Scurvy complicated with Capnocytophaga sputigena sepsis as a possible cause of death of king Saint-Louis of France (1270 AD).

The cause of death of Saint-Louis is not known, but recent findings indicated that he presented scurvy and inflammatory jaw disease, which has been associated with infection by oral commensals. Here, we have the exceptional opportunity to analyze the relics of the viscera of King Saint-Louis. A 4.3 g sample from the viscera relics of King Saint-Louis conserved in Versailles' cathedral was subjected to radiocarbon dating, electronic and optic microscopy, and elementary, palynological, molecular, proteomics and microbiological analyses including specific PCR and v3v4 16 S rRNA gene amplification prior to large-scale sequencing using an Illumina MiSeq instrument. The measured radiocarbon age was Cal 1290 CE-1400, which was compatible with that of the viscera of St Louis viscera, considering the addition of lime, incense and vegetables within the human organs. Elemental and palynological analyses confirmed a medieval embalming process. Proteomics analysis identified mainly human muscle and blood proteins. Specific PCR for plague, amoebiasis, shigellosis and typhoid fever was negative. C. sputigena was identified as the main pathogenic species representing 10.8 % of all microbial sequences. In contrast, C. sputigena was found in only 0.001 % of samples sequenced in our center, and the 23 positive human samples showed a dramatically lower abundance (0.02-2.6 %). In the literature, human infections with C. sputigena included odontitis, dental abscess, sinusitis, thoracic infections and bacteremia, particularly in immunocompromised patients with oral and dental diseases consistent with recent analysis of King Saint-Louis' jaw. C. sputigena, a commensal of the mouth that is potentially pathogenic and responsible for fatal bacteremia, may have been the cause of the king's death.

Modern Day Scurvy in Pediatric Orthopaedics: A Forgotten Illness.

INTRODUCTION: Scurvy, or vitamin C deficiency, is rare. The goal of this study is to highlight the common risk factors and identify the orthopaedic presentation of scurvy in children. METHODS: A retrospective chart and radiograph review was performed of all patients consulted to the pediatric orthopaedic service from 2010 to 2019 who ultimately had the diagnosis of scurvy confirmed by an abnormally low serum vitamin C level. Data extracted included: patient age, sex, neurological conditions, prematurity, psychiatric conditions, dietary abnormalities, bone pain, arthritis, limb swelling, inability to walk, skin changes, child abuse evaluations, radiographic findings, additional vitamin deficiencies, lab studies, additional tests, response to treatment. Descriptive statistics were performed. RESULTS: Nine patients (7 males, 2 females) with scurvy were studied. The average age was 7 years (range 3 to 13 y). The average body mass index was 21.4 (range, 14 to 30). Five had autism, 2 had a neurological disorder. Two had been born premature. Two had a psychiatric disorder. Seven had an abnormal diet. One presented with bone pain. Four presented with limb swelling. Seven had unilateral and 2 had bilateral leg symptoms. Five presented with inability to walk. Six demonstrated skin changes with ecchymosis or petechiae. Three presented with gingival bleeding. Radiographic findings included subperiosteal hematoma in 2, ring epiphysis in 3, Pelkan spurs in 1, metaphyseal white lines (Frankel sign) in 6, and a metaphyseal zone of rarefaction (Trummerfeld zone) in 3. Seven had additional vitamin deficiencies including: A, B1, B6, B9, D, E, K, iron and zinc. Four had a bone marrow biopsy and 1 had lumbar puncture. All were anemic. The average erythrocyte sedimentation rate was 25.7 (range 6 to 35) and C-reactive protein was 1.5 (range 0.55 to 5.64). Six had a computed tomography, 3 had a magnetic resonance imaging. After treatment with vitamin C lasting 3.4 months (range, 2 wk to 7 mo), all symptoms gradually resolved, including leg pain and swelling. All children began to walk. CONCLUSION: The pediatric orthopaedic surgeon should have an increased awareness about the diagnosis of scurvy when consulted on a child with bone pain or inability to walk. The most common orthopaedic presentation was the refusal to bear weight, the most common radiographic finding was the metaphyseal line of increased density (Frankel sign) and treatment with vitamin c supplementation was excellent in all cases.

