Familial adenomatous polyposis (Gardner's syndrome) and thyroid carcinoma

Summary The case history of a 24-year-old woman with Gardner's syndrome [familial adenomatous polyposis (FAP)] and papillary thyroid carcinoma is presented, representing the 37th report of this association. Although FAP is transmitted as an autosomal dominant trait with similar penetrance in both sexes, thyroid carcinoma has been found almost exclusively in women (94.3%). The majority have been papillary carcinomas (88.5%), which have become apparent during the third decade (average 23.6, range 16–40 years). Most (55.5%) thyroid carcinomas have been discovered 1–17 years after FAP was identified, although some have been found before (29.6%), or at the same time (14.8%) FAP was diagnosed. Multicentric papillary carcinomas have been reported in 64% (14 of 22) of FAP patients, a frequency at least twofold greater than usual. Although papillary carcinoma found before age 30 (as it was in most patients with FAP) typically has an excellent prognosis, one patient with FAP developed distant metastases from thyroid carcinoma and a 28-year-old woman's death was attributed to papillary carcinoma. The high frequency of multicentric papillary thyroid carcinoma in young patients with FAP and the potential for metastases and death due to thyroid carcinoma warrant aggressive diagnostic screening at regular intervals with neck palpation, ultrasonography, and if necessary, fineneedle aspiration biopsy. When thyroid carcinoma is found, total or near-total thyroidectomy should be considered because of the tumor's high likelihood of being multifocal. Since almost 30% of the thyroid carcinomas associated with FAP have been diagnosed 4–12 years before polyposis was identified, young patients presenting with thyroid carcinoma should be questioned regarding bowel function and a family history of gastrointestinal disease, and consideration should be given to periodic testing for fecal occult blood.The views expressed herein are those of the authors and do not necessarily reflect those of the Army or the Department of Defense.     

A nationwide population-based study of migraine in Brazil

The aim of this study was to estimate the 1-year prevalence of migraine and the degree of the association of migraine with some sociodemographic characteristics of a representative sample of the adult population of Brazil. This was a cross-sectional, population-based study. Telephone interviews were conducted on 3848 people, aged 18–79 years, randomly selected from the 27 States of Brazil. The estimated 1-year gender- and age-adjusted prevalence of migraine was 15.2%. Migraine was 2.2 times more prevalent in women, 1.5 times more in subjects with > 11 years of education, 1.59 times more in subjects with income of < 5 Brazilian Minimum Wages per month, and 1.43 times more in those who do not do any physical exercise. The overall prevalence of migraine in Brazil is 15.2%. Migraine is significantly more prevalent in women, subjects with higher education, with lower income, and those who do not exercise regularly, independently of their body mass index.     

THE EUGLYCAEMIC HYPERINSULINAEMIC CLAMP: AN EVALUATION OF CURRENT METHODOLOGY

1. The recognition of the role of insulin resistance in disease states and the recent development of new drugs that modify insulin-dependent metabolism has led to increased use of the euglycaemic hyperinsulinaemic clamp to measure in vivo insulin sensitivity, but several key aspects of the technique are poorly documented in the literature. 2. We have evaluated the reproducibility and intersubject variation of measurements of insulin sensitivity in groups of insulin-sensitive and insulin-resistant subjects and assessed the effects of hand warming on haemodynamic and metabolic responses. 3. Subjects participated in one of two protocols: (i) 18 healthy male volunteers and 18 patients with hypertension and glucose intolerance were clamped on two occasions, 1 week apart with measurements of insulin sensitivity (M) derived after 120 and 180 min of hyperinsulinaemia; and (ii) six healthy volunteers were clamped on one occasion with simultaneous sampling of antecubital and ‘arterialized’ (dorsal hand) venous blood for comparison of plasma glucose concentrations and oxygen saturation and a further six volunteers were clamped on two occasions with and without the use of hand warming. 4. Measurements of M derived after 120 min (M120) and 180 min (M180) of hyperinsulinaemia were reproducible: the coefficients of repeatability (mg/kg per min) of M120 and M180 were 1.0 and 0.9 for volunteers and 1.0 and 1.0 for the patient group, respectively. The intersubject variation in insulin stimulus was high: coefficients of variation for M180 were 22% for volunteers compared with 38% for the patient group. In volunteers compared with the patient group, hand warming significantly increased venous oxygen saturations (95 ± 2 vs 79 ± 18%, respectively) and glucose concentrations (5.2 ± 0.2 vs 4.5 ± 0.4 mmol/L, respectively) and measurements of M were significantly higher using arterialized compared with antecubital venous blood. However, local hand warming was associated with systemic vasodilatation: blood pressure decreased (e.g. 6mmHg diastolic; P < 0.05) with a compensatory increase in heart rate (8 b.p.m.). 5. In conclusion, clamps of 120 and 180 min duration yielded measurements of M that were reproducible. The technique is much more robust when used in the context of a crossover design because of the significant (20–40%) intersubject variation in M, even among apparently homogeneous male volunteers. Hand warming effectively arterializes venous blood and gives significantly higher M values, but induces systemic vasodilatation, which may confound measurements of M.     

