1/86. 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) |
2/86. Pulmonary hypertension secondary to systemic lupus erythematosus: prolonged survival following treatment with intermittent low dose iloprost.Pulmonary hypertension (PHT) associated with systemic lupus erythematosus (SLE) has a dismal prognosis. Vasodilators and immunosuppressive therapy have been tried over the years with discouraging results. Prostacyclin (PGI2) which has potent vasodilatatory and anti-platelet effects has been demonstrated to significantly decrease pulmonary arterial pressure and pulmonary vascular resistance during acute infusion. Satisfactory response has been reported in SLE patients with PHT treated with short-term intravenous continuous PGI2 infusion. We report here a 48-month experience of the use of monthly low dose infusion of a PGI2 analogue, iloprost, in a SLE patient with pulmonary hypertension in new york Heart association functional Class III. There was an initial haemodynamic response to an acute infusion of iloprost. Repeated infusions were followed by marked improvement in her functional status and her mean pulmonary arterial pressure dropped from 80 mmHg in the first few months and remained static at around 55 mmHg for the subsequent years.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
3/86. blindness from bad bones.Progressive visual loss is the most common neurologic finding in osteopetrosis. Several mechanisms may explain this phenomenon, including compression of the optic nerves caused by bony overgrowth of the optic canals and retinal degeneration. We report a child with osteopetrosis and progressive visual loss, even though patent optic canals were demonstrated by computed tomography and digital holography. This patient's visual loss was caused by increased intracranial pressure secondary, to obstruction of cerebral venous outflow at the jugular foramen. This case points to the importance of a full evaluation of the skull base foramina in the diagnostic workup of visual loss in patients with osteopetrosis.- - - - - - - - - - ranking = 0.5keywords = pressure (Clic here for more details about this article) |
4/86. Unilateral capsular glaucoma after long-standing bilateral pigmentary glaucoma.In 1964 we treated a 42-year-old woman diagnosed to have classical bilateral pigment dispersion syndrome combined with an intraocular pressure (IOP) of up to 28 mmHg in both eyes. The patient now has a daughter, also with pigment dispersion syndrome. Miotic treatment brought the IOPs to normal, and 8 years later when the disease was in the inactive phase all treatment could be discontinued. At the age of 67 years, exfoliation deposits became visible in the right eye only, but the IOPs were still below 21 mmHg. Four years later, the pressure of the right eye rose to 31 mmHg. During the next few years all available IOP-lowering medications and laser trabeculoplasty failed, and finally trabeculectomy had to be performed to keep the pressure of the right eye under control and to halt visual field changes, which had already appeared. This case illustrates that development of exfoliation syndrome may take place irrespective of pigment dispersion, and that their simultaneous occurrence may lead to an IOP rise that is resistant to medical therapy and laser trabeculoplasty. It additionally provides further clues to the pathogenesis of capsular glaucoma.- - - - - - - - - - ranking = 1.5keywords = pressure (Clic here for more details about this article) |
5/86. Juvenile onset primary open-angle glaucoma: three case studies and review.BACKGROUND: Common clinical characteristics of juvenile onset primary open-angle glaucoma (JPOAG) include increased intraocular pressure, optic nervehead damage, visual-field loss, and a normal-appearing iridocorneal angle by gonioscopy. Histologic analysis of the angle structures may show varying developmental abnormalities that are observed to be less obvious as age of onset increases. Individuals who are African-American, male in gender, and myopio, are at highest risk--particularly if a positive family history exists. A genetic locus for juvenile onset primary open-angle glaucoma has been isolated to the long arm of chromosome 1. case reports: Three separate cases of JPOAG diagnosed in a 16-year-old African-American girl, a 9-year-old African-American boy, and a 28-year-old African-American woman are presented and reviewed. The symptoms, clinical presentation, disease progression, and treatment options are discussed in detail. CONCLUSION: Early recognition of this disease is possible through screening on all patients--particularly those who exhibit increased cupping with increased intraocular pressures and risk factors, such as youthful age, male gender, myopic refractive error, and African-American heritage with a positive family history of glaucoma. family members should also be examined.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