Do You C What I C: Emergency Department Evaluation and Diagnosis of Pediatric Scurvy in an Autistic Child With a Restricted Diet.

Scurvy in modern times may not be as rare as previously thought. The link between adequate intake of vitamin C and scurvy has been known since ancient times and is recorded in Ebers Papyrus. Recent reports indicate that, with restricted diets, vitamin C deficiency is being seen in infants exclusively fed plant-based formula and children with oral aversion, autism, restricted diets, and cerebral palsy. Additional at-risk groups include the older adults and patients having alcoholism. Often costly, emergency department visits and elaborate diagnostic studies lead to fruitless results when a simple diet history is often overlooked. Here, we report a case of pediatric scurvy in an 11-year-old autistic child with a restricted diet who presented with refusal to walk, fatigue, a purpuric rash, and gingival bleeding. The diagnosis was made based on diet history, physical examination findings, and symptom resolution with vitamin C supplementation. Our case report reaffirms that vitamin C deficiency still occurs and should be considered in children with restrictive diets. Early recognition of this disease by physicians provides early diagnosis, avoids costly diagnostic workup and hospitalization, and expedites effective treatment.

Scurvy Outbreak Among South Sudanese Adolescents and Young Men - Kakuma Refugee Camp, Kenya, 2017-2018.

Scurvy is a relatively rare micronutrient deficiency disease that can occur among refugees dependent on food aid (1). Inadequate access to fresh fruits and vegetables in refugee camps can result in scurvy (2,3). Kakuma Refugee Camp in Kenya's Turkana District is home to 148,000 refugees, mostly from Somalia and South Sudan, who receive food assistance. In August 2017, a number of South Sudanese adolescent and young adult male refugees were evaluated at a health clinic in the camp for calf pain, chest pain, and gingival swelling. Because the symptoms were nonspecific, no diagnosis was made, and some patients received antibiotics and analgesics. All were managed as outpatients, but symptoms did not improve. During subsequent months, more young men with similar symptoms were reported. On January 20, 2018, the United Nations High Commissioner for Refugees (UNHCR) was informed and conducted clinical examinations. Signs and symptoms included lower limb pain and swelling (in some cases involving joints), lethargy, fatigue, gingival swelling and pain, hyperkeratotic skin changes, and chest pain. Based on these clinical findings, micronutrient deficiency, particularly vitamin C deficiency (scurvy), was considered a possible diagnosis, and an investigation of a possible outbreak was conducted. The suspected scurvy cases all occurred in young men from South Sudan who were living and cooking together in one geographic section of the camp. All patients who received treatment with vitamin C noted improvement of symptoms within <1 week. Patients were provided with food and cash assistance, the latter to allow dietary diversification (i.e., fresh fruits and vegetables). However, both forms of assistance were inadequate to allow access to sufficient amount of calories and the dietary diversification needed for intake of micronutrients, such as vitamin C. It is important to consider these needs when determining the amount of food or cash assistance provided to adolescents and young adult male refugees.

A 3-Year-Old With Gingival Hemorrhage and Musculoskeletal Pain.

A 3-year-old had spontaneous gingival hemorrhage and bilateral limb weakness with inability to bear weight. He had no preceding oral trauma or recent infection, took no regular medications, and had no recent use of aspirin or nonsteroidal anti-inflammatory drugs; his diet was limited to primarily chicken nuggets and milk. What is the diagnosis and what would you do next?

Macroscopic features of scurvy in human skeletal remains: A literature synthesis and diagnostic guide.

