Cases reported "Disease Progression"

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1/63. Rapid progression of cardiomyopathy in mitochondrial diabetes.

    Cardiac involvement and its clinical course in a diabetic patient with a mitochondrial tRNA(Leu)(UUR) mutation at position 3243 is reported in a 54-year-old man with no history of hypertension. At age 46, an electrocardiogram showed just T wave abnormalities. At age 49, it fulfilled SV1 RV5 or 6>35 mm with strain pattern. At age 52, echocardiography revealed definite left ventricular (LV) hypertrophy, and abnormally increased mitochondria were shown in biopsied endomyocardial specimens. He was diagnosed as having developed hypertrophic cardiomyopathy associated with the mutation. However, at age 54, SV1 and RV5,6 voltages were decreased, and echocardiography showed diffuse decreased LV wall motion and LV dilatation. Because he had mitochondrial diabetes, the patient's heart rapidly developed hypertrophic cardiomyopathy, and then it seemed to be changing to a dilated LV with systolic dysfunction. Rapid progression of cardiomyopathy can occur in mitochondrial diabetes.
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ranking = 1
keywords = diabetic
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2/63. University of Miami Division of Clinical pharmacology Therapeutic Rounds: ischemic renal disease.

    Ischemic renal disease (IRD) is defined as a significant reduction in glomerular filtration rate and/or loss of renal parenchyma caused by hemodynamically significant renal artery stenosis. IRD is a common and often overlooked clinical entity that presents in the setting of extrarenal arteriosclerotic vascular disease in older individuals with azotemia. IRD is an important cause of chronic renal failure and end-stage renal disease (ESRD), and many patients with a presumed diagnosis of hypertensive nephrosclerosis may actually have undiagnosed ischemic nephropathy as the cause of their ESRD. The primary reason for establishing the diagnosis of IRD is the hope that correction of a renal artery stenosis will lead to improvement of renal function or a delay in progression to ESRD. There are six typical clinical settings in which the clinician could suspect IRD: acute renal failure caused by the treatment of hypertension, especially with angiotensin-converting enzyme inhibitors; progressive azotemia in a patient with known renovascular hypertension; acute pulmonary edema superimposed on poorly controlled hypertension and renal failure; progressive azotemia in an elderly patient with refractory or severe hypertension; progressive azotemia in an elderly patient with evidence of atherosclerotic disease; and unexplained progressive azotemia in an elderly patient. It is important for the clinician to identify IRD, because IRD represents a potentially reversible cause of chronic renal failure in a hypertensive patient.
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ranking = 0.17800859250739
keywords = vascular disease
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3/63. 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.
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ranking = 1.6020773325665
keywords = vascular disease
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4/63. 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.
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ranking = 0.17800859250739
keywords = vascular disease
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5/63. Treatment of severe proliferative retinopathy and diabetic maculopathy.

    Strict blood glucose control, early detection and surveillance of diabetic retinopathy by means of validated screening programmes, and judicious use of laser photocoagulation can greatly reduce the risk of visual loss in diabetes. Some patients however, have aggressive neovascular disease resistant to laser treatment, or present at a late stage with advanced fibroproliferative disease, and may progress rapidly to blindness. In the elderly with Type 2 disease, diabetic maculopathy is more common and requires a different therapeutic approach. The present article describes two diabetic patients and discusses the management of patients with severe proliferative retinopathy or diabetic maculopathy.
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ranking = 8.1780085925074
keywords = diabetic, vascular disease
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6/63. osteomyelitis of the skull base with atypical onset and evolution.

    skull base osteomyelitis arises as a complication of malignant external otitis, but it can be also due to middle ear and/or mastoid infection. Other causes can be infections of the paranasal sinuses or of the mandible or maxilla due to odontic caries. Generally, osteomyelitis involves elderly patients affected by diabetic immunodeficiency or microvascular disease. In this paper, we present 3 new cases of skull base osteomyelitis with atypical onset and evolution. The difficulties of diagnosis and details of the management are discussed.
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ranking = 1.1780085925074
keywords = diabetic, vascular disease
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7/63. Ruptured sinus of a Valsalva aneurysm associated with autosomal-dominant polycystic kidney disease in an elderly patient: report of a case.

    We report herein the case of a 71-year-old woman with autosomal-dominant polycystic kidney disease (ADPKD), who was referred to our hospital for investigation of facial edema. echocardiography demonstrated a large aneurysm arising from the non-coronary sinus of valsalva, with a left to right shunt and jets of blood passing from the aneurysm toward the septal leaflet of the tricuspid valve. Surgical treatment was successfully carried out by resecting the aneurysmal wall and performing a patch closure of the orifice. It is well known that ADPKD predisposes patients to cardiovascular disease, and this case report serves to demonstrate that when a patient with ADPKD presents with progressive heart failure, the possibility of a ruptured sinus of a Valsalva aneurysm must be considered.
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ranking = 0.17800859250739
keywords = vascular disease
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8/63. Possible activation of auto-immune thyroiditis from continuous subcutaneous infusion of genapol-containing insulin.

    A case of a type 1 diabetic woman with auto-immune thyroiditis is reported, in whom repeated exposure to insulin containing Genapol(R) (polyethylen-polypropylenglycol) over 3 years reproducibly parallels with an increase of serum TSH (thyroid-stimulating hormone) above the normal limit. Previously, adverse effects of Genapol(R) insulin have been related to its intraperitoneal application, and thought to be restricted to anti-insulin-immunity; activating effects on thyroid auto-immunity have been repeatedly disputed. We suggest that Genapol(R) insulin should be replaced by other insulin preparations with a better safety record.
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ranking = 1
keywords = diabetic
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9/63. Pyogenic vertebral osteomyelitis presenting as single spinal compression fracture: a case report and review of the literature.

    STUDY DESIGN: A case report of pyogenic vertebral osteomyelitis (PVO) presenting as single collapsed vertebral body without narrowing of the intervertebral disc space, and review of the literature. OBJECTIVE: To describe an unusual case of PVO showing atypical radiological change and call attention to this condition so that others may avoid this diagnostic pitfall. SETTING: japan. methods: A 62-year-old diabetic woman with suspected T12 pathological fracture of malignant spinal tumor and neurological involvement received urgent anterior decompression and spinal reconstruction without biopsy. RESULTS: Anterior decompression and spinal reconstruction was performed, but histological examination of the specimen after surgery unexpectedly revealed PVO. The surgery was followed by therapy with antibiotics for 7 months. A follow-up radiograph at 5 years after surgery revealed that solid consolidation has been achieved. CONCLUSIONS: Diagnosis of PVO presenting with single spinal compression fracture is very difficult. Although the finding of the high signal intensity in the lesion equal to or higher than that of the cerebrospinal fluid on T2-weighted MR image seemed to be the most reliable diagnostic modality retrospectively, diagnosis of this type of PVO is impossible without histology. A needle biopsy before surgery is strongly recommended.
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ranking = 1
keywords = diabetic
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10/63. diabetic retinopathy and serum lipids.

    The association between serum lipid levels and diabetic retinopathy has been investigated in many studies. Some studies show a positive relationship between serum cholesterol and low-density lipoprotein levels and retinal hard exudation. Other studies show serum triglyceride levels as being important in the progression of retinopathy. Certain other studies show no relationship between serum lipid levels and diabetic retinopathy. We review the literature on this subject and illustrate this report with an example of a diabetic with severe diabetic maculopathy and high serum lipid levels.
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ranking = 4
keywords = diabetic
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