Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 74-year-old woman with chronic auricular fibrillation, arterial hypertension, hypercholesterolemia, ischemic cardiopathy, and peripheral arteriopathy presented with purpuric lesions on the lower limbs (Fig. 1) and, to a lesser extent, on the anterior area of the chest. The mucous membranes were not affected. In 1989, she was diagnosed with anemia that evolved until 1998, when a bone marrow biopsy revealed a myelodysplastic syndrome unclassified in French-American-British Group (FAB). The patient has required periodic transfusions since February 1999. A skin biopsy of the purpuric lesions revealed a leukocytoclastic vasculitis; the lesions cleared with topical corticosteroid treatment. In May 1999, the patient presented with inflammatory and painful lesions localized on the vulva (Fig. 2), which had evolved over several days, without fever. No lesions were observed in other locations. A cutaneous biopsy showed an intense dermal edema and a diffuse and polymorphous dermal infiltrate involving the follicular structures. Exocytosis, spongiosis, and mucin deposits, demonstrated by Alcian blue stain, were observed in the follicular epithelium. Mature neutrophils were predominant in the dermal infiltrate, but a small number of eosinophils and immature cells were also present (Fig. 3). The myelogenous origin of the immature lining cells was further confirmed by positive staining of intracytoplasmic granules with naphthol-ASD chloroacetate sterase (Leder's stain). Vasculitis was not observed. Routine laboratory tests revealed 3030 leukocytes/mm(3) (60% neutrophils), a hemoglobin level of 8.4 g/dL, and 92,000 platelets/mm(3). Treatment with 30 mg/day of prednisone was started, and the lesions cleared slowly within 4 weeks. A new bone marrow biopsy in September 1999 showed a similar appearance to that taken in 1998. The patient died in January 2000 as a result of pneumonia with cardiac and respiratory failure. A 66-year-old man presented with a febrile syndrome that had evolved over 5 days, and painful and pruritic cutaneous lesions on the face and posterior neck (Fig. 4). Three months before, the patient was diagnosed with chronic myelogenous leukemia in acceleration phase. Examination revealed an edematous and erythematous face with pustular lesions on the surface, also involving the neck and the upper part of the back. The histopathologic examination revealed an intense edema and abscesses in the dermis. The infiltrate of these lesions was composed of mature neutrophils with the presence of abundant immature cells with a myelogenous aspect (Fig. 5). Analytical studies revealed 26,130 leukocytes/mm(3) (42% blasts). No specific treatment for Sweet's syndrome was administered and the lesions showed an improvement within 5 days. Eight days after admission, the patient died as a result of acute hemorrhage, before treatment for leukemia was initiated.
Int J Dermatol 2005 Aug
PMID:Concurrent Sweet's syndrome and leukemia cutis in patients with myeloid disorders. 1610 72

Most public health statements regarding exposure to solar ultraviolet radiation (UVR) recommend avoiding it, especially at midday, and using sunscreen. Excess UVR is a primary risk factor for skin cancers, premature photoageing and the development of cataracts. In addition, some people are especially sensitive to UVR, sometimes due to concomitant illness or drug therapy. However, if applied uncritically, these guidelines may actually cause more harm than good. Humans derive most of their serum 25-hydroxycholecalciferol (25(OH)D3) from solar UVB radiation (280-315 nm). Serum 25(OH)D3 metabolite levels are often inadequate for optimal health in many populations, especially those with darker skin pigmentation, those living at high latitudes, those living largely indoors and in urban areas, and during winter in all but the sunniest climates. In the absence of adequate solar UVB exposure or artificial UVB, vitamin D can be obtained from dietary sources or supplements. There is compelling evidence that low vitamin D levels lead to increased risk of developing rickets, osteoporosis and osteomaloma, 16 cancers (including cancers of breast, ovary, prostate and non-Hodgkin's lymphoma), and other chronic diseases such as psoriasis, diabetes mellitus, hypertension, heart disease, myopathy, multiple sclerosis, schizophrenia, hyperparathyroidism and susceptibility to tuberculosis. The health benefits of UVB seem to outweigh the adverse effects. The risks can be minimized by avoiding sunburn, excess UVR exposure and by attention to dietary factors, such as antioxidants and limiting energy and fat consumption. It is anticipated that increasing attention will be paid to the benefits of UVB radiation and vitamin D and that health guidelines will be revised in the near future.
J Cosmet Dermatol 2003 Apr
PMID:Sunshine is good medicine. The health benefits of ultraviolet-B induced vitamin D production. 1716 34

