Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0009443 (cold)
 92,137 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Digital blood pressure was measured using photoplethysmography in patients with cold sensitivity of the hand. In 19 patients with Buerger's disease, or arteriosclerosis obliterans (arterial occlusion group), 90 of 91 fingers with cold sensitivity showed significantly low pressures. In 17 patients with typical Raynaud's phenomena due either to primary Raynaud's disease or secondary to collagen disease (Raynaud's group), decreased digital pressure was noted in only five of 123 fingers with cold sensitivity. Blood pressure measurements in the fingers after local cooling of the hand showed a more severe response to cold in the Raynaud group than in the arterial occlusion group. These results indicate that the pathophysiologic mechanism for cold sensitivity in arterial occlusive disease is different from that in Raynaud's disease. In the arterial occlusion group impaired circulation due to occlusions in the digital arteries or more proximal arteries is a necessary precondition for cold sensitivity, and an increased sympathetic response to cold is of less importance as an etiologic factor. Thus a patient with cold sensitivity of the hand and normal digital blood pressure should not be considered to have arterial occlusive disease as the underlying cause of cold sensitivity.
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PMID:Cold sensitivity of the hand in arterial occlusive disease. 41 55

Raynaud's phenomenon (RP) which is the episodic and reversible vascular spasm of the extremities can be elicited by cold or emotional changes. RP has been classified into two groups. One is primary Raynaud's phenomenon (PRP) with no identifiable underlying disease and/or cause, and the other is the secondary form of RP (Raynaud's syndrome) with underlying disease and/or cause, e.g., collagen disease, trauma, vibration from vibrating tools, etc. In the present study using this definition of RP the prevalences of RP were studied in general populations in a mountain village "T" and in a town "K" located in a plain area in Gifu prefecture, Japan. The prevalences of RP in T Village were 8.0% (25/314) in males and 3.5% (16/454) in females, and in K Town 2.7% (7/259) in males and 3.2% (21/657) in females. In 52% of the male cases in T Village it was estimated that the exposure to vibration from vibrating tools was the cause of RP. In K Town no male cases of RP were induced by vibration. Exposure to vibration may be one of the important factors when prevalences of RP are investigated in various areas. There were no significant differences between the two areas in the prevalences of PRP (T Village: male 2.9%, female 3.1%; K Town: male 2.7%, female 2.9%). The annual mean air temperature was usually 5-6 degrees C lower in T Village than that in K Town. From the results of this study the differences in the mean air temperature did not appear to have a significant influence on the prevalence of PRP in each area.
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PMID:[Differences in the prevalences of Raynaud's phenomenon in general populations living in a mountain area and in a plain area]. 150 36

Numerous methods for the exploration of the microangiopathy of collagen disease exist, but their perspective differs as a function of their use to assist early diagnosis, to establish a prognosis, to provide follow-up data or to attempt better understanding of the mechanism of these affections. Progressive systemic sclerosis is a very suitable collagen disease with which to illustrate these methods, since in this affection the microangiopathy is frequent, diffuse, of early onset and also formally implicated in the pathogenic rank. Biopsy specimens provide details of morphologic anomalies of the microcirculation. Capillary changes have been demonstrated in muscle and the choroid but in clinical practice histology samples involve mainly the digital pulp, subungual fold and accessory salivary glands. Isotopic and fluorescent angiography are, on the contrary, in vivo methods for dynamic exploration of skin microcirculation, but the invasive nature of these methods is an obstacle to their repetition in a same patient and to follow-up of the microangiopathy. Non-invasive vascular explorations avoid this pitfall. Thermometry, thermography, digital plethysmography under hot and cold conditions, videomicroscopy of the nailbed, percutaneous PO2 and laser-doppler tests measure functional parameters, the hemodynamic significance of which require discussion. Capillaroscopy, mainly oriented towards the study of capillary morphologic changes, also allows, together with plethysmography, measurement of digital systolic pressure. Exploration of the microangiopathy of collagen disease requires a judicious combination of currently available morphologic and functional techniques which, far from being superimposable are on the contrary complementary.
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PMID:[How to explore the microangiopathy of collagen diseases]. 409 14

