1/28. Progressive pulmonary vascular disease after pulmonary artery banding and total correction in a case of ventricular septal defect and pulmonary hypertension.A 7-month-old infant with ventricular septal defect and pulmonary hypertension underwent pulmonary artery banding, which resulted in a decrease in the pulmonary arterial peak pressure from 102 to 54 mmHg. lung biopsy findings showed at most an early grade 3 Heath-Edwards classification, and an index of pulmonary vascular disease of 1.4, both of which indicated operability for total correction. Small pulmonary arteries less than 100 microns in diameter, however, showed marked hydropic changes in the medial smooth muscle cells. Total correction was performed at the age of 2 years, but the pulmonary arterial pressure failed to decrease. A lung biopsy taken just after the closure of the ventricular septal defect contraindicated operability due to progressive pulmonary vascular disease at a grade 6 Heath-Edwards classification and an index of pulmonary vascular disease of 2.4. The patient died at 8 months after the operation, and an autopsy revealed still more advanced pulmonary vascular disease at a grade 6 Heath-Edwards classification and an index of pulmonary vascular disease of 2.8. The pathogenesis of arterial changes is discussed.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
2/28. Congenital hypertrophy of the lateral nail folds of the hallux: clinical features and follow-up of seven cases.Congenital hypertrophy of the nail folds of the hallux describes an abnormality of the periungual soft tissues of the big toe characterized by hypertrophy of the nail fold which partially covers the nail plate and is frequently associated with inflammation and pain due to an ingrowing nail. We describe the clinical picture and follow-up of seven patients with this abnormality. In three patients the affected toe showed an asymptomatic, dome-shaped, hypertrophic lip that partially covered the nail plate. Four patients had acute inflammatory changes due to toenail ingrowth, with considerable swelling and reddening of the hypertrophic lip that was painful on pressure. Topical treatment with steroids was useful to reduce inflammation and produced persistent remission in two patients. Follow-up showed a spontaneous disappearance of the hypertrophic nail fold in one of the seven patients. In two patients the hypertrophic lip partially regressed, but remained clearly visible, while in two patients it remained unchanged. In two patients surgical correction of the soft tissue abnormality was necessary due to painful nail ingrowth unresponsive to topical treatment.- - - - - - - - - - ranking = 0.5keywords = pressure (Clic here for more details about this article) |
3/28. Hypertrophic chronic pachymeningitis as a localized immune process in the craniocervical region.Hypertrophic chronic pachymeningitis (HCP) is a rare disorder that causes intracranial or spinal thickening of the dura mater. This report describes a patient with progressive HCP in the craniocervical region associated with signs of rheumatic disease. A ventricular-atrial shunt had to be inserted because of increased intracranial pressure. The patient improved after suboccipital craniotomy, C1 to C6 laminectomy, and removal of the thickened dura. Additional therapy with methotrexate stopped progression, which was documented by MRI and PET.- - - - - - - - - - ranking = 0.5keywords = pressure (Clic here for more details about this article) |
4/28. Bilateral optic disk edema and blindness as initial presentation of acute lymphocytic leukemia.PURPOSE: To report bilateral optic disk edema and blindness as the unusual initial presentation of acute lymphocytic leukemia (ALL) in an adult. methods: A 19-year-old man presented with a history of headaches, back pain, and 10 days of worsening vision that progressed to blindness. Ocular examination revealed light perception acuity in the right eye and no light perception in the left eye. Fundus examination revealed bilateral profound optic disk edema, tortuous vessels, and retinal hemorrhages. Acute lymphocytic leukemia was diagnosed with complete blood count and bone marrow biopsy. head computed tomography and magnetic resonance imaging, were normal. Lumbar puncture revealed normal opening pressure. Ocular ultrasonography showed bilateral optic nerve enlargement. DESIGN: Interventional case report and literature review. ESULTS: The presumptive diagnosis of leukemic infiltration of the optic nerves was made, and urgent radiotherapy, intrathecal methotrexate, and intravenous daunorubicin were instituted. visual acuity improved to hand motions in the right eye. CONCLUSIONS: Acute lymphocytic leukemia can rarely present in adults as visual changes due to leukemic optic nerve infiltration. radiation treatment should be considered as an urgent treatment modality for this rare condition.- - - - - - - - - - ranking = 0.5keywords = pressure (Clic here for more details about this article) |
5/28. Sonographic detection of diffuse peripheral nerve enlargement in hereditary neuropathy with liability to pressure palsies.Hereditary neuropathy with liability to pressure palsies (HNPP) is a disorder characterized by a tendency to develop focal neuropathies after trivial traumas. On teased nerve fiber studies, sausage-shaped myelin sheath swellings (tomacula) are found. We report the sonographic findings in a patient with genetically proven HNPP. We were able to demonstrate enlargement of several peripheral nerves, even nerves that were clinically unaffected. Enlargement was found not only at typical nerve entrapment sites but also outside these sites. This diffuse nerve enlargement may play an important role in the pathogenesis of entrapment neuropathies in HNPP patients.- - - - - - - - - - ranking = 2.5keywords = pressure (Clic here for more details about this article) |
