Cases reported "Syndrome"

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1/34. Healing of skin necrosis and regression of anticardiolipin antibodies achieved by parathyroidectomy in a dialyzed woman with calcific uremic arteriolopathy.

    AIM: To present the impact of parathyroidectomy on the spontaneous healing of necrotic lesions of the skin of the lower leg and on anticardiolipin antibodies regression in a 68-year-old female dialyzed patient with hyperparathyroidism and calcific-uremic arteriolopathy (CUA). methods: After the occurrence of initial lesions of the lower leg skin, the intact parathyroid (iPTH) level, calcium (Ca) and phosphorus (P) product were measured, and on two occasions at 6-week intervals, the titer of anticardiolipin antibodies was determined, followed by a clinical monitoring of the progress of necrotic skin lesions. Two months after the occurrence of the skin lesions, the patient's right leg was amputated below the knee due to gangrene, and a histopathological analysis of the skin tissue sample of the amputated lower leg was made. After parathyroidectomy, iPTH, Ca x P product were measured, and on two occasions at 6 weeks' intervals, anticardiolipin antibodies titer was determined, followed by a clinical monitoring of lesions of the left lower leg skin. RESULTS: Before parathyroidectomy, iPTH level and Ca x P product were increased, as well as IgG anticardiolipin antibody titer measured on two occasions 6 weeks apart. The histopathological analysis of the skin tissue sample of the amputated right lower leg showed mural calcification of artery walls and thrombotic occlusions of small arteries, arterioles, and dermal capillaries, in addition to epidermolysis. A week after parathyroidectomy, iPTH level and Ca x P product were within normal range. Two measurements 6 weeks apart revealed no anticardiolipin antibodies. Eight weeks after parathyroidectomy, spontaneous healing of necrotic skin lesions of the left lower leg was observed. CONCLUSION: Regression of anticardiolipin antibodies, normalization of Ca x P product, and healing of the skin lesions after parathyroidectomy all pointed to the elevated PTH level as a crucial factor in the pathogenesis of CUA.
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2/34. Acute glaucoma in hemorrhagic fever with renal syndrome (nephropathia epidemica).

    Bilateral eyelid edema, chemosis, conjunctival injection and hemorrhages, shallowing of the anterior chamber with transitory myopia, and acute glaucoma were observed in three patients with hemorrhagic fever with renal syndrome (nephropathia epidemica). One patient had anterior uveitis with posterior synechiae. Two patients had congestion of retinal venules and retinal edema, and one patient had small retinal hemorrhages. fluorescein angiography revealed early leakage of the conjunctival capillaries and delayed filling of the lower retinal arterioles with retinal edema. The acute glaucoma subsided rapidly. The myopia disappeared slowly with a gradual deepening of the anterior chamber after onset of the diuretic phase of the illness. In nephropathia epidemica, the transitory shallowing of the anterior chamber, myopia, and acute glaucoma are due to lesions in the small blood vessels of the ciliary body, resulting in its swelling, with relaxation of the zonules and forward movement of the lens.
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3/34. susac syndrome: report of four cases and review of the literature.

    susac syndrome is a rare disease of unknown pathogenesis. It is caused by a microangiopathy affecting the arterioles of the brain, retina, and cochlea, giving the classic clinical triad of subacute encephalopathy, visual loss secondary to retinal branch occlusions, and sensorineural hearing loss. The features of four cases of this syndrome are presented. MR imaging, retinal fluorescein angiography, and audiography findings enable diagnosis. Early therapy may reduce sequelae and improve recovery.
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4/34. Blue rubber bleb nevus syndrome and pulmonary hypertension: an unusual association.

