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CLICK ON THE IMAGE

Syn: Scurvy

Ascorbic acid is a powerful biologic reducing agent that is involved in numerous oxidation-reduction reactions and the transfer of protons.

Nutritional Pathology Online : click

This vitamin is important in the synthesis of chondroitin sulfate and in the hydroxylation of proline to form the hydroxyproline of collagen.

 Vitamins : click

It serves many other important functions, such as preventing the oxidation of tetrahydrofolate and augmenting the absorption of iron from the gut.

Without vitamin C the biosynthesis of certain neurotransmitters is impaired because of a reduction in the activity of dopamine-beta-hydroxylase.

Wound healing and immune functions are also under the influence of ascorbic acid.

The best dietary sources of vitamin C are citrus fruits, green vegetables, and tomatoes.

The clinical vitamin C deficiency state is termed scurvy.

The first demonstration of the need for this vitamin was the remarkable effect of lime in preventing scurvy among 18th century British sailors. The distribution of limes in the British navy led to the term "limey" for the sailors.

Scurvy is uncommon in the Western world, but is often seen in non-industrialized countries in which other forms of malnutrition are prevalent.

In the industrialized countries, scurvy is now a disease of people  with chronic diseases who do not eat well, the neglected aged, and malnourished alcoholics.

Elderly individuals who consume a “tea and toast” diet are particularly vulnerable to ascorbic acid deficiency because of an inadequate intake of the vitamin.

The stress of cold, heat, fever, or trauma (accidental or surgical) leads to an increased requirement for vitamin C.

Children who are fed milk for the first year of life develop scurvy, as do alcoholics.

However, in alcoholics, vitamin C deficiency is not as common as deficiency of the B vitamins.

The rate of catabolism of ascorbic acid is about 3% of the body pool a day, a value which is consistent with the fact that on a diet lacking in vitamin C the symptoms of scurvy take some months to develop.

In the early stages of vitamin C deficiency, nonspecific symptoms of weakness and lethargy are noted.

Most of the subsequent events are caused by the formation of abnormal collagen that lacks tensile strength.

Within 1 to 2 months, subperiosteal hemorrhages lead to pain in the bones and joints.

Petechial hemorrhages, ecchymoses, and purpura are common, particularly after mild trauma or at pressure points.

Perifollicular hemorrhages in the skin are particularly typical of scurvy.

In advanced cases, swollen, bleeding gums are a classic finding.

Alveolar bone resorption results in loosening and loss of teeth.

Wound healing is poor and dehiscence of previously healed wounds occurs.

Anemia may result from prolonged bleeding, impaired iron absorption, or an associated folic acid deficiency.

In children, vitamin C deficiency leads to growth failure and collagen-rich structures such as the teeth, bones, and blood vessels develop abnormally.

The effects on developing bone are conspicuous and relate principally to impaired calcification.

In addition to poor wound healing, patients have difficulty in preventing an infection to form an abscess, and infections therefore spread more easily.

The diagnosis of scurvy is confirmed by finding low levels of ascorbic acid in the serum.

Mild depression of ascorbate levels also occurs in other conditions, however, including cigarette smoking, tuberculosis, rheumatic fever, and many debilitating disorders.

Some women who use oral contraceptives may have a mild decrease in serum vitamin C levels.

                         

Infantile scurvy: an old diagnosis revisited with a modern dietary twist. Am J Clin Dermatol. 2007;8(2):103-6.

Ascorbic acid (vitamin C) is necessary for the formation of collagen, reducing free radicals, and aiding in iron absorption. Scurvy, a disease of dietary ascorbic acid deficiency, is uncommon today. Indeed, implementation of dietary recommendations largely eradicated infantile scurvy in the US in the early 1900s. We present a case of an otherwise healthy 2-year-old Caucasian girl who presented with refusal to walk secondary to pain in her lower extremities, generalized irritability, sleep disturbance, and malaise. The girl's parents described feeding the patient an organic diet recommended by the Church of Scientology that included a boiled mixture of organic whole milk, barley, and corn syrup devoid of fruits and vegetables. Physical examination revealed pale, bloated skin with edematous, violaceous gums and loosening of a few of her teeth. Dermatologic findings included xerosis, multiple scattered ecchymoses of the extremities, and perifollicular hemorrhage. Laboratory and radiographic evaluation confirmed the diagnosis of scurvy. The patient showed dramatic improvement after only 3 days of treatment with oral ascorbic acid and significant dietary modification. In this case report, we revisit the old diagnosis of scurvy with a modern dietary twist secondary to religious practices. This case highlights the importance of taking a detailed dietary history when evaluating diseases involving the skin.

Vitamin C measurements in vulnerable populations: 4 cases of scurvy.Ann Biol Clin (Paris). 2007 Jan-Feb;65(1):65-9.

