Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0036572 (seizures)
80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Transfusion of homologous blood components is associated with immunological (incompatibility, alloimmunization, immunosuppression) and infectious risks (hepatitis, cytomegalovirus, HIV and other agents). Endoprosthetic surgery of the hip and knee frequently requires transfusion. Preoperative deposit of autologous blood can reduce homologous transfusion requirements. The simplest method is liquid storage of whole blood. In order to re-examine the efficiency of our present scheme of preoperative deposit, we studied patients scheduled for endoprosthetic surgery with respect to the amount of blood deposited, stimulation of erythropoiesis, and homologous blood requirements at the time of operation. PATIENTS AND METHODS. Sixty-seven consecutive patients (33 men, 34 women) scheduled for endoprosthetic replacement of hip or knee or for revision arthroplasty of the hip were studied. Patients with anemia, coagulopathies, coronary heart disease, severe obstructive or restrictive pulmonary disease, cerebral sclerosis, syncopes and seizures were excluded from preoperative deposit. Patients deposited 450 ml at weekly intervals, with occasionally slightly higher or lower volumes. A patient was temporarily deferred when the hemoglobin concentration prior to donation fell below 11 g/dl. Blood was collected in CPDA-1 buffer. The aim was a deposit of three units. In patients undergoing exchange reoperation of a total hip arthroplasty, intra- and postoperative autotransfusion with a cell separator was employed in addition to preoperative donation. RESULTS. The age of the patients ranged from 43 to 83 years (mean +/- SD: 61.2 +/- 9.1). The differences between men and women with respect to height (172.9 +/- 6.8 vs 160.6 +/- 7.4 cm; p less than or equal to 0.001), weight (75.7 +/- 11.2 vs 69.1 +/- 11.0 kg; p less than or equal to 0.05), calculated blood volume (p less than or equal to 0.001), and erythrocyte volume prior to donation (p less than or equal to 0.001) were significant. A total of 185 units was deposited. Men donated 1350 (450-1970) ml blood (median, range) and women 1260 (340-1450) ml (p less than or equal to 0.01). Hemoglobin concentrations decreased significantly from an average of 14.7 g/dl in men and 13.8 g/dl in women prior to donation to 13.4 g/dl and 12.3 g/dl preoperatively (p less than or equal to 0.0001 for both groups). The donation was not associated with serious complications. For 4 patients the scheduled operation was deferred for a longer term. Forty-six patients (23 men, 23 women) underwent total hip arthroplasty, 12 (4 men, 8 women) exchange of total hip arthroplasty, and 5 (3 men, 2 women) endoprosthetic knee surgery. In total hip arthroplasty men required 0 to 500 ml homologous packed red cells (median=0), women 0 to 1250 ml (median=0;p less than or equal to 0.05). Thirty-nine (69.6%) of the patients, 19 (82.6%) men and 13 (56.5%) women, did not require homologous transfusion.(ABSTRACT TRUNCATED AT 400 WORDS)
...
PMID:[Preoperative autologous blood deposit and liquid storage for replacement arthroplasty]. 258 31

Tuberous sclerosis is a neurologic disease affecting various organs with a triade: sebaceum adenoma, mental retardation and seizures. This report presents a case of a patient with tuberous sclerosis and third degree A-V block with complete invasive and non-invasive evaluation. The patient had sincope and complete A-V block with QRS complexes showing right bundle branch block morphology. The echocardiogram showed dilated cardiomyopathy with diffuse left ventricular dysfunction and had normal coronary arteriography. The eletrophysiologic evaluation showed complete infra-hisian A-V block and QRS with left bundle branch block pattern with normal sinus nodal and A-V nodal function. It was not possible to induce ventricular tachtyarrhythmias up to two extrastimuli. Histologic study showed normal myocardium under light and electronic microscopy. After permanent VVI pacemaker implant, the patient in follow-up for 16 years. This case seems to be the first in the international medical literature of tuberous sclerosis with complete heart block.
...
PMID:[Total A-V block due to tuberous sclerosis. A case report]. 262 81

Renal angiomyolipoma associated with tuberous sclerosis is well known. On the other hand, few cases of renal cell carcinoma in connection with tuberous sclerosis have been reported. We report a patient with tuberous sclerosis whose kidney was involved with renal cell carcinoma. A 18-year-old woman was first admitted in August 1987 for evaluation of left renal tumor. Diagnosis of tuberous sclerosis was made when she was 11 years old on the basis of mental retardation, papules on her face, seizures, white leaf-shaped macules and periventricular calcifications. Computerized tomographic scan demonstrated a large mass arising from the left kidney and small masses in the right kidney. Angiography confirmed bilateral hypervascular renal tumors. On these bases, a clinical diagnosis of bilateral renal angiomyolipomas was made and surgical treatment of the left kidney was recommended because of its large size. However, her parents did not permit treatment until March, 1988. Finally, left nephrectomy of 4,750 g was performed and histological examination revealed renal cell carcinoma with clusters of spindle cells. In the literature available to us, we found twelve reports of malignant renal tumors associated with tuberous sclerosis including five renal cell carcinomas in Japan.
...
PMID:[Renal cell carcinoma associated with tuberous sclerosis: a case report]. 269 34

