Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020538 (hypertension)
170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The middle aortic syndrome (MAS) is a rare disease affecting children and young adults, and it occurs in about 0.5% to 2.0% of all aortic coarctation cases. Congenital, acquired, inflammatory, and infectious etiologies have been described. In the majority of cases, there is a short, isolated or diffuse tubular narrowing of the descending thoracic and abdominal aorta, often accompanied by ostial stenosis or occlusion of the renal and visceral branches, which leads to renovascular hypertension and visceral ischemia. Surgical treatment should be considered in cases of uncontrollable hypertension, evidence of end-organ damage such as cardiac failure, progressive renal insufficiency, or severe intermittent claudication. Several surgical treatments for this condition have been reported, including bypass grafting, graft replacement, or patch angioplasty. We report a successful case of ascending aorta-abdominal aorta bypass for MAS in a 11-year-old boy.
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PMID:A successful case of ascending aorta--abdominal aorta bypass for middle aortic syndrome. 1879 99

Dispensing medication at the discharge of patients is an activity that allows to the Pharmacy Department to have a privileged point of view about prescribing new drugs and therapies. During last year we met several prescriptions for new drugs utilized in the treatment of pulmonary arterial hypertension (PAH), These prescriptions are the mirror of the avalaibility of new specific drugs. PAH is a rare and fatal disease; current disease-specific therapeutic interventions in PAH established pathways in disease treatment: prostacyclin, endothelin receptors antagonist. Several studies indicate that sildenafil, an oral phosphodiesterase type-5 inhibitor, may also offer benefits in the pharmacological management of PAH as well as tadalafil or vardenafil too. We collected data from our Pharmacy patient's database to evaluate the impact of this rare disease on our setting, to analyse the patterns of prescription of our physicians and to think specific health policy or pathways for the patients suffering from this disease.
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PMID:[Pulmonary arterial hypertension in Health District 11: a patient-oriented drug utilization analysis]. 1904 53

The goal of combination therapy in patients with pulmonary arterial hypertension (PAH) is aimed at maximizing therapeutic efficacy while limiting toxicity and drug interactions. Because PAH is a rare disease, it is difficult to adequately power therapeutic trials to evaluate significant morbidity or mortality differences between various drug therapies. At this point, it is premature to either dismiss or strongly favor any one combination of therapies over another. There is debate among PAH specialists as to whether combination therapy should be reserved for patients whose condition deteriorates (add on therapy) or should be started up front and followed by maintenance therapy with one or more agents once patients have improved. Careful design of future trials testing these comparisons is very important.
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PMID:[Combination therapy]. 1905 37

Sometimes, a clinical course that initially appears to be 'classic' turns out to be a manifestation of a rare disease. We report on a 62-year-old woman who presented initially with episodic headache, followed by cardiogenic shock. What was first thought to be an ST-segment elevation acute myocardial infarction, later to be a takostubo cardiomyopathy, finally appeared to be a catecholamine-induced cardiomyopathy due to a pheochromocytoma. This case illustrates that in a patient with presumed takotsubo cardiomyopathy and episodic headache, sweating, hypertension or tachycardia, pheochromocytoma needs to be excluded.
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PMID:From trivial headache to life-threatening disease. 1917 13

Failure to thrive is frequently seen in breastfed infants. The most common diagnosis is insufficiency of breast milk in an otherwise healthy child. However, several differential diagnoses need to be considered. Progressive feeding difficulties and failure to thrive can be the first manifestation of group B streptococcal ventriculitis. This rare disease does not present with acute symptoms of sepsis or meningitis but evolves insidiously with no fever. Diagnosis is therefore often delayed and made only when intracranial hypertension develops. Cerebrospinal fluid (CSF) culture confirming the group B streptococcal infection and cerebral imaging are the necessary investigations for diagnosis. To our knowledge, only 10 cases have been previously reported.
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PMID:[A 6-week-old infant with failure to thrive: insidious presentation of group B streptococcal ventriculitis]. 1924 27

