as a function of myelomeningocele area showed that 0 of 10 (0%) with an area of less than 15 cm2, 2 of 13 (15.4%) with an area equal to 15 to 20 cm2, 3 of 7 (42.9%) with an area of 21 to 25 cm2, and 4 of 9 (44%) with an area greater than 25 cm2 were referred (p = 0.001). Data from the interpretation of maximum myelomeningocele dimension also showed statistically significant trends in referral. Using multiple logistic regression, it was found that the odds of referral increased by a factor of 3.3 for every 1 cm increase in maximum dimension.
A case of purpura fulminans secondary to pneumococcal septicemia is presented in an 8-month-old girl. The purpuric lesions on the fingers of both hands, as well as on the lower extremities, were treated by the local application of medicinal leeches. There was nearly complete salvage of the threatened tissues and the baby made a complete recovery. The possible mechanisms by which the leeches may have contributed to the clinical salvage are discussed.
We retrospectively assessed the intermediate and long-term results of craniofacial surgery in 22 consecutive patients with nonsyndromic bicoronal synostosis to determine the outcome of corrective surgery. The study population consisted of 13 males and 9 females whose ages ranged from 6 weeks to 24 months (mean, 5.6 months) at the time of initial surgery. All patients had been assigned a diagnosis of nonsyndromic bicoronal synostosis. Each patient underwent resection of both coronal sutures and frontal orbital advancement with cranial vault remodeling using a floating forehead technique. Age at initial operation was 5 months or less in 13 patients and 6 months or more in 9. Complications occurred in 5 patients (23%), and 1 patient with an associated metabolic disorder died from respiratory arrest postoperatively. Follow-up ranged from 6 to 168 months (mean, 53.2 months). Results were graded according to the need for and extent of reoperation. Residual aesthetic deformities were documented in 12 patients (55%). Three patients (14%) required calvarial recontouring or cranioplasty to achieve satisfactory forehead contour or bony continuity. Total reoperation for recurrent deformity was required at a mean age of 29.7 months in 8 patients (36%) and is pending in another (4%). Four patients (18%) required a third operation (two total reoperations and two cranioplasties) to achieve satisfactory results. Eight of 13 patients (62%) operated on at 5 months of age or younger required total reoperation compared with 1 of 9 (11%) operated at 6 months of age or older. When analyzed alone, age of operation was a statistically significant determinant of the need for reoperation (p < 0.03). However, when subjected to multivariate analysis, neither age at operation nor the presence of an associated anomaly or positive family history had a significant effect on outcome.
Trisomy 21 develops as a result of nondisjunction of two homologous chromosomes during either the first or second meiotic division. One of the more important consequences of these genetic alterations is the predictable, although variable disturbance in the architecture of the craniofacial region [1]. Postnatal craniofacial morphology has been extensively studied in Down's syndrome (DS). However, little information is available on human prenatal development of the head and face in such patients. The time at which changes in craniofacial phenotype first emerge in Down's syndrome fetuses and at which physical growth begins to diverge from normal is unknown. To explore these questions, we compared prenatal craniofacial growth in 50 Down's syndrome fetuses with that of 555 fetuses judged to be "typical for body weight and age" using the method of log-linear allometry [2].
Normative data on human craniofacial growth during the fetal period and important to provide a broader perspective on normal morphogenesis as well as to serve as reference for analyzing craniofacial syndromes in which growth has gone awry. Over a 19-year period, the Teratology Unit at the University of Michigan Medical Center has collected data on 2,568 legally donated fetuses that have undergone necropsy examination at various gestational ages. From previous analyses, 609 of the total fetal population (25%) were designated as typical for age or body weight on the basis of normal morphology, absence of maceration, and general growth symmetry. Of the 609 fetuses reviewed, 54 were excluded secondary to incomplete data. The remaining 555 constitute the basis of this study. Seven craniofacial measurements were recorded, including head circumference (HC), brain weight, inner canthal and outer canthal distances, and distances from nasion to menton, outer canthus to tragus and auditory meatus to vertex. Statistical analysis was carried out using the single-factor allometric model of Sewall Wright. Size was estimated as the first unstandardized principal component of the logarithms of lengths and of cube roots of weights, and then allometry was expressed in the regressions of each log variable on size. Significant allometry was found as were significant differences in errors about the allometric relation, but no evidence for more than a single factor or of "nonlinearity" in the regression curves was noted. Although there were differences of specific allometric coefficients between the various measurements (i.e., the slope of the curve for IC was significantly smaller than the slope of the curve generated for HC), these specific growth rates remain in relatively strict proportion to one another from early in gestation (body weight, 54.2 gm) to later in gestation (body weight, 1,000 gm).