6/86. Progressive splenomegaly after epoprostenol therapy in portopulmonary hypertension.patients with end-stage liver failure, portal hypertension, and associated pulmonary artery hypertension (portopulmonary hypertension [PPHTN]) have a high mortality when undergoing liver transplantation. Successful transplantation in these patients may depend on efforts to reduce pulmonary artery pressure (PAP). To this end, a number of centers are using a continuous intravenous (IV) infusion of epoprostenol, which has been shown to improve symptoms, extend life span, and reduce PAP in patients with primary pulmonary hypertension. We report four cases in which treatment of patients with PPHTN with continuous IV epoprostenol was followed by the development of progressive splenomegaly, with worsening thrombocytopenia and leukopenia. This finding may limit the usefulness of epoprostenol in PPHTN and influence the timing of transplantation in such patients.- - - - - - - - - - ranking = 0.5keywords = pressure (Clic here for more details about this article) |
7/86. Diagnosing and treating primary pulmonary hypertension.Primary pulmonary hypertension (PPH) is a pulmonary vascular disease characterized by an elevation in mean pulmonary artery pressure and pulmonary vascular resistance. Recently, PPH gained national attention because of its association with appetite suppressants. PPH may also be associated with pregnancy, hypothyroidism, autoimmune disorders, human immunodeficiency virus infection, and the use of drugs such as oral contraceptives and cocaine. patients with PPH may report dyspnea on exertion and fatigue. early diagnosis is crucial. New therapeutic regimens have dramatically reduced mortality rates and improved quality of life by halting the progression of pulmonary vascular remodeling and averting right-sided heart failure. These therapies include high-dose calcium channel antagonists, anticoagulants, and continuous intravenous prostacyclin. Lung or heart-lung transplantation remains a viable therapeutic option for patients who are treated late in the disease process, who are not responsive to medical management, or who remain symptomatic and continue to deteriorate.- - - - - - - - - - ranking = 0.5keywords = pressure (Clic here for more details about this article) |
8/86. Progression of first degree atrioventricular block to second degree Mobitz type I block during spinal anesthesia associated with induced hypertension.A case is presented in which an elderly patient with preexisting first degree atrioventricular (AV) block progressed to second degree Mobitz Type I AV block during spinal anesthesia and associated with hypertension induced by a pure alpha 1 agonist. Second degree AV block caused by increased vagal tone was transient, which resolved as the blood pressure normalized. hypotension due to spinal anesthesia treated with pure alpha 1 agonist can increase AV block in patient with pre-existing first degree heart block.- - - - - - - - - - ranking = 0.5keywords = pressure (Clic here for more details about this article) |
9/86. Visual loss in idiopathic intracranial hypertension after resolution of papilledema.PURPOSE: To demonstrate that progressive visual field loss may occur after resolution of papilledema in patients with idiopathic intracranial hypertension and persistently elevated intracranial pressure. methods: A patient with idiopathic intracranial hypertension was evaluated with serial Humphrey automated static perimetry after initial treatment and resolution of papilledema. RESULTS: The patient developed recurrent headache and elevated cerebrospinal fluid pressure. optic nerve head appearance did not change. Automated perimetry demonstrated reproducible, worsening visual field loss; mean deviation decreased 11 dB in each eye. Visual field defects resolved after optic nerve sheath fenestration. CONCLUSIONS: Increased intracranial pressure caused visual field loss after resolution of papilledema. optic nerve sheath fenestration improved visual function in this patient.- - - - - - - - - - ranking = 1.5keywords = pressure (Clic here for more details about this article) |
10/86. Progressive bone resorption after pathological fracture of the femoral neck in Hunter's syndrome.We report a case of Hunter's syndrome associated with a transverse fracture of the left femoral neck after minor trauma, followed by progressive resorption of the femoral head at 12 years of age and a stress fracture of the right femoral neck at 16 years of age. MRI performed at 15 years of age revealed intra-articular low intensity on T1-weighted and T2-weighted images of both hip joints. The MR finding may represent fibrous synovial thickening, which caused pressure erosion of the femoral neck, resultant pathological and/or stress fractures, and subsequent osteonecrosis with rapid absorption of the femoral head.- - - - - - - - - - ranking = 0.5keywords = pressure (Clic here for more details about this article) |
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