1/9. proteus syndrome.proteus syndrome is a rarely described dysplasia syndrome of the group of congenital hamartomas that arises from mosaic mutation. An extraordinary case history including imaging studies will be reported. This 17-year-old girl suffered from cachexia, lifelong chronic obstipation, different dysplasias, and lipomatous tumor-like lesions. The following findings were marked: macrodactyly, nevi, hemihypertrophy, aggressive lipomatosis, hemangiomas of the spleen, and skull and cerebral malformations. Additionally, an intestinal affection with fatty wall thickening was detected. In contrast to reports in the literature describing a reduced lifespan with a mean of few years, our patient is still alive. The treatment should take a palliative symptomatic approach considering the clinical situation.- - - - - - - - - - ranking = 1keywords = affect (Clic here for more details about this article) |
2/9. cachexia in patients with advanced cancer.Cancer cachexia generally is considered to be the end stage in the progression of nutritional deterioration and wasting of malignancy (Ottery, 1995). In patients with advanced cancer, this condition is very common and decreases quality of life, as well as survival (Fearon et al., 2001; Ottery; Smith & Souba, 2001; Whitman, 2000). However, if early diagnosis and intervention can control cachexia, the potential exists to greatly improve a patient's quality of life and prolong survival. Because metabolic alterations inhibit the effective use of conventional nutritional support, anti-inflammatory agents or fish oil are possible options. Orexigenic agents may be prescribed if patients wish to improve oral intake. steroids and progestational agents may be used to attempt to improve mood and appetite. Nutrition affects symptoms that need to be managed effectively. nurses should work aggressively to correct factors that contribute to decreased food intake (e.g., nausea, pain) and correct factors that worsen debility (e.g., anemia). Information must be presented so that informed choices can be made and realistic eating goals set. An interdisciplinary approach that involves the nurse, physician, dietician, and possibly social worker or case manager, as well as the patient and family, is necessary to identify nutritional alterations, assess specific needs, and plan individual interventions. Whitman (2000) stated that counseling is the most effective and least expensive intervention. It may be conducted by any member of the healthcare team and should be combined with other interventions. Palliation of cachexia in patients with advanced cancer is a challenge for nurses. Hopefully, early and judicious use of these interventions may decrease the significant morbidity and mortality that result from cancer cachexia.- - - - - - - - - - ranking = 96.94323426307keywords = mood, affect (Clic here for more details about this article) |
3/9. Giant uterine tumors: two cases with different clinical presentations.BACKGROUND: Giant uterine tumors are uncommon. However, they may be life threatening because of pressure effects on the lungs and other adjacent organs. Proper surgical management and careful perioperative care are essential to assure a good outcome after excision. CASE: Two women with giant uterine leiomyomata (weighing more then 40 kg [88 lb]) are discussed. In one case the leiomyoma led to severe pulmonary hypertension and respiratory failure necessitating an emergency operation. Abdominal hysterectomy and bilateral salpingo-oophorectomy were successfully carried out in both cases, which are among the largest tumors ever removed with survival of the patient. CONCLUSION: Different clinical manifestations can be expected in cases of giant uterine tumors according to which other organs are secondarily affected. Numerous difficulties may be encountered in the evaluation and removal of these tumors. A combined team consisting of gynecologic, general, and plastic surgeons is necessary for a successful outcome.- - - - - - - - - - ranking = 1keywords = affect (Clic here for more details about this article) |
4/9. pellagra: a rare disease observed in a victim of mental and physical abuse.Lesions of the brain stem and cerebellum due to nutritional deficiencies are mostly seen in chronic alcohol abuse and more rarely in severe malnutrition. We report the case of a 27-year-old woman, found dead in the family flat. She presented cachexia (167 cm, 25 kg) and multiple hematomas of the limbs. Postmortem examination revealed lesions due to peritonitis. Neuropathological examination showed severe atrophy of the corpus callosum and central neuronal chromatolysis, which are observed in pellagra. Inflammatory colitis or celiac disease was not found. Toxicological analysis was negative, in particular no alcohol absorption. pellagra, which is due to nicotinamide deficiency, is a disease rarely seen in this country. In this case, nutritional deficiency was the consequence of failure to eat in a context of abuse. The woman was born of an incestuous relationship and presented intellectual retardation due to poor affective relations with her mother.- - - - - - - - - - ranking = 1keywords = affect (Clic here for more details about this article) |
