A macroscopic assessment of porosity and new bone formation on the inferior pars basilaris: Normal growth or an indicator of scurvy?

OBJECTIVES: This research aims to determine the aetiology of porosity and subperiosteal new bone formation on the inferior surface of the pars basilaris. MATERIALS: A total of 199 non-adult individuals aged 36 weeks gestation to 3.5 years, from a total of 12 archaeological sites throughout the UK, including Iron Age (n=43), Roman (n=12), and post-medieval (n=145) sites, with a preserved pars basilaris. METHODS: The pars basilaris was divided into six segments, with porosity (micro and macro) and subperiosteal new bone formation recorded on the inferior surface in scorbutic and non-scorbutic individuals. Scurvy was diagnosed using criteria from the palaeopathological literature that was developed using a biological approach. RESULTS: There was no statistically significant difference in microporosity between scorbutic and non-scorbutic individuals in four out of the six segments analysed. There was a significant negative correlation between age and microporosity in non-scorbutic and scorbutic individuals. A significant difference in subperiosteal new bone formation was observed between scorbutic and non-scorbutic individuals. CONCLUSIONS: Microporosity on the inferior pars basilaris should not be considered among the suite of lesions included in the macroscopic assessment of scurvy in non-adult skeletal remains (less than 3.5 years). SIGNIFICANCE: This study highlights the risk of over diagnosing scurvy in past populations. LIMITATIONS: It is difficult to distinguish between physiological (normal) and pathological (abnormal) bone changes in the skeleton of individuals less than one year of age. SUGGESTIONS FOR FURTHER RESEARCH: Future research should focus on the analysis of individuals over 3.5 years of age.

Evidence of non-adult vitamin C deficiency in three early medieval sites in the Jaun/Podjuna Valley, Carinthia, Austria.

OBJECTIVE: This study aims to determine and discuss the prevalence of non-adult scurvy cases from the early medieval Jaun/Podjuna Valley in southern Austria. MATERIALS: 86 non-adult individuals were assessed from three early medieval sites. METHODS: Morphological characteristics associated with suggestive and probable scurvy were observed macroscopically and under 20-40x magnification. RESULTS: A significant relationship between the prevalence of scurvy and age group was observed. Perinates (46%, 6/13) and children (27.5%, 8/28) showed a high prevalence of skeletal features indicating a diagnosis of scurvy, while no cases of scurvy were observed in adolescents and adults. CONCLUSIONS: In this Alpine region, scurvy occurred frequently in infants and children. Seasonal fluctuations of diet are discussed as factors triggering scurvy. SIGNIFICANCE: This study sheds new light on the prevalence of scurvy in the Alpine region and how the region developed after the fall of the Roman Noricum. It also models ways in which multiple lines of evidence can contribute to the diagnostic process. LIMITATIONS: Poor preservation posed a challenge to identifying probable cases of scurvy. Likewise, non-adult remains are difficult to diagnose due to their developing nature and it is not always possible to distinguish between normal bone growth and pathological growth. SUGGESTIONS FOR FURTHER RESEARCH: Future applications of biomolecular studies will help illustrate changes in diet that may have contributed to vitamin deficiencies.

Infantile scurvy as a consequence of agricultural intensification in the 1st millennium BCE Etruria Campana.

The 1st millennium BCE in Italy was a time of agricultural intensification of staple cereal production which shaped sociocultural, political, and economic spheres of pre-Roman groups. The lifeways and foodways of the Etruscans, the greatest civilization in western Europe before Roman hegemony, are traditionally inferred from secondary written sources, funerary archaeology, archaeobotany, and zooarchaeology. However, no direct data extrapolated from the study of human skeletal remains are available to evaluate the extent to which agricultural intensification and decreased dietary diversity impacted health and the expression of skeletal indicators of metabolic disease. Macroscopic and radiological analyses were conducted on an archaeological skeletal sample of non-adults (n = 29) recovered from Pontecagnano (southern Italy) dating to the Orientalizing period (730-580 BCE). This allowed us to identify five cases of scorbutic non-adults and to assign diagnostic values to skeletal lesions of scurvy that have not been previously described in the literature. The onset of scurvy in the examined sample is related to the increased reliance of Etruscans on crops lacking vitamin C in this period of agricultural intensification. The skeletal expression of scurvy varied among the non-adults, with differences in location and disease severity; these were interpreted considering the age-at-death of the individuals coupled with feeding behaviors and interindividual variability.

