[Diagnostic image (40). Scurvy].
In a 17-year-old woman with anorexia nervosa, scurvy was diagnosed as the cause of fatigue, abdominal pain, vaginal bleeding and several distinctive skin lesions.
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In a 17-year-old woman with anorexia nervosa, scurvy was diagnosed as the cause of fatigue, abdominal pain, vaginal bleeding and several distinctive skin lesions.
An 8-year-old child with cerebral palsy came with progressive purpuric rash affecting the trunk and legs. He had tenderness on palpation of his extremities. Physical examination revealed a moderately pale and cachectic boy. There was bleeding per swollen gums and petichiae on the hard palate. Generalized multiple discrete palpable petichiae spots at hair follicles along the whole body, more on both legs, were observed. He also had tenderness on palpation of his extremities. His hemoglobin was 6.6 g/dl. Platelet count and coagulogram were normal. Roentgenographic findings showed generalized osteoporosis, metaphyseal white line of distal femur, proximal tibia. proximal fibula, distal radius, and distal ulna with submetaphyseal lucency bilaterally. Skin biopsy showed dilated hair follicles, filled with keratinous material and a small corkscrew hair. A diagnosis of scurvy was made; and vitamin C at a dosage of 300 mg per day was given. His swollen gums, bleeding per gums and muscle tenderness improved within 2 days. Perifollicular hemorrhage, follicular hyperkeratosis, and anemia improved in 2 and 3 weeks respectively.
The authors report the case a 11-month-old baby girl admitted by the "Mother and Child Welfare" doctor. On admission, she was very hypotrophic, febrile and pale. Furthermore, she had anaemia complicated with painful invalidating oedema of both thighs. X-ray film show moderate lesions of the metaphysis, but very rapidly, there was a hugh bilateral sub-periosteal hematoma. They insist on the value of periosteal hematoma as a characteristic sign of scurvy. This was later confirmed by laboratory investigations and by the finding of urinary infection due to bilateral reflex on cystography and to right uretero-hydronephrosis on I.V.P. This case is singular because it associates three findings: severe vitamin deficiency by dietetic error due to modern industrial alimentation and a major urinary tract malformation with refractory urinary tract infection.
An 91 years old woman was hospitalized because of lethargy, shortness of breath and diffuse subcutaneous hemorrhage of legs. Clinical features were consistent with the diagnosis of vasculitis with systemic involvement. However dermatologic characteristics of the legs, in association with malnutrition, suggested vitamin C deficiency which was confirmed by laboratory test. Ascorbic acid supplement dramatically improved her clinical symptoms. This case remarks how scurvy may mimmick a systemic vasculitis.
In 1535, when Jacques Cartier landed in Canada for the second time, he sailed up the Saint-Lawrence, and spent the winter at Stadaconé. His ships were frozen in the ice for five months. A strange disease attacked the indigenous population, and then the French sailors. In a two month period, almost all Cartier's men became sick. Facing such a tragic situation, the explorer carried out an examination on a dead sailor and described what would later be called scurvy. The Indians were able to extract a medicine from a tree called Anneda. This medicine worked quickly and allowed the sailors to leave Canada in May. However, 25 of the 112 sailors had died during that icy winter.
The osteogenic disorder Shionogi (ODS) rat is a mutant Wistar rat that is subject to scurvy, because it lacks L-gulono-gamma-lactone oxidase, a key enzyme in L-ascorbic acid biosynthesis. Sequencing of polymerase chain reaction-amplified cDNAs for mutant and normal rat L-gulono-gamma-lactone oxidases demonstrated that the mutant cDNA has a single base mutation from G to A at nucleotide 182, which mutation alters the 61st amino acid residue from Cys to Tyr. To test the effect of this mutation on the expression of L-gulono-gamma-lactone oxidase, we inserted a region of the cDNAs coding for normal and mutant L-gulono-gamma-lactone oxidases into an expression vector, pSVL, and transfected COS-1 cells with such vectors. The result indicated that the defined amino acid substitution does decrease both the amount of immunologically detectable protein and the level of enzyme activity to about one-tenth of their normal values, while it does not affect the amount of the mRNA produced in the transfected cells. This situation is similar to our previous observation that L-gulono-gamma-lactone oxidase is expressed in the liver of the ODS rat at a very low level irrespective of the presence of a normal amount of L-gulono-gamma-lactone oxidase-specific mRNA of a normal size (Nishikimi, M., Koshizaka, T., Kondo, K., and Yagi, K. (1989) Experientia (Basel) 45, 126-129). Thus it became clear that the Cys-->Tyr substitution is responsible for the L-gulono-gamma-lactone oxidase deficiency in the ODS rat.
Alvaro Velho's account of Vasco da Gama's first journey to India (1497-1499), describing, among other things, the anguish and pains suffered by the crew because of scurvy, represents, with the striking vividness of its images, one of the oldest references to a terrifying disease to which seamen then began to be exposed, as well as to the use of fresh fruit, and vegetables as a successful method to rescue them, and in a certain degree, from this distemper at sea. This paper analyzes Alvaro Velho's statements from a scientific point of view, whereas it stresses the accent on what contemporary Portuguese historians, and chroniclers, as well as foreign travellers, and merchants, thought about it, often misinterpretating its causes, just like Alvaro Velho did.
