Generalized Disease Process Research Articles

Disorders of perfusion of the anterior segment of the eye.

We have investigated the vascular perfusion of a wide variety of conditions of the anterior segment using fluorescein angiography. The conditions were classified and findings reported according to the system set out below. Patients underwent full ocular examination. Fluorescein angiography of the anterior segment was carried out when indicated to investigate iris atrophy and neovascularisation. Specular microscopy of the corneal endothelium was used to detect changes in this tissue. The hypoperfusion was variable in degree and accompanied by varying degrees of iris hypoplasia and atrophy with neovascularisation. The degree of neovascularisation depended upon its rapidity of development, the pre-existing state of vascular perfusion and the underlying pathological condition. Hypoperfusion with resultant ischaemia and neovascularisation is common in conditions of the anterior segment. An understanding of the changes is valuable in treating many conditions affecting the anterior segment. The changes observed may also occur elsewhere in the physical system and may be a significant part of the ageing process, either as scattered, disparate processes or as part of a general disease process.

Primary and secondary disorders to gut muscle and nerve.

There have been many recent advances in understanding primary disorders of enteric nerve and muscle. Although not common in clinical practice, most gastroenterologists and surgeons will encounter a few such patients. Management is often difficult but these patients form a fascinating area of practice, and put to the test a clinician's skills. There are also exciting discoveries still waiting to be made in this field. This article focuses on conditions which lead to gut dilatation, or more rarely conditions in which there is no gut dilatation but there is definitive proof of an abnormality of gut nerve or muscle. Disorders of enteric nerves or muscles, or of the extrinsic innervation of the gut, can lead to an acute or chronic gut dilatation. These disorders can be primary or secondary to a generalized disease process outside the gut. These conditions tend to fall into clinically homogeneous syndromes, although within each syndrome the underlying pathologies are heterogeneous. The field of primary abnormalities of the enteric nerve and muscle are only beginning to be explored. Many conditions remain to be discovered, each bringing with them new insights into the mechanisms of normal and abnormal gastrointestinal function.

BURNING MOUTH SYNDROME

Burning mouth syndrome (BMS) is a frequent process that produces marked oral dysesthetic symptoms. There is controversy over its etiopathogenesis and diagnosis. Therapeutic results are unsatisfactory. The objectives of this study were: (1) to analyze a possible agreement on the definition of BMS; (2) to analyze its cause; (3) to propose a therapeutic scheme that has been shown to be highly effective in our experience and has not been previously reported. Five hundred patients with symptomatology of BMS were retrospectively evaluated with a study protocol, specially designed for this disease. Patients with local or general disease processes that could cause secondary asymptomatic stomatodynia were excluded from the study. Most of the patients evaluated were women over 60 years of age with oral symptoms, including a sensation of heat and burning, and pain, lasting for months or years, and a history of multiple unsuccessful treatments. The patients showed depression associated with anxiety, with evidence of psychiatric disorders in the family. Based on these findings and eliminating symptomatic painful conditions of the mouth that may stimulate this syndrome, we diagnosed a genuine or basic BMS of psychosomatic origin. Therapies used in the treatment of these neurotic conditions were not always useful. Tranylcypromine associated with anxiolytics and hypnotics in low doses plus the support of psychotherapy by the stomatologist were the most effective treatments. The BMS should be defined as a psychosomatic process causing oral dysesthesias for months or years.

Diseases of the Fetus and Newborn: Pathology, Radiology and Genetics

This textbook contains four sections: first, an overview of general disease processes, and then a section for each of the title categories: pathology, radiology, and genetics. Two chapters in the general disease section nicely address embryology, the neonatal period, and placenta with pathophysiology and morphological development adequately covered. Another chapter evaluates spontaneous abortion, recurrent fetal loss, and the malformed fetus. This chapter is excellent and provides a good review and approach for the obstetrician, pediatrician, pathologist, and geneticist. Evaluation of the fetus or tissue in pregnancy loss is a crucial examination, which is too often avoided or inadequately performed. It is unfortunate to discard or neglect the only potential source of explanation for a family of such a devastating event. Chapter four discusses congenital anomalies in a basic, general format, probably obviating its usefulness within this text. The Brain initiates the section on pathology. Although such important associations as occipital

The Pathogenesis of Cervical Spondylosis

Cervical spondylosis is a generalized disease process affecting all levels of the cervical spine. Cervical spondylosis encompasses a sequence of degenerative changes in the intervertebral discs, osteophytosis of the vertebral bodies, hypertrophy of the facets and laminal arches, and ligamentous and segmental instability. The natural history of cervical spondylosis is associated with the aging process. Senescent and pathologic processes are thus morphologically indistinguishable. Clinical manifestations of cervical spondylosis may arise when morphologic sequelae are superimposed on a developmentally narrow spinal canal. The two clinical syndromes of spondylotic radiculopathy and myelopathy are distinct, yet they may overlap.