Deficiency diseases in the Kyiv Rus' subadult population: The issue of the small sample effects.

This paper underlines the importance of the small sample effects consideration in paleopathological research providing an example of children from Kyiv Rus' cemeteries dated to the 10th-13th century AD. The study presents data introduction and systematization, i.e., construction of the statistically significant disease profiles combining the analyzed sites according to the distribution of anemia, scurvy, and rickets. Then we present usually provided (often 'associative') interpretations for the obtained results and question their reliability from the perspective of the small samples effects. This leads to the discussion of explanatory limits of bioarcheological research basing on the small sample analysis.

Manifestation of infantile scurvy in a skeleton from the high medieval village of Trutmanice (South Moravia, Czech Republic).

The study describes the pathological findings recorded on the human remains of a 3 to 4-year-old child found in the burial site of Trutmanice (Czech Republic), dated to the 13th-15th century. The human remains were examined using standard macroscopic osteological and paleopathological methods, supplemented by radiographic examination. Although the preservation of the bones limited the analysis, multiple pathological changes were observed mainly on the skull: cribra orbitalia, small pores and fine deposits of newly formed bone tissue, hypertrophic diploë, grooves indicating rich branching of meningeal arteries on the intracranial surface of the cranial vault. A very fine periostotic deposition of newly formed bone tissue was also observed at the distal end of the diaphysis of the right tibia and both distal ends of the femora. The radiographs of the femora showed white lines of Frankel, scurvy lines, and Wimberger's rings. The observed pathological changes were possibly consistent with scurvy based on differential diagnosis. Reports about cases of juvenile scurvy from rural areas within the context of landlocked countries of Central Europe are rare. While scurvy is now more frequently reported in the paleopathological literature, evidence for scurvy within one of the most natural agricultural areas in the Czech lands remains rare. We recommend using radiographic examination to complement the diagnosis of scurvy in future paleopathological and epidemiological studies of past populations.

A Prehispanic infant from Tenerife with diffuse microporotic lesions.

We describe diffuse microporotic lesions observed in most of the scattered skeletal remains belonging to a ≈ 6 months-old female (genetic sexing) prehispanic (antiquity ≈ 600 years BP) individual recovered from a small recess of a basaltic burial cave in the highlands (2300 m above sea level) of Tenerife. Although sphenoid wings were lacking, microporotic lesions were present in several bones, especially in the hard palate, basilar part of the occipital bone, outer aspect of the maxilla, and proximal half of the right humerus, accompanied by a subtle periosteal reaction. Although non-specific, bone lesions may be compatible with scurvy, possibly in the context of malnutrition, that probably also affected the mother, given the young age of the infant and her dependence on maternal feeding. Pathophysiological connections among iron deficiency, vitamin C deficiency and vitamin D deficiency are discussed. Both observational reports on paleopathological cases of diffuse microporotic lesions as well as experimental studies devoted to discern the relative and combined effects of hypoxia-mediated bone marrow expansion, protein-calorie malnutrition, ascorbate, vitamin D or iron deficiency on such lesions are needed.

Scurvy and Tinea Corporis Simulating Leukocytoclastic Vasculitis.

ABSTRACT: Leukocytoclastic vasculitis (LCV) is a small vessel inflammatory condition considered to be caused by circulating immune complexes and often occurs after an acute infection or exposure to a new medication, although it may be associated with an underlying systemic disease or be idiopathic in nature. It is important to determine the etiology, identify the extent of the disease for early intervention and appropriate management, and treat and/or eliminate the underlying cause. Here, we report cases of scurvy and tinea corporis that presented with histopathologic features of LCV and had significant clinical improvement with treatment of the underlying etiologies. These cases emphasize that histopathologic features of early evolving LCV may be seen in other settings including scurvy and tinea corporis. Appropriate treatment of the underlying condition is important for optimized patient management.

An Epidemic of Scurvy, Identified Based on Lower Extremity Swelling, in a Southern Ethiopian Prison.

