Pleuropulmonary aspergilloma: clinical spectrum and results of surgical treatment

From 1974 to 1991, 77 patients were admitted for pulmonary (55), pleural (16), or bronchial (6) aspergilloma. About 50% were asymptomatic. Sixty-three underwent operation. Pulmonary aspergillomas were operated on for therapeutic need in 26 and on principle in 18; the procedures were 28 lobar or segmental resections, 10 thoracoplasties, and 5 pleuropneumonectomies (1 patient had exploration only). Pleural aspergillosis was treated by operation on principle in 5 and for therapeutic need in 8 patients; 10 thoracoplasties, 1 attempt at pleuropneumonectomy, and 2 decortications were performed. All six bronchial lesions were operated on as a rule. Overall postoperative mortality was 9.5%. Major complications were bleeding (n = 37), pleural space problems (n = 24), respiratory failure (n = 6), and postpneumonectomy empyema (n = 4). All patients with pleural disease experienced complications. The outcome was better after lobar or segmental resection than after thoracoplasty (mortality, 6% versus 15%). Asymptomatic and nonsequellary pulmonary or bronchial aspergilloma also had an improved outcome. We conclude that operation is at low risk in pulmonary or bronchial locations in asymptomatic patients and in the absence of sequellae; the risk is high in symptomatic patients for whom operation is the only definite treatment. Pleuropneumonectomy should be avoided. Only symptomatic pleural aspergilloma should be operated on.     

Regional pulmonary function in scimitar syndrome

Analysis of regional pulmonary function distribution using radioactive 133xenon gas and eight scintillation detectors was performed in a patient with scimitar syndrome. Zonal ventilation per unit of lung volume within the slightly hypoplastic right lung was normal. The pulmonary blood flow to the lower right zone was significantly reduced due to aberrant systemic arterial supply to the right lower lobe. The 133xenon technique provides a simple nontraumatic method of evaluating the functional integrity of the right lung in this cardiopulmonary syndrome.     

Ehlers-Danlos syndrome and type III collagen abnormalities: a variable clinical spectrum

Ehlers Danlos syndrome (EDS) comprises ten types. EDS IV is the most severe type because of its often lethal complications, such as arterial rupture. EDS IV is caused by an abnormality of collagen type III as a result of mutations in the corresponding gene COL3A1. A collagen type III abnormality is also seen in patients with EDS without the classical severe EDS IV phenotype. We report on 11 patients with type III collagen abnormality and normal collagen V in whom clinically EDS II, III, and IV were diagnosed. There is no correlation between the type of collagen III anomaly and the clinical phenotype. It is concluded that type III collagen abnormality may lead to a phenotypic spectrum and that it does not predict the severity and course of the disease.     

Kidney in Galloway-Mowat syndrome: clinical spectrum with description of pathology

The Galloway-Mowat syndrome, a rare inherited disorder, is characterized by congenital microcephaly with hypotonia and developmental delay, often hiatus hernia, and nephrotic syndrome manifested in infancy or in early childhood. The glomerular lesion has been poorly characterized in the few previous reports of this syndrome. We studied three siblings with microcephaly and nephrotic syndrome occurring during the first two weeks of life. Hematuria, glycosuria and renal failure were also present. Renal biopsy and postmortem specimens of two patients were studied. Glomerular structure was disorganized; capillary lumina were of varying calibers, capillary walls were adherent to one another, and mesangial zones were poorly demarcated. Glomerular basement membrane ultrastructure was markedly altered. The normal trilaminar structure was obscured or replaced by flocculent material; furthermore, 6 to 8 nm fibrils of unknown nature permeated the space between endothelial and epithelial cells. Non-glomerular basement membranes were unaltered in appearance. This syndrome apparently represents, in part, a new disorder of glomerular basement membrane formation and function.     

