Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018801 (heart failure)
 72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The course of arterial hypertension (AH) was evaluated retrospectively in 306 patients, who had undergone annual hospital examination and treatment during the previous five years. After five years, AH had got complicated with ischemic cerebral stroke (ICS) in 147 patients (group 1). AH had not got complicated with ICS in 149 patients (group 2). These patients had been observed for another five years. By the end of the study, 98 patients had remained in group 1, and 100 patients had remained in group 2. Mean term of observation had been 10.3 +/- 0.8 years in group 1, and 10.1 +/- 0.9 years in group 2. Clinico-anamnestic analysis revealed the following peculiarities of the course of AH complicated by ICS: a longer AH course before the development of ICS; faster deterioration of AH in a period of 10 years; faster deterioration of chronic cardiac insufficiency and discirculatory encephalopathy; more frequent combination with coronary heart disease and carbohydrate exchange; more frequent development of transitory ischemic attacks (TIA); TIA became more frequent before the development of ICS; in 70% of group 1 patients TIA was located in the place of a future ICS. The authors suggest that slowly and quickly progressing AH forms should be differentiated according to the progression of the risk of cardiovascular complications.
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PMID:[The clinical features of arterial hypertension complicated by an old ischemic cerebral stroke]. 1803 67

Although linked with cardiac dysfunction, the association of MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes) and pulmonary artery hypertension (PAH) has not been previously described. PAH and right ventricular heart failure were identified by echocardiography in a 3-year-old boy with a history of hypotonia, microcephaly and developmental delay. He initially presented with a 10-day history of dyspnoea, dependent oedema and reduced oral intake. Lactic acidosis was noted on serial arterial blood sampling and cerebrospinal fluid. Muscle biopsy demonstrated cytochrome-c oxidase-positive 'ragged-red' fibres consistent with MELAS; subsequent analyses revealed the m.3243A>G point mutation most commonly associated with MELAS. The mutation was heteroplasmic, representing 92% of the total mtDNA from a lung sample. Nitric oxide and epoprostenol were administered without significant clinical or echocardiographic improvement of his PAH. A 'mitochondrial cocktail' including biotin, riboflavin, carnitine and coenzyme Q10 also was provided. Five months after presentation, he developed seizures; MRI imaging of his brain demonstrated multiple focal lesions. His clinical status worsened with increasing cardiopulmonary failure. He died two months later. Although therapy for both MELAS and PAH remains limited, recent investigations suggest a beneficial role for l-arginine in both conditions, implying a possible common pathophysiology. Mitochondrial diseases such as MELAS should be considered in cases of idiopathic PAH, particularly when associated with multisystem involvement including short stature, hearing loss, renal dysfunction, retinopathy, diabetes mellitus, migraines, seizures, ophthalmoplegia, fatigability and weakness.
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PMID:Pulmonary artery hypertension in a child with MELAS due to a point mutation of the mitochondrial tRNA((Leu)) gene (m.3243A>G). 1818 Oct 29

We retrospectively analyzed 92 cases of severe rickettsial infections in patients (median age = 49 years, 57% male, 37.0% with scrub typhus) in Hong Kong. Immunofluorescence assay was used for diagnostic confirmation. Identification of > or = 1 diagnostic sign (exposure history, rash, or eschar) was possible in 94.6% of the cases. Multivariate analysis suggested that pulmonary infiltrates (odds ratio [OR] = 25.2, 95% confidence interval [CI] = 3.9-160.9, P = 0.001) and leukocytosis (OR = 1.3, 95% CI = 1.0-1.5 per unit increase, P = 0.033) were independent predictors of admission to an intensive care unit (14.1%). Delayed administration of doxycycline was independently associated with major organ dysfunction (23.9%; oxygen desaturation, renal failure, severe jaundice, encephalopathy, cardiac failure) (OR = 1.2, 95% CI = 1.0-1.5 per day delay, P = 0.046; adjusted for age and rickettsia biogroup) and prolonged hospitalization > 10 days (25%) (OR = 1.4, 95% CI = 1.1-1.9 per day delay, P = 0.014). Treatment with fluoroquinolone/clarithromycin did not correlate with clinical outcomes (P > 0.05). Early empirical doxycycline therapy should be considered if clinico-epidemiologic signs of rickettsial infections are present.
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PMID:Risk factors associated with life-threatening rickettsial infections. 1854 79