    INTRODUCTION: Blue rubber bleb nevus syndrome (BRBNS) is a rare congenital systemic angiodysplasia with multiple vascular malformations in the skin, gastrointestinal tract and, less often, in other internal organs and the brain. CASE REPORT: A 36-year-old man with past history of BRBNS was admitted to our hospital for progressive dyspnea and fatigue. Primary pulmonary hypertension (PPH) was diagnosed. He then developed acute abdominal pain and dyspnea, dying in a few hours due to sudden cardiac arrest. Postmortem examination demonstrated angiomatous lesions located in the skin, small bowel, heart, lungs, liver and thyroid. The lesions were slightly raised, soft and compressible and microscopically consisted of dilated vascular channels lined by a flattened endothelium. The vascular wall was formed by several layers of smooth muscle cells, intermixed with abundant aggregates of elastic lamellae and thin collagen fibers. Luminal thrombi were a frequent finding. In the small bowel, we identified the presence of an abnormally large artery directly opening into a thin-walled venous channel. The most striking finding in the lungs was the presence of thrombi of varying age in the lumen of segmental and elastic arteries, as well as muscular arteries and arterioles. Severe medial hypertrophy of muscular arteries and muscolarization of arterioles were also present. Intimal proliferative lesions and plexiform lesions were never observed. CONCLUSION: The pulmonary findings are consistent with recurrent thromboembolic events from shunts in the visceral lesions. To our knowledge, this is the first report of BRBNS with visceral arterovenous (AV) fistulae complicated by thromboembolic pulmonary hypertension (PH).
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5/34. MELAS of infantile onset: mitochondrial angiopathy or cytopathy?

    An 83-day-old male infant had convulsions, hypertrophic cardiomyopathy, and lactic acidosis. Cranial computed tomography revealed low-density areas in both parieto-occipital lobes and in the left temporal lobe. Muscle biopsy did not reveal ragged-red fibers, but abnormal mitochondria were found in the capillary endothelial cells as well as in the muscle fibers. At 5 months of age, the patient developed purpura on the soles and palms. skin biopsy showed degeneration of the endothelial cells with abnormal mitochondria in the arterioles and capillaries. Myelinated nerves in the skin had vacuolated axons with swollen mitochondria, and their myelin sheaths showed vacuolation. At 9 months of age, he died of heart failure, and autopsy revealed abnormal mitochondria in the myocardium but not in the coronary vessels. Our findings indicate that the symptoms of the mitochondrial encephalopathy, myopathy, lactic acidosis, and strokelike episodes (MELAS) syndrome cannot be fully explained by the mitochondrial angiopathy alone.
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6/34. Systemic and renal fibrinolytic activity in a patient with anticardiolipin syndrome and renal thrombotic microangiopathy.

    A 24-year-old white woman with a past history of recurrent venous thromboses of the lower extremities was admitted for hypertension and renal failure. She had a chronic cutaneous ulcer on the anterior side of the left leg and oral ulcers of the palatum. Laboratory tests demonstrated rapidly progressive renal failure and the presence of an anticardiolipin antibody (ELISA). thrombosis of the inferior vena cava was shown by phlebocavography. Renal biopsy revealed typical thrombotic microangiopathy. Tissue-type plasminogen activator (tPA) was visualized by immunofluorescence in endothelial cells of renal arterioles and glomeruli. Normal plasma levels of tPA, urokinase and plasminogen activator inhibitor 1 were found by ELISA, and tPA antigen levels rose after desmopressin acetate infusion. Thus, in this case, the diffuse thrombotic process was not related to defective circulating or renal fibrinolytic systems and could be promoted by the procoagulant effect of antiphospholipid antibodies.
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7/34. Pseudo-Foster Kennedy syndrome in a patient with anterior ischemic optic neuropathy and a nonbasal glioma.

    A 49-year-old woman with a 6-year history of headaches was found to have a pale right optic disc with narrowed retinal arterioles and a congested left optic disc. Her visual acuity was 20/20 in each eye with normal visual fields in May 1983. These findings were attributed to a previous attack of non-arteritic anterior ischemic optic neuropathy (AION). She had a normal neurologic examination and a normal head computed tomographic (CT) scan performed 2 years prior to her initial ophthalmologic evaluation. She was followed over the next 2 years without change in her fundus examination. In December 1987, after a generalized tonic-clonic seizure, she was found to have a large right frontoparietal mass without direct impingement on the optic nerves, or chiasm on neuroradiological studies. At this time she developed marked papilledema in the left eye with a pale optic disc in the right eye remaining unchanged. Histopathological diagnosis of malignant glioma was made. Two diseases, ischemic optic neuropathy and glioma, in one patient represents a bizarre example of the pseudo-Foster Kennedy syndrome.
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keywords = arteriole
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8/34. Quadrantic venous-stasis retinopathy secondary to an embolic branch retinal artery obstruction.