Altered neuronal excitability in cerebellar granule cells of mice lacking calretinin

Calcium-binding proteins such as calretinin are abundantly expressed in distinctive patterns in the CNS, but their physiological function remains poorly understood. Calretinin is expressed in cerebellar granule cells, which provide the major excitatory input to Purkinje cells through parallel fibers. Calretinin-deficient mice exhibit dramatic alterations in motor coordination and Purkinje cell firing recorded in vivo through unknown mechanisms. In the present study, we used patch-clamp recording techniques in acute slice preparation to investigate the effect of a null mutation of the calretinin gene on the intrinsic electroresponsiveness of cerebellar granule cells at a mature developmental stage. Calretinin-deficient granule cells exhibit faster action potentials and generate repetitive spike discharge showing an enhanced frequency increase with injected currents. These alterations disappear when 0.15 mm of the exogenous fast-calcium buffer BAPTA is infused in the cytosol to restore the calcium-buffering capacity. A proposed mathematical model demonstrates that the observed alterations of granule cell excitability can be explained by a decreased cytosolic calcium-buffering capacity resulting from the absence of calretinin. This result suggests that calcium-binding proteins modulate intrinsic neuronal excitability and may therefore play a role in information processing in the CNS.     

A technique for correction of carotid kinks and coils following endarterectomy

This study presents a technique to correct kinking or coiling of the internal carotid artery using patch angioplasty following endarterectomy, resection, and anastomoses of the vessel. Since 1984, 579 carotid endarterectomies have been performed with 19 patients (3.3%) having arteriosclerotic carotid bulb and internal carotid artery disease associated with a carotid kink or coil. These have been treated successfully using the technique of resection and patch angioplasty. The indications for surgery included seven patients with transient ischemic attacks (36.8%); seven patients who had suffered a cerebrovascular accident (36.8%); amaurosis fugax in two patients (10.5%); and one patient each with Hollenhorst plaque (5.3%), central retinal artery occlusion (5.3%), and an asymptomatic critical stenosis (5.3%). All patients had successful repair of the vessel using saphenous vein or Dacron patch angioplasty. There were no perioperative strokes or deaths. Follow-up ranged from four months to 58 months (mean 25 months). All vessels are patent with no evidence of stenosis. One patient had an ipsilateral cerebrovascular accident but had no evidence of recurrent carotid disease. Surgical correction of the carotid kink or coil can present a difficult surgical problem, and resection, fixation, or transposition can be complicated. The technique of endarterectomy, resection of the redundant vessel with anastomosis of the back wail, and patch angioplasty has been used effectively and safely in this series of patients.Presented at the Fifteenth Annual Meeting of the Peripheral Vascular Surgery Society, June 2, 1990, Los Angeles, California.The views and opinions expressed in this paper are those of the authors and do not reflect those of the Department of the Army, Department of Defense, or of the Uniformed Services University of the Health Sciences.     

Percutaneous cecostomy: controlled transperitoneal approach

The authors present two cases of percutaneous cecostomy performed with a modified approach previously described for percutaneous gastrostomy and cholecystostomy. T-fastener devices were used to affix the cecum to the anterior abdominal wall; thus, the potential problem of fecal spillage was prevented. In both cases, adequate fecal drainage was provided without complication.     

Elongated Styloid Process (Eagle''s Syndrome) Causing Hemicrania

SYNOPSIS
Hemifacial pain and hemicrania can be caused by an elongated styloid process (Eagle's syndrome). This entity is reviewed historically with six case histories and x–rays presented. The etiology of the symptom complex is unclear, but possible mechanisms such as cranial nerve pressure and carotid sympathetic nerve involvement are discussed. Specific treatment is surgical removal of the tip of the styloid process.     

White cells in fresh-frozen plasma: evaluation of a new white cell- reduction filter

BACKGROUND: Fresh-frozen plasma (FFP) has generally been regarded as an acellular component. Recently, viable lymphocytes have been detected in this component and the question of irradiation of FFP for certain patients has been raised. Whether the numbers of white cells (WBCs) in FFP are sufficient to require WBC-reduction of acellular components for patients receiving WBC-reduced cellular components has not been determined. WBC numbers in FFP were examined, and the performance of a new commercial WBC-reduction filter for FFP was assessed. STUDY DESIGN AND METHODS: WBC numbers in plasma processed for use as FFP and in thawed FFP were counted before and after WBC-reduction filtration by the use of flow cytometry Fast and slow filtration was used to simulate laboratory and bedside filtration, respectively. Three different methods for plasma harvesting (soft-spin, hard-spin, and second-spin methods) were assessed. The filter capacity was also examined. RESULTS: The numbers of WBCs in plasma covered a three-log10 range (soft-spin method, 0.04-3.6 × 10(6); hard-spin method, 0.47-45.4 × 10(6); second- spin method, 0.4–37.2 × 10(6)). For the hard-spin and second-spin methods which produced the greatest plasma yields, 92 percent and 85.7 percent of bags, respectively, had counts>1 × 10(6) and 43 percent (hard-spin method) and 45.7 percent (second-spin method) had counts>5 × 10(6). There was no significant difference between the counts obtained in plasma and thawed FFP. The filter reduced WBC numbers to <1 × 10(5) in all but 3 of 49 bags. In the remaining three, there were <2 × 10(5) WBCs. Five bags of plasma could be processed effectively through each filter. CONCLUSION: FFP may contain WBC numbers above the threshold at which the use of WBC-reduction filters for cellular components in some patients is necessary. Confirmation of these findings and similar investigations on plasma prepared by other methods may help in defining a role for the use of WBC-reduction filters for FFP     

Isolated polyarteritis nodosa affecting the cecum

Summary We report the case of a 52-year-old white male who developed low back pain and 35-pound weight loss and whose barium enema revealed a constricting lesion of the cecum. After resection, polyarteritis was found to be the cause of the lesion. There was no other histological evidence for arteritis in this patient.The opinions expressed herein are those of the authors and not necessarily those of the Department of Defense, U.S. Air Force, or the Uniformed Services University of the Health Sciences.Figures are published with permission from the Armed Forces Institute of Pathology.