Platelets and whole blood coagulation.

In our early work in developing activated clotting time (ACT) assays, it became apparent that changes occurred in coagulation times as a whole blood sample aged (0-6 h). Subsequent studies showed that the coagulation parameters of plasma obtained from the samples remained stable during this time frame. These changes in whole blood clotting times during storage were eventually traced to the platelets. Several years of work demonstrated that this change was due to the removal of the blood from the vascular lining. This recalled a mechanism that was originally put forth in the 1970s with the discovery of prostacyclin. In this postulated mechanism, platelets are 'time-bombs'. They are kept under control by prostacyclin (PGI2) secreted by the vascular lining. Without this prostacyclin, platelets 'preactivate'. Since that time, additional substances secreted by the vascular endothelium have been identified, such as nitric oxide, that also influence platelet activity. The 'preactivation' of platelets in a blood sample can be followed using an ACT. In the same donor, the preactivation is uniform and reproducible over an extended period (months). There is, however, considerable variability between donors. Some donors' platelets preactivate dramatically, while other donors show hardly any change. Prostacyclin, added to the blood sample when it is collected, prevents this preactivation. The clinical significance of these observations has yet to be clearly established, but these observations raise a number of questions with respect to methods for improving platelet function during bypass and in evaluating the risk of platelet-mediated cardiovascular disease.

Otolaryngologic features of Laurence-Moon-Bardet-Biedl syndrome.

The most common otolaryngologic features associated with LMBBS include SNHL, speech and language disorders, and oral and dental abnormalities. Early otolaryngologic, audiologic, speech pathology, and dental evaluation of these individuals is recommended. This is the first reported case of bifid epiglottis, a rare congenital laryngeal anomaly, found in association with LMBBS. Most patients with bifid epiglottis have additional congenital anomalies, most commonly polysyndactyly. Polysyndactyly is a feature of both LMBBS and bifid epiglottis and may be an early hallmark for the presence of other congenital anomalies.

Otolaryngology manifestation of postpolio syndrome.

Twenty-one patients with postpolio syndrome were surveyed to determine otolaryngologic symptoms. An alteration in voice, dysphagia, and fatigue were the most common symptoms reported. Prevailing etiologic theories are presented, and treatment recommendations are offered.

Eagle's syndrome: a reappraisal.

W.W. Eagle in 1937 described two patients with elongated styloid processes, cervico-facial pain and a history of pharyngeal trauma. Selected case reports are used to illustrate the spectrum of Eagle's Syndrome. An analysis of the prevailing theories of etiology and causation is undertaken correlating anatomy, embryology, and physiology to derive a clearer understanding of Eagle's Syndrome.

Airway manifestations of chondrodysplasia punctata.

Chondrodysplasia punctata is a heterogeneous skeletal dysplasia characterized by small focal calcifications in articular and other cartilages in infancy, referred to as stippled epiphyses, with subsequent epiphysial dysplasia and associated anomalies of the face, eyes and skin. Nasal hypoplasia is commonly seen but secondary respiratory distress is infrequently described. We present two siblings with different degrees of involvement and a review of the different forms of this disorder. When an infant presents with a small nasal airway, the diagnosis of chondrodysplasia punctata should be considered and appropriate evaluations obtained.

Heparinase in the activated clotting time assay: monitoring heparin-independent alterations in coagulation function.

The activated clotting time (ACT) is routinely used to monitor heparin during cardiopulmonary bypass surgery. Activated clotting times may be influenced by a number of factors other than heparin. The presence of heparin in blood samples disguises the occurrence of non-heparin-related changes in coagulation function. During cardiopulmonary bypass, it is difficult to ascertain baseline clotting time fluctuations with ACT alone. Previous attempts to establish accurate baseline data were imprecise and involved extensive sample handling. In this study, we present data obtained using a modified (ACT) assay that incorporates heparinase. The heparinase test cartridge (HTC) instantaneously, specifically, and completely removes heparin in the blood sample at the initiation of the test. In conjunction with standard ACT techniques, the clinician is provided with heparin-independent (baseline) and functional clotting data. The HTC/ACT assay was used in a case study involving 19 patients undergoing cardiopulmonary bypass surgery. The data gathered indicate the usefulness of this assay in monitoring incidents of baseline drift, hemodilution, and hypercoagulation and the efficacy of protamine reversal.

Tonsillar abscess.

This article presents seven cases of patients with tonsillar abscess formation and discusses the pathophysiology of intratonsillar abscess formation.

Heparin monitoring in the coronary care unit after percutaneous transluminal coronary angioplasty.

The rate of acute restenosis in patients after percutaneous transluminal coronary angioplasty (PTCA) is related to thrombotic complications triggered by the PTCA. This risk is reduced by anticoagulating the patients with heparin after the procedure. The anticoagulation state of patients receiving heparin therapy is routinely monitored with the activated partial thromboplastin time (APTT) test. In an effort to provide more timely results regarding the status of patients who are receiving heparin after PTCA, a study was conducted to see whether low-range activated clotting time measurement (LR ACT) performed at the bedside could provide information comparable to that from APTT values determined in the laboratory. The study showed that the LR ACT values were comparable to laboratory-generated APTT values (R2 = 0.68). The LR ACT data generated were superior to the APTT data in terms of timeliness and the wider range of heparin levels covered. Having these values available allowed the CCU staff to react rapidly to changes in the patient's coagulation status.

Vocal rehabilitation of tracheoesophageal speech failures.