    Venous-stasis retinopathy is a term used to describe the posterior segment findings of the ocular ischemic syndrome. These include midperipheral blot retinal hemorrhages, dilated retinal veins, attenuated arterioles, peripheral retinal microaneurysms, macular edema, as well as retinal and optic disc neovascularization. The authors recently evaluated a 71-year-old woman who presented with an asymptomatic calcific embolus in her right infero-temporal branch retinal artery. Classic venous-stasis retinopathy that was limited to the territory of the obstructed arteriole was present concurrently. This case appears to demonstrate that chronic retinal hypoperfusion and resultant venous-stasis retinopathy can be produced by not only high-grade, fixed stenosis but also by embolic disease.
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keywords = arteriole
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9/34. Neuro-ophthalmic presentation of cone dysfunction syndromes in the adult.

    Cone dysfunction syndromes are probably part of the spectrum of cone-rod degenerations and can present with widely varying clinical pictures. Thus, although the age of onset is usually before the third decade, patients can present at any age, and, although family history is usually positive, in typical cases it may be quite negative. patients can have initially very subtle, bizarre, or poorly described visual complaints so that numerous examiners may label them "functional" or "malingering." They can present with the classic symptoms of hemeralopia, poor acuity, and reduced color vision, but these complaints may be absent. visual acuity and color vision can be normal or severely reduced and the fundi may show classic changes such as bulls-eye maculopathy, macular choroidal atrophy, pigment clumping in the maculae, mild peripheral pigmentary changes, or a fundus flavimaculata-like change. The patients here reported were considered as having normal fundi by several competent ophthalmologists as a rule, however. visual fields can vary from normal to ring scotomas, central scotomas, and other interesting types of defects, even simulating a hemianopia. Although involvement is usually symmetrical between the two eyes, this is not always the case, and one of our patients had a strictly uniocular cone dystrophy. Cone dysfunction can be considered in a patient of any age even with normal acuity, good color vision, and a normal ophthalmoscopic examination. A high index of suspicion should prompt specific questioning about hemeralopia, or reduced visual function in brightly illuminated situations, and better vision in twilight or under dim illumination. patients may falsely describe hemeralopia as "glare" or "photophobia." Careful testing of color vision, a meticulous tangent screen examination, and specifically looking for diffuse narrowing of retinal arterioles in a patient with an otherwise normal fundus appearance will usually suffice to prompt the clinician to order electroretinography, which is the definitive diagnostic criterion for the cone dystrophies. It is important to consider this diagnosis before embarking on an otherwise fruitless and expensive neuroimaging investigation.
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keywords = arteriole
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10/34. Facioscapulohumeral dystrophy associated with sensorineural hearing loss, tortuosity of retinal arterioles, and an early onset and rapid progression of respiratory failure.

    Two sibling cases of facioscapulohumeral dystrophy (FSHD) are described. One was characterized by sensorineural hearing loss, marked tortuosity of retinal arterioles, an early onset and progression of severe restrictive-type pulmonary dysfunction, and cor pulmonale. The other had a mild course of FSHD without involvement of any other organ than muscles at the time of diagnosis. Recently, a new nosological entity of FSHD, with sensorineural hearing loss and tortuosity of retinal arterioles, was advocated. Our cases, especially the first case, seem to belong to this newly recognized entity of FSHD. Moreover, it is noteworthy that our first case exhibited rapid aggravation of severe restrictive-type respiratory failure and cor pulmonale, leading to death, which was never seen in any other reported cases.
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ranking = 6
keywords = arteriole
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