Impending paradoxical embolus: clinical imaging and successful surgical treatment

AIM OF THE STUDY: Postoperative sensory component of the facial nerve after acoustic tumor surgery has received little attention in the literature. The object of the present investigation was to review this specific topic analyzing the postoperative frequency of taste and lacrimation (crocodile tears or dry eye) abnormalities. MATERIAL AND METHODS: 54 patients who underwent acoustic tumor removal were selected for this study. Each of these patients was recalled and pre and postoperative evolution of the sensory dysfunction were assessed. The latters were correlated with the facial function evaluated according to the House-Brackmann classification. RESULTS: Postoperative taste dysfunction (reduced or changed sensation) was complained by 38.5% of the patients. After surgery, 42.3% of the cases had crocodile tears, while in 59.6% altered tearing occurred. DISCUSSION: The present study, according to the Irving et al's experience confirmed a significant incidence of postoperative abnormal function of the sensory facial nerve. The influence of the motor component on these outcomes was variable. Lacrimation worsened when facial function was poor. On the other hand, grades V or VI did rarely manifest crocodile tears. Clinically, these findings implies the importance of a preoperative counseling of such particular aspect in the candidates to surgery of acoustic neuroma in order to adequately motivate them and, at the same time, to reduce their psychological discomfort.     

Amplification of the clinical spectrum of Robinow syndrome

Calcifying tendinitis is a relatively common disorder of the rotator cuff. For symptomatic patients, excision of the calcium deposits offers reliable pain relief. The arthroscopic technique is demanding. Arthroscopic localization of the deposit is frequently demanding. The technique described in this article facilitates the localization of calcium deposits based on preoperative ultrasonography. Knowing the exact topography of the deposit relative to the visible landmarks allows the surgeon to localize the site of the deposit from the intra-articular view. Probing the rotator cuff with a spinal needle and looking for calcific material at the tip of the needle is an important step in verifying the location of the deposit.     

Interdisciplinary treatment for fibromyalgia syndrome: clinical and statistical significance

ATM has been identified as a gene that is responsible for ataxia telangiectasia (AT), a pleiotropic disorder of autosomal recessive inheritance. While many mutations of this gene in AT patients of various ethnicities have been reported, data on Japanese patients are scarce. In this report, we present the results of a thorough survey of ATM mutations in 14 unrelated AT patients, with an emphasis on Japanese subjects. We used a hierarchical strategy in which we extensively analyzed the entire coding region of the cDNA. In the first stage, point mutations were sought by PCR-SSCP in short patches. In the second and third stages, the products of medium- and long-patch PCR, each covering the entire region, were examined by agarose gel electrophoresis to search for length changes. We found a total of 15 mutations (including 12 new) and 4 polymorphisms. Abnormal splicing of ATM was frequent among Japanese, and no hotspot was obvious, suggesting no strong founder effects in this ethnic group. Eleven patients carried either one homozygous or two compound heterozygous mutations, one patient carried only one detectable heterozygous mutation, and no mutation was found in two patients. Overall, mutations were found in at least 75% of the different ATM alleles examined. Possible reasons for the inability to detect mutations in some patients are discussed.     

The spectrum of generalised anxiety in clinical practice: the role of short-term, intermittent treatment

BACKGROUND: DSM-IV generalised anxiety disorder (GAD) has a high lifetime prevalence, but subthreshold anxiety states are even more common, particularly in family practice. METHOD: Generalized anxiety is conceptualised as a spectrum of disorders, with transient anxiety at one end and GAD at the other. RESULTS: Based on long-term experience with family practice patients, the authors suggest that most anxious patients, wherever on this continuum they are placed, could be treated with short-term, possibly intermittent, rather than chronic anxiolytic therapy. Data are presented which show that 50% of chronic GAD patients are only in need of such short-term intermittent therapy. CONCLUSIONS: Further clinical research is needed to refine short-term, intermittent treatments for anxiety spectrum disorders, to make effective treatments available to those suffering from anxiety but falling short of diagnostic criteria for GAD, and to target more effectively the different treatment strategies.     