Scurvy is caused by severe deficiency of vitamin C or ascorbic acid, and is usually diagnosed by the overt clinical signs which appear at an advanced stage. Laboratory examinations, and in particular ascorbic acid assays, do not yet enable subclinical vitamin C deficiency to be reliably detected; hence the importance of knowing the situation which expose to this deficiency.
Ascorbic acid (AsA) concentrations in plasma exert a modulating effect on the activity of liver enzymes. Since UDP-glucuronyl transferase is a liver enzyme, which is responsible for bilirubin glucuronidation, the effect of varied amounts of AsA on this enzyme activity was studied. Sixty male guinea-pigs were randomly allocated to the following six groups: controls, scorbutic and groups given 2, 5, 10 or 20 mg of ascorbic acid, respectively. All the animals with the vitamin C deficient diet presented clinical signs of scurvy at the end of the experimental period, and had lost both body and liver weight compared to all other groups. Scorbutic animals had very low levels of AsA in the liver compared with controls (0.20 +/- 0.10 and 1.65 +/- 0.45 mg/g liver, respectively) (p less than 0.001). Liver AsA levels increased as the AsA dose increased. The UDP-glucuronyl transferase activity was lower in scorbutic animals than in controls (6.20 +/- 1.95 and 23.85 +/- 4.20 mg bilirubin/g protein/h, respectively) (p less than 0.001). The other groups C, D, E and F also had higher mean levels of UDP-GT activity than the scorbutic group B. Finally, no correlation was found between UDP-glucuronyl transferase activity and ascorbic acid intake.
The translation form Latin of a XVIIth century medical document concerning scurvy is reported. Stress is laid on the importance of historical research of this type as an intellectual exercise and as a historical curiosity.
The case of a 10-month-old boy with scurvy and epiphyseal displacement of both the distal femur and the right proximal tibia is presented. He was treated without reduction of the displacement.
Vitamin C (ascorbic acid) has been known to prevent scurvy for many years. Recent research has shown its importance in lipid and iron metabolism. Vitamin C may also have some effect on the immune system. There is not as yet conclusive evidence that ascorbic acid may cure or prevent colds or cancer. The vitamin has few side effects even when ingested in large quantities. Several methods for analysis of ascorbic acid have been developed. These include titration and fluorometric methods, a ferrozine technique automated for centrifugal analyzers, a high performance liquid chromatography method, and a dip-stick for urine ascorbic acid.
Scintigraphic bone changes in two cases of scurvy are described, the cause being subperiosteal hematoma. Generalized increased uptake was shown in the affected femur at an early state of the disease. At a late stage, marked uptake both in the femoral shaft and surrounding tissue was seen causing scintigraphic appearance of widened ("club-shaped") femur.
Bilateral epiphyseometaphyseal cupping of the distal femoral epiphysis occurred in a 9-year-old girl with a history of infantile scurvy. This rare condition is characterized by a cup-shaped depression of the metaphyseal end, growth disturbance at the distal femoral epiphysis and knee flexion contracture which develops slowly over many years of bone growth. Conservative treatment by corrective plasters and back slabs brought about recovery of the right knee flexion contracture and halted the growth disturbance. However, in the left knee, extension supracondylar femoral osteotomies had to be performed and severe growth disturbance of the distal femoral epiphysis led to premature epiphyseal fusion.
We report a case of scurvy. This case is interesting by the unilateral character of the ecchymotic leg's purpura. Ascorbic acid assays do not yet enable subclinical vitamin C deficiency to be reliably detected. Hence the importance of knowing the situation which expose to this deficiency.
We report a 66 years-old man case that was admitted with abdominal wall hemorrhagic swelling ecchymosis on inferior extremities and perifollicular purpura with hyperkeratosis of the follicles, which gives it an appearance of palpable purpura, together with gingival hemorrhage and moderate anemia. He was a well-nourished sick man but who made a peculiar diet, practically free from fruit and vegetables which developed a pure and serious form of scurvy. We report this case because we consider that the features which appear are illustrative to provide the immediate clinical diagnosis of this uncommon disease, potentially mortal, but easy to diagnose, if we think about it in the adequate clinical contexts, and which it is quickly curable with the administration of ascorbic acid.
In scurvy-prone animals ascorbic acid uptake is regulated by the number of intestinal "brush-border" carriers available for transport. The number of carriers is negatively affected by an increasing dietary ascorbic acid intake, thus limiting the maximum attainable uptake. We tested a new approach towards manipulating the accumulation of ascorbic acid in rainbow trout by repeatedly feeding a 10 day ascorbic acid allowance in a 1 or 3 day "pulse" followed by withdrawal, rather than spread out over the entire 10 day period. Pulse dosing led to initially increased mortalities compared to continuous dosing, but this was reversed after 55 days. Although individual weight and cumulative mortality were not significantly different after 88 days, total liver ascorbic acid levels were significantly higher in the 1-day pulse group (16.3 +/- 3.9 micrograms/g) than in the 3-day pulse (9.8 +/- 0.9 micrograms/g) or continuous (9.3 +/- 0.9 micrograms/g) groups. This led us to conclude that after an initial adaptation phase a feeding profile with intermittent ascorbic acid withdrawal results in a compensatory increase in uptake of ascorbic acid from the diet and/or a better conservation of the body pool of ascorbic acid, opening interesting new avenues for ascorbic acid dosing and therapy.
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