Hypovitaminose C, at the origin of the scurvy, did not disappear. We report 4 cases of desocialized patients and whose very unbalanced diet was at the origin of the vitamin deficit. In addition to the hemorrhagic demonstrations described among 4 patients, one finds a modification of the superficial body growths. Physiologically, the vitamin C takes part in the stability of the collagen of the vascular wall and the bone. The biological assessment of the 4 patients was normal apart from the low ascorbemy, a syndrome of denutrition and anaemia related to the haemorrhage. The quality of the vitamin C determination requires to respect the recommended pre-analytical conditions. The treatment quickly effective, consists of an oral administration of ascorbic acid. The evolution for 3 of the 4 described patients was favorable without after-effects. The proportioning of the vitamin C should be more often prescribed in geriatrics, psychiatry and cancerology. It should systematically form part of the biological assessment of the people living alone and/or homelessness.

Vitamin C. Actas Dermosifiliogr. 2006 Nov;97(9):557-68.

Vitamin C or ascorbic acid is a hydrosoluble vitamin derived from glucose metabolism. It acts as a reductor agent required for synthesis of collagen fibers through hydroxylation of proline and lysine. It also protects the body against damage caused by the free radicals. Humans cannot synthesize ascorbic acid as they lack an enzyme called gulonolactone oxidase. Concentrations in plasma and leukocytes reflect the levels of the diet and body deposits respectively of this vitamin. Among foods with high vitamin C levels are tomatoes, potatoes, and citrus fruits such as limes, oranges and lemons. The current recommendation of daily intake of vitamin C is 90 mg/d for men and 75 mg/d for women. Patients with chronic diseases such as cancer or diabetes or those who smoke need higher doses in their usual diet. Ascorbic acid deficiency gives rise to the appearance of scurvy. This disease is rarely seen in developed countries. The symptoms develop with plasma levels below 0.15 mg/dL. Scurvy is characterized by the presence of weakness, joint pain or skin lesions in form of petechias, gum bleeding, ease of developing bruises or delay in wound healing. The most characteristic skin manifestations are purpuric perifollicular hyperkeratotic papules and the presence of kinky hair.

Scurvy: a disease almost forgotten.Int J Dermatol. 2006 Aug;45(8):909-13.

BACKGROUND: Although much decreased in prevalence, scurvy still exists in industrialized societies. Few recent large studies have examined its pathogenesis, signs, and symptoms. METHODS: After we diagnosed scurvy in a 77-year-old female patient in 2003, we conducted a retrospective records review to identify patients with scurvy treated between 1976 and 2002 at Mayo Clinic (Scottsdale, Arizona; Rochester, Minnesota; or Jacksonville, Florida). We also searched the English-language medical literature for published reports on scurvy. RESULTS: In addition to our patient, seven of 11 patients whose records in the institutional database mentioned vitamin C deficiency were women. The age ranged from a neonate to 77 years (mean, 48 years). The most common associated causes were concomitant gastrointestinal disease, poor dentition, food faddism, and alcoholism. Vitamin or mineral deficiencies other than vitamin C deficiency were also found in our patients who had scurvy. The most common symptoms were bruising, arthralgias, or joint swelling. The most common signs were pedal edema, bruising, or mucosal changes. Four patients had vague symptoms of myalgias and fatigue without classic findings, and five had concomitant nutritional deficiencies. Follow-up available for six of 12 patients treated by vitamin C supplementation showed complete resolution of symptoms in five. CONCLUSIONS: Patients with scurvy may present with classic symptoms and signs or with nonspecific clinical symptoms and an absence of diagnostically suggestive physical findings. Concomitant deficiency states occur not uncommonly. Taking a thorough dietary history and measuring serum ascorbic acid levels should be considered for patients with classic signs and symptoms, nonspecific musculoskeletal complaints, or other vitamin or mineral deficiencies.

Is sudden death with vitamin C deficiency caused by lack of carnitine?: J Clin Forensic Med. 2006 Jan;13(1):26-9. 

We investigated the effect of carnitine supplementation during vitamin C (ASC) deficiency by measuring the levels of ASC and carnitine in plasma and cardiac muscle cells (CMC), and histological analysis with electron microscopy. The levels of carnitine were significantly decreased in ASC-deficient rats in plasma and the heart than those in the control. In carnitine supplemented ASC-deficient rats, a significant increase of carnitine levels were observed in both plasma and heart. The number of lipid droplets significantly increased in the ASC-deficient rats compared to the control rats, but did not increase in carnitine supplemented rats. These results indicate that ASC deficiency causes a generalized mitochondrial abnormality and accumulation of lipid droplets in CMC as observed in carnitine deficiency, and supplementation of carnitine prevented these changes even in the presence of ASC deficiency.

Scurvy: follicular purpura as a diagnostic sign. Actas Dermosifiliogr. 2005 Jul-Aug;96(6) :400-2.