The past two decades have seen a proliferation in bioarchaeological literature on the identification of scurvy, a disease caused by chronic vitamin C deficiency, in ancient human remains. This condition is one of the few nutritional deficiencies that can result in diagnostic osseous lesions. Scurvy is associated with low dietary diversity and its identification in human skeletal remains can provide important contextual information on subsistence strategy, resource allocation, and human-environmental interactions in past populations. A large and robust methodological body of work on the paleopathology of scurvy exists. However, the diagnostic criteria for this disease employed by bioarchaeologists have not always been uniform. Here we draw from previous research on the skeletal manifestations of scurvy in adult and juvenile human skeletal remains and propose a weighted diagnostic system for its identification that takes into account the pathophysiology of the disease, soft tissue anatomy, and clinical research. Using a sample of individuals from the prehistoric Atacama Desert in Northern Chile, we also provide a practical example of how diagnostic value might be assigned to skeletal lesions of the disease that have not been previously described in the literature.

[Atypical scurvy associated with anorexia nervosa]. / Scorbut atypique associé à une anorexie mentale.

INTRODUCTION: Scurvy, or "Barlow's disease", is a widely described disease involving cutaneous and mucosal lesions resulting from vitamin C deficiency. Herein, we report a case of scurvy in a 48-year-old woman that was unusual in its atypical cutaneous-mucosal presentation as well as its association with anorexia nervosa. PATIENTS AND METHODS: A 48-year-old woman treated for depression for several years was admitted to hospital for her impaired general state of health. Over the last year, she had presented palmoplantar rash and episodes of perimalleolar oedema. The clinical examination showed the patient to have wasting syndrome, with a BMI of 11.9kg/m2, lower-limb oedema, palmoplantar fissures, geographic tongue, telogen effluvium and purpuric petechiae on her right knee. However, no gingival bleeding was noted and there was no loss of tooth enamel. The remainder of the clinical examination was normal. Blood tests revealed extremely low vitamin C levels without any other associated deficiencies, as well as laboratory signs of cytolysis and anicteric cholestasis without inflammatory syndrome. The diagnosis of anorexia nervosa was made by psychiatrists, despite the unusual age of onset. Favorable clinical outcome was rapidly achieved via a one-month course of vitamin C supplements at a daily dose of 1g. DISCUSSION: The absence of classical buccal-dental symptoms and the presence of keratotic dermatosis with fissures and ulcers on the hands and feet are atypical in scurvy; however, this diagnosis was confirmed by the existence of purpura evoking capillary fragility, the patient's drastically low vitamin C level and the rapid subsidence of symptoms following treatment with oral vitamin C alone. Anorexia nervosa was doubtless the cause of deficiency. This situation is rare and a systematic review of the literature in Medline via PubMed showed that only three reports of scurvy associated with mental anorexia have been published since 1975.

Gingival Bleeding and Bloody Dialysate: A Case Report of Scurvy in a Child With End-Stage Renal Disease Receiving Peritoneal Dialysis.

Patients with chronic kidney disease (CKD) or end-stage renal disease are at risk for vitamin C deficiency and scurvy due to diet restriction, increased urinary loss of the water-soluble vitamin C with diuretics, and in case of patients who are on dialysis, through dialysates. The condition may be overlooked as the clinical manifestation of scurvy may be subtle, and some presentations may mimic clinical signs in CKD. We reported a case of scurvy presenting with gingival bleeding and blood dialysate in a 6-year-old girl with end-stage renal disease who was on continuous ambulatory peritoneal dialysis. Physical examination showed gingival hyperplasia and bleeding, and the pathognomonic bleeding of perifollicular hemorrhage. The typical radiographic changes were present. The clinical signs and symptoms resolved after ascorbic acid treatment. This case underscores the importance of awareness of the increased risk for vitamin C deficiency in patients with CKD and receiving dialysis.

A Case of Scurvy-Uncommon Disease-Presenting as Panniculitis, Purpura, and Oligoarthritis.

IMPORTANCE: Scurvy remains prevalent in certain populations, including addicts, people of low socioeconomic status, and the severely malnourished. It classically presents as follicular hyperkeratosis and perifollicular hemorrhage of the lower extremities, as well as bleeding in other areas such as the gingiva and joints. This case presentation and literature review highlights the common pathophysiological findings associated with scurvy and current methods of diagnosis and treatment. OBSERVATION: The patient described in this case presented with sudden oligoarthritis and purpura of the lower extremities. Following progression of the patient's symptoms and a low vitamin C serum concentration, the patient was treated with vitamin C supplementation and dramatically improved. This was considered to be the result of an underlying vitamin C deficiency secondary to insufficient fruit and vegetable intake due to allergies. CONCLUSIONS AND RELEVANCE: This case highlights the importance of maintaining a high index of suspicion for scurvy in atypical presentations of purpura not better explained by another disease or in additional populations at high risk of vitamin C deficiency. Early diagnosis by either a primary care physician or dermatologist can expedite the treatment process and improve patient prognosis.