Twenty-three tracheoesophageal speech failures were prospectively evaluated by clinical parameters and transnasal air insufflation at 3 L per minute. The results of testing allow an accurate indication of the etiology of the speech failure. Pharyngoesophageal spasm accounted for 79% of the failures; hypopharyngeal strictures for 26%. One patient was found to have both pharyngoesophageal spasm and a hypopharyngeal stricture. A modified air insufflation test result greater than 20 mm Hg reliably identified all tracheoesophageal speech failures prior to tracheoesophageal puncture. Clinical parameters were not helpful in identifying speech failures. Successful treatment of the specific etiology of the failure resulted in a reduction of the measured intraesophageal pressures. Ninety-one percent of the tracheoesophageal speech failures were successfully rehabilitated and achieved fluent tracheoesophageal speech. Successful rehabilitation was associated with long-term tracheoesophageal speech use.

Surgical treatment of pharyngoesophageal spasm.

The clinical course of 17 laryngectomees with pharyngoesophageal spasm who underwent pharyngeal myotomy was studied to determine clinical response and complications. The influence of speech therapy on the development of volitional control of pharyngoesophageal spasm, tracheoesophageal speech fluency, and the efficacy and complications of surgical treatment for pharyngoesophageal spasm were assessed. Volitional control of tracheoesophageal speech was never achieved. Ninety-four percent of the patients (16/17) were successfully rehabilitated following surgical therapy. The complications following pharyngeal myotomy were acceptable.

Supraglottitis in young adults.

Supraglottitis is a disorder with the potential for significant morbidity and mortality in adults. Of the 25 patients we reviewed, 18 were black, representing the largest series of black adults with this condition reported in the medical literature. The mean age for black men was 14 years younger than previously described adults with supraglottitis. The index of suspicion for supraglottitis should be high in young adults with odynophagia out of proportion to findings on oropharyngeal examination. Indirect laryngoscopy will confirm the diagnosis and allow prompt initiation of treatment. Early recognition and treatment will avoid complications.

An objective method for prediction of tracheoesophageal speech production.

Current methods of esophageal air insufflation testing to predict postoperative tracheoesophageal speech success lack procedural objectivity and rely on subjective interpretation of the voice production results. A range of intraesophageal pressure measurements was obtained prior to tracheoesophageal (TE) puncture in each of 27 laryngectomized patients in an attempt to predict TE speech outcome. Postoperatively, three levels of speech production were identified. Fluent speakers, nonfluent speakers, and nonspeakers demonstrated low, intermediate, and high intraesophageal pressures, respectively. Patients with intermediate and high preoperative pressures did not achieve fluent speech without myotomy. This technique offers a reliable, objective preoperative indication of expected TE speech fluency.

Laryngeal paraganglioma.

Paraganglioma is an uncommon tumor of the larynx. The diagnosis and management of laryngeal paragangliomas remain problematic for the clinician. A recent experience with this uncommon tumor provides a forum for a discussion of laryngeal paraganglioma.

Carcinoid (neuroendocrine carcinoma) of the larynx.

Carcinoid of the larynx is a rare aggressive malignancy with few long-term survivors. Three cases of laryngeal carcinoid treated at the University of Michigan and 23 cases reported previously are reviewed. The clinical behavior, patterns of metastasis, and response to therapy were analyzed to determine optimal therapeutic evaluation and treatment strategies. Patient survival did not correlate with clinically evident regional adenopathy, length of symptoms, extent of laryngeal surgery, or retrospective staging according to American Joint Committee guidelines on cancer of the larynx (AJC-1980). Distant metastatic spread occurred frequently and was the most common cause of death. Surgical therapy is currently the only effective treatment for primary or recurrent disease. Future treatment regimens must address distant spread of tumor.

Aldred Scott Warthin. The man behind the tumor.

The personal milestones of Aldred Scott Warthin are detailed. One of the pathologic specimens that served as the basis for his original description of papillary cystadenoma lymphomatosum is reviewed.

Preoperative assessment of tracheoesophageal speech.

Air insufflation testing of tracheoesophageal (TE) puncture candidates as currently practiced lacks objectivity and relies on subjective evaluation of the sound produced. Intraesophageal pressure measurements were obtained from 21 consecutive laryngectomized patients during air insufflation by either experienced examiners or a compressed air source at 1 liter or 3 liters per minute. Postoperatively, three levels of TE speech fluency were identified. Preoperative intraesophageal pressure measurements were associated with a specific post-TE puncture speech fluency result. Intraesophageal pressure measurements may offer a reliable objective preoperative assessment of post-TE puncture speech fluency and identify patients who would benefit from pharyngeal myotomy at the time of TE puncture.

Small cell carcinoma of the larynx: results of therapy.

Primary small cell carcinoma of the larynx is a rare malignancy with a dismal prognosis. A survey of the long-term follow-up from reported cases of small cell carcinoma of the larynx and a review of the recent experience with this tumor at the University of Michigan Hospitals was undertaken to determine if newer treatment approaches incorporating adjuvant chemotherapy were associated with prolonged survival. Median survival for those patients receiving adjuvant chemotherapy was 19 months compared to 11 months for patients treated with surgery and/or radiation therapy alone. Among patients treated initially with primary radiation therapy and adjuvant chemotherapy median survival was 55 months, which was significantly longer than any other treatment regimen (P = 0.02). Systemic chemotherapy and therapeutic irradiation appears to offer the least disabling and most efficacious form of current therapy.

Papillary cystadenoma lymphomatosum syndrome.

"Papillary cystadenoma lymphomatosum syndrome" is an unusual clinical constellation that was first described in 1970 as infected adenolymphoma syndrome. A case of papillary cystadenoma lymphomatosum syndrome is presented demonstrating the specific histopathology of the lesion as well as the typical clinical history of all reported papillary cystadenoma lymphomatosum complicated by acute onset of pain and sudden increase in size. Five other cases noted in the literature are compared to complete the clinical and histopathologic review of the entity.