Scurvy on the rise in the United States: Pediatric cases triple in five years

By Hugo Francisco de SouzaReviewed by Susha Cheriyedath, M.Sc.Jul 19 2024

In a recent review published in the journal JAAOS: Global Research and Reviews, researchers compile available information on the symptoms, diagnosis, radiological evidence, and treatment of Scurvy, a disease caused by the severe deficiency of vitamin C (ascorbic acid). They further conducted a retrospective investigation of pediatric patients from the United States (US) National Inpatient Sample Database (n = 19,413,465) between 2016 and 2020. Their findings highlight an alarming trend – in the span of just five years, the incidence of Scurvy in children more than tripled from 8.2 to 26.7 cases per 100,000.

More than half (64.2%) of scurvy patients were found to suffer from concomitant autism spectrum disorder, with male obese individuals, especially those in the lowest income quartiles, representing the highest-risk population. This study highlights the need for pediatricians and care providers to be aware of the potential for scurvy diagnoses, especially in patients with risk factors presenting vague musculoskeletal symptoms.

Study: The Troubling Rise of Scurvy: A Review and National Analysis of Incidence, Associated Risk Factors, and Clinical Manifestations. Image Credit: Iryna Imago / Shutterstock

Background

Scurvy is a nutritional disorder caused by the severe and prolonged deficiency of ascorbic acid (vitamin C), characterized by mucocutaneous and musculoskeletal symptoms such as petechiae, bone pain in lower appendages, and gingival bleeding. Formerly a common and often lethal disease plaguing ancient mariners and responsible for millions of maritime deaths, particularly in the Ages of Sail and Exploration, Scurvy is now a rare disease thought to be restricted to underdeveloped regions and in refugee populations with limited access to fresh fruits and healthy nutrition.

Unfortunately, given this preconception and in tandem with Scurvy's symptomatic similarity to other rheumatological and musculoskeletal conditions (juvenile idiopathic arthritis, bone tumors, and septic arthritis), pediatricians in the developed world frequently fail to diagnose the condition, resulting in delayed treatment of this otherwise easy-to-treat disease.

"Often thought of as an outdated diagnosis, scurvy is frequently not included on the list of differential diagnoses for pediatric musculoskeletal reports, which results in delayed diagnosis, increased morbidity, and unnecessarily invasive, costly testing."

Symptoms, diagnosis, and treatment

Typical disease symptoms, especially in younger populations, include pseudoparalysis, swollen and externally rotated knee or ankle joints (in infants), and flexion contractures (in older children). In cases where Scurvy has sufficiently advanced, scorbutic rosary (beading of the costochondral joints) and skeletal growth abnormalities may additionally manifest. Given the essential role of vitamin C in collagen manufacture and iron absorption, more than 80% of patients may also present concomitant iron-deficiency anemia.

Radiographs have historically been considered vital and sufficient evidence in diagnosing Scurvy, presenting characteristic subperiosteal hemorrhages, physeal widening, cortical irregularities, and osteopenia. Orthopedic physicians usually consider the presence of any of the following – Wimberger ring sign, Pelkan spurs, Trümmerfeld zone rarefaction, or the white line of Frankel – as necessary observations in scurvy differentials.

Unfortunately, previous research by Pan et al. has highlighted the low sensitivity of these diagnostic factors, suggesting that their absence is insufficient to exclude scurvy diagnoses. Advanced imaging techniques such as ultrasonography and magnetic resonance imaging (MRI) may be required to confirm scurvy suspicions. Still, these tests are substantially more expensive than conventional radiological approaches and present their own concerns for economically backward, at-risk populations.

Encouragingly, despite its severe physiological impacts and complicated diagnosis, Scurvy is exceedingly easy to treat, only requiring changes in diet to include fresh fruits and vegetables (citrus fruits produce best results) in its early stages and vitamin C supplementation (100-300 mg per day for children and 500-1000 mg per day for adults) in advanced cases. However, the physiological outcomes of delayed disease diagnoses may include fractures, physeal separation, and other severe musculoskeletal manifestations that require hospitalization. This, in turn, exposes patients and their families to avoidable financial and psychological burdens, necessitating investigations into the frequency of scurvy incidences and risk factor analysis.

About the study

In addition to the above-summarized review of scurvy diagnoses and treatment, the present study further comprises a retrospective investigation of the incidence and risk factors associated with Scurvy in the United States (US). Data for the study was derived from the Healthcare Cost and Utilization Project National Inpatient Sample (HCUP-NIS), the US's largest publicly available all-payer inpatient care database, representing approximately 20% of all inpatient documentation nationwide. Study inclusion criteria included all patients under 18 years of age between 2016 and 2020.

Data collection comprised demographic data (age, sex, ethnicity and race, household income, and insurance status) and medical data (including International Classification of Diseases [ICD-10] codes for scurvy or comorbidity identification). Patients identified as having Scurvy were compared with the general inpatient population using statistical analysis comprising analysis of variance (ANOVA) or chi-square tests (with Kendall tau).

Study findings

Nineteen million four hundred thirteen thousand four hundred sixty-five patients met the study inclusion criteria for the period under investigation and were included in statistical analysis. Of these, 265 individuals were diagnosed with Scurvy. Time series analyses revealed that during the course of the five years under study, the incidence of Scurvy increased from 8.2 per 100,000 (2016) to 26.7 per 100,000 children (2020). Population demographic data revealed that the mean age of scurvy patients was 2.15 years, with most being male (69.8%) and belonging to the lowest economic quartile (36.5%).

"Younger age (P < 0.001), male sex (P = 0.010), and socioeconomic status in the lowest quartile (P = 0.013) were significantly associated with a diagnosis of Scurvy. No notable associations were observed between scurvy diagnosis and race, residential setting (urban/rural), or payer."

Concurrent diagnostic analyses revealed that Scurvy was frequently manifested in patients with autism spectrum disorder (ASD; 64.2%), cerebral palsy (5.7%), vitamin D deficiency (50.9%), and anemia (62.3%). Scurvy was also concurrently associated with abnormal weight gain – 7.6% and 3.8% of scurvy patients were found to be obese or morbidly obese, respectively. Compared to inpatients without the condition, scurvy patients were observed to experience substantially higher inpatient days (10.83 vs. 4.05) and hospitalization costs ($163,415.90 vs. $30,552.73), especially alarmingly given the low-income risk factor associated with the disease.

"Results show that Scurvy places a sizable burden on patients, families, and the hospital system as measured by length of stay, cost of hospitalization, and rate of adverse discharges. However, it is important to note that these findings may be confounded by the complexity of these patients' care because of concomitant diagnoses. Nonetheless, these burdens further suggest the importance of readily considering Scurvy when forming a differential diagnosis, especially given its available and efficacious treatments."

Conclusions

Despite being written off as an ancient disease or one restricted to the underdeveloped world, pediatric Scurvy seems to be making a comeback even in developed nations like the US. The present study highlights that in a span of just five years between 2016 and 2020, scurvy incidence more than tripled in the documented inpatient population. Younger male individuals, particularly those from low-income households, were found to represent the highest-risk populations in the nation, with ASD, anemia, and obesity identified as common concurrent diagnoses.

These findings suggest that pediatricians, orthopedic physicians, and caregivers need to be aware of the looming risk of rising scurvy incidence and include the disease in their workup differential, potentially saving these children and their families from prolonged hospitalizations, adverse musculoskeletal outcomes, and substantial hospitalization costs.