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Biomedical subjects

T J Sullivan

Publications and source records attributed to T J Sullivan.

At least 19 recordsLinked to original sources

Iris sector heterochromia as a marker for neural crest disease.

A 6-month-old female infant with biopsy-proved Hirschsprung's disease had associated sector heterochromia of the irides. The association between sector heterochromia and Hirschsprung's disease has been previously reported and both conditions have been ascribed to neural crest defects. Histologic characteristics of the ocular involvement have not previously been reported, to our knowledge. Histopathologic examination of the globes revealed decreased iris stroma, decreased pigmentation in the anterior stroma, and reduced numbers of pigment-producing cells in the affected areas. Both the ocular and gastrointestinal findings reflect abnormalities in tissues of neural crest origin.

Female

Comparative investigation of the influence of nizatidine, ranitidine, and cimetidine on the steady-state pharmacokinetics of theophylline in COPD patients.

The influence of usual regimens of the H2 blocking drugs, cimetidine, ranitidine, and nizatidine on the steady-state plasma concentrations and pharmacokinetic characteristics of theophylline was studied in seventeen patients with chronic obstructive pulmonary disease (COPD). Patients were dosed to steady-state with an oral, sustained-release formulation of theophylline given in therapeutic doses twice daily for 2 weeks. Over the next 4 weeks, each patient received a week-long regimen of each H2 blocker concomitantly with theophylline, and a week-long regimen of theophylline alone (control). At the end of each of the latter 4 weeks the steady-state pharmacokinetics of theophylline were assessed. Neither ranitidine nor nizatidine treatment altered the steady-state pharmacokinetics of theophylline relative to the control phase (i.e. no H2 blocker treatment). Values for theophylline C(ave), Cssmax, AUC0-12, and CLoral were significantly different during cimetidine treatment compared with all other treatments (ranitidine, nizatidine, and control). Cimetidine increased theophylline Cssmax, AUC0-12 and Cave by approximately 32%, and decreased theophylline oral clearance by approximately 23%. The authors conclude that cimetidine alters the steady-state pharmacokinetics of theophylline in COPD patients, whereas ranitidine and nizatidine are without effect.

Administration, Oral

Is asthma curable?

The information reviewed here supports the concept that asthma is potentially curable. Reports of complete, durable remission of asthma can no longer be regarded as fortuitous occurrences, unrepresentative of asthma in general. Systematic studies of anti-inflammatory drug therapy designed to explore possible induction or remission of asthma clearly are warranted. Studies of aggressive anti-inflammatory drug therapy of asthma at the onset, to avoid establishment of chronic asthma, also are desirable. The current goals of therapy of asthma have been revised to include reduction of airway hyperreactivity with topical anti-inflammatory drugs, in addition to relief of current symptoms. This approach may provide valuable resistance to exacerbations in response to antigen exposures, infections, exercise, or irritants. Pathophysiologic mechanisms apparently essential to the establishment and perpetuation of chronic asthma have been identified. These processes may be vulnerable to eradication by combination therapy with existing pharmacologic agents such as cyclosporin A or FK-506 (to suppress cytokine production), gold, methotrexate, and other anti-inflammatory drugs, alone or in combination. Equally important, the vigorous anti-inflammatory therapy may be necessary only long enough to achieve a resolution of the chronic pulmonary inflammation. Systematic studies of the use of these agents to induce partial, or complete, stable remissions of asthma should be performed. In the past, remissions of asthma in children with neoplasia and the other patients presented herein were complete, durable, and welcome, but they were largely unexpected and unpredictable. For the future, there is increasing reason to believe that predictable pharmacologically induced remission of asthma will be feasible.

Adult

A functional scoring method for the field of binocular single vision.