Fiber size and energy metabolites in five separate muscles from patients with chronic obstructive lung diseases.

The purpose of this study was to examine whether morphologic abnormalities in human respiratory muscles are related to increased airway obstruction. 43 patients who were undergoing thoracotomy for suspected neoplasm had biopsies taken from one or more of the following muscles: external intercostal (EXT), internal intercostal (INT), diaphragm (DIA), latissimus dorsi (LAT), and quadriceps femoris (LEG). Mean FEV1/FVC was 65% of predicted (range 43–90%). 21 of the 43 patients had a malignancy. Atrophy of type I fibers was found in 27% of respiratory and 11 % of nonrespiratory muscles. Type II fiber atrophy was more common, being found in 58% of all muscles studied. The degree of type II fiber atrophy correlated significantly with the amount of weight loss, but not with age or the presence of malignancy. A unique and significant relationship was found between type II fiber atrophy in the INT (an expiratory muscle) and all measured indices of airway obstruction. This relationship did not exist in the DIA, EXT, or LAT, ordinarily considered muscles of inspiration. The percentage of type I and type II fibers bore no relationship to indices of airway obstruction. Depletion of muscle metabolites was common to all muscles and could not be related to airway obstruction or fiber atrophy. These data suggest that fiber atrophy and metabolite depletion occur commonly in both respiratory and nonrespiratory muscles in patients with stable obstructive lung disease. These changes probably reflect a generalized disease process and may predispose to muscle fatigue. Whether or not airway obstruction produces fiber atrophy in expiratory muscles requires further investigation.

An Unusual Presentation of Pigmented Villonodular Synovitis

A case of multiple joint and tendon involvement by PVS seems not to have been preciously reported. In a 7-year-old girl there were other organ system problems usually not seen with PVS, and the entire complex may represent a more generalized disease process. The diffuse form of the disease was refractory to multiple attempts at surgical excision. The similarities between PVS and synovial hemangioma suggest that it is important to establish the diagnosis after the first operation.

Surgical aspects of peripheral vascular disease.

ABSTRACT: This article reviews current surgical concepts regarding the management of elderly patients with peripheral vascular disease. The indications for surgical reconstruction may be either relative or absolute, but only absolute indications (e.g., incipient gangrene) apply in the elderly. A review of the natural history of the disease shows that although some cases remain stable for long periods, many progress to a state of serious deterioration with painful intermittent claudication, incipient gangrene and threatened loss of the limb. The mechanisms of deterioration include thrombosis, embolism and minor trauma. Peripheral vascular occlusive disease is only one manifestation of a generalized disease process, and vascular reconstruction is only a palliative measure. However, since high morbidity and mortality are associated with amputation, reconstruction should always be considered, even in the geriatric patient. The aorto‐iliac reconstruction technique is associated with a fairly low mortality rate and a limb‐salvage rate of 85 per cent; simpler alternatives are available in high‐risk patients. For femoro‐popliteal procedures the mortality rate is low and the limb‐salvage rate is 75 per cent. Such results emphasize that vascular reconstruction usually is preferable to amputation. Measures for conservative management, particularly in aged patients, are discussed.

Oral extension of regional enteritis (Crohn's disease): Report of a case

The clinician must remain alert for oral manifestations of systemic and extraoral disease conditions, however remote the possibility may seem. An unusual case of an oral extension of Crohn's disease is described. Some speculations regarding the significance of the oral lesion to treatment of the general disease process are discussed.

"Bell's Palsy" Caused by Basal Cell Carcinoma

Facial palsy may represent a serious local or generalized disease process which should not be labeled Bell's palsy until all possible causes have been excluded. Evaluation of such a patient includes topographical diagnostic studies of the facial nerve, and complete otolaryngologic examination, as well as appropriate laboratory studies. Symptoms in a region of a previous tumor may represent residual tumor even when the clinical pattern is atypical, as in this case of invasive basal cell carcinoma.

Current concepts of pneumoconiosis; pathological aspects.