Scurvy in the present times: vitamin C allergy leading to strict fast food diet.

Scurvy results from a deficiency of vitamin C, a nutrient otherwise known as ascorbic acid. Today, scurvy is rare yet emerges in select patients. The patient reported herein developed scurvy secondary to deliberate avoidance of vitamin C-rich foods. Classic cutaneous manifestations of scurvy include follicular hyperkeratosis and perifollicular hemorrhage encompassing coiled "corkscrew" hairs and hairs bent into "swan-neck" deformities. Ecchymoses, purpura, and petechiae are also characteristically prominent. Classic oral abnormalities include erythematous, swollen gingivae that hemorrhage from subtle microtrauma.Subungual linear splinter hemorrhages may also manifest as a sign of the disease. To establish the diagnosis requirements include characteristic physical exam findings, evidence of inadequate dietary intake, and rapid reversal of symptoms upon supplementation. Although unnecessary for diagnosis, histological findings demonstrate perifollicular inflammation and hemorrhage, fibrosis, and hyperkeratosis, amongst dilated hair follicles and keratin plugging. Although citrus fruit allergies have been historically documented, ascorbic acid has not been previously reported as an allergen. Although lacking absolute certainty, this report suggests a presumed case of ascorbic acid allergy based on patient history and favorable response to ascorbic acid desensitization therapy.

Traumatic Injury in a Child with Scurvy: A Case Report.

This case report aimed to describe the clinical presentation, treatments and prognosis of a child who had scurvy and traumatic injury of the left thigh. A 30-month-old boy had presented with left hip pain two weeks after falling down on the floor while walking. He developed pain, warmness of the left hip and thigh, and finally was unable to bear weight. He also had a high fever gingival hemorrhage, dental caries, petechiae, positive rolling test and limited range of motion of the left hip. The radiographs revealed Wimberger's ring and Frenkel line as scurvy. Vitamin C supplement had been prescribed for one week. However, there was no clinical response and magnetic resonance imaging (MR) suggested subperiosteal abscess as well as osteomyelitis of bilateral femurs and tibias. Debridement and biopsy of the left femur were performed and found only subperiosteal blood. A clinical improvement was noted on the second day after surgery. Vitamin C level was reported at 0.03 mg/dl which was very low. Bacterial culture was negative and the pathological findings were callus formation with hemorrhage. The patient continued the treatment for two months and all conditions were healed eventually. In severe scurvy with trauma, prolonged subperiosteal hematoma was susceptible to infection, and may need debridement simultaneously with vitamin C supplement to shorten the clinical course.

[Infantile scurvy: Two cases]. / Scorbut chez l'enfant: deux cas.

BACKGROUND: Scurvy is the classic and most severe form of vitamin C deficiency. This condition has become extremely rare among children in the industrialized countries. PATIENTS AND METHODS: We report the case of two boys presenting bone pain associated with haemorrhagic gingivitis, with perifollicular purpura of the lower limbs in one boy. The children had an unbalanced diet. Scurvy was associated with vitamin D and iron deficiency. The dermatological and radiological abnormalities seen were characteristic and a favourable outcome was rapidly obtained following supplementation. DISCUSSION: The possibility of this forgotten historical illness should not be overlooked in the presence of these dermatological and rheumatologic signs, since this can help avoid unnecessary or excessively aggressive investigations.

Scurvy: a new problem for patients with chronic GVHD involving mucous membranes; an easy problem to resolve.