Psoriasis has been traditionally viewed as an inflammatory skin disorder of unknown origin. Recent advances in the immunopathogenesis and genetics of psoriasis have broadened our understanding of psoriasis. Psoriasis is now considered a systemic inflammatory condition analogous to other inflammatory immune disorders. Patients with other immune disorders, such as systemic lupus erythematosus or rheumatoid arthritis, are known to be at increased risk of heart disease. Similarly, patients with psoriasis may carry an excess risk of heart disease, which would represent an important previously unrecognized cause of morbidity and mortality. This review summarizes the current evidence for an increased cardiovascular risk in patients with psoriasis and outlines deficits in our knowledge in this area.
J Am Acad Dermatol 2007 Aug
PMID:Heart disease in psoriasis. 1822 38

Bardet-Biedl syndrome (BBS) is a genetically heterogeneous autosomal recessive disorder characterized by progressive retinal dystrophy, polydactyly, obesity, hypogonadism, mental retardation, and renal dysfunction. Other manifestations include diabetes mellitus, heart disease, hepatic fibrosis, neurological features, and multiple pigmented nevi. To date, twelve BBS genes have been cloned (BBS1-BBS12). Herein we discussed a patient with BBS who had multiple pigmented nevi.
Dermatol Online J 2008 Jan 15
PMID:Bardet-Biedl syndrome: a case report. 1831 26

Several reports have demonstrated an association between psoriasis and cardiovascular diseases such as hypertension, valvular disease and arrhythmia. However, the data is scarce. Forty-seven psoriasis patients and 20 healthy people underwent transthoracic echocardiographic examination including pulse- and tissue Doppler analysis and 24-h ambulatory electrocardiographic monitoring including heart rate variability (HRV) analysis. Patients having systemic hypertension, diabetes mellitus, history of structural or ischemic heart disease, chronic obstructive pulmonary disease and any associated systemic disease were excluded. Psoriasis Area and Severity Index (PASI) was calculated and severe psoriasis was defined in the case of history of hospitalizations for psoriasis and/or getting systemic therapy. Mean age of the patients was 35.7 +/- 12.9 years and disease duration was 123.2 +/- 84.3 (3-360) months. PASI ranged from 0.4 to 34.0 (mean +/- SD: 7.1 +/- 6.6) and 20 (42.6%) patients had severe psoriasis. There were no significant differences between psoriasis patients and control group with respect to mean values of blood pressure, body mass index, lipid profile and cardiac dimensions. However, frequency of being overweight was significantly higher in psoriasis patients (42.6 vs. 10.0%, P = 0.011). No patient had valvular disease. Mild pulmonary hypertension (PH) (30-40 mmHg) was significantly more frequent in psoriasis patients (31.9 vs. 0%, P = 0.003). Pulse wave mitral Doppler deceleration and isovolumetric relaxation times were significantly longer in psoriasis patients (195.9 +/- 29.7 vs. 191.6 +/- 14.7 ms, P = 0.002 and 91.6 +/- 14.7 vs. 79.6 +/- 10.5 ms, P = 0.001, respectively). However, frequency of diastolic dysfunction was not significantly different than the control group (8.5 vs. 0%, P = 0.309). HRV parameters and frequency of supraventricular and ventricular premature beats were not significantly different between the groups. No patient had ventricular tachycardia. Echocardiographic follow-up of psoriasis patients may be important due to possible association of PH. However, incidences of structural heart disease and arrythmia are not increased in psoriasis according to our results.
Arch Dermatol Res 2008 Sep
PMID:Increased frequency of pulmonary hypertension in psoriasis patients. 1844 54

Lymphedema-distichiasis syndrome (LD, OMIM 153400) is an autosomal dominant disorder with variable expression. It is caused by mutations in the FOXC2-gene, which codes for a forkhead transcription factor involved in the development of the lymphatic and vascular system. LD is characterized by late childhood or pubertal onset lymphedema of the limbs and distichiasis (double row of eyelashes). While the latter is the most common expression of LD, venous insufficiency occurs in half of the patients. Other associations have been reported, including congenital heart disease, ptosis, cleft lip/palate and spinal extradural cysts. Here we describe a family with classical lymphedema-distichiasis syndrome caused by a duplication in the FOXC2-gene.
Int J Dermatol 2008 Nov
PMID:Lymphedema-distichiasis syndrome: a distinct type of primary lymphedema caused by mutations in the FOXC2 gene. 1898 89