Application of distraction osteogenesis to the human craniofacial skeleton in properly selected cases represents a major advance in the treatment of craniofacial deformities. We report our initial clinical experience with a system of miniature distraction devices that permitted maxillary, orbital, and mandibular distraction in a 4-month-old boy with unilateral craniofacial microsomia and anophthalmia. At 6 months of age, after maxillary repositioning and orbital expansion, a costochondral rib graft was used to construct the missing left mandibular ramus and condyle.
Plastic surgeons who treat congenital giant nevi should be aware of the neurocutaneous melanosis syndrome and its variants. When neurologic symptoms are present concurrent with MRI evidence of central nervous system involvement, treatment of the cutaneous lesion must be tempered by knowledge of a poor prognosis. In the otherwise asymptomatic patient, the plastic surgeon should consider screening with MRI imaging, realizing that the clinical significance of a positive MRI scan without neurologic symptoms is unknown.
Obstructive sleep apnea in children may result in hypoxia, right-sided heart failure, and sudden death. Children with craniofacial deformities and/or cerebral palsy are at high risk for the development of obstructive sleep apnea. Prompted by the excellent results obtained in adults when sleep apnea was managed by an aggressive surgical approach, we undertook a similar treatment philosophy in children. Twenty-eight patients representing four diagnostic groups were evaluated and operated on for severe upper airway obstruction: Down syndrome (n = 5), cerebral palsy (n = 12), Goldenhar syndrome (n = 4), and a mixed apnea group (n = 7). Tracheostomy was avoided in 25 of 28 patients (89 percent), with a marked decrease in apnea (median 90 percent) and hypopnea (median 87 percent) episodes. Tongue hyoid suspension and skeletal expansion procedures, which were the mainstay of treatment, were applied for the first time in children and adolescents with obstructive sleep apnea.
Sixty-seven consecutive patients over the age of 14 with either unilateral (n = 38) cleft lip and palate or bilateral (n = 29) cleft lip and palate seen over a 15-month period at the University of Michigan Craniofacial Program were reviewed to determine the total number of surgical procedures performed over the course of treatment. The demographics of the two groups differed: There were 25 males and 13 females who were a mean age of 17 years and 9 months with unilateral cleft lip and palate and 23 males and 6 females who were a mean age of 18 years and 5 months with bilateral cleft lip and palate. Lip and palate repairs were carried out on all patients. Lip adhesions were performed in 29 and 62 percent; pharyngoplasties (either pharyngeal flap or modified Ortichochea) in 39 and 38 percent; alveolar bone grafts in 82 and 79 percent; Abbé flaps in 0 and 10 percent; and orthognathic surgery was done in 10.5 and 13.8 percent and recommended and/or done in 26 and 24 percent of patients with unilateral cleft lip and palate and bilateral cleft lip and palate, respectively. Lip revisions averaged 1.13 and 2.17 per patient and secondary nasal surgeries averaged 1.13 and 1.18 per patient in the unilateral cleft lip and palate and bilateral cleft lip and palate, respectively. All totaled, the average number of operations was 6.12 per patient (range 3 to 12) in the unilateral cleft lip and palate and 8.04 per patient (range 5 to 15) in the bilateral cleft lip and palate.(ABSTRACT TRUNCATED AT 250 WORDS)
Infantile (desmoid-type) fibromatosis is an uncommon, locally aggressive tumor that is seldom reported in the dermatologic literature. We describe a case of infantile (desmoid-type) fibromatosis in a 2-year-old girl. Surgical disposition entailed amputation of the affected digit. Our report is intended to heighten awareness of this potentially fatal tumor, and emphasize the importance of timely diagnosis and treatment.
This is the first report on the McGill Quality of Life Questionnaire (MQOL), a questionnaire relevant to all phases of the disease trajectory for people with a life-threatening illness. This questionnaire differs from most others in three ways: the existential domain is measured; the physical domain is important but not predominant; positive contributions to quality of life are measured. This study was conducted in a palliative care setting. Principal components analysis suggests four subscales: physical symptoms, psychological symptoms, outlook on life, and meaningful existence. Construct validity of the subscales is demonstrated through the pattern of correlations with the items from the Spitzer Quality of Life Index. The importance of measuring the existential domain is highlighted by the finding that, of all the MQOL subscales and Spitzer items, only the meaningful existence subscale correlated significantly with a single item scale rating overall quality of life.