6/28. Disproportionate hypertrophy of the interventricular septum and its regression in Cushing's syndrome. Report of three cases.Three patients of Cushing's syndrome with severe disproportionate hypertrophy of the interventricular septum are reported. All three underwent adrenalectomy and the diagnosis of Cushing's syndrome was confirmed by the presence of adrenal adenoma. All three showed hypertension before the adrenalectomy and two remained hypertensive following adrenalectomy. Before the operations the electrocardiograms revealed inverted T waves in broad leads, and mechanocardiogram systolic and diastolic dysfunction. After the adrenalectomies, abnormal findings on electrocardiograms were normalized and septal hypertrophy was completely regressed. It appears that not only high aortic pressure but also excessive plasma cortisol may be an etiologic factor of the left ventricular hypertrophy in Cushing's syndrome.- - - - - - - - - - ranking = 0.5keywords = pressure (Clic here for more details about this article) |
7/28. Pre-operative magnetic resonance imaging in Lhermitte-Duclos disease.A 30-year-old woman presented with a 2.5 year history of symptoms and signs of raised intracranial pressure and unsteadiness of gait. CT showed a non-enhancing hypodense mass lesion in the left cerebellar hemisphere with compression and displacement of the fourth ventricle to the right and hydrocephalus of the third and lateral ventricles. magnetic resonance imaging showed increased signal intensity in the mass lesion with the preservation of the gyral pattern on T2 weighted sequence. Imaging after gadolinium-DTPA injection showed non-enhancement and slight hypointensity of the lesion on T1 weighted sequence. These MRI findings are helpful in the preoperative diagnosis of Lhermitte-Duclos disease and allow the planning of an appropriate line of treatment for this rare condition.- - - - - - - - - - ranking = 0.5keywords = pressure (Clic here for more details about this article) |
8/28. Obstructive sleep apnea leading to increased intracranial pressure in a patient with hydrocephalus and syringomyelia.Rises in intracranial pressure from normal baseline values up to 50 cm H2O occurred shortly after the onset of obstructive sleep apnea in a patient with myelomeningocele, hydrocephalus, arnold-chiari malformation, and syringomyelia. Tonsillar hypertrophy caused the airway obstruction during sleep, because the obstructive sleep apnea and also the periodic elevation of intracranial pressure disappeared after tonsillectomy. Only one report from japan has previously described three patients with elevated cerebrospinal fluid pressures during obstructive sleep apnea. It is conceivable that episodic airway obstruction and concurrent intracranial hypertension may have contributed to the development of syringomyelia in our patient.- - - - - - - - - - ranking = 3.5keywords = pressure (Clic here for more details about this article) |
9/28. Glucocorticoid-suppressible hyperaldosteronism. Ultrastructural observation of a case.A 56-year-old woman presented with intracranial hemorrhage. Laboratory examinations revealed severe hypertension, hypokalemia, elevated aldosterone excretion, and suppressed plasma renin activity. Left adrenocortical tumor was suspected and adrenalectomy was performed. The laboratory data after operation, however, showed no significant difference from the preoperative data. On the basis of further examinations, dexamethasone was administered and returned blood pressure to normal, and also normalized serum potassium, plasma aldosterone, and renin activity. The patient's illness was diagnosed as glucocorticoid-suppressible hyperaldosteronism. light microscopically, the zona glomerulosa was hypertrophic and the outer zona fasciculata decreased in lipid droplets and was centrifugally arranged in small alveoli. Electronmicroscopically, the cells of the outer zona fasciculata had several lipid droplets and well-developed sER. mitochondria were round to oval with lamellar or lamellovesicular cristae. These findings were evidence of hyperfunction. The cytoplasm of the cells also contained spironolactone bodies. Therefore, it is assumed that the aldosterone, which induced the disorder, was produced mainly in the outer zona fasciculata.- - - - - - - - - - ranking = 0.5keywords = pressure (Clic here for more details about this article) |
10/28. Respiratory muscle weakness and ventilatory failure in AL amyloidosis with muscular pseudohypertrophy.Generalized muscle weakness culminating in ventilatory failure developed in a 59-year-old man with kappa light chain multiple myeloma. physical examination demonstrated skeletal muscle enlargement, severe proximal muscle weakness, and macroglossia, consistent with amyloid-associated muscle pseudohypertrophy. Pulmonary function studies revealed a severe restrictive abnormality with a low maximal inspiratory pressure and maximal voluntary ventilation. Arterial blood gas values and chest radiographic results were normal. There was no clinical evidence of cardiac or central nervous system disease. At autopsy, skeletal muscles and diaphragm were diffusely infiltrated by amyloid. There was also multifocal deposition of amyloid in alveolar septae, esophagus, and subendocardium. This report suggests that ventilatory failure may occur as a complication of myeloma-associated (AL) amyloidosis involving the respiratory muscles.- - - - - - - - - - ranking = 0.5keywords = pressure (Clic here for more details about this article) |
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