Clinician’s Corner Case 2: An adolescent refugee with lower-extremity pain and weakness A 12-year-old boy reported to a tertiary-care paediatric emergency department with gradual, intense pain and weakness in the lower limbs. As an Iraqi refugee who had resided in Canada for almost a year, he was living in a precarious social and economic condition with his mother and three siblings. He had transfusiondependent β-thalassemia major with associated hemosiderosis, splenectomy and hepatitis C. At presentation, he was taking an iron chelator and was receiving blood transfusions every three weeks. He had been recently hospitalized for cellulitis at the site of catheterization for his chelation sessions and treated with intravenous antibiotics. Pain and weakness in both legs were the only symptoms reported and were interfering with his school attendance. He denied experiencing other neurological symptoms. He had neither voiding problems nor fecal incontinence. He reported no fever, night sweats or weight loss. The physical examination revealed a frail and small-for-age boy with normal vital signs. He had extensive gingivitis and his neurological examination was somewhat inconsistent, revealing symmetrical hyperesthesia and moderate weakness of the lower limbs. His gait was laboured, but his deep tendon reflexes were normal. There was no joint or soft tissue swelling, and his skin showed no rash. Further investigations revealed the diagnosis. Correspondence (Case 2): Dr Richard E Bélanger, Centre mère-enfant Soleil du CHU de Québec, 2705, Laurier Boulevard – R1742, Quebec G1V 4G2. Telephone 418-654-2282, fax 418-654-2137, e-mail [email protected] Case 2 accepted December 17, 2013 Paediatr Child Health Vol 19 No 6 June/July 2014 ©2014 Pulsus Group Inc. All rights reserved 299 Clinician’s Corner CASE 2 DIAGNOSIS: SCURVY Because of the severity of the clinical presentation and the overall complexity of the case, the patient was hospitalized. Blood samples were drawn, showing moderate anemia (hemoglobin 81 g/L), with a hemoglobin level similar to his baseline level. His white blood cell (18.4×109/L) and platelet (856×109/L) counts were high but also unchanged. C-reactive protein and creatine kinase levels were normal. In the following days, he underwent electromyography and magnetic resonance imaging (cerebral and spinal), which showed no neurological cause for his symptoms. Extended radiographs revealed low bone mineralization. Otherwise, psychosocial assessments pointed toward intimidation and rejection at school, mainly from cultural barriers, as important factors to consider in the understanding of his symptomatology. However, a few weeks after discharge, the nutritional work-up revealed a very low vitamin C level (6 µmol/L; normal 40 µmol/L to 90 µmol/L) and the diagnosis of scurvy was reached. Although historically an important issue among mariners, scurvy is currently rare, especially in industrialized countries. It results from a deficiency in vitamin C (ascorbic acid), which is derived exclusively from the diet. After absorption in the distal small bowel, a one- to three-month supply of ascorbic acid can be stored in the body, while some is used immediately as a cofactor for many biological processes. The first symptoms to arise are nonspecific: malaise, lethargy, low-grade fever and poor weight gain. Once the body pool of ascorbic acid is depleted, patients may complain of bruises/petechiae, bleeding gums, arthralgia and poor wound healing. Scurvy may also cause generalized tenderness and weakness (especially in the lower limbs), neuropathy and multifactorial anemia. Severe deficiency may even result in skeletal muscle degeneration, growth delay, cardiac hypertrophy and adrenal atrophy. In the present case, scurvy explained the neurological symptoms reported as well as the poor oral condition, and may have contributed to previous cellulitis. Thus, when faced with vague symptoms, nutritional deficiency should be considered as an underlying cause whenever risk factors are present. Regarding scurvy, the latter include exclusive cow’s milk feeding during the first year of life, poverty, refugee life conditions, anorexia, type 1 diabetes, small intestinal diseases and restricted diets due to developmental or behavioural factors such as autism. In the present case, the cause was most likely multifactorial including low vitamin C intake associated with life as a poor and chronically ill refugee. Moreover, iron overload promotes irreversible ascorbic acid oxidation into oxalic acid, which then becomes largely excreted in the urine (1). The patient’s condition improved after oral ascorbic acid supplementation was initiated. He left with nutritional advice and social interventions were undertaken to alleviate the psychosocial difficulties, including at school. Since then, he has not experienced any cellulitis or limb pain, and his oral condition has completely resolved. Throughout follow-up, his plasma vitamin C concentration normalized. Acknowledgements: The authors thank Richard Poulin PhD for his help in the preparation of the manuscript. Sébastien Bergeron MD Valérie Larouche MD Department of Paediatrics Centre mère-enfant Soleil du CHU de Québec Laval University Richard E Bélanger MD Department of Paediatrics Centre mère-enfant Soleil du CHU de Québec Population Health and Optimal Health Practices Research Unit CHU de Québec Research Center Laval University, Laval, Quebec References 1. Cohen A, Cohen IJ, Schwartz E. Scurvy and altered iron stores in thalassemia major. New Engl J Med 1981;304:158-60. 2. Islam B, Ali S. Demographic characteristics and needs of families at an urban, low-income, multicultural paediatric clinic. Paediatr Child Health 2012;17:181-4. Clinical Pearls • Health care providers should remember that scurvy still occurs and must be considered as a diagnosis, especially in high-risk groups with restricted diets. • Nutritional deficiencies should be considered to explain atypical symptoms, even in the presence of strong psychosocial stressors. • In providing culturally adapted care, paediatricians should consider that poverty, social stigmatization and language barriers are only some of the hurdles new immigrants and refugees face after their arrival (2). 300 Paediatr Child Health Vol 19 No 6 June/July 2014
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