In October 2016, we received reports of five deaths among prisoners with leg swelling of unknown etiology in southwestern Ethiopia. A descriptive cross-sectional study was conducted to investigate the outbreak. A suspected case was defined as a prisoner with leg swelling of unknown etiology noted between May 15, 2016 and November 29, 2016. A total of 118 suspected cases were identified with unilateral or bilateral leg swelling without an identifiable cause from a total of 2,790 prisoners. Eight of the suspected cases were thoroughly examined, and seven of these suspected cases had clinical findings consistent with scurvy. Three of the clinical cases had undetectable vitamin C levels in the serum. The attack rate for the prison was 4.2% (118/2,790), and 11 deaths were identified, making the case fatality rate 9.3% (11/118). Clinical cases of scurvy had symptoms of fatigue, myalgia, arthralgia, and signs of follicular hyperkeratosis, petechiae, peripheral edema, and oral lesions. All clinical cases had severe anemia with hemoglobin < 6.0 g/dL. The diet provided by the prison excluded fruits and vegetables. Scurvy was determined to be the cause of the outbreak, and vitamin C supplementation was promptly initiated. All symptomatic prisoners improved, and no further cases were identified in a 4-week follow-up period of active surveillance.

Ascorbic Acid Route to the Endoplasmic Reticulum: Function and Role in Disease.

Significance: Humans cannot synthesize ascorbic acid (AscH2) (vitamin C), so deficiencies in dietary AscH2 cause the life-threatening disease of scurvy and many other diseases. After oral ingestion, plasma AscH2 concentrations are strictly controlled by transporters, which are required for entry into the cell and into intracellular organelles. Recent Advances: Besides its general antioxidant function, AscH2 is a cofactor for endoplasmic reticulum (ER)-localized collagen hydroxylases. Its important role in ER homeostasis is also highlighted by the fact that AscH2 deficiency in auxotrophic species triggers ER stress. Critical Issues: Characterizations of the molecular basis of diseases suggest that intracellular AscH2 deficiency is due not only to limited dietary access but also to its limited intracellular transport and net loss under conditions of intracellular hyperoxidation in the ER. This essay will offer an overview of the different transporters of vitamin C regulating its intracellular concentration, its function inside the ER, and the phenotypes of the diseases that can be triggered by increased depletion of this vitamin in the ER. Future Directions: When considering the benefits of increasing dietary AscH2, it is important to consider pharmacokinetic differences in the bioavailability between orally and intravenously administered AscH2: the latter bypasses intestinal absorption and is, therefore, the only route that can lead to the high plasma concentrations that may provide some health effects, and it is this route that needs to be chosen in clinical trials for those diseases associated with a deficiency of AscH2. Antioxid. Redox Signal. 34, 845-855.

Scottish soldiers from the Battle of Dunbar 1650: A prosopographical approach to a skeletal assemblage.

After the Battle Dunbar between English and Scottish forces in 1650, captured Scottish soldiers were imprisoned in Durham and many hundreds died there within a few weeks. The partial skeletal remains of 28 of these men were discovered in 2013. Building on previous osteological work, here we report wide-ranging scientific studies of the remains to address the following questions: Did they have comparable diet, health and disease throughout their lives? Did they have common histories of movement (or lack of movement) during their childhoods? Can we create a collective biography of these men? Strontium and oxygen isotope analysis of tooth enamel investigated childhood movement. Carbon and nitrogen isotope analysis of incrementally sampled dentine addressed childhood diet and nutrition. Metaproteomic analysis of dental calculus investigated oral microbiomes and food residues; this was complemented by microscopic analysis of debris in calculus from ingested materials. Selected individuals were examined for dental microwear. The extent of hydroxylation of proline in collagen was examined as a potential biomarker for scurvy. An osteobiography for each man was created using the full range of data generated about him, and these were synthesised using an approach based on the historical method for a collective biography or prosopography. The childhood residences of the men were primarily within the Midland Valley of Scotland, though some spent parts of their childhood outside the British Isles. This is concordant with the known recruitment areas of the Scottish army in 1650. Their diets included oats, brassicas and milk but little seafood, as expected for lowland rather than highland diets of the period. Childhood periods of starvation or illness were almost ubiquitous, but not simultaneous, suggesting regionally variable food shortages in the 1620s and 1630s. It is likely there was widespread low-level scurvy, ameliorating in later years of life, which suggests historically unrecorded shortages of fruit and vegetables in the early 1640s. Almost all men were exposed to burnt plant matter, probably as inhaled soot, and this may relate to the high proportion of them with of sinusitis. Interpersonal violence causing skeletal trauma was rare. Based on commonalities in their osteobiographies, we argue that these men were drawn from the same stratum of society. This study is perhaps the most extensive to date of individuals from 17th century Scotland. Combined with a precise historical context it allows the lives of these men to be investigated and compared to the historical record with unprecedented precision. It illustrates the power of archaeological science methods to confirm, challenge and complement historical evidence.

