1/222. Acute disseminated encephalomyelitis in childhood: report of 10 cases.We report 10 children with the diagnosis of acute disseminated encephalomyelitis. diagnosis was based on clinical and radiologic findings, and after acute encephalitis was excluded by negative culture and antibody results. The most common presenting symptom was ataxia, followed by optic neuropathy, cranial nerve palsy, convulsions, motor dysfunction, and loss of consciousness. Brain magnetic resonance imaging showing bilateral symmetrical hyper-intense lesions of the same age in brain stem, subcortical white matter, thalamus, basal ganglia, or cerebellum was the mainstay of the diagnosis. The presence of a preceding event (either an infection or vaccination) was present in 8 of 10 patients. Brain computed tomographic scans were abnormal in 3 of 10, and electroencephalogram was normal in all patients. High-dose corticosteroids were given to six patients, one received low-dose steroids, and the other three had symptomatic follow-up. Those who relapsed were mainly from the symptomatic follow-up group. Only one patient (the youngest) receiving high-dose methylprednisolone relapsed. Therefore, early high-dose steroid treatment seems to be the most effective treatment in acute disseminated encephalomyelitis and can prevent relapses.- - - - - - - - - - ranking = 1keywords = dysfunction (Clic here for more details about this article) |
2/222. angioedema presenting in the retropharyngeal space in an adult.PURPOSE: Hereditary angioedema is a rare disorder of deficient or dysfunctional C1-esterase inhibitor and usually manifests as edema of the face, tongue, supraglottis, extremities, or gastrointestinal tract. We report the case of a 40-year-old man with known hereditary angioedema who presented with a sore throat and a sensation of evolving airway obstruction. After a thorough search of the medical literature, we believe this to be the first reported case of angioedema manifesting in the retropharyngeal space. The pathophysiological factors of angioedema are discussed, along with its variable presentation and management issues. METHOD: Laryngoscopic examination was suggestive of posterior pharyngeal fullness; therefore, a computed tomographic scan of the neck was obtained, which showed a non-contrast-enhancing retropharyngeal edema from the base of the skull to below the level of the glottis. The patient had a history of multiple episodes of angioedema requiring hospitalization and three prior tracheotomies. RESULTS: Familiarity with the patient's history directed his rapid treatment course (including intravenous stanozolol, Solu-Medrol, and diphenhydramine), which significantly reduced his edema and avoided the need for tracheotomy. CONCLUSION: Hereditary angioedema may present in atypical locations, and expeditious treatment in a patient with a known history may avert the sequelae of evolving airway obstruction.- - - - - - - - - - ranking = 1keywords = dysfunction (Clic here for more details about this article) |
3/222. The combination of risk factors for sudden death in a resuscitated elderly patient with an exceptional cause of left ventricular hypertrophy.The work-up of a previously asymptomatic 72-year-old man presenting with sudden cardiac death revealed a coarctation of the aorta as the cause of arterial hypertension, severe left ventricular hypertrophy, in combination with coronary artery disease with an apical myocardial infarction, severe autonomic dysfunction, and AV-nodal reentrant tachycardia. All these elements and their complex, probably synergistic interactions might have been involved in the development of sudden cardiac death.- - - - - - - - - - ranking = 1keywords = dysfunction (Clic here for more details about this article) |
4/222. plasmapheresis for prevention of recurrent focal segmental glomerulosclerosis of kidney allograft in adult recipients.nephrotic syndrome due to focal segmental glomerulosclerosis (FGS) frequently recurs even after renal transplantation and may cause renal allograft failure. From January 1983 though April 1995, 11 adult recipients with primary FGS received 11 kidney transplants at our institution, and 3 of them were treated with pretransplant plasma exchange (PE). Other patients did not receive any preoperative PE, and 4 patients lost their grafts due to recurrent FGS (50%). PE was completed 3 times before the transplantation to prevent posttransplant recurrence of FGS. Two recipients did not have any proteinuria or graft dysfunction without posttransplant PE. One patient had mild proteinuria immediately after transplantation, and histological examination showed recurrent FGS. The patient has been undergoing PE once a month (2 years posttransplant). Her renal function is excellent (sCr 1.2 mg/dl), and her FGS is being well controlled by PE. PE seems to be effective for the prevention of the recurrence of FGS following renal transplantation.- - - - - - - - - - ranking = 1keywords = dysfunction (Clic here for more details about this article) |