The effectiveness of a new, nifedipine-like calcium antagonist (Bay K 9320) on the severity and frequency of episodic digital vasospasms was studied in a randomized double-blind trial of 24 patients (10 women, 14 men) with Raynaud's phenomenon. During the winter months the patients took one tablet three times daily for three weeks, each tablet either containing 20 mg of the calcium antagonist or a placebo. Three patients had collagen disease, 21 a primary form of Raynaud's phenomenon. Subjective improvement was reported by nine of twelve patients receiving the drug, while no improvement occurred in nine patients on the placebo. Light-plethysmography demonstrated under calcium antagonist treatment a reduction in the cold-provoked acral blood flow decrease more than four-fold compared with the unchanged value in the placebo group.
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PMID:[Calcium antagonists for the treatment of Raynaud's phenomenon]. 635 92

A 70-year-old woman was admitted to our hospital in November 1992 for evaluation of anemia. Physical examination revealed anemia, jaundice, swelling of axial and inguinal lymph nodes, and splenomegaly. Abnormal hematological findings were as follows: Hb of 3.9 g/dl, reticulocyte count of 58.2% (61.7 x 10(4)/microliters), hyperplasia of normal erythroblasts in bone marrow, and eosinophilia (21.0%, 2352/microliters) in peripheral blood. Routine laboratory examinations revealed polycolonal hypergammaglobulinemia 3.0 g/dl, a high level of serum LDH (797 IU/I) and a total bilirubin of 2.4 mg/dl (indirect, 1.6 mg/dl). The serum haptoglobin level was very low (< 5 mg/dl). Results of serological examinations were as follows: IgG of 3366 mg/dl, CH50 of 16.0 U/ml, positive Coombs test 2+, and positive tests for antinuclear antibody, rheumatoid factor, and cold agglutinin. CRP was negative. PHA-stimulated lymphocyte blast formation, NK activity, and ADCC activity were found to be suppressed, and the percentage of CD4-positive lymphocytes in peripheral blood was also low. An axillary lymph node biopsy revealed reactive lymphadenitis. No signs or history suggested allergy, collagen disease, or parasitic infection. Autoimmune hemolytic anemia (AIHA) complicated by immunologic abnormalities and eosinophilia was diagnosed. Oral prednisolone markedly reduced the hemolytic anemia, eosinophilia, lymph node swelling, and splenomegaly, but NK activity remained low.
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PMID:[Autoimmune hemolytic anemia with eosinophilia in elderly patient]. 892 99

