A case report of a 4-year-old child with glucose-6-phosphate dehydrogenase deficiency: An evidence based approach to nutritional management

The objective was to describe the nutritional management of a 4-year-oldchild with glucose-6-phosphate dehydrogenase (G6PD) deficiency.A 4-year-old male child, African descent, born from non-consanguineousparents presented with a clinical history of frequent respiratory infections,usually treated with antibiotics. At 30 months of age, G6PD diagnosis wasmade after eating one portion (40 – 60 g) of fava beans, resulting in severehemolytic anemia hospitalization for 5 days. Diagnosis was confirmed byG6PD activity measurement. Nutritional counseling was given to avoid dietaryoxidative stressors particularly the exclusion of fava beans and accidentalingestion of other similar beans. Dietary intake of high vitamin C containingfoods was discouraged and adequate hydration advised.Nutritional management is crucial in preventing acute stress events in patientswith G6PD deficiency.

___

1. Cappellini MD, Fiorelli G. Glucose-6-phosphate dehydrogenase deficiency. Lancet 2008; 371: 64-74.

2. Frank JE. Diagnosis and management of G6PD deficiency. Am Fam Physician 2005; 72: 1277-1282.

3. Odièvre M-H, Danékova N, Mesples B, et al. Unsuspected glucose-6-phosphate dehydrogenase deficiency presenting as symptomatic methemoglobinemia with severe hemolysis after fava bean ingestion in a 6-yearold boy. Int J Hematol 2011; 93: 664-666.

4. Zuccotti GV, Redaelli F, Gualdi V, et al. Hemolytic crisis in a G6PD-deficient infant after ingestion of pumpkin. Ital J Pediatr 2014; 40: 71.

5. Laquale KM. Glucose-6-phosphate dehydrogenase (G6PD) deficiency and exercise. Athlet Ther Today 2009; 14: 26-31.

6. Finaud J, Lac G and Filaire E. Oxidative stress: relationship with exercise and training. Sports Med 2006; 36: 327-358.

7. Nikolaidis MG, Jamurtas AZ, Paschalis V, et al. Exerciseinduced oxidative stress in G6PD-deficient individuals. Med Sci Sports Exerc 2006; 38: 1443-1450.

8. Beutler E. G6PD deficiency. Blood 1994; 84: 3613-3636.

9. Hafez M, Amar ES, Zedan M, et al. Improved erythrocyte survival with combined vitamin E and selenium therapy in children with glucose-6-phosphate dehydrogenase deficiency and mild chronic hemolysis. J Pediatr 1986; 108: 558-561.

10. Tsakiris S, Reclos GJ, Parthimos T, Tsakiris T, Parthimos N, Schulpis KH. α-tocopherol supplementation restores the reduction of erythrocyte glucose-6-phosphate dehydrogenase activity induced by forced training. Pharmacol Res 2006; 54: 373-379.

11. Schulpis KH, Reclos GJ, Parthimos T, Parthimos N, Gavriilidis A, Tsakiris S. L-cysteine supplementation protects the erythrocyte glucose-6-phosphate dehydrogenase activity from reduction induced by forced training. Clin Biochem 2006; 39: 1002-1006.

12. Georgakouli K, Deli CK, Zalavras A, et al. α-lipoic acid supplementation up-regulates antioxidant capacity in adults with G6PD deficiency. Food Chem Toxicol 2013; 61: 69-73.
Turkish Journal of Pediatrics-Cover
  • ISSN: 0041-4301
  • Yayın Aralığı: Yılda 6 Sayı
  • Başlangıç: 1958
  • Yayıncı: Hacettepe Üniversitesi Çocuk Sağlığı Enstitüsü Müdürlüğü
Sayıdaki Diğer Makaleler

Unilateral pityriasis rosea in a child: A rare clinical presentation

Atiye OĞRUM

Drug reaction with eosinophilia and systemic symptoms in a child on multiple antiepileptics

Jyotindra Narayan GOSWAMI, Pankaj C. VAIDYA, Arushi Gahlot SAINI, Dipankar DE, Bishan Dass RADOTRA, Pratibha D. SINGHI

High frequency oscillatory ventilation versus conventional mechanical ventilation in pediatric acute respiratory distress syndrome: A randomized controlled study

Ahmed EL NAWAWY, Azza MOUSTAFA, Hassan HESHMAT, Ahmed ABOUAHMED

The use of nasal trumpet as a non-invasive treatment method in congenital nasal stenosis

Özgür KEMAL, Sinan ATMACA, Ayşe BEL ÇEÇEN, Berkan DÜZGÜN, Hasibe Canan AYGÜN

Non-suicidal self-injury behaviors’ features and relationship with adolescents’ daily life activities and mental status

Ayşen COŞKUN, İrem Damla ÇİMEN, Nilay ETİLER

Truncus arteriosus with double aortic arch: A rare association

Selman Vefa YILDIRIM, Ali YILDIRIM

The long-term neurodevelopmental outcomes of infants born full-term with low birth weight

Hatice AKSU, Ayşe TOSUN, Börte GÜRBÜZ ÖZGÜR, Münevver KAYNAK TÜRKMEN

SNAPPE-II and risk of neonatal morbidities in very low birth weight preterm infants

Zeynep GENÇTÜRK, Ahmet Yağmur BAŞ, Özge AYDEMİR, Beyza ÖZCAN, Nihal DEMİREL, A. Sumru KAVURT

A case report of a 4-year-old child with glucose-6-phosphate dehydrogenase deficiency: An evidence based approach to nutritional management

Alex PINTO, Anita MACDONALD, Esmeralda CLETO, Manuela ferreira ALMEIDA, Paula cristina RAMOS, Julio cesar ROCHA

Association of STAT4 gene single nucleotide polymorphisms with Iranian juvenile-onset systemic lupus erythematosus patients

Arash SALMANİNEJAD, Mahdi MAHMOUDI, Saeed ASLANI, Shiva POURSANI, Vahid ZIAEE, Nima REZAEI