Wiskott-Aldrich syndrome: histopathologic findings in the lymph nodes and spleens of 15 patients.

Seventeen lymph nodes and 13 spleens from 15 patients with the Wiskott-Aldrich syndrome were examined histologically. The material included both biopsy and autopsy specimens. Consistent findings included depletion of small lymphocytes from T cell areas (all cases), prominence of the reticulum cell stroma (all cases), the presence of atypical plasma cells with and without plasmacytosis (16 lymph nodes and 11 spleens), and extramedullary hematopoiesis (13 lymph nodes and 9 spleens). Less frequent features noted were tissue eosinophilia, hemophagocytosis, focal fibrosis, and progressive depletion of germinal centers. One case with a unique abundance of transformed lymphocytes is described.

Histologic study of the ocular lesions in multiple endocrine neoplasia syndrome type IIb.

We studied the clinical and histopathologic findings in the eyes of two patients with multiple endocrine neoplasia type IIb. A prominent clinical feature of this condition is enlarged nonmyelinated corneal nerves composed of bundles of axons associated with Schwann cells. Prominent corneal nerves within an otherwise normal corneal stroma are an important clinical feature of multiple endocrine neoplasia type IIb. Because of this, ophthalmologists are in a position to make an early diagnosis and hence to recommend diagnostic procedures and treatment for the associated potentially fatal medullary carcinoma of the thyroid and pheochromocytomas.

Noninvasive fluorescent identification of bacteria causing acute otitis media in a chinchilla model.

OBJECTIVES: To evaluate a noninvasive method of bacterial identification via fluorescence spectroscopy in the setting of acute otitis media in a chinchilla model. STUDY DESIGN: Prospective. METHODS: For each chinchilla, transbullar inoculation with Streptococcus pneumoniae, Haemophilus influenzae, or Staphylococcus aureus was performed bilaterally and clinical infection was determined by otoscopy. An optical fiber coupled to a spectrofluorometer allowed for the delivery of an excitation wavelength to the inflamed tympanic membrane and the acquisition of the resulting emission signal. Sequential emission spectra obtained over a range of excitation wavelengths were assembled by a computer algorithm, and a single, three-dimensional plot was created for each test ear. Similarly, plots from the healthy external auditory canal (EAC) were also recorded. Twelve animals were used to establish a library of four reference plots representing the three bacteria and the EAC. Of the 24 ears available for study, 10 were excluded from analysis because of lack of clinical infection or presence of tympanic membrane perforation with purulent drainage. From four additional animals, four samples from ears infected with the above bacteria and three samples of the EAC served as unknowns. The unknown plots were analyzed by an investigator blinded to their identity. RESULTS: Using a multiple correlation of the unknown to the reference plots, seven of seven samples were correctly identified. CONCLUSIONS: We were able to establish a prototype method for the noninvasive identification of a limited library of pathogens in a chinchilla model of acute otitis media.

The effect of absorbable stenting on postoperative stenosis of the surgically enlarged maxillary sinus ostia in a rabbit animal model.

OBJECTIVE: To determine the effect of absorbable stenting on postoperative stenosis of the surgically enlarged maxillary sinus in a rabbit model. DESIGN: A randomized controlled animal study with each specimen serving as its own control. Animals had their maxillary ostia surgically enlarged bilaterally. Video images were made of each enlarged ostia. Unilateral stenting was performed using rolled absorbable stenting (Gelfilm, Upjohn Co, Kalamazoo, Mich). The animals were killed at 3 months and repeated images were made of each ostia. An image processing system (IBAS-AT, Kontron Instruments, Milan, Italy) was used to individually calibrate each image and then measure areas. Surgical and 3-month areas were compared with the examiner blinded to the stented side. SUBJECTS: Twelve specific pathogen-free New Zealand white rabbits. RESULTS: Stented ostia had an increased stenosis compared with unstented ostia, but this increase was not statistically significant (P = .08, Student t test). There was no within-animal change in nonstented ostia compared with an 18% decrease in the stented ostia. CONCLUSIONS: Stenting did not statistically change the amount of stenosis of the surgically enlarged ostia in a rabbit model, although a trend toward more scarring when compared with the nonstented side was observed.

Quality-of-life assessment in patients with unilateral vocal cord paralysis.

OBJECTIVE: Our objective was to identify the impact of unilateral vocal cord paralysis (UVCP) on an individual's quality of life both before and after thyroplasty. STUDY DESIGN AND SETTING: This was a prospective observational outcome study of consecutive patients presenting to a laryngology clinic with UVCP. Participants received The Medical Outcomes Study Short Form 36-Item Health Survey (SF-36), the Voice Handicap Index (VHI), and the Voice Outcome Survey (VOS). Patients underwent medialization laryngoplasty with silastic, with or without arytenoid adduction. Outcome measures were repeated after surgery. RESULTS: A review of 45 patients at presentation revealed statistically significant reductions in quality of life as measured by each survey. Marked improvements were noted after surgery. CONCLUSIONS: Our preoperative data support a profile of significant general health-related and voice-related limitations caused by UVCP. Patient perceptions improved significantly after the surgical treatment of glottal insufficiency. SIGNIFICANCE: The SF-36, VHI, and VOS provide an important complement to traditional endpoints in the analysis of patients with UVCP.

Voice outcome following thyroplasty in patients with cancer-related vocal fold paralysis.