52 patients (30 male, 22 female) with paroxysmal cold haemoglobinuria (PCH) were identified by critically examining the records of all cases with Donath-Landsteiner antibodies seen over a 37 year period. Although ages ranged from 1-82 years, PCH was much commoner in young children; the median age at presentation was 5 and the peak incidence, 0.4 per year per 100,000 of the population at risk, was in the 4 years and under group. 44 patients had acute transient PCH, 3 chronic non-syphilitic PCH and 1 chronic syphilitic PCH; 4, in whom the positive Donath-Landsteiner tests were incidental findings, could not be classified. Acute PCH typically presented in young children as sudden onset of malaise, haemoglobinuria and pallor, often associated with mild jaundice - all 30 patients who were 13 or younger had this type. There was usually a history of a recent viral type infection, most commonly of the upper respiratory tract. The occurrence of acute PCH had no obvious relation to exposure to cold. Dramatic and rapid falls in haemoglobin level were common, often accompanied initially by relative or absolute reticulocytopenia. The illness was severe, but the prognosis was generally good and the majority of patients had completely recovered within one month, some requiring no treatment. In approximately 68% of patients, blood transfusion was needed; the P blood group was not taken into account, but the patients were kept warm throughout. Steroids (usually prednisolone) were given in many cases; but since there was no evidence to support their benefit, it was recommended that they were stopped as soon as the diagnosis was confirmed. Chronic non-syphilitic PCH was much rarer; the patients had a characteristic presentation of severe systemic symptoms (paroxysms) and haemoglobinuria brought on by exposure to cold. The clinical manifestations varied in intensity between individuals; at the extreme, severe debility was experienced over many years. Warmth and avoidance of cold were an effective treatment, though in a severely afflicted patient, an attack could be precipitated by relatively little exposure to cold. With chronic syphilitic PCH there was the added need to treat the specific infection. The direct antiglobulin test was almost always positive (50 out of 51 cases tested), with C3d coating the red cells. The Donath-Landsteiner antibodies were of IgG class, but this was rarely demonstrated unless direct antiglobulin tests were carried out at 4 degrees C. The antibodies showed the classical anti-P specificity in 27 of the 30 patients tested; other specificities were unusual. Although acting much better as haemolysins, Donath-Landsteiner antibodies could also cause weak agglutination at room temperature. This was paralleled in vivo by predominantly intravascular haemolysis with an extravascular component. Diagnosis was usually easy when PCH was suspected, though in some patients Donath-Landsteiner tests did not become positive until more sensitive techniques involving papainised red cells or two-stage procedures were employed. Of particular interest was the association in some cases with lymphoproliferative disorders, collagen disease, myelodysplastic syndrome, delayed haemolytic transfusion reaction and other types of autoimmune haemolytic anaemia. In one patient, an aetiological relationship was confirmed by a saline extract of lymphoma tissue behaving as a strong Donath-Landsteiner antibody with the same anti-P specificity as the serum. 4 patients had other types of autoimmune haemolysis concomitant with, but distinct from, the PCH; in 3 cases this was cold haemagglutinin disease and in one it was warm type autoimmune haemolytic anaemia.
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PMID:Erythropoiesis: Paroxysmal Cold Haemoglobinuria: A Clinico-Pathological Study of Patients with a Positive Donath-Landsteiner Test. 1139 60

The term autoinflammatory syndromes describes a distinct group of systemic inflammatory diseases apparently different from infectious, autoimmune, allergic and immunodeficient ones. Originally, it was almost synonymous with clinically defined hereditary periodic fever syndromes, including familial Mediterranean fever, hyper immunoglobulin D syndrome with periodic fever and tumor necrosis factor receptor-associated periodic syndrome. Similar but distinct periodic fever syndromes accompanied by urticarial rash, familial cold autoinflammatory syndrome, Muckle-Wells syndrome and chronic infantile neurological cutaneous articular syndrome, have all been reportedly associated with CIAS1 mutations and are collectively called cryopyrin-associated periodic syndromes. Consequently, the concept of autoinflammatory syndromes has been spread to contain other systemic inflammatory diseases: rare hereditary diseases with or without periodic fevers, such as pyogenic sterile arthritis, pyoderma gangrenosum and acne syndrome, Blau syndrome and chronic recurrent multifocal osteomyelitis, and the more common collagen disease-like diseases, such as Behcet's disease, Crohn's disease, sarcoidosis and psoriatic arthritis. These diseases are all caused by or associated with mutations of genes regulating innate immunity and have common clinical features accompanied with activation of neutrophils and/or monocytes/macrophages. In this review, major autoinflammatory syndromes are summarized and the pathophysiology of related skin disorders is discussed in association with dysregulated innate immune signaling.
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PMID:Autoinflammatory syndromes with a dermatological perspective. 1772 63