Asbestosis: clinical spectrum and pathogenic mechanisms

Asbestosis is a diffuse pulmonary fibrotic process caused by the inhalation of asbestos fibers. Despite extensive investigations, the precise mechanisms regulating asbestos-induced lung damage are not fully understood. This review summarizes the important clinical manifestations and pathogenic mechanisms of asbestosis. We focus on the relatively new information that has emerged over the last several years. The diagnosis of asbestosis is often easily established by well-characterized criteria. Pulmonary physiologic testing and high-resolution computed tomography can detect clinically occult disease. The finding of asbestos bodies in the bronchoalveolar lavage fluid confirms that an individual has been exposed to asbestos but is of unclear significance in diagnosing asbestosis. Evidence reviewed herein suggests that asbestos pulmonary toxicity is due in part to the physical properties of the fibers, iron-catalyzed reactive oxygen species (ROS), and macrophage-derived cytokines and growth factors. Special emphasis is given to the hypothesis that iron-catalyzed hydroxyl radicals (HO.-) have a pivotal role in causing asbestosis. Definitive proof of this hypothesis is difficult to obtain since HO.- are highly reactive and their deleterious effects to cells may have occurred years prior to disease presentation. Despite these limitations, considerable data firmly support the notion that ROS have an important role in causing asbestos toxicity. Further, the iron content of asbestos or the redoxactive iron associated with or mobilized from the surface of the fibers is important in generating HO.- as well as in activating inflammatory cells. There also appears to be a close association between asbestos-induced ROS production and cellular toxicity and DNA damage. The full expression of asbestos-induced diseases likely involves the contribution of cytokines, growth factors, proteases, and other inflammatory cell products. Many of the mechanisms by which asbestos- and inflammation-induced ROS activate specific genes in pulmonary cells remain to be elucidated.     

The clinical spectrum of narcolepsy and idiopathic hypersomnia

To better define the clinical spectra of narcolepsy and idiopathic hypersomnia, we retrospectively compared clinical and polygraphic findings and questionnaire results in groups of subjects with narcolepsy with or without cataplexy, idiopathic hypersomnia, insufficient sleep syndrome, mild sleep apnea, and excessive daytime sleepiness not otherwise specified. Sleep paralysis and sleep-related hallucinations were most frequent in narcolepsy-cataplexy, but their frequency did not differ between narcolepsy without cataplexy and idiopathic hypersomnia. Mean durations of nocturnal sleep, daytime naps, and morning grogginess were not increased in idiopathic hypersomnia compared with other groups. Among subjects without cataplexy, symptoms of sleep paralysis and sleep-related hallucinations were equally common in subjects with and without frequent sleep-onset REM periods. These findings suggest that the occurrence of these symptoms in subjects without classical narcolepsy-cataplexy is a function of factors other than a propensity for early onset of REM sleep and indicate a need to reevaluate diagnostic criteria for narcolepsy and idiopathic hypersomnia.     

Anomalous venous drainage of the hypoplastic right lung and other malformations (scimitar syndrome)

Patients with lower abdominal symptoms, indicating colonic disorder, were classified into predefined clinical syndromes. Two important syndromes were colicky sigmoid syndrome and chronic diverticular disease. The first one, characterized by presence of colicky lower abdominal pains but absence of colonic diverticula, probably covers what is generally referred to as 'irritable bowel syndrome'. The second one was characterized by presence of lower abdominal colics and of colonic diverticula. The results showed a significant correlation between the presence of lower abdominal colics and a high pressure activity in the sigmoid colon after intravenous neostigmine. There was, however, no correlation between the presence of diverticula and a high pressure activity. The generally accepted theory of a high pressure activity as the dominant factor in the pathogenesis of colonic diverticula, therefore, was questioned, as was the equally accepted theory of a gradual development of chronic diverticular disease from the adiverticular colicky sigmoid syndrome.     

Efficacy of surgical treatment in traumatic central cord syndrome

BACKGROUND: Controversy surrounds the treatment of traumatic central cord syndrome (TCCS), as there are strong advocates for nonsurgical treatment for most patients. However, conservative treatment has been shown to yield a longer period of discomfort from pain and weakness in certain cases. METHODS: In a retrospective review of 114 patients presenting with acute or chronic TCCS from 1988-94, four different age groups were separately observed under different treatments. Motor and sensory recovery were assessed. RESULTS: Better results were achieved in younger patients, with or without radiographic abnormalities, and in patients with clinically correlated encroaching cord lesions who received early surgical decompression. CONCLUSIONS: Surgical intervention for TCCS must be addressed with careful clinical and radiographic survey. Removal of offending lesions in the subacute period results in significant motor and sensory improvement in short-term and long-term follow-up.     

The surgical treatment of hyperhidrosis

The diagnosis of primary hyperhidrosis is one of exclusion, but it is not a difficult one to make. It is a common, embarrassing and even disabling condition. If it does not respond to simple conservative therapy, the practitioner should not hesitate to advise sympathectomy for hyperhidrosis of the upper or lower limbs, or some simple local operation on the affected skin area for localized excessive axillary sweating.