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular disorder that can involve the liver diffusely in the form of vascular malformations ranging from small telangiectases to discrete arteriovenous malformations. Anatomically, three different patterns of abnormal vascular communications can occur in liver: portal vein to hepatic vein (portovenous), hepatic artery to hepatic vein (arteriovenous) and hepatic artery to portal vein (arterioportal), with the most common being arteriovenous. Only 5 to 8% of patients with these vascular malformations are symptomatic. When symptomatic, patients present with high-output cardiac failure, biliary ischemia (which, when severe, can progress to biliary and hepatic necrosis and lead to acute liver failure), or portal hypertension. Other less common presentations include portosystemic encephalopathy and abdominal angina. Diagnosis is confirmed by Doppler ultrasonography or multidetector computed tomography. The hallmark findings are intrahepatic hypervascularization and an enlarged common hepatic artery. Focal nodular hyperplasia and nodular regenerative hyperplasia are common findings. Symptomatic patients are treated with intensive medical treatment aimed at the predominant clinical presentation. Patients who fail aggressive medical therapy and those with acute biliary/hepatic necrosis should be considered for liver transplantation.
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PMID:Hepatic vascular malformations in hereditary hemorrhagic telangiectasia. 1881 78

Hyperammonemic encephalopathy is a rarely reported complication of multiple myeloma (MM). We describe an illustrative case of hyperammonemia in the setting of an immunoglobulin (Ig) D-lambda MM, and perform a systematic review of the English-written literature. Our search yielded 26 more cases. Median age was 64 years, and 54% of patients were male. All presented with progressive impairment of their level of consciousness. Median ammonium concentration was 109 micromol/L (interquartile range, 73-149 micromol/L). Most were IgA type (10 cases), and there were 2 cases of IgD type. Most cases were aggressive or chemotherapy-resistant forms of MM. Eight patients were diagnosed with MM at the same time as the episode of hyperammonemia. Only 1 patient had signs of portal hypertension as a result of concomitant hyperdynamic heart failure. Determination of amino acid in 10 patients showed high levels of glycine, low levels of tyrosine, and a low Fischer ratio. Two patients did not receive chemotherapy and died. Twenty-two out of 25 patients who received chemotherapy against MM showed a decrease in ammonium blood concentration, and of those, 15 survived the episode (68%). Overall mortality was 44%. In conclusion, hyperammonemia is a severe complication of MM, associated with a high mortality. It should be considered in any patient with MM and a low level of consciousness. Chemotherapy directed against MM seems to be the most effective measure in order to achieve normal ammonium levels and clinical improvement.
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PMID:Multiple myeloma and hyperammonemic encephalopathy: review of 27 cases. 1906 3

A 49-year-old woman was admitted to hospital because of heart failure. She was diagnosed as having mitochondrial cardiomyopathy and diabetes mellitus. Echocardiography revealed a hypertrophic and poorly contracting left ventricle. A diagnosis of mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes was established by muscle biopsy. She underwent technetium-99m-sestamibi ((99m)Tc-MIBI) and beta-methyl-p-(123)I-iodophenyl-pentadecanoic acid ((123)I-BMIPP) scintigraphic examinations. (99m)Tc-MIBI single-photon emission computed tomography revealed reduced tracer uptake in the hypertrophic left ventricular inferior wall. In contrast, there was an increase in (123)I-BMIPP uptake in the in the region of reduced (99m)Tc-MIBI uptake ((99m)Tc-MIBI/(123)I-BMIPP mismatch). There was rapid washout of (99m)Tc-MIBI from the myocardium (washout rate increased by 30%). Decreased (99m)Tc-MIBI and increased (123)I-BMIPP uptake ((99m)Tc-MIBI/(123)I-BMIPP mismatch) were the characteristics of cardiac involvement in mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes.
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PMID:Cardiac scintigraphic findings of mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes: A case report. 1934 24