A 5-year-old boy with the epidermal nevus syndrome and hemimegalencephaly is reported. He had pigmented nevi on the forehead and neck, and hemihypertrophy of the body from the birth. He developed intractable seizures, mental retardation, and right hemiparesis. His seizure pattern changed from early infantile epileptic encephalopathy to infantile spasms at 2 months of age. Electroencephalograms showed a suppression-burst pattern in the neonatal period, subsequently changing to hypsarrhythmia. Computerized tomography of the brain disclosed slight dilatation of the posterior horn of the lateral ventricle at the age of 2 months. Later, hemimegalencephaly with calcification on the left side of the brain was noted. Histological examination of the pigmented nevus on the neck showed it to be an acanthosis nigricans-like lesion. Clinical differences between tuberous sclerosis and epidermal nevus syndrome with hemimegalencephaly are discussed.
...
PMID:Epidermal nevus syndrome with hemimegalencephaly: a clinical report of a case with acanthosis nigricans-like nevi on the face and neck, hemimegalencephaly, and hemihypertrophy of the body. 275 Oct 68

1. Tuberous sclerosis is a hereditary disorder characterized by mental retardation, epilepsy, skin papules, and multiple developmental tumors, which involve every organ of the body including the eye. 2. Treatment is directed to control seizures and eliminate surface and systemic lesions by dermabrasion and surgery. 3. Genetic counseling is important for patients, their parents, and their siblings.
...
PMID:Tuberous sclerosis (Bourneville's disease). 276 69

Six patients with tuberous sclerosis were evaluated with computed cranial tomography (CCT) and magnetic resonance imaging (MRI). The results were assessed in comparison with the clinical severity of the disease. The brain lesions were shown by MRI as low-signal areas on IR images (T1 weighted sequences) and high-signal areas on SE images (T2 weighted sequences) Three patients, who had severe psychomotor retardation (DQ less than 70) and intractable epileptic seizures following infantile spasms, had many cortical and subcortical lesions. In the other three patients, intelligence was normal or slightly retarded (DQ or IQ greater than 70) and epileptic seizures were well controlled, and small subependymal lesions were observed. Cortical lesions were rare. These results indicate that MRI can detect more precisely intracranial lesions in tuberous sclerosis.
...
PMID:[Magnetic resonance imaging of intracranial lesions of tuberous sclerosis]. 278 56

A follow-up study was performed on 40 children with tuberous sclerosis and epilepsy. In 68% of the patients with infantile spasms and 73% of those without them, complex partial seizures were observed. Thus, patients with tuberous sclerosis are subject to not only infantile spasms but also complex partial seizures. Patients with infantile spasms showed mental retardation more often than those without. Seizures which had evolved from infantile spasms were more difficult to control than those which had not. The prognosis of infantile spasms associated with tuberous sclerosis was better than that of prenatal group other than tuberous sclerosis. Forty percent of the 40 cases showed asymmetry on EEG, and the asymmetry tended to disappear as the patients grew up.
...
PMID:Long-term prognosis of tuberous sclerosis with epilepsy in children. 311 77

Six children affected by tuberous sclerosis and severe multifocal epilepsy were studied by MRI. In all cases, MRI disclosed multiple cortical lesions and the relationship between these lesions and the EEG localization of epileptogenic foci has been studied. In all cases, a relationship between the localization of persistent EEG foci and the site of the major MRI lesions was found. The presence of frontal cortical tubers, mainly localized in the parasagittal region, was related to more intractable seizures.
...
PMID:[Magnetic resonance imaging in Bourneville's disease: relation to the EEG]. 318 65

The data emerging from our study are the following: the presence of an identifiable cause is important: complications like tuberous sclerosis or signs of marked cerebral damage represent an adverse risk factor for IE. The presence of epilepsy among relatives, evidence of pre- or perinatal cerebral damage, mental retardation, and early onset, long periods of uncontrolled seizures before starting an adequate therapy and frequency of seizures appear to be indicative of an adverse prognosis, since differences between the two groups of responsive or unresponsive patients are statistically significant. On the contrary, the occurrence of febrile convulsions in the past history does not seem to have an adverse prognosis. Temporal lobe epilepsy and IS bear the worst prognosis. ME, CPS, GTCS, SPS, LGS and PM have a progressively better outcome in responsiveness to AEDs. Concerning therapy in patients with IE, studies indicate the results of high dose monotherapy appear to be equal or better than with polypharmacy. Because of the gravity of the situation, trials with unconventional drugs have been performed, but it is too early to draw definite conclusions about the long-term usefulness of most of them. In conclusion, our data indicate that the appearance of an IE can be predicted utilizing the above mentioned criteria, considered either alone or in combination. The issue of IE remains undoubtedly an important one among the group of convulsive disorders. Further studies considering a greater number of patients and new therpeutic strategies are to be recommended.
...
PMID:Intractable epilepsy: etiology, risk factors and treatment. 329 46

Tuberous sclerosis is a multisystem disorder characterized by changes primarily involving the skin, eye, and central nervous system. Although the disease often produces mental retardation and seizures, this is not universal, and some patients with tuberous sclerosis lead a relatively normal life.
...
PMID:Tuberous sclerosis. 330 30


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>