Vitamin C deficiency in developed countries is typically observed in patients with unique clinical conditions such as cystic fibrosis or anorexia nervosa, or in patients on long-term tube feeds. We report here a clinical observation in six pediatric and adolescent patients (median age 17.5 yr, range 9.8-23.5 yr) with chronic GVHD with mucous membrane involvement found to be vitamin C deficient. These patients' baseline serum vitamin C levels ranged from <0.12 to 0.94 mg/dL (normal value 0.20-1.90 mg/dL), with a mean level 0.56 ± 0.36 mg/dL and a median level 0.6 mg/dL. Among these patients, signs and symptoms of mucositis failed to respond to standard chronic GVHD therapy. After receiving treatment with 2000 mg of ascorbic acid by mouth, daily patients displayed increased serum vitamin C levels. Clinically, this correlated with a remarkable improvement in patients' mucositis and ability to eat.

Scurvy revealed by difficulty walking: three cases in young children.

Scurvy is rare in developed countries but is known to cause lower-extremity pain and refusal to ambulate in children. Since the discovery of the link between scurvy and dietary deficiency of ascorbic acid, there has been a substantial decrease in its prevalence and recognition. Here we describe 3 cases of scurvy in young children presenting with difficulty walking. Only 1 of 3 patients had gingival lesions at the initial presentation. Two cases underwent an extensive evaluation for hematologic and rheumatologic diseases before the diagnosis of scurvy was made. Dietary histories eventually revealed that all 3 patients had sharply limited intake of fruits and vegetables secondary to oral aversion, and 1 patient had autism. Radiographic changes of long bones were observed in all patients. Interestingly, all patients had concomitant vitamin D deficiency. After replacement with vitamin C, all patients recovered and started to walk again with improved leg pain. These clinical manifestations and radiologic findings highlight the importance for rheumatologists to have a higher index of suspicion for scurvy in nonambulatory children.

A rare cause of petechial rash in the 21st century.

Scurvy was first described by Hippocrates (460-370 BC) as a condition characterised by poor dental health, bruising, bleeding and fragile skin. Despite the widespread availability of foods rich in vitamin C in Australia, scurvy continues to afflict certain high-risk subgroups of the population. Cutaneous signs may be the only manifestation of the disease, as in the case presented below, and therefore scurvy continues to be relevant to dermatologists.

Scurvy masquerading as IgA vasculitis.

BACKGROUND: Vitamin C deficiency, or scurvy, is rare but poses risks for children with poor diets, limited resources, or malabsorption issues. It may also be common in children with restrictive or selective dietary habits in children with global developmental delay, autism spectrum disorder, and physical disabilities. Symptoms include fatigue, irritability, joint and muscle pain, joint swellings, edema, swollen gums, easy bruising, and delayed wound healing. Early recognition and prompt intervention are essential to prevent the progression of symptomatic vitamin C deficiency in children. CASE PRESENTATION: We present a case of a 13-year-old boy with developmental delay secondary to Lennox Gastaut syndrome referred for suspected recurrent, severe, and atypical IgA vasculitis. He presented with irritability, loss of appetite, petechial and ecchymotic lower limb lesions, unilateral gum swelling, severe arthritis, peripheral oedema, severe weight loss, anaemia, and raised inflammatory markers. Multiple investigations were performed before the diagnosis of scurvy was made. A surgical finding of friable gingival tissue with multiple loose teeth, a skin biopsy with follicular hyperkeratosis and extravasated perifollicular red blood cells, and a typical X-ray finding led to the diagnosis of scurvy. CONCLUSION: Scurvy should be given careful consideration as a differential diagnosis in patients presenting with musculoskeletal issues, mucocutaneous complaints, and constitutional symptoms such as malaise, asthenia, irritability, and loss of appetite. A focused and detailed dietary history looking for a lack of good sources of vitamin C can be an easy indicator of this differential. Imaging studies revealing the typical features can also help make the diagnosis. Pathology of the skin revealing pathognomonic features can add to the certainty of the diagnosis. In the absence of all else, the rapid response to treatment with an appropriate dose of vitamin C has a diagnostic and therapeutic role.

[Vitamin C]. / Vitamine C.