A 52-year-old woman was admitted to the hospital because of appetite loss, unsteadiness, psychogenic symptoms, ataxia, and consciousness disturbance as a result of the ingestion of a diet restricted to only carbohydrates for a long term. Laboratory examination indicated the presence of pancytopenia with macrocytic anemia; further, decreased vitamin B1 and B12 levels were detected in her serum. Magnetic resonance imaging fluid attenuated inversion recovery (FLAIR), revealed high-signal intensity in the bilateral corpus striatum, third ventricle circumference, and cerebellar cortex. Thereafter, she received drip infusion that did not include vitamin B1 or B12 and subsequently suffered a cardiac arrest due to the aggravation of cardiac insufficiency; consequently, she was transferred to our hospital. Upon admission the patient was diagnosed to have obvious cardiomegaly with pleural effusion; further, a negative T-wave was obtained on the electrocardiogram. A diagnosis of beriberi heart disease was made because of thiamine deficiency. She was treated by thiamine administration, following which the cardiac symptoms improved immediately. Various neurological symptoms caused by encephalopathy, peripheral neuropathy and subacute combined spinal cord degeneration improved by treatment with thiamine and cyanocobalamine administration; however, some of these symptoms still remained. General awareness of the fact that neurological symptoms can be caused by vitamin deficiency is essential.
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PMID:[Case of Wernicke's encephalopathy and subacute combined degeneration of the spinal cord due to vitamin deficiency showing changes in the bilateral corpus striatum and cardiac arrest due to beriberi heart disease]. 1980 6

Hepatic involvement in hereditary hemorrhagic telangiectasia (HHT) consists of vascular malformations associated with arteriovenous (AV), arterioportal, and/or portovenous shunting. Most patients with HHT have liver involvement. Symptoms, although rare, consist of cardiac failure, pulmonary hypertension, portal hypertension, portosystemic encephalopathy, cholangitis, and atypical cirrhosis. Reported treatments for symptomatic AV malformations have been associated with substantial morbidity and mortality. This report describes a case of hepatic HHT that required liver transplantation after hepatic artery embolization. Recurrent vascular malformations developed in the transplant, resulting in portal hypertension and life-threatening variceal hemorrhage that was controlled with transjugular intrahepatic portosystemic shunt creation.
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PMID:Transjugular intrahepatic portosystemic shunt for variceal hemorrhage due to recurrent of hereditary hemorrhagic telangiectasia in a liver transplant. 1992 94

We investigated a unique method utilising a Heartport bifurcated arterial cannula and an aortic occlusion balloon to provide both distal perfusion and bloodless, distal arterial exposure during repair of thoracic and thoracoabdominal aortic pathology. Between February 2002 and September 2008, 21 patients underwent thoracic or thoracoabdominal aortic repair with this method. Because of an inability or potential difficulty in achieving distal aortic control secondary to anatomic or technical challenges, a compliant balloon was passed through the accessory arterial channel using fluoroscopic guidance. Adjunctive cerebrospinal drainage was used in all cases. Ballon deployment and satisfactory occlusion was attained in all cases. The overall 30-day mortality rate was 4.75% (1 of 21 patients). Renal failure occurred in one patient (4.75%) and heart failure occurred in two patients (9.5%). Six patients (29%) had pulmonary complications. Transient encephalopathy occurred in two patients (9.5%). Spinal cord neurologic deficit and stroke were avoided in all patients. We recommend the application of this simple method to obtain a bloodless anastomotic field and maintain ongoing distal aortic perfusion in instances where distal control with a clamp is challenging, not feasible or presents the potential for catastrophic intra-operative bleeding.
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PMID:Combined distal aortic perfusion and balloon occlusion to facilitate the repair of complex thoracic and thoracoabdominal aortic aneurysms. 2018 8

Medium-chain acyl-CoA dehydrogenase deficiency (MCADD) is the most common fatty acid oxidation disorder. Typically, undiagnosed individuals are asymptomatic until an episode of increased energy demand and fasting occurs, resulting in metabolic derangement. Phenotypic heterogeneity has been increasingly realized, with reports of both neonates and adults manifesting with life-threatening symptoms including encephalopathy, rhabdomyolysis, and cardiac failure. If diagnosed presymptomatically, outcome is favorable basically by avoidance of fasting. Early detection by newborn screening (NBS) has significantly reduced the incidence of severe adverse events including deaths. In this manuscript we focus on the natural course of the disease in both children and adults. Although NBS for MCADD has been successfully established, continuing efforts need to be made to avoid acute crises and deterioration of outcome in screened patients entering adolescence and adulthood.
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PMID:The clinical manifestation of MCAD deficiency: challenges towards adulthood in the screened population. 2053 24


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