5/9. osteoporosis associated with megestrol acetate.megestrol acetate is a progestational agent for treatment of metastatic breast cancer and endometrial cancer. Megestrol has also been used as an appetite stimulant for patients with human immunodeficiency virus and malignancy who experience cachexia and wasting; also, megestrol can be beneficial in relieving hot flashes in women and men. Megestrol has been shown to have a glucocorticoidlike effect and has been associated with substantial suppression of plasma estradiol levels. We describe 2 patients who recently presented to our Metabolic Bone Disease Clinic with severe osteoporosis complicated by multiple vertebral fractures experienced while the patients were receiving high-dose megestrol therapy. The patients had evidence of adrenal axis suppression but recovered fully after megestrol was discontinued. We speculate that megestrol was an important factor in the development of osteoporosis and subsequent fractures. Further study is warranted to clarify the relationship between megestrol and its potential for adversely affecting the skeleton.- - - - - - - - - - ranking = 1keywords = affect (Clic here for more details about this article) |
6/9. Beneficial response to megoestrol acetate in AIDS-related cachexia and a possible megoestrol withdrawal-associated syndrome?A man with AIDS is described in whom a profound weight loss was converted into a weight gain by treatment with megoestrol acetate, a synthetic progesterone. His appetite improved and was accompanied by a feeling of improved well-being. Following abrupt discontinuation of the drug, there was a significant but transient depression of mood and appetite associated with loss of energy; it is suggested that this complex of symptoms might represent a megoestrol acetate withdrawal-associated syndrome.- - - - - - - - - - ranking = 95.94323426307keywords = mood (Clic here for more details about this article) |
7/9. The "FACES" syndrome: a new syndrome with unique facies, anorexia, cachexia, and eye and skin lesions.A new syndrome with unique facial features, anorexia, cachexia, eye and skin anomalies affecting three family members is described. A possible mode of inheritance is suggested and various aspects of the phenotype are discussed.- - - - - - - - - - ranking = 1keywords = affect (Clic here for more details about this article) |
8/9. Delayed radiation-induced bulbar palsy.We report a man with a slowly progressive bulbar palsy 14 years after radiation therapy for nasopharyngeal carcinoma. electromyography demonstrated prominent myokymic and neuromyotonic discharges in muscles innervated by the lower cranial nerves. Late effects of radiation therapy can occur in the cranial nerve musculature that are similar to well-recognized syndromes affecting the brachial plexus and spinal cord.- - - - - - - - - - ranking = 1keywords = affect (Clic here for more details about this article) |
9/9. Pathologic findings of latissimus dorsi muscle graft in dynamic cardiomyoplasty: clinical implications.BACKGROUND: We hypothesize that the integrity of the latissimus dorsi muscle graft used to wrap the heart may affect the clinical outcome of patients undergoing dynamic cardiomyoplasty. methods: By correlating the pathologic findings with their clinical course in five patients who died 1 month to 6 years after dynamic cardiomyoplasty operation, we sought to discern findings that might shed light on the pathophysiology of cardiomyoplasty. RESULTS: Of the two patients who had a limited clinical response, one had an atrophic, edematous latissimus dorsi muscle with fatty infiltration resulting from cardiac cachexia, and the other had insufficient length of latissimus dorsi muscle to cover a large heart. The remaining patients responded well clinically without signs of pump failure and died at various intervals, mostly of arrhythmias. autopsy findings included the following: (1) one patient with ischemic cardiomyopathy as the underlying disease had development of rich vascularity in the interface between the muscle wrap and the epicardium; whereas in four others with idiopathic cardiomyopathy, such evidence of collateralization was far less evident. (2) There was a variation in the skeletal muscle transformation achieved, with the fraction type I fatigue-resistant fiber in the muscle wrap ranging from 60% to 100%, in spite of the identical transformation protocol used. Such variation is believed to be genetically based. (3) In one patient, the skeletal muscle was paced to contract at 30 to 50 times/minute (2:1 ratio) for more than 5 years. Nevertheless, the pathologic specimen of the muscle wrap showed only minimal interstitial fibrosis. (4) Relatively thin muscle wrap around the heart found at autopsy could be atrophy but most likely was related to muscle transformation, which is known to reduce muscle mass and increase capillary density. (5) All skeletal muscle grafts showed geometric conformation to the shape of the epicardium and grossly looked as if they were an additional layer of the ventricular wall. Such conformation may facilitate the modulation of the ventricular remodelling process in the failing heart, as has been described both in clinical and experimental studies. CONCLUSIONS: Our findings are consistent with and support a number of mechanisms proposed for cardiomyoplasty. Thus preservation of latissimus dorsi muscle graft integrity may be important in the success of dynamic cardiomyoplasty.- - - - - - - - - - ranking = 1keywords = affect (Clic here for more details about this article) |