Scurvy is a set of clinical manifestations characterized by general weakness, anemia, gingivitis and cutaneous bleeding, caused by a lack of ascorbic acid in the diet. This pathology is currently a clinical rarity, although it can still be seen in cases of malnutrition associated with alcoholism or with dietary deficiencies, especially in childhood and old age. We present the case of a 45-year-old male who consulted his physician because of lower limb edema with follicular purpura, accompanied by asthenia, polyarthralgia and bleeding gums. After treatment with 1 g/day of vitamin C was initiated, the patient's symptoms quickly improved. The diagnosis of scurvy was based on the patient's clinical symptoms, dietary history and the rapid resolution of the symptoms when vitamin C supplements were initiated.

Musculoskeletal manifestations of scurvy.Joint Bone Spine. 2005 Mar;72(2):124-8.

Scurvy occurs in individuals who eat inadequate amounts of fresh fruit or vegetables, often because of dietary imbalances related to advanced age or homelessness. Asthenia, vascular purpura, bleeding, and gum abnormalities are the main symptoms. In 80% of cases, the manifestations of scurvy include musculoskeletal symptoms consisting of arthralgia, myalgia, hemarthrosis, and muscular hematomas. Vitamin C depletion is responsible for structural collagen alterations, defective osteoid matrix formation, and increased bone resorption. Imaging studies may show osteolysis, joint space loss, osteonecrosis, osteopenia, and/or periosteal proliferation. Trabecular and cortical osteoporosis is common. Children experience severe lower limb pain related to subperiosteal bleeding. Laboratory tests show nonspecific abnormalities including anemia and low levels of cholesterol and albumin. The finding of a serum ascorbic acid level lower than 2.5 mg/l confirms the diagnosis. Vitamin C supplementation ensures prompt resolution of the symptoms.

Vitamin C deficiency. Rev Med Interne. 2004 Dec;25(12):872-80.

BACKGROUND: Hypovitaminosis C is frequent in populations at risk (men who live alone, old people, homelessness, patients with psychiatric diseases, foodfaddists,...) and is underestimated in the general population. STRONG POINTS: Scurvy occurs after 3 months without consumption of ascorbic acid, and is due to lack of consumption fresh fruits and vegetables. Clinical manifestations are weakness, myalgia and arthralgia, vascular purpura and hemorrhagic syndrome, and later the stomatologic manifestations: gingivorragia and loss of teeth. Biological signs are nonspecific: anemia, hypocholesterolemia, hypoalbuminemia. Clinical suspicion must be confirmed by a low level of ascorbic acid (<2.5 mg/l), but this value needs to be interpretated according to the presence of an acute phase response. Leucocyte ascorbic acid level reflects total body store and is more reliable, but not available in practice. Treatment consists in administration of 1 g vitamin C per day during 15 days. PERSPECTIVES: Vitamin C depletion (serum ascorbic acid level between 2 and 5 mg/l) may occur long-term complications such as increase cardiovascular and neoplasic risks or cataract. The new recommended dietary allowance of vitamin C (110 mg per day for an adult) takes into account of these risks.

Scurvy: historical review and current diagnostic approach.Am J Emerg Med. 2003 Jul;21(4):328-32.

Scurvy, a deficiency of vitamin C, now most often occurs in disadvantaged groups seen frequently in EDs: alcoholics with poor nutrition, the isolated elderly, and the institutionalized. Its prominent clinical features are lethargy; purpuric lesions, especially affecting the legs; myalgia; and, in advancing disease, bleeding from the gums with little provocation. Common misdiagnoses are vasculitis, blood dyscrasias, and ulcerative gingivitis. Untreated, scurvy is inevitably fatal as a result of infection or sudden death. Fortunately, individuals with scurvy, even those with advanced disease, respond favorably to administration of vitamin C.

Vitamin C and bone markers: investigations in a Gambian population.Proc Nutr Soc. 2003 May;62(2):429-36.

Vitamin C is an essential micronutrient. Absence from the diet will result in the deficiency disease scurvy, typically characterised by weakening of collagenous structures. High intakes of vitamin C have been associated with decreased incidence or severity of a number of diseases, including cancer and cardiovascular disease. These beneficial effects may be attributed to its antioxidant properties, although the exact mechanisms of action remain elusive. It is also unclear what intake levels are required for optimal health benefits. The task of defining optimal intakes is hindered by the lack of a reliable functional marker of tissue vitamin C status in man. Many different pathways have been investigated, but none of them have measurable outcome variables relating directly to scorbutic changes. The bone-collagen formation pathway has the potential to provide a functional index of tissue vitamin C adequacy. Vitamin C acts as a cofactor for the enzyme lysyl hydroxylase, which is required for the hydroxylation of lysine residues in procollagen chains. Pyridinoline is a mature collagen cross-link formed from three hydroxylysine residues, deoxypyridinoline is formed from two hydroxylysine and one lysine residue. Guinea-pig studies have shown an alteration in the pyridinium cross-link ratios in response to graded vitamin C intakes (Tsuchiya & Bates, 1998). In order to investigate whether these changes can be seen in a human population group, a study was carried out in rural Gambia, where there is a marked seasonal variation in dietary vitamin C. The present review discusses the rationale behind the study and presents some preliminary results.

 


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