[Cutaneous manifestations of nutritional deficiencies]. / Manifestations cutanées de carences nutritionnelles, ou quand l'examen de la peau alimente le diagnostic.

In elderly or chronically ill patients, nutritional deficiencies are common and the presence of related skin lesions is not unusual. Recognition of such cutaneous involvement is important regarding the diagnosis essentially based on clinical elements. By using some clinical case reports, we will describe several pathologies related to nutritional deficiencies like scurvy, pellagra and acquired acrodermatitis enteropathica.

Dans nos populations de patients vieillissants ou atteints de maladies chroniques, les carences nutritionnelles sont fréquentes et la présence de manifestations dermatologiques associées n'est pas rare. La reconnaissance de telles atteintes cutanées est importante pour le diagnostic qui est essentiellement clinique. A l'aide de vignettes cliniques, nous allons discuter ici de pathologies carentielles comme le scorbut, la pellagre ainsi que l'acrodermatite entéropathique acquise.

Scurvy, an old story in a new time: The hematologist's experience.

BACKGROUND: Scurvy is a rare entity in developed countries and the diagnosis may often be delayed resulting in unnecessary investigations and/or potentially severe complications. A recent increase in the number of patients diagnosed with scurvy in our hematology clinics indicated the need to review the literature on the diagnosis and optimal management of similar patients. METHODS: We conducted a retrospective chart review of patients referred to hematology at our tertiary care centre between 2010 and 2018, who were ultimately diagnosed with scurvy. Data collected from electronic medical records included baseline characteristics, clinical features on presentation, bloodwork results from initial consultation, treatment plan as well as response to treatment. FINDINGS: Twenty-two adults patient had a diagnosis of scurvy with a mean vitamin C level of 6 µmol/L. Iron deficiency anemia (54%) and gastrointestinal disorders (54%) were the most common comorbidities noted in our cohort. Proton-pump inhibitors use was noted in 54% of patients. Bleeding (45%) and bruising (45%) were the most commonly reported clinical features. Eleven patients received oral supplementation, five had intravenous (IV) vitamin C and six were not treated. Two patients required a transition from oral to IV supplementation. Vitamin C dosing ranged between 250 and 2000 mg and the frequency varied from daily for oral therapy to every few weeks or months for IV. INTERPRETATION: Awareness of scurvy and its associated risk factors and clinical presentation is important in the evaluation of a patient with bleeding tendency. Treatment plan should be individualized, and a careful review of patients' diet, medial history and medications is warranted.

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.

Juvenile scurvy from Late Medieval Knin, Croatia.

In this study, we examine the scurvy associated skeletal lesions among juveniles from the late Medieval site Uzdolje-Grablje in Croatia. The study aims to establish a demographic pattern of scurvy using existing diagnostic criteria. The sample consists of 16 well-preserved and fairly complete juveniles, excavated from a cemetery dating to 1420-1920. Skeletal remains were analyzed macroscopically for signs of porosity and new bone formation. Scurvy is diagnosed using the "Ortner criteria" of cranial and postcranial lesions. Depending on the location of the lesions, individuals were diagnosed with either scurvy or possible scurvy. Half of the individuals in our sample are diagnosed with scurvy, with additional 25% being diagnosed with possible scurvy. Along with porotic lesions, all individuals diagnosed with scurvy/possible scurvy showed new bone formation on at least one skeletal element. The occurrence of lesions associated with scurvy in our sample is unusually high. Instead of focusing solely on diet and insufficient intake of the vitamin C, a more likely explanation lies in the synergy between diet, exposure to infections, and the environment.

Lesson of the month 1: Massive spontaneous haematomas in an elderly man.

A 73-year-old man presented with bilateral leg pain and swelling, and no history of trauma or bleeding disorders. Clinical examination, biochemistry and magnetic resonance imaging of the thighs were suggestive of muscle haematomas. These progressed significantly during the admission, requiring blood transfusion. Normal vascular anatomy on computed tomography, renal and liver function, and absence of infection made a bleeding diathesis more likely. This may be caused by coagulation defects, platelet disorders and vascular fragility. An undetectable serum ascorbic acid level confirmed the clinical suspicion of scurvy, and administration of vitamin C resulted in rapid improvement. Our case provides a structured approach to the diagnosis of bleeding disorders and scurvy, a treatable and potentially fatal disease which is often forgotten.