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Biomedical subjects

S R Cohen

Publications and source records attributed to S R Cohen.

At least 19 recordsLinked to original sources

Hypopigmented variant of mycosis fungoides: demography, histopathology, and treatment of seven cases.

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.

Adult

Decision making in primary surgical repair of myelomeningoceles.

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.

Decision Making

Successful use of leeches in the treatment of purpura fulminans.

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.

Animals

Primary temporal melanoma without diffuse leptomeningeal involvement: a variant of neurocutaneous melanosis.

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.

Humans

Surgical therapy for severe refractory sleep apnea in infants and children: application of the airway zone concept.

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.

Adolescent

Cumulative operative procedures in patients aged 14 years and older with unilateral or bilateral cleft lip and palate.

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)

Adolescent

Infantile (desmoid-type) fibromatosis.

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.

Adipose Tissue

The McGill Quality of Life Questionnaire: a measure of quality of life appropriate for people with advanced disease. A preliminary study of validity and acceptability.

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.

Adult

The association between pH level and corneal recovery from induced edema.

Corneal acidosis has been shown to reduce corneal hydration control (CHC) as measured by the rate, expressed as the percent recovery per hour (PRPH), at which the thickness of the cornea decreases exponentially after an increased hydration load. Since the effect of pH on corneal function is of scientific interest and may have clinical implications, we explored the relationship between pH and PRPH in greater detail by examining the effect of different stromal pH levels on corneal hydration control. Corneal edema was induced using a 90-min exposure to wearing a hypoxic contact lens (CL). Following removal of the CL, random assignment over four eye-test combinations of either 0, 3, 5, and 7% CO2 were made while pH and corneal thickness were monitored using slit lamp fluorophometry and optical pachometry to measure corneal pH and corneal thickness, respectively. From these measurements we determined the pH-dose/PRPH relationship. The average stromal pH +/- 1SD resulting from exposure to either the 0, 3, 5, and 7% CO2, was 7.65 +/- 0.11, 7.30 +/- 0.09, 7.15 +/- 0.08 and 7.04 +/- 0.07 (p < 0.001), respectively. Analysis based on a quadratic model of the dose-response relationship between PRPH and corneal pH indicates that PRPH is relatively unchanged for pH in the physiological range (pH = 7.40-7.65) and then decreases notably below the physiological range.

Acidosis

Managed care.

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Cleft Palate

Piriform aperture stenosis: a rare cause of neonatal airway obstruction.

Because neonates are obligate nasal breathers, neonatal nasal obstruction may have serious consequences. Prompt diagnosis and appropriate treatment are essential to avoid severe hypoxia. Anterior inlet, piriform aperture stenosis is an extremely rare cause of neonatal nasal airway obstruction and can easily be confused with choanal atresia or stenosis. Computed tomography with direct coronal scans is the best means of establishing a definitive diagnosis. We present our experience with four neonates having nearly complete piriform aperture stenosis. Prompt surgical enlargement of the nasal inlet through an upper buccal sulcus approach is recommended.

Constriction, Pathologic

Phototherapy in the treatment of depression in the terminally ill.

Research in affective disorders has shown that there is a clear link between mood and light exposure, and that exposure to bright wide-spectrum light (phototherapy) may be an effective antidepressant treatment in some clinical situations. Cancer patients, especially those in the terminal phase of illness, have a high incidence of depression. Furthermore, their mobility is often severely reduced, resulting in little exposure to direct sunlight. We report the use of phototherapy in three terminally ill patients to alleviate symptoms of depression.

Aged

Surgical correction of severe scaphocephalic deformities.

Sagittal synostosis may result in severe skull deformities. Characteristic components of the deformity include extreme elongation, frontal and occipital bossing, temporal pinching, and angulatory apical skull deformation. Conventional strip craniectomy often fails to correct these complex problems completely in severe early or late cases of sagittal synostosis. Techniques for total calvarial vault reconstruction have previously been reported, but a single large series has not been presented. Eighteen consecutive patients ranging in age from 3 months to 5 years (mean = 12 months) with severe early and late scaphocephalic skull deformities underwent total calvarial vault reshaping. All children required transfusions ranging from 250 to 1,100 mL. Operative times averaged 6 hours, and hospital stay ranged from 4 to 7 days. There was no perioperative mortality. Two patients experienced transient syndrome of inappropriate secretion of antidiuretic hormone, which responded to fluid restriction. One patient was noted to have a 2-cm parietal craniectomy defect 9 months after operation. Microscrews, which were used in all 18 patients, had to be removed in 2 patients when they became palpable. Excellent aesthetic results were noted in all 18 patients up to 36 months of follow-up.

Child, Preschool

Patterns of abnormal myogenesis in human cleft palates.

To test the hypothesis that soft palate muscles are abnormal in cleft palate, we compared soft palate morphogenesis in fetuses with cleft palate (n = 4) to age-matched (n = 3) and nonmatched (n = 1) control specimens. The morphologic status of all soft palate and masticatory structures were classified into one of six stages based on the level of histogenesis. At 54 mm crown-rump length (CRL), the levator veli palatini (L), palatopharyngeus (PP), and palatoglossus (PG) in cleft subjects demonstrated mesenchymal condensation into myoblastic fields, lagging behind the control specimens (97 mm CRL), which displayed definitive fields of myoblasts and myotube formation. In the 175 mm and 225 mm cleft and the 170 mm and 192 mm control specimens, muscular morphology was similar and had reached its postnatal appearance for the tensor veli palatini (175 m only) and L, PP, PG (225 mm only). Muscle fiber directions were, however, disoriented and disorganized, especially close to the medial epithelial edge of the cleft. The levator veli palatini, could not be distinguished as a discrete muscle in the cleft specimens, and what we believed to be the PP and PG seemed "normal" at the level of light microscopy, but malpositioned in a superior direction. This preliminary study demonstrates for the first time that early myogenesis in cleft palates differs from normal.

Case-Control Studies

Metopic synostosis: evaluation of aesthetic results.

Analysis of intermediate- and long-term results of surgical treatment of metopic synostosis is lacking. We therefore retrospectively studied 23 patients with metopic synostosis (14 males, 9 females) who have been followed from 3 months to 8.1 years (mean 42.5 months) after operation. Age at first operation ranged from 2 to 56 months (mean 8.2 months), with 15 patients operated on before 6 months and 8 after 7 months. Fronto-orbital remodeling and calvarial vault reshaping with floating forehead techniques were carried out in all patients. Stabilization of bony segments was accomplished with microplates and screws in 7 patients (30 percent), wires in 15 (65 percent), and absorbable sutures in 1. Complications included minor wound dehiscence (n = 1), seizures (n = 1), and increased intracranial pressure (n = 1). Postoperative photographic documentation of surgical results was available in 17 of the 23 patients. Aesthetic outcome in these 17 patients was graded (I = none or minor contour irregularities; II = moderate; and III = severe) by one of the authors (Cohen) and by a lay panel (n = 3) according to the degree of residual cranio-orbital deformity. Judged by the surgeon, grade I results were present in 53 percent, grade II in 35 percent, and grade III in 12 percent. To date, total reoperation (reoperative fronto-orbital remodeling and calvarial vault reshaping) was necessary in 2 patients (9 percent), one of whom had signs of increased intracranial pressure 3 years after the original craniofacial procedure, while partial reoperation (temporal cranioplasty) was carried out (n = 2) or recommended (n = 3) in another 5 patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Child