Vitamin C is a water soluble vitamin which is mainly fresh fruits and vegetables foodborne. Vitamin C deficiency is most often due to a lack of daily amount. Scurvy is characterized by the occurrence of fatigue, myalgia, arthralgia, purpura, bleeding disorders, and later by dental manifestations. Biological signs are nonspecific: anemia, hypocholesterolemia, hypoalbuminemia. Clinical suspicion is confirmed by the decrease in ascorbic acid level (< 2 mg/L). It must be interpreted in light of the acute phase reactants. The treatment is the administration of 1 g of vitamin C per day for 15 days. Vitamin C depletion (ascorbic acid: 2 to 5 mg/L) could induce long-term complications. The recommended dietary allowance of vitamin C protect from these risks.

Scurvy and food selectivity in childhood: a case report.

Despite its rarity, symptomatic micronutrient deficiency remains a public health problem. Scurvy is the differential diagnosis for bleeding disorders and hematological and rheumatological diseases, especially in patients with eating disorders. However, it is unrelated to autism spectrum disorders or other neurodevelopmental disorders. A previously healthy 10-year-old boy living in São Paulo, Brazil, had a history of significant food selectivity unrelated to autism spectrum disorder, resulting in symptomatic ascorbic acid deficiency (scurvy). This resulted in pain and purpuric lesions on the lower limbs, gingival edema, bleeding during tooth brushing, asthenia, weakness, malaise, and sadness. Therefore, dietary anamnesis is important for routine monitoring of child growth and development. This process helps prevent nutritional deficiencies, facilitates early diagnosis of eating disorders, and enables multidisciplinary follow-up for these patients.

Unusual Presentation of Pediatric Scurvy: A Necrotic Gastrostomy Tube Site in a 14-Year-Old Boy.

BACKGROUND Despite being considered a disease of the past, pediatric scurvy is increasingly reported in developed countries, especially among children with autism spectrum disorder, developmental delays, or a restrictive diet. Pediatric patients typically present with lower extremity pain or refusal to walk. This case study features an atypical presentation of scurvy in a non-ambulatory patient. CASE REPORT A 14-year-old boy with arthrogryposis multiplex congenita displayed a late-stage scurvy symptom: a necrotic gastrostomy tube site, indicative of poor wound healing due to vitamin C deficiency. The usual telltale symptoms of scurvy were camouflaged due to his non-ambulatory status, which may have contributed to a delayed presentation. Nevertheless, a comprehensive clinical evaluation, incorporating diet history, gingival symptoms, petechiae, and characteristic radiological signs, eventually led to the correct diagnosis. Although acute osteomyelitis was initially suspected, it was subsequently ruled out. Upon initiation of vitamin C therapy, the patient's symptoms subsided within a few days, and the necrotic tissue surrounding the gastrostomy tube healed completely within two weeks. CONCLUSIONS The highlighted case underscores the importance of including scurvy in the differential diagnosis for pediatric patients with lower extremity pain without fever. A detailed dietary history focusing on vitamin C intake is crucial during clinical evaluation. Early initiation of vitamin C therapy, when scurvy is suspected, may prevent unnecessary and extensive diagnostic workup for other potential causes, offering timely relief to the patient.

Difficult to think about but easy to treat: scurvy.

OBJECTIVES: Severe vitamin C deficiency, or scurvy, presents as a syndrome of multisystem abnormalities associated with defective collagen synthesis and antioxidative functions. The many clinical features of scurvy lead to frequent misdiagnoses, as they can often point to other diseases, such as vasculitis, venous thrombosis and musculoskeletal disorders. As such, an extensive workup is recommended in cases in which scurvy is suspected. CASE PRESENTATION: A 21-month-old male patient and a 36-month-old female patient presented with difficulty in walking, painful joint movements, irritability, gingival hypertrophy and bleeding. After exhaustive investigations and risky invasive procedures, vitamin C deficiency was diagnosed in both cases, and the symptoms improved dramatically with vitamin C treatment. CONCLUSIONS: The importance of taking a dietary history in pediatric patients is highly recommended. In cases where scurvy is considered, serum ascorbic acid levels should be checked to confirm the diagnosis prior to conducting invasive tests.