A Case Report

Scurvy May Occur Even in Children With No Underlying Risk Factors

Romina Gallizzi; Mariella Valenzise; Stefano Passanisi; Giovanni Battista Pajno; Filippo De Luca; Giuseppina Zirilli

Disclosures

J Med Case Reports. 2020;14(18) 

In This Article

Discussion

An unusual aspect of the present case was the absence of any potential risk factors, which demonstrates that scurvy may still be occasionally encountered in the pediatric population, even in individuals with no predisposing disorders, living in families without unusual eating habits.

Vitamin C is a necessary cofactor in collagen biosynthesis; capillary fragility, which is a typical feature of scurvy, depends on the depletion of pericapillary collagen. As in our case, the earliest manifestations in patients with scurvy are at mucosal and cutaneous levels, with petechiae, ecchymoses, and gingival bleeding. Gastrointestinal manifestations are very rare in patients with scurvy. Submucosal hemorrhages involving the stomach, duodenum, and the colon, may cause gastrointestinal bleeding mimicking an inflammatory bowel disease.

Another typical hallmark of scurvy is anemia, which is multifactorial in its pathogenesis. In fact, it may be secondary to a combination of bleeding, decreased iron absorption, and other dietary deficiencies.

Finally, a further frequent manifestation in pediatric age is bone disease, which may be debilitating, as in our case. In fact it has to be underlined that our patient was hospitalized due to a clinical picture characterized by musculoskeletal pain and refusal to walk. These symptoms may be explained on the basis of a defect in osteoid matrix formation and cartilage reabsorption, leading to disordered bone structure and bone pain.[5]

Radiologic studies can be useful for the diagnosis of scurvy, although the most specific signs are likely to be unfamiliar to many radiologists, owing to the rarity of this condition. The main radiographic findings are: white line of Fraenkel, which is an irregular, thickened white line that appears at the metaphysis and represents an increased calcification of the cartilage matrix; the Trummerfeld zone, which is a zone of rarefaction beneath the Fraenkel line, which denotes subperiosteal hemorrhage; Wimberger ring sign, which is a white line that surrounds ossification nucleus in the epiphysis; and the Pelkan sign, which is the presence of metaphyseal spurs that appear later due to the repair of microfractures.[6]

Therefore, the diagnostic suspicion of scurvy is based on a combination of clinical signs and radiographic findings. The dosage of serum vitamin C levels is considered specific but laboratory tests are insensitive. It is known that serum concentrations do not always correspond with tissue storage of ascorbic acid. A reliable indicator of body storage is the measure of urinary excretion after intravenous ascorbic acid administration. Normally, 80% of absorbed vitamin C should be excreted within 3–5 hours. Lower levels of urinary excretion suggest vitamin deficiency.[5] Finally, the prompt resolution of symptoms after substitutive treatment represents the main evidence to confirm the diagnosis of scurvy.[21]

The dose and duration of treatment should be individualized. It is demonstrated that ascorbic acid administration at an initial dose of 300 mg per day leads to complete resolution of symptoms within 4 weeks.[1] Then, vitamin C supplementation at a lower dose should be extended for another 2–3 months. Hemorrhagic manifestations, oral symptoms, and constitutional symptoms disappear within a very few days of starting the treatment, while the resolution of bone changes may take several weeks.[22]

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