Spontaneous spinal epidural hematoma (SSEH) is a rare disease that requires emergency surgical intervention because it can cause serious and permanent neurological damage. Its etiology is related to coagulopathy, vascular malformation, hypertension, neoplasms, infections and idiopathic causes. It is frequently observed in the cervicothoracic and thoracolumbar regions. Inadequate spinal vascularization of the thoracolumbar junction increases the risk of spinal infarcts in lesions in this region. Therefore, prompt and effective surgical intervention is critical in patients with a thoracolumbar SSEH. We conclude that prognosis would be better than it is if patients with complete neurological damage were to undergo spinal decompression within the first 36 hours, and patients with incomplete neurological damage were operated on in the first 48 hours. We report a 71-year-old female patient with a history of aspirin use who developed paraparesis accompanied by SSEH following straining, and emphasize the importance of early surgical treatment.
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PMID:Paraparesis following straining accompanied by spontaneous thoracolumbar spinal epidural hematoma: a case report. 1954 46

Acromegaly is a rare disease caused by a growth-hormone-secreting pituitary adenoma. Symptoms include enlargement of the hands, feet, and jaw with growing dental interspaces, as well as hypertrophy of the tongue and nasal and sinusoidal mucosa. The two latter symptoms are mostly responsible for the accompanying obstructive sleep apnea syndrome. Besides these "cosmetic" symptoms, the disease is associated with hypertension and diabetes mellitus, as well as with an increased risk for adenomas and carcinomas of the colon. The average time span from first symptom to diagnosis is well over 6 years; a single determination of insulin-like growth factor 1 in serum can confirm the disease. The treatment of choice remains surgical resection of the adenoma in suitable patients, whereas in extensive disease with invasion of surrounding tissue, drug therapy and/or radiotherapy may be necessary.
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PMID:[Acromegaly-associated lesions of the nasal mucosa. Case report]. 1955 21

Botulism is a rare disease in Chile and of the known clinical presentation, infant botulism is the most common. We report the case of a previously healthy seven month old male infant with a two weeks history of rinorrea, cough, fatigue, constipation and progressive weakness after the consumption of honey. Stool cultures were positive for Clostridium botulinum group 1 type A and electromyography was compatible with the diagnosis. The patient evolved with arterial hypertension, interpreted as secondary to autonomic dysfunction, which responded to calcium channel blockers. Muscle tone improved progressively during the following four weeks. Infant botulism is a potentially fatal disease; diagnosis can be difficult given the broad clinical manifestations. Prevention should focus on education of parents of infants as well as medical personnel.
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PMID:[Infant botulism: case report and review]. 1962 Nov 49

(1) Human insulin-like growth factor type 1 (IGF-1) is the main effector of growth hormone action. Primary IGF-1 deficiency is a rare disease, mainly resulting in very short stature; (2) Mecasermin is a recombinant IGF-1 marketed for this indication as a twice daily subcutaneous injection; (3) Clinical evaluation is mainly based on a non-comparative follow-up study of 76 children with an average age of 7 years, some of whom were treated for 8 years. The mean height at treatment initiation was 6.7 standard deviations below normal. Eight years later, it was 5.2 standard deviations below normal, i.e. their growth failure remained very severe; (4) The main short-term adverse effects of mecasermin are hypoglycaemia, headache and intracranial hypertension. Nearly one in 5 children developed tonsillar hypertrophy, resulting in otitis and hypoacusis; (5) Animal studies showed hypertrophy of other organs (kidneys, spleen and heart) as well as carcinogenic effects. The risk in humans is unknown; (6) The mecasermin packaging is not well-adapted (a multidose vial designed to be punctured several times), and is a potential source of contamination and errors. Prefilled pens or syringes would be easier to use; (7) In practice, the limited clinical benefits of mecasermin do not justify exposure to its potential risks.
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PMID:Mecasermin: new drug. Insufficient improvement in statural growth. 1963 20

Non-compaction cardiomyopathy is a rare disease, anatomically characterized by a prominent trabecular pattern and deep intertrabecular recesses. Its clinical manifestations include severe left ventricular dysfunction, arrhythmias, systemic embolism, and sudden death. In this report, two cases of patients of different ages with non-compaction cardiomyopathy are described: a male schoolboy whose pathology was associated with mitral stenosis and regurgitation and a 50-year-old female with history of high blood pressure and cardiac failure.
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PMID:Echocardiographic features of non-compaction cardiomyopathy: missed and misdiagnosed disease. 1983 76


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