Search PubMed⌕ Search

Biomedical subjects

J M Pensler

Publications and source records attributed to J M Pensler.

At least 37 records · Page 2Linked to original sources
PubMed1994

Disseminated Rhizopus infection in a premature infant.

A 16-day-old premature male infant developed rapidly expanding necrotic facial plaques. Histology and cultures of tissue biopsy specimens revealed infection with Rhizopus arrhizius. The patient was treated aggressively with surgical debridement, amphotericin B, and rifampin, but had a progressive spread of the cutaneous infection and a fatal clinical course. He had gastrointestinal and pulmonary involvement on autopsy. Zygomycoses are rapidly progressive infections which generally affect immunocompromised hosts. Premature infants should be included as individuals at risk for disseminated zygomycotic disease.

Anti-Bacterial Agents↗
PubMed1993

Metabolic changes in osteoclasts isolated from children with fibrous dysplasia.

Children with fibrous dysplasia have specific areas of local bony overgrowth. The in vitro metabolism of osteoclasts obtained from involved disease areas of two patients with idiopathic monostotic fibrous dysplasia and one patient with polyostotic fibrous dysplasia were compared with osteoclasts isolated from noninvolved bone in the same individuals and normal controls. The osteoclasts from the involved regions formed similar resorption lacunae compared with osteoclasts isolated from both normal and noninvolved bone. Calcium release into the media, a function of cell-mediated resorption, was decreased in involved osteoclasts compared with osteoclasts derived from normal or noninvolved bone (p < 0.05). Osteoclasts from regions of in vivo abnormal growth exhibited normal mineral metabolism in vitro.

Acid Phosphatase↗
PubMed1993

Necrotizing periorbital cellulitis.

We report traumatic necrotizing periorbital cellulitis attributed to group A beta-hemolytic streptococci in a 4-year-old child. The infection was successfully treated via surgical cleansing, drainage, and grafting. The virulence of this organism requires an aggressive approach to the patient with periorbital cellulitis, which is refractory to intravenous antibiotics. Early treatment may limit extensive eyelid necrosis, the resultant secondary deformity, and the need for multiple reconstructive procedures.

Cellulitis↗
PubMed1993

Surgical treatment of patients with lobar holoprosencephaly: a personal note.

The diagnosis of holoprosencephaly usually implies a poor prognosis and is often regarded as a contraindication for surgical correction of associated clefts of the lip or palate. Between 1985 and 1991, 4 patients with lobar holoprosencephaly were evaluated and studied with computed tomography or magnetic resonance imaging. All had cleft palate and 3 had paramedian cleft lip; all 4 exhibited some degree of nasal dysplasia. All patients are presently alive, at a mean age of 26.6 +/- 2.4 months (mean +/- SD). Three of the children showed normal or near normal development, whereas the fourth was severely retarded. Our experience suggests that some children with lobar holoprosencephaly have a highly variable degree of intellectual development and that long-term survival may be expected.

Agenesis of Corpus Callosum↗
PubMed1993

Correction of micrognathia with ankylosis of the temporomandibular joint in childhood.

Insult of temporomandibular joints in infancy may result in severe micrognathia with ankylosis. These patients require tracheostomy and a pureed diet for survival. Between July of 1987 and December of 1990, we treated eight patients with severe micrognathia and ankylosis of one or both temporomandibular joints. Preoperative range of motion at the central incisors was 3.2 +/- 2.1 mm (mean +/- SD). Patients underwent functional reconstruction of one (n = 3) or both (n = 5) temporomandibular joints using costochondral grafts and mandibular advancement at 10.4 +/- 1.7 years of age (mean +/- SD). Rigid fixation of bone grafts with early remobilization was achieved in all cases. Average mandibular advancement was 23.8 +/- 3.7 mm (mean +/- SD), and average elongation was 21.4 +/- 3.3 mm (mean +/- SD). Range of motion at the central incisors was 27.4 +/- 4.2 mm (mean +/- SD) 1.7 +/- 0.8 years (mean +/- SD) postoperatively. Seven patients had tracheostomies prior to surgery, and all were successfully decannulated within 6 months postoperatively. One patient was treated with closed capsulotomy under general anesthesia. Two were treated with open capsulotomy and fascial flap transposition. Functional reconstruction of this deformity in mixed dentition offers these unfortunate individuals significant predictable improvement.