5/222. Reversible catecholamine-induced cardiomyopathy in a heart transplant candidate without persistent or paroxysmal hypertension.BACKGROUND: Both dilated and hypertrophic cardiomyopathy have been reported in patients with pheochromocytoma, who were almost always hypertensive. The outcome frequently has been fatal, yet cardiac dysfunction can be reversible after medical or surgical therapy for the pheochromocytoma. methods: We report the case of a patient with dilated cardiomyopathy without persistent or paroxysmal hypertension, who was found to have a pheochromocytoma during initial medical evaluation. RESULTS: The identification and treatment of the pheochromocytoma led to significant improvement in cardiac function and cardiac transplantation was avoided. CONCLUSIONS: This case illustrates some unusual features in pheochromocytoma-induced cardiomyopathy: (1) absence of persistent or paroxysmal hypertension, (2) initial presentation with acute myocardial infarction and normal coronary arteries, and (3) recurrent episodes of nonsustained ventricular tachycardia.- - - - - - - - - - ranking = 1keywords = dysfunction (Clic here for more details about this article) |
6/222. Recurrent Pseudomonas infection associated with neutrophil dysfunction.A 72-year-old male is described with a history of 4 episodes of pseudomonas aeruginosa sepsis and chronic otitis media caused by pseudomonas species. in vitro testing of the patient's polymorphonuclear leukocytes (PMNs) revealed profoundly abnormal chemotactic responses and defective intracellular killing of Ps. aeruginosa, staphylococcus aureus and escherichia coli. Chemiluminescence production by the patient's PMNs in response to opsonized zymosan as well as endotoxin stimulated nitroblue tetrazolium dye reduction were markedly depressed. These data indicate the presence of a profound, apparently acquired, defect in PMN function in an elderly male. Detailed evaluation of adult patients with recurrent infections may reveal similar, apparently acquired defects in PMN function.- - - - - - - - - - ranking = 4keywords = dysfunction (Clic here for more details about this article) |
7/222. A case of peripartum eosinophilic myocarditis.A 19-year-old postpartum patient with a previous history of asthma and eosinophilic myocarditis is described. Eosinophilic myocarditis is thought to be caused by exacerbation of the idiopathic hypereosinophilic syndrome by pregnancy. The diagnosis was made by a right ventricular endomyocardial biopsy, which showed an eosinophilic infiltrate with a few scattered foci of myonecrosis, but no fibrosis, vasculitis or granulomas. The patient's myocardial function continued to decline over a two-year follow-up period, despite normal levels of eosinophils. She developed echocardiographic evidence of diastolic and systolic dysfunction.- - - - - - - - - - ranking = 1keywords = dysfunction (Clic here for more details about this article) |
8/222. Cytophagic histiocytic panniculitis improved by combined CHOP and cyclosporin A treatment.In a 31-year-old Japanese man with cytophagic histiocytic panniculitis (CHP) remission was achieved by a combination of combined chemotherapy CHOP and cyclosporin A treatment. He was admitted to our hospital in January 1994 with recurrent high fever of 40.2 degrees C and tender and violaceous subcutaneous nodules on his trunk, arms and legs. He developed pancytopenia, hemorrhagic diathesis, liver dysfunction. Histological examination of the biopsied subcutaneous nodule revealed a lobular panniculitis with fat necrosis and a massive infiltration of histiocytes phagocytosing nuclear debris. He was treated initially with 40 mg/day prednisolone. However, following a reduction in prednisolone dosage, his symptoms reappeared. CHOP (cyclophosphamide, doxorubicin, vincristine and prednisolone) therapy was then initiated. Three courses of CHOP treatment alleviated his symptoms and cyclosporin A was used to maintain his condition for 15 months. His medication was then discontinued and he has been in complete remission for 10 months. Combined treatment of cyclosporin A and CHOP combined chemotherapy was shown to be effective for this patient with severe CHP.- - - - - - - - - - ranking = 1keywords = dysfunction (Clic here for more details about this article) |
9/222. Familial relapsing haemolytic uraemic syndrome and complement factor h deficiency.BACKGROUND: In a recent study of three families we have found that inherited haemolytic uraemic syndrome (HUS) maps to a region of chromosome 1q containing the gene for complement factor h. In one of these families and also in a case of sporadic D-HUS, we have identified mutations in the factor H gene. A further family with inherited HUS has therefore been investigated. methods: dna extracted from the family members and dna extracted from archival post-mortem material from a deceased family member, was studied. review of renal biopsies and study of complement components was also undertaken. RESULTS: This family demonstrates an inherited deficiency of complement factor h. Non-diarrhoeal HUS has affected at least two family members with half normal levels of factor H. CONCLUSION: These findings represent further evidence of the association between factor H dysfunction and HUS.- - - - - - - - - - ranking = 1keywords = dysfunction (Clic here for more details about this article) |
10/222. cholesteatoma in a child with congenital ichthyosiform erythroderma.A 7-year-old girl had non-bullous congenital ichthyosiform erythroderma (NBCIE) in treatment with retinoids. She also suffered continuous desquamation of the external ears, recurrent external otitis, chronic adenoiditis, tubaric dysfunction and intermittent episodes of otitis media and developed a cholesteatoma in her left ear. Chronic scaling disorders, such as NBCIE and other ichthyosis, can be entities at risk for the development of cholesteatoma in childhood.- - - - - - - - - - ranking = 1keywords = dysfunction (Clic here for more details about this article) |
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