A 58-year-old female with systemic lupus erythematosus (SLE) and anti-phospholipid syndrome (APS) was referred and admitted to our hospital because of fever and pancytopenia. She had been taking small dose of prednisolone and azathiprine since 2003 with stable SLE and APS. Two weeks before the admission, she developed fever and common cold-like symptoms and was admitted to a hospital. Antibiotics were ineffective and thrombocytopenia manifested. Under a diagnosis of progressive SLE, bolus methylprednisolone was administrated. However, she became pancytopenic and was transferred to our hospital. A bone marrow aspirate showed the hemophagocytosis and chest CT scanning revealed interstitial pneumonia. Cytomegalovirus (CMV) antigenemia test gave a positive result. A diagnosis of CMV-induced hemophagocytic syndrome (HPS) and CMV pneumonia was made. Gancyclovir resolved the pancytopenia, pneumonia, and fever. There have been only 3 reported cases of CMV-related HPS in collagen disease.
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PMID:Systemic lupus erythematosus complicated by cytomegalovirus-induced hemophagocytic syndrome and pneumonia. 1831 Oct 46

Cryofibrinogenemia is a cryoprotein that was first identified in 1955 by Korst and Kratochvil. Unlike cryoglobulin, the precipitate forms only in plasma and not in the serum. The presence of cryofibrinogen in plasma can be asymptomatic. Cryofibrinogenemia is considered a rare disorder: its prevalence varies from 0% to 7% in healthy subjects and from 8% to 13% in hospitalized patients. Nevertheless, cryofibrinogenemia, when a cryopathy is clinically suspected, has been reported in 12% to 51% of patients. Skin manifestations are usually the first signs and are usually moderate; in addition, cold intolerance, Raynaud phenomenon, purpura, or livedo reticularis often occurs. Skin necrosis, acral ulcers, and gangrene can lead to surgery and amputation. Systemic manifestations are common, and arterial or venous thrombotic events are frequent. Cryofibrinogenemia may be primary (essential) or secondary to other underlying disorders, such as carcinoma, infection, vasculitis, collagen disease, or associated with cryoglobulinemia. The histological features of cryofibrinogenemia can confirm the presence of cryofibrinogen within small and medium arteries, plus occlusive thrombotic diathesis composed of eosinophilic refractile deposits within vessel lumina. Cryofibrinogenemia is a treatable and potentially reversible disease.In moderate forms, it can be treated by simply avoiding cold temperatures. The use of corticosteroids in association with low-dose aspirin is the treatment of choice for moderate forms, although stanozolol is an alternative maintenance therapy. Immunosuppressive therapies, plasmapheresis, and/or intravenous fibrinolysis are useful at treating severe forms of cryofibrinogenemia. The use of anticoagulants is limited to the management of thrombotic events. Treatment of secondary cryofibrinogenemia involves the management of associated diseases. Regular follow-ups are needed because of the high risk of recurrence. Moreover, up to half of patients with cryofibrinogenemia considered as essential may develop lymphomas in the following years. Compared with cryoglobulinemia, less is known about cryofibrinogenemia. Its diagnosis should be considered when suggestive clinical manifestations are present and when there are specific biopsy findings. Although identification of cryofibrinogen in blood samples is simple and inexpensive, cryofibrinogenemia can be asymptomatic, and a lack of diagnosis criteria can make diagnosis difficult to confirm. This review describes the clinical manifestations and the biological and pathological features and discusses the criteria used to diagnose and manage cryofibrinogenemia.
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PMID:Cryofibrinogenemia. 2351 83

A 58-year-old man was admitted with shortness of breath in September 2019. He had a severe hemolytic anemia with a high cold agglutinin (CA) titer. He also had arthralgia and finger deformation. He was diagnosed with cold agglutinin syndrome (CAS) secondary to rheumatoid arthritis (RA) based on the clinical course. Occasionally, CAS has been reported to occur in parallel with collagen disease, infectious disease, or malignant tumor. CAS developing secondary to collagen disease occurs less frequently than that to infectious disease or malignant tumors. Furthermore, CAS caused by RA is very rare, even among patients with collagen diseases. Our patient was effectively treated with immunosuppressive therapy including abatacept, which attenuated the symptoms of CAS and RA.
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PMID:[Successful management of cold agglutinin syndrome developing subsequent to rheumatoid arthritis with immunosuppressive therapy]. 3275 59


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