Ankylosis↗
PubMed1993

The anatomy of Cupid's bow in normal and cleft lip.

This is a gross and microscopic examination of the cutaneous-vermilion-mucosal region (Cupid's bow) in infants with normal lip as compared to cleft lip. Morphologic observations of lips in situ were made with magnification. Specimens for histology were from the normal upper lip of infants (n = 3) and adults (n = 4), and from redundant tissue made available during primary repair of cleft lip (n = 13) and cleft lip revision (n = 4). Tissues were analyzed with hematoxylin and eosin, Gomori trichrome, and Fontana-Masson stains. Immunoperoxidase stains were used to emphasize vascular endothelium and epithelial cytokeratins. Our observations support the hypothesis that the anterior projection of the pars marginalis of the musculus orbicularis oris gives rise to the normal cutaneous-vermilion junction ("white skin roll" of Gillies-Millard). The band of vellus hairs, found atop the vermilion-cutaneous junction, also highlights the rim of Cupid's bow. There was no abrupt alteration in vasculature, melanin, or cytokeratin content in the white roll. Proceeding caudally, the vermilion-mucosal region was characterized by progressively increasing epidermal thickness and size of rete ridges, decreasing melanin, more superficial capillaries, and an abrupt transition from keratinized to nonkeratinized squamous epithelium ("red line" of Noordhoff). In the cleft lip specimens, where white roll was absent, there was hypoplasia and disorientation of the underlying pars marginalis component of m. orbicularis oris. Vellus hair follicles were seen in the zone of presumptive white roll in clefts, just as in normal lip. Frequently, these pilar units contained disproportionately large sebaceous glands. In some cleft lip specimens, ectopic sebaceous glands were noted in the oral mucosa. We also observed a deficiency of vermilion width on the medial side of unilateral cleft lip, whereas the width of the vermilion at the crest of the bow was normal or slightly increased in the lateral cleft segment. A narrow vermilion band and exposed hypoplastic mucosa were characteristic of the prolabium in the bilateral complete cleft deformity. The findings of this study are pertinent to technical details for construction of the Cupid's bow and vermilion in unilateral and bilateral cleft lip.

Cleft Lip↗
PubMed1991

A comparison of speech results after the pharyngeal flap and the dynamic sphincteroplasty procedures.

Eighty-five patients underwent surgery to reduce velopharyngeal incompetence with either a pharyngeal flap (n = 75) or a dynamic sphincteroplasty (n = 10) performed between April 1958 and August 1989, and were evaluated preoperatively and postoperatively by a plastic surgeon, speech pathologist, and otolaryngologist. Improvement in speech was noted in 75% (n = 56) of the patients with pharyngeal flaps and 70% (n = 7) of the patients with dynamic sphincteroplasties postoperatively. Thirty percent of the patients in both groups showed no improvement postoperatively in speech. Three patients (4%) who underwent pharyngeal flap procedures developed sleep apnea postoperatively. Persistent velopharyngeal incompetence may be treated effectively with either a pharyngeal flap or a dynamic sphincteroplasty. Either procedure appears to result in improved speech in most patients.

Adolescent↗
PubMed1991

Nasal midline masses in infants and children. Dermoids, encephaloceles, and gliomas.

Nasal dermoids, gliomas, and encephaloceles are uncommon congenital lesions that result from aberrant embryologic development. We have treated 46 children with these nasal lesions. In view of the potential intracranial connection, patients are at risk for intracranial infection, and early surgical correction is thus imperative. Neuroimaging studies may help to predict intracranial involvement.