The authors present a new method for plotting and quantitating the field of binocular single vision (BSV). They modified a Goldmann perimeter to project a fusion target in the form of a cross (+). Unlike previous methods that used a spot, which is a poor fusion target that cannot detect cyclotorsion, the authors' method permits a single tester to plot BSV fields in patients who have any combination of horizontal, vertical, and cyclotorsional diplopia. The BSV fields were plotted in 23 patients with acquired diplopia using both the fusion target and the spot. Scoring was performed using a new scoring template weighted to the functionally important areas around primary and reading positions. Nine patients (39%) had scores for the two targets that differed by more than 10%. The authors' method yields BSV scores that are reproducible and easy to perform and that can be used for preoperative and postoperative assessments, serial monitoring of changes over time, and disability evaluations.

Adult

Eyelid and fornix reconstruction in bilateral abortive cryptophthalmos (Fraser syndrome).

Cryptophthalmos refers to a group of uncommon congenital anomalies of eyelid formation that can occur alone or in combination with multiple congenital anomalies as part of the Fraser syndrome. We present a case of bilateral abortive cryptophthalmos in a child with Fraser syndrome and discuss the problems of surgical management. A brief discussion of isolated and syndromic cryptophthalmos, including normal eyelid development, the pathogenesis of cryptophthalmos, and the management options follows.

Abnormalities, Multiple

Management of congenital dacryocystocoele.

Congenital dacryocystocoele occurs when the nasolacrimal drainage apparatus in the newborn has concomitant blocks at the level of the junction of the common canaliculus with the lacrimal sac and at the distal end of the nasolacrimal duct. This results in a typical pink or blue swelling in the region of the medial canthus. Spontaneous resolution is common, although dacryocystitis may supervene. Treatment should be conservative unless dacryocystitis occurs, or intranasal extension coexists. A series of seven consecutive cases is presented and a management plan for the neonate with congenital dacryocystocoele is proposed.

Cysts

The surgical management of congenital lacrimal fistulae.

Congenital lacrimal fistulae are developmental anomalies of the lacrimal apparatus that are usually symptomatic, frequently causing epiphora and occasionally causing fistulitis or dacryocystitis. They may be associated with other abnormalities of the lacrimal system or with systemic anomalies. Complete excision alone, or in combination with nasolacrimal intubation and/or dacryocystorhinostomy is recommended for treatment.

Dacryocystorhinostomy

Haemangiopericytoma of the orbit.

Orbital haemangiopericytomas are ideally managed by complete surgical excision in the first instance. This is frequently not achieved, because difficulty in making the diagnosis preoperatively results in incisional biopsy and the highly vascular nature of the tumour makes complete excision difficult. A series of 12 patients with orbital haemangiopericytoma seen over a 23-year period is presented. The following combination of clinical and radiological features is suggestive of haemangiopericytoma. 1. Painless non-axial proptosis with downward displacement of the globe. 2. Intermittent upper lid swelling. 3. A soft, superiorly located mass with poorly defined borders, especially with a blue hue. 4. A superiorly located, rounded or elongated extraconal mass on CT, isodense with brain, with smooth, well-defined borders and moderate to marked enhancement with the injection of intravenous contrast medium. 5. Significant blush in all three phases of carotid angiography, without prominent arteriovenous shunting. Once haemangiopericytoma is suspected, complete surgical excision is recommended.

Adult

Familial congenital corneal anaesthesia.

Congenital corneal anaesthesia is a cause of severe corneal ulceration and scarring in childhood. Although uncommon, it may be underdiagnosed when present as an isolated entity. Measures such as the use of elbow splints and tarsorrhaphy may be necessary to prevent visual loss. In rare instances, the condition may be inherited. A family is presented with autosomal dominant isolated congenital corneal anaesthesia, and the systemic associations and treatment of the condition are reviewed.

Adult

Actinomycosis of the orbit.

Actinomycosis is a very rare cause of orbital abscess usually attributable to direct spread from adjacent structures. A case of actinomycosis of the orbit is presented, which was treated as orbital pseudotumour for 3 months before progression of signs and symptoms, despite high dose steroids, led to the diagnosis being reconsidered.

Actinomycosis

Endogenous cortisol regulates immunoglobulin E-dependent late phase reactions.