Kawasaki disease is a systemic acute vasculitis of unknown etiology. It is the leading cause of acquired heart disease in children in the USA. It occurs more frequently in boys and eighty percent of the cases occur in children under five years of age. The disease rarely occurs after eight years and it can affect children of all races, with higher incidence among Asian descendants. Kawasaki disease is characterized by fever, bilateral non-exudative conjunctivitis, redness and swelling of the tongue, lips and oral mucosa, abnormalities in the extremities, cervical lymph node, and polymorphic exanthema. Aneurysms and stenoses of coronary arteries occur in approximately 20 to 25% of untreated patients and subsequently can lead to acute myocardial infarction and sudden death. Treatment with intravenous immunoglobulin is effective and should be initiated early to prevent cardiac sequel. The development of diagnostic tests, more specific treatment approaches and prevention of this potentially fatal disease in children depends on continuous advances in the determination of its pathogenesis.
An Bras Dermatol
PMID:[Kawasaki disease]. 1985 63

We have previously postulated that as well as T-helper (Th) 1 and Th17 cells, the transforming growth factor (TGF)-beta/fibronectin (FN)/alpha5beta1 pathway is central to psoriasis pathogenesis. EDA+ FN refers to an alternatively spliced isoform of FN with an additional domain known as extra domain A. EDA+ FN has two important properties pertinent to psoriasis lesions: it stimulates keratinocyte hyperproliferation, and, through stimulation of Toll-like receptor (TLR) 4, stimulates production of proinflammatory cytokines. EDA+ FN production induced by TGF-beta stimulation can be maintained in psoriasis lesions via two main feedback loops. Firstly, EDA+ FN stimulates proliferation of keratinocytes, which, in an autocrine fashion, will release more EDA+ FN. Secondly, EDA+ FN stimulates TLR4 expressed by antigen-presenting cells resulting in the production of proinflammatory cytokines such as tumour necrosis factor-alpha, interleukin (IL)-1, IL-6 and IL-12. The resultant promotion of cutaneous inflammation results in the recruitment of Th1 cells, which also produce EDA+ FN. We propose that these 'FN loops' contribute to the maintenance and progression of psoriatic lesions. Finally, although the association between psoriasis and heart/thrombotic disease remains unclear one plausible link may be the promotion of atherosclerosis and thrombotic heart disease by EDA+ FN.
Br J Dermatol 2010 Jul
PMID:Psoriasis and extra domain A fibronectin loops. 2019 57

Adherens junctions and desmosomes are intercellular adhesive junctions and essential for the morphogenesis, differentiation, and maintenance of tissues that are subjected to high mechanical stress, including heart and skin. The different junction complexes are organized at the termini of the cardiomyocyte called the intercalated disc. Disruption of adhesive integrity via mutations in genes encoding desmosomal proteins causes an inherited heart disease, arrhythmogenic right ventricular cardiomyopathy (ARVC). Besides plakoglobin, which is shared by adherens junctions and desmosomes, other desmosomal components, desmoglein-2, desmocollin-2, plakophilin-2, and desmoplakin are also present in ultrastructurally defined fascia adherens junctions of heart muscle, but not other tissues. This mixed-type of junctional structure is termed hybrid adhering junction or area composita. Desmosomal plakophilin-2 directly interacts with adherens junction protein alphaT-catenin, providing a new molecular link between the cadherin-catenin complex and desmosome. The area composita only exists in the cardiac intercalated disc of mammalian species suggesting that it evolved to strengthen mechanical coupling in the heart of higher vertebrates. The cross-talk among different junctions and their implication in the pathogenesis of ARVC are discussed in this review.
Dermatol Res Pract 2010
PMID:A new perspective on intercalated disc organization: implications for heart disease. 2058 98

Healthy-appearing hair is a sign of excellent general health, as well as good hair care practices. Most healthy individuals have adequate nutrients in their diet; however, many people do not have access to good nutrition, and others have medical illnesses that predispose them to nutritional deficiency. This is often reflected in changes of scalp and, at times, body hair. Malnutrition, congenital heart disease, neuromuscular disease, chronic illnesses, malignancy, alcoholism, and advanced age can cause hair to change color, be weakened, or lost. Recognition of the populations at risk for vitamin deficiency is the first step to their detection. Changes in skin and hair can provide clues to the presence of an underlying vitamin deficiency.
Clin Dermatol
PMID:Nutrition and hair. 2062 Jul 58


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