Cases reported "Disease Progression"

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1/29. 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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2/29. 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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3/29. 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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4/29. 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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5/29. 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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6/29. 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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7/29. A possible association between Chlamydiae psittacci infection and temporal arteritis.

    Some arguments are in favor of the role of Chlamydia in the pathogenesis of atherosclerosis and some vasculitis. Illustrating this possible relation, we report the case of a patient developing consecutively a Chlamydia psittacci infection and a temporal arteritis. A 73-year-old woman, with no significant medical history, was hospitalized for constitutional symptoms. Three weeks before, she had described fever and sore throat of two days' duration. Since that time, she had remained exhausted and developed a mild intermittent claudication of the jaws. Clinical examination was poor. A biological inflammatory syndrome was noticed. Chest X-ray revealed bilateral interstitial opacities. The titer of anti-C. psittaci antibodies was significant (positive 1g G at 1/2048). Soon after initiation of doxycycline, a temporal arteritis biopsy was performed, due to the persistence of clinical symptoms and high inflammatory syndrome, conclusive for the diagnosis of temporal arteritis. Corticotherapy was added to antibiotic therapy, resulting in the decrease of inflammatory syndrome and an improvement in the general status of the patient. X-ray opacities decreased in three weeks. Serological control after three months showed a decrease of the titer of anti-C. psittacci antibodies to 1/256, confirming the initial diagnosis of Chlamydia pneumopathy. Our observation could provide one more argument for the role of bacteria-like Chlamydia in the pathogenesis of vascular diseases. Prospective seroepidemiological and molecular biology studies could allow us to clarify the association between chlamydia infections and inflammatory vasculitis-like temporal arteritis.
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8/29. coronary angiography and intravascular ultrasound.

    Clinicians have long used the size of the lumen and the angiogram as a predictor of coronary events. However, cardiovascular disease is not a disease of the lumen but a disease of the vessel wall. In early stages, atherosclerosis outwardly remodels the external elastic membrane; only late in the disease process does luminal narrowing occur, enabling angiographic detection. This has profound implications for drug therapy, because approximately 70% of patients present with acute myocardial infarction (MI) or sudden death, not angina as the first symptom of coronary disease. Intravascular ultrasound (IVUS) can provide detailed images of the artery and is the only technique currently available that enables physicians to routinely visualize coronary plaques. Due to its sensitivity in measuring plaque volume and content, IVUS may be a useful surrogate marker to evaluate the atherosclerotic process in smaller numbers of patients than required for conventional clinical endpoint trials.
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keywords = vascular disease
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9/29. 11: disorders of memory and intellect.

    The clinical approach to the patient with a suspected disorder of memory and intellect is to establish whether it is dementia, which parts of the brain are affected, what is the cause, what is the prognosis, and what can be done about it. The diagnosis of dementia usually requires the involvement of memory and at least one other cognitive system. delirium and depression are important differential diagnoses. patients with dementia should usually have some simple investigations after a careful history-taking and examination to identify "reversible" causes. The commonest cause of dementia is Alzheimer's disease, in which short-term memory disturbance is usually prominent. Other causes of dementia include cerebrovascular disease, Lewy-body disease and Pick's disease. There is now hope for patients with Alzheimer's disease (which can be treated with some success with cholinesterase inhibitors) and patients with vascular dementia, in whom aggressive control of causal risk factors may retard progression.
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10/29. Does type 2 diabetes mellitus delay renal failure in autosomal dominant polycystic kidney disease?

    Autosomal dominant polycystic kidney disease (ADPKD) is a common renal disease without an effective therapeutic intervention to delay renal failure. Within kindreds, renal dysfunction often develops at a similar age in affected individuals, although there are known modifying factors. Two kindreds with ADPKD have shown a striking pattern of delayed onset of renal insufficiency in those individuals also suffering from type 2 diabetes mellitus. Eight nondiabetic patients with ADPKD had onset of dialysis or renal death at ages 38-52 years, (mean /- SEM 46 /- 1.9, n = 7) as compared with four diabetics who started dialysis or are still off dialysis at the age of 61 /- 2.8 years (p < 0.01). Two of the four diabetics still have reasonable renal function at age 61 and 66. The diabetes was diagnosed at age 32 /- 2 years and was treated with oral hypoglycemics for 19 /- 2 years before institution of insulin. Cardiovascular disease dominated the clinical picture in the diabetics. In conclusion, onset of renal failure in ADPKD was delayed for over 15 years in individuals who also suffered from type 2 diabetes mellitus, in two ADPKD kindreds. Possible mechanisms are discussed, including glibenclamide inhibition of the cystic fibrosis transmembrane conductance regulator. The striking delay associated with type 2 diabetes mellitus in ADPKD induced renal failure should be evaluated further.
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keywords = vascular disease
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