Medialization laryngoplasty restores voice in patients with unilateral laryngeal paralysis. Of question was whether patients with vocal fold paralysis resulting from cancer or its treatment had as good a post-operative voice result as patients with vocal fold paralysis of benign etiology. The purpose of the present study was to compare post-operative perceptual, acoustic, aerodynamic, and quality of life data in these two patient groups. Twenty-eight patients with vocal fold paralysis secondary to malignancy or its treatment were age and gender-matched with patients with paralysis resulting from benign origin. Pre- and post-operative perceptual judgments of pitch, loudness and quality were rated independently by two speech-language pathologists. A digital audiotape of the patient's voice was analyzed using Soundscope software. Fundamental frequency, conversational intensity and perturbation were evaluated. Glottal flow rates in propositional speech, phonation times and extent of pitch and loudness ranges were also measured. Three quality of life surveys, the Short Form-36 general health survey, the Voice Handicap Index, and the Voice Outcomes Study were administered. Results of voice testing indicated that perceptual, acoustic and aerodynamic data were significantly improved 3 months after thyroplasty in all patients regardless of whether they had a history of cancer. Quality of life data, however, distinguished the two groups. In particular, the general health measure found a significant difference in physical functioning and overall vitality, although satisfaction with improved voice was equally appreciated in both patient groups. Of clinical significance is that though general health may differ, patients with cancer-related laryngeal paralysis can expect to have as good a voice outcome following thyroplasty as patients with paralysis of benign etiology.

Why change programs don't produce change.

Faced with changing markets and tougher competition, more and more companies realize that to compete effectively they must transform how they function. But while senior managers understand the necessity of change, they often misunderstand what it takes to bring it about. They assume that corporate renewal is the product of company-wide change programs and that in order to transform employee behavior, they must alter a company's formal structure and systems. Both these assumptions are wrong, say these authors. Using examples drawn from their four-year study of organizational change at six large corporations, they argue that change programs are, in fact, the greatest obstacle to successful revitalization and that formal structures and systems are the last thing a company should change, not the first. The most successful change efforts begin at the periphery of a corporation, in a single plant or division. Such efforts are led by general managers, not the CEO or corporate staff people. And these general managers concentrate not on changing formal structures and systems but on creating ad hoc organizational arrangements to solve concrete business problems. This focuses energy for change on the work itself, not on abstractions such as "participation" or "culture." Once general managers understand the importance of this grass-roots approach to change, they don't have to wait for senior management to start a process of corporate renewal. The authors describe a six-step change process they call the "critical path."

Video integrated measurement system.

A Video Integrated Measurement (VIM) System is described which incorporates the use of various noninvasive diagnostic procedures (moire contourography, electromyography, posturometry, infrared thermography, etc.), used individually or in combination, for the evaluation of neuromusculoskeletal and other disorders and their management with biofeedback and other therapeutic procedures. The system provides for measuring individual diagnostic and therapeutic modes, or multiple modes by split screen superimposition, of real time (actual) images of the patient and idealized (ideal-normal) models on a video monitor, along with analog and digital data, graphics, color, and other transduced symbolic information. It is concluded that this system provides an innovative and efficient method by which the therapist and patient can interact in biofeedback training/learning processes and holds considerable promise for more effective measurement and treatment of a wide variety of physical and behavioral disorders.

Primary immunodeficiency and malignancy.

Evidence indicating an essential relationship between immunologic deviation and cancer has accumulated rapidly over the past 15 years. Following a brief review of the enormous body of literature linking experimental immunodeficiency and cancer, this discussion will center on humans with primary immunodeficiency disorders and the inordinate number of malignancies which develop in these patients.

Lymphoreticular disorders in primary immunodeficiencies: new findings based on an up-to-date histologic classification of 35 cases.

A histologic review was undertaken of 35 lymphoreticular disorders that developed in primary immuno-deficiency patients from the Immunodeficiency Cancer Registry. Twenty-one (60%) of the lesions were non-Hodgkin's lymphomas: these included eight B-immunoblastic sarcomas. Eight (23%) of the lesions were Hodgkin's disease, with a high frequency of lymphocytic depletion type in an unusually young age group. Three lesions (8.5%) represented abnormal proliferative processes, which could not be definitely categorized as either benign or malignant. There were only two acute lymphoblastic leukemias (6%). Differences were found between lymphomas arising in Wiskott-Aldrich syndrome and those occurring in ataxia-telangiectasia; this suggests that different pathogenetic mechanisms might operate in their development. The lymphomas in Wiskott-Aldrich syndrome were all of non-Hodgkin's type, predominantly B-immunoblastic sarcomas, and presented as localized extranodal infiltrates. The lymphomas in ataxia-telangiectasia were either Hodgkin's disease, mostly of lymphocytic depletion type, or non-Hodgkin's lymphomas of the histologic subtypes associated with 14q translocations.

The Wiskott-Aldrich syndrome in the United States and Canada (1892-1979).

Information was collected on 301 cases of the Wiskott-Aldrich syndrome in the United States and Canada Examination of available medical records, death certificates and published case reports on these patients showed that they came from a wide geographic area and many diverse ethnic and racial groups. No significant difference was found in the incidence of cases born between 1947 and 1976; the overall rate was 4.0 per million live male births in the United States. Median survival has increased with time from eight months for patients born before 1935 to 6.5 years for those born after 1964. Seventy-six of the 301 patients (25%) were still alive at last follow-up and ranged in age from 1 to 36 years with a median of 10 years. Causes of death were primarily limited to infections or bleeding, but malignancy represented a significant problem. Twelve percent of the group (36 of 301) developed malignancy, the predominant types being lymphorecticular tumors (23 of 36) and leukemia (7 of 36). The overall relative risk for malignancy was found to be greater than 100 times that of the general population and was found to increase with increasing age.