The clearance of dust particles from the pulmonary alveoli toward the bronchi depends to some extent on adequate respiratory excursions of alveolar walls. Dust particles not so removed can penetrate the alveolar membrane instead; it is now believed that naked dust particles can pass directly through the alveolar membrane and are not carried by macrophages. In silicosis the silica-bearing cells in the characteristic nodules are thought to be proliferated alveolar cells that have remained in situ instead of desquamating. In explaining the subsequent fibrosis it is now postulated that silica crystals possess surface activity which denatures proteins and that the deposition of beta and gamma globulins in the nodules is indicative of a subsequent immune reaction. Tuberculosilicosis must be considered as a special case of a general disease process for which the more inclusive term tuberculopneumoconiosis is proposed.

Biliary tract surgery

1. 1. Experience with 109 consecutive cases of biliary tract disease over a ten-year period from 1940 to 1950 demonstrates that no absolute rule can be applied in any series of cases. A guide to therapy is offered through the posing of three questions regarding the warranty of surgical intervention, proper time for surgery and operative procedure of choice. 2. 2. Indications for surgery, other factors permitting, are as follows: cholelithiasis, extrahepatic biliary obstruction, hydrops, empyema and perforation of the gallbladder. Establishing these diagnoses is the major problem. 3. 3. Empyema with acute manifestations or perforation of the gallbladder are the only conditions in which emergency surgery is indicated in extrahepatic biliary disease. Conservative therapy is preferred in all other cases until the patient has been nutritionally and medicinally prepared to withstand surgery in so potentially hazardous an area. 4. 4. The procedure of choice cannot be determined beforehand. It is dependent upon findings at the operating table at which time the patient's general condition, disease process and prognosis are all considered prior to the selection of the type of operation.

Familial Involvement of the Nervous System by Multiple Tumors of the Sheaths and Enveloping Membranes: Hereditary, Clinical, and Pathological Study of Central and Peripheral Neurofibromatosis

Von Recklinghausen (1) in 1882 recognized the fact that the presence of “multiple neuromas” could not be considered as a purely acquired disease, but Thomson (2) in 1900 was the first to point out clearly that the condition was hereditary. Since that time the hereditary and familial character of the disease has been fully established and is generally recognized. Cases of multiple neurofibromatosis with diffuse involvement of the central nervous system are unusual. The association of this condition with multiple meningeal tumors is rare and more so when there is a distinct hereditary background. The present communication is a clinico-pathological study of familial von Recklinghausen9s disease of the central and peripheral nervous system associated with meningeal and glial tumors. Review of the Literature Multiple neurofibromatosis or von Recklinghausen9s disease may be looked upon as a congenital anomaly of the nervous system in a hereditary and dysontogenetic sense, according to Hosoi (3). Penfield (4) describes the condition as a “familial disease of diffuse character in which meningeal fibro-blastomas, gliomas and sarcomas may occasionally be encountered.” Preiser and Davenport (5) reviewed the literature and collected 30 cases of the familial type. They were able to show that the condition appears in both sexes and follows the mendelian law as a dominant trait. Hoekstra (6) in 1922 also pointed out the hereditary tendency in this condition. While multiple neurofibromatosis is a system disease affecting mainly the peripheral nerves (7), involvement of the central nervous system, though unusual, is by no means rare. German (8), reporting upon the changes in the ear in bilateral acoustic nerve tumors, states that over 30 cases of central neurofibromatosis have been recorded. Cases with acoustic nerve involvement have been reported recently by van Bogaert (9), Lewin (10), de Klejn and Gray (11), and others. Of interest in this respect is the occurrence of bilateral acoustic nerve tumors. Although these may rarely occur without evidence of von Recklinghausen9s disease elsewhere in the nervous system, at the present time it is generally conceded that they represent a local manifestation of a general disease process. Gardner and Frazier (12), in a review of the literature, were able to collect 44 cases of bilateral tumors of the acoustic nerve, 37 of which were associated with neurofibromatosis. In addition, they report a family in which 38 members were affected with bilateral deafness which in the autopsied cases was shown to be due to bilateral acoustic tumors. The condition in this family was transmitted as a mendelian dominant. Roger, Alliez and Sarradon (13) have described a family in which bilateral acoustic nerve tumors were verified at autopsy in one member and clinical evidence of a cerebellar tumor was present in four others. These writers believe that there is not sufficient evidence to warrant the full acceptance of Gardner and Frazier9s view that bilateral acoustic nerve tumors, as a part of neurofibromatosis, are transferred as a mendelian dominant. In reviewing the literature Roger and his associates (13) were able to find only 7 convincing family histories of intracranial involvement.