Adolescent↗
PubMed1990

Sex steroid hormone receptors in normal and dysplastic bone disorders in children.

Children with monostotic and polyostotic bone dysplasias often exhibit localized bone overgrowth. We investigated the presence of nuclear estrogen and nuclear progesterone receptors by solid-phase radioimmunoassay, immunocytochemistry, and radioligand binding in osteoblast cell cultures derived from the areas of overgrowth of membranous bone, noninvolved membranous bone, and normal membranous bone from children undergoing elective craniotomy. Membranous bone of normal children had demonstrable levels of nuclear estrogen and progesterone receptors identified by radioimmunoassay and immunocytochemical assay. Two- to threefold increased levels of these receptors (p less than 0.001 versus normals) were found in cultures derived from the involved bone of two children with monostotic fibrous dysplasia and in one patient with polyostotic dysplasia (McCune-Albright syndrome). The noninvolved bone in our patients with fibrous dysplasia exhibited nuclear sex steroid hormone receptor levels similar to those in the normal children. Radioligand binding studies demonstrated increased sex steroid hormone receptors in cultures derived from involved osteoblasts. The presence of an increased level of sex steroid hormone receptor was accompanied by increased alkaline phosphatase activity and increased production of osteocalcin in vitro compared to normal or noninvolved bone. The mechanisms by which sex steroid hormone receptor levels are increased in the ostotic dysplasias remain to be established.

Alkaline Phosphatase↗
PubMed1990

Osteoclasts isolated from membranous bone in children exhibit nuclear estrogen and progesterone receptors.

Osteoclasts were isolated from membranous bone from four children without metabolic bone disease who were undergoing craniotomy for either tumor or trauma. Both freshly isolated osteoclasts and those cultured for 4-7 days exhibited the following characteristics: production of tartrate-resistant acid phosphatase (9.5-14.8 units), contraction in response to application of 100 mg/ml of human calcitonin, and formation of resorption lacunae on devitalized bone wafers. Nuclear estrogen and progesterone receptors were demonstrated by immunohistochemical techniques and quantitated in two of the patients by radioimmunoassay (estrogen receptor RIA, 23.6 and 23.8 cpm/micrograms protein; progesterone receptor RIA, 36.7 and 74.2 cpm/micrograms protein). The demonstration of sex steroid hormone receptors in the nucleus of osteoclasts derived from children with normal membranous bone has established a potential mechanism whereby direct modulation of bone resorption by the sex steroid estrogen and progesterone may occur.

Acid Phosphatase↗
PubMed1990

The efficacy of tongue resection in treatment of symptomatic macroglossia in the child.

Nine patients with symptomatic macroglossia, 3 with Beckwith-Wiedemann syndrome, 3 with Down's syndrome, and 3 with lymphatic malformation of the tongue, were evaluated for postoperative improvement following partial glossectomy. Symptoms evaluated were speech intelligibility, oral competence at rest, drooling, and normal deglutition. All patients with Down's syndrome and Beckwith-Wiedemann syndrome were improved postoperatively. All patients with Down's syndrome exhibited improved deglutition and reduced drooling. Only 1 patient (33%), however, achieved oral competence. Speech was improved postoperatively in 2 patients (67%) with Down's syndrome. Patients with lymphatic malformation of the tongue did not exhibit consistent long-term improvement postoperatively, suggesting that conservative management of macroglossia in this patient group may be indicated.

Adolescent↗
PubMed1990

Anterior nasal stenosis secondary to accessory nasal bones.

Choanal atresia may result from a posterior or anterior obstruction of the airway. Treatment is predicated on an accurate identification of the underlying pathological condition by means of physical examination, fluoroscopy, and computed tomographic scan. Expeditious surgical management after appropriate diagnosis appears to result in effective relief of the obstruction.