To investigate the impact that physiological variation in serum cortisol has on IgE-mediated events, 10 atopic subjects underwent cutaneous antigen challenge with measurement of the early phase wheal (EPW) at 20 min and the late phase reaction (LPR) at 6 h. All subjects were challenged during control conditions between 8:00 and 9:00 a.m. Repeat challenges were performed in five subjects at 6:00 p.m. and in eight subjects after ingestion of metyrapone, a specific inhibitor of cortisol synthesis. Compared with control values, mean serum cortisol was suppressed in the evening and after metyrapone (P less than 0.05 all time points). No effect was seen on the EPW, but mean LPR diameters at three antigen dilutions were significantly increased by cortisol suppression (P less than 0.05). Replacement doses of hydrocortisone given in the evening and with metyrapone abrogated these increases. Blinded analysis of LPR biopsies from cortisol-suppressed subjects revealed increases in leukocytoclasis (P less than or equal to 0.0001), interstitial leukocytes (P less than or equal to 0.01), and eosinophils (P less than or equal to 0.04). These results indicate that physiological levels of serum cortisol can regulate IgE-dependent cutaneous inflammation by affecting the expression of cellular events at late phase sites.

Circadian Rhythm

Detection of human IgE to sulfamethoxazole by skin testing with sulfamethoxazoyl-poly-L-tyrosine.

Adverse reactions to sulfamethoxazole (SMX) occur in 4% to 6% of normal individuals. Many of these reactions resemble immunopathologic reactions, but skin test or in vitro evidence of a role for IgE is limited. Earlier RAST studies in our laboratory provided evidence that the N4-SMX hapten was a major determinant in immediate hypersensitivity reactions to SMX. We tested the hypothesis that IgE to this hapten is present on the mast cells of patients who have experienced immediate hypersensitivity reactions temporally related to exposure to SMX. A multivalent skin test reagent, SMX168-poly-L-tyrosine, and a univalent hapten, SMX-tyrosine, were synthesized. Forty-four patients with histories of allergic reactions to SMX and six subjects who had been exposed to the drug, but who had not reacted, were skin tested. Twenty-seven percent of the history-positive patients were skin test positive. None of the control individuals was positive. The immunologic responses to SMX in three patients who had experienced allergic reactions during SMX/trimethoprim therapy were analyzed in serial skin test and RAST assessments. One to three years after the clinical reactions, IgE to SMX could be demonstrated by skin testing in all three patients with a SMX-poly-L-tyrosine skin test reagent. Skin test reactions were inhibited by the monovalent reagent, SMX-tyrosine, in a dose-dependent manner. SMX-specific IgE antibodies could also be detected by RAST in serum obtained within days of the reactions from two of the three individuals.(ABSTRACT TRUNCATED AT 250 WORDS)

Acquired Immunodeficiency Syndrome

Medical canthal resection: an effective long-term cure for medial ectropion.

The results of 37 medial canthal resection procedures performed for the correction of severe paralytic or involutional medial ectropion are presented with an average follow-up of 5.4 years. Epiphora was improved in 33 out of the 37 cases and all but one patient had an anatomically improved lid-globe apposition, medial canthal angle, and posterofixation of the medial canthus. These results confirmed the long-term value of the operation.

Aged

Building a professional environment in long-term care. The role of clinical career development.

1. To be successful, an organizational career development program must include a differentiation of the responsibilities for which the various parties (employer, employee, career counselor) will be held accountable. 2. Project outcomes revealed that the career mobility program was attractive to nursing personnel and facility management personnel alike. However, it was more attractive to nonlicensed than licensed personnel. 3. Of the staff who participated and were promoted, the majority remained in their jobs. The program was most successful in enhancing retention with personnel who received within-level promotions. 4. The process of career development requires collaboration and support from all levels of leadership and staff throughout the organization. A career development program, including a career mobility program with a strong career counseling component, can serve as a catalyst in professionalizing the long-term care work environment.

Career Mobility