Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0033377 (prolapse)
11,717 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The involvement of the central nervous system in acute leukemia was studied in 15 adult patient. 60% of the patients were up to 35 years of age and 12 of them (80%) were with acute lymphoblastic leukemia. In 12 of the patients the nervous system was affected in the acute course of the disease, in one female patients--in the course of induction treatment and in the remaining patients--after the induction treatment. The manifestations of neuroleukemia are predominantly meningoradicular irritation with headache--in 86.7% of the patients, diplopia, papilledema, dysarthria and palpebral ptosis with affection of the cranial nerves (III, IV, V, VI, VII, VIII, IX, XII). In 12 patients (87.5%) blast cells were found in the cerebrospinal fluid in numbers ranging from 70 up to 36,000/mm3. In 26.7% of the patients other extramedullary localizations of leukemia were found parallelly (testes, thyroid gland hypopharynx, muscles). The application of methotrexate intrathecally and radiation therapy of the cranium led to a remission in 43% of the patients. Favourable results with considerably prolonged survival can be achieved also in patients with many recurrences of the disease. The need of neuroleukemia prophylaxis is proved (it is obligatory for the patients with acute lymphatic leukemia and with some forms of acute myeloblastic leukemia).
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PMID:[Extramedullary localization of acute leukemia. I. The involvement of the central nervous system]. 316 77

Light and electronmicroscopic findings in two cases. Neuropediatrics 12: 215-31 (1981). Two cases of infantile spinal muscular atrophy (Werdnig-Hoffmann disease) are described in unrelated children deceased at 11 months (acute clinical onset at 6 months) and 2 years (onset at birth). Severe respiratory difficulties, hypotonia, muscular weakness and depressed tendon reflexes were the main clinical features. Bulbar palsy, bilateral ptosis, pale optic discs and atactic movements of the hands were observed in the child deceased at 11 months. Besides severe loss of anterior horn cells and neurogenic muscle atrophy there was evidence of an extensive sensory involvement in both cases. Shrinkage, vacuolation as well as chromatolytic changes of dorsal root ganglion cells, together with the evidence of a primary axonal damage in sural nerve biopsies were interpreted in terms of ganglioneuropathy of the primary sensory neurons. An invasion of fibrous astrocytes into dorsal roots constituted another striking anomaly in one case as well as a pronounced degeneration of cranial nerves V and VIII in the other case, a finding not hitherto reported in Werdnig-Hoffmann disease.
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PMID:Sensory ganglioneuropathy in infantile spinal muscular atrophy. Light and electronmicroscopic findings in two cases. 729 Mar 43

We performed an open heart surgery for ventricular septal defect and right coronary cusp prolapse in a 5-year-old boy with hemophilia A. He has only 3.5% of factor VIII activity. This paper describes a successful perioperative management of hemophilia A. Previous infusion study of factor VIII concentrate showed an administration of 500 units of factor VIII increased its level from 3.5 to 25% two hours after the infusion. With this result we administered 1,500 units of concentrate 14 and 2 hours before the operation, factor VIII level was 201% after the induction of anesthesia. Sternotomy was uneventfully performed. Subarterial VSD was patch closed and right coronary cusp hung down into the defect was plicated. After intra cardiac repair, we administered 2,000 units of VIII factor concentrate, followed by an injection of protamin and its level was 240% at CCU. During the initial 3 postoperative day, patient was maintained with 1,500 units of factor VIII every 12 hours. Chest drains were removed on 2 postoperative day with no evidence of bleeding. Additional infusion of 1,000 units per a day was continued on postoperative days 4 through 17th. Subsequently factor VIII level was maintained more than 60%, so that we achieved successful perioperative course in a patient with hemophilia A.
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PMID:[Perioperative management of ventricular septal defect and right coronary cusp prolapse in a patient with hemophilia A]. 783 Mar 56

66-year-old female patient presents palpebral bilateral ptosis and external ophthalmoplegia with a progressive evolution. The clinical aspects are completed with the affectation of the sensitive and motor trigemen, with the masseter atrophy and bilateral trophic keratitis. The affectation of the facial and acoustic nerves was associated with bilateral perception surditate. The hereditary transmission was dominant, with incomplete penetration. The neurogenic pathogeny was sustained on the basis of the association of neurological multilesions with III, IV, V, VI, VII, VIII nerves, with progressive extension.
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PMID:[Progressive nuclear external ophthalmoplegia]. 828 17