We are always on the lookout for the big, bad, and ugly causes of our patients’ complaints in the Emergency Department. Someone has epistaxis and we think about bleeding disorders. If a patient is limping, we know it may be relatively benign, but we focus on the concerning causes (ex, Septic Arthritis, Osteomyelitis, Discitis, JIA) . If there is a rash (… well… let’s be honest… we try to ignore it), we carefully walk through the Ddx list searching for concerning signs and symptoms. What, however, should we do if there are all three issues? Let’s review one reason to ask a dietary history – Vitamin C Deficiency and Scurvy in Children:

Vitamin C Deficiency: Basics

• Vitamin C (ascorbic acid) is important (not just for mild URIs)
  • Cofactor and antioxidant in many reactions [Fortenberry, 2020]
  • Essential vitamin involved in hydroxylation and cross-linking of collagen [Ravi, 2020]
  • This collagen is critical in ensuring the integrity and strength of blood vessels.

• Vitamin C is Essential, and as such, is only obtained from our diet. [Fortenberry, 2020; Nastro, 2019]
  • It cannot be manufactured by the body.
  • It is also not stored in the body… so without continual repletion, it will become deficient.
  • Any condition or situation that prevents the consumption of Vitamin C can lead to significant deficiency in it.

• Vitamin C Deficiency (Scurvy) is rare in developed countries… [Ravi, 2020; Fortenberry, 2020]
  • In developed countries, generally, food is in abundance and fortified.
  • This is true for populations at large, but individuals may still develop Vitamin C Deficiency.

• At risk populations in developed countries include: [Ravi, 2020; Fortenberry, 2020; Brambilla]
  • Those with inadequate access to food (sadly, this is a real problem)
  • Those with concomitant disease:
    • Intestinal malabsorption states (ex, Celiac Disease, Crohn Disease)
    • Neurologic Disorders
    • Bone Marrow Transplantation
    • Hemodialysis
    • Substance abuse
    • Eating disorders
    • Iron Overload (from multiple transfusions)
  • Those with severe dietary restrictions
    • Imposed dietary restrictions from caregivers
    • Behavioral conditions that lead to restricted diets
      • Autism
      • Patients with oral aversions
      • Self-imposed, “selective eater”
  • Of note, the restricted diets do NOT have to produce a malnourished, failure to thrive state. Patients may maintain normal weight and height for age.

Vitamin C Deficiency: Presentation

• Vitamin C Deficiency (Scurvy) is a collection of non-specific symptoms [Ravi, 2020; Fortenberry, 2020; Nastro, 2019; Brambilla]
  • Often go unrecognized or misdiagnosed for prolonged time
  • Often has had multiple, expensive, and expansive evaluations
  • Symptoms usually begin 1-3 months after inadequate intake.

• Constellation of symptoms can include: [Ravi, 2020; Nastro, 2019]
  • Early Symptoms: (likely to be missed)
    • Fatigue
    • Weakness
    • Anorexia
  • Late Symptoms:
    • Easy Bleeding, Bruising
    • Petechiae
    • “Dermatitis”
    • Follicular Hyperkeratosis
    • Corkscrew hairs
    • Perifollicular hemorrhage or petechiae
    • Gingival disease
    • Arthralgias
    • Bone Pain
    • Microfractures
    • Osteoporosis

• Often initially misconstrued as: [Ravi, 2020; Brambilla]
  • Rheumatologic Disorders (ex, Lupus, Juvenile Idiopathic Arthritis)
  • Vasculitis (ex, HSP)
  • Bleeding Disorders (ex, Christmas Disease, VonWillebrand Disease)
  • Musculoskeletal conditions (ex, Osteomyelitis, Septic Arthritis)
  • Oncologic conditions

• Diagnosis is made based on History and Physical, but can be supported by: [Ravi, 2020; Fortenberry, 2020; Nastro, 2019]
  • Lab values:
    • Vitamin C Level low
    • Low Hemoglobin
    • ESR can be elevated
    • CRP and Fibrinogen can also be elevated
  • Radiographs: [Nastro, 2019]
    • Commonly Knee x-rays
      • Pelkan Spur – healing metaphysical pathologic fracture
      • Wimberger Ring Sign – thin sclerotic cortex surrounding a lucent epiphysis
      • Frankel Line – dense calcification adjacent to the physis
      • Trummerfeld line – lucent band adjacent in diaphysis
    • MRI
      • Often obtained in the course of evaluating other possible concerns
      • No specific finding
      • Findings can be differentiated from hematologic malignancies or infectious causes by the fact that Scurvy will have symmetric bilateral findings, have periosteal reactions, and adjacent soft-tissue edema.

Vitamin C Deficiency: Management

• A Multidisciplinary team is typically required to appropriately manage these patients. [Ravi, 2020]
  • Nutrition
  • Physical therapy
  • Pharmacy
  • Psychology

• Vitamin C can be delivered in a variety of forms: [Fortenberry, 2020]
  • Oral is preferred
    • Least expensive (ie, pennies)
    • Many types of formulation available
    • The “picky” eater or child with oral aversions may still refuse this form.
  • IM/IV/SQ
    • Doesn’t require patient cooperation…
    • Much more expensive (~2,000% more expensive)
    • IM is preferred of these 3 options.
    • Requires specialized training to give
  • Rectal
    • Avoids the issue of oral aversion… but, clearly, still requires patient cooperation.
    • More expensive than oral formulation.

• Initial therapy of Scurvy is typically: [Ravi, 2020; Fortenberry, 2020]
  • Vitamin C 100 mg IV 3 times a day x 7 days.
  • Signs and symptoms will often improve within 3 days of therapy.

Moral of the Morsel

• Vitamin C is for Collagen! That means it deserves more attention than just when I have a cold.
• What we eat (and don’t) is important! Yes, even for Emergency providers.
• Considering a rheumatologic Dx for that funny rash and joint pain?? Ask about the diet.
• Considering a hematologic problem is causing the anemia and petechiae in the limping child? Ask about the diet.

References