Choanal Atresia↗
PubMed1990

Maxillary deformation in unilateral coronal synostosis.

Distinct osseous structural differences exist between patients with unilateral coronal synostosis and those with "deformational plagiocephaly." Patients with unilateral coronal synostosis exhibit significant anterior displacement of the infraorbital rim relative to the ipsilateral frontal bone and contralateral infraorbital rim (p less than 0.005). Patients with deformational plagiocephaly are noted to have retrusion of the frontal bone and infraorbital rim on the involved side. The observed differences, documented utilizing computerized tomography, are easily appreciated on clinical examination, facilitating rapid distinction between these two entities.

Craniosynostoses↗
PubMed1990

Clinical and ultrastructural studies of Romberg's hemifacial atrophy.

Romberg's disease is an uncommon and poorly understood condition manifested by progressive hemifacial atrophy of skin, soft tissue, and bone. In order to better define the natural history and anatomic variation of this disorder, we evaluated 41 patients by history, physical examination, and facial radiographs. Light microscopic studies were performed on tissue from 19 patients, and ultrastructural analysis was performed on specimens from 6 patients. The average age at inception of the disease was 8.8 years. Atrophy, within one or more trigeminal nerve dermatomes, progressed at a variable rate (mean period of active tissue dissolution = 8.9 +/- 6 years). In 26 patients with skeletal involvement, the mean age of onset was 5.4 years, versus 15.4 years for 15 patients without skeletal involvement, a statistically significant difference (p less than 0.01). However, there was no correlation between the severity of soft-tissue deformity and the age of onset. Electron microscopy demonstrated lymphocytic infiltrates in neurovascular bundles and abnormalities of vascular endothelium and basement membranes. We hypothesize that the pathogenesis of Romberg's disease involves chronic cell-mediated vascular injury and incomplete endothelial regeneration along branches of the trigeminal nerve (lymphocytic neurovasculitis).

Adult↗
PubMed1989

Orbital roof fractures in childhood.

The authors documented 36 orbital roof fractures in 32 children over a 5-year period. With the aid of computed tomography (CT), 16 fractures were classified as isolated and 20 were found to be associated with more extensive damage to the skull. Isolated fractures typically occurred in younger children (mean age, 2.8 years) after relatively minor trauma, most often a fall from a height of less than 10 feet. Birth trauma was the cause in one case. Three fourths of the isolated fractures were linear; with extensive trauma, the orbital roof was usually comminuted. Upper eyelid hematoma characteristically developed hours after the traumatic event. There were no significant chronic disturbances of vision, motility, or lid function. Acute neurologic concerns were common, and two patients died, but full recovery was otherwise the rule. In two cases, late development of ocular pulsation led to CT demonstration of encephaloceles. Both of these children had comminuted roof fractures with displacement of bone fragments into the orbit. The authors conclude that fracture of the orbital roof is a common but frequently overlooked occurrence in childhood. Anatomic factors may account for differences in the effect of frontal impact at different ages.

Accidental Falls↗
PubMed1989

Monomorphic adenoma of the parotid in a premature neonate.

We report the case of a monomorphic adenoma of the parotid in a neonate. Although solid tumors of the parotid are rare in the pediatric population, these lesions may develop in utero and present at any time after birth. A solid mass in the parotid must be considered malignant until proved otherwise regardless of the age of the patient.

Adenoma↗
PubMed1989

Treatment of mandibular fractures in children.

Thirty-seven mandibular fractures were treated in 25 patients between January 1984 and January 1988. The patients' ages ranged from 4 months to 16 years (6.9 +/- 4.6 years [mean +/- standard deviation]). Nineteen of the patients were male and 6 were female, indicating a significant gender predilection (p less than 0.05). A treatment approach based on the patients' dental maturity and fracture location has evolved that stresses rapid restoration of osseous continuity and preoperative occlusion while striving to minimize risk to unerupted dentition.

Adolescent↗