Artículos de revistas
Kwashiorkor And Coagulation Disturbance - Atypical Presentation Of Cystic Fibrosis [kwashiorkor E Distúrbio De Coagulação - Apresentação Atípica De Fibrose Cística]
Registro en:
Revista Paulista De Pediatria. , v. 30, n. 1, p. 139 - 143, 2012.
1030582
10.1590/S0103-05822012000100020
2-s2.0-84859298432
Autor
Sundell M.O.T.
Meneses D.G.M.
Ribeiro A.F.
Pinto E.A.L.C.
Hessel G.
Institución
Resumen
Objective: To address the clinical presentation of cystic fibrosis (CF) in an infant presenting Kwashiorkor along with coagulation disturbance due to vitamin K deficiency. Case description: A female baby aged three and a half months, born at term, with birth weight of 2655g, and height of 46cm, was referred to a university center due to perineal moniliasis refractory to therapy, including antifungal drugs and corticosteroids. She had poor weight gain, edema, and diarrhea. After hospital admission under the diagnostic hypothesis of Kwashiorkor of primary or secondary origin, the child received exclusive breastfeeding, but lost weight and maintained the diarrhea. At admission, a urinary tract infection was detected and treated. The child developed bleeding of upper digestive tract and phlebotomy incision at the right saphenous vein treated with vitamin K and fresh frozen plasma. Laboratory exams showed steatorrhea and hypoalbuminemia. Serology was negative for syphilis, toxoplasmosis, mononucleosis, cytomegalovirus, rubella, HIV and hepatitis B. Heterozygous ΔF508 mutation for CF was positive. The patient died with a septic shock. Necropsy showed that the septic shock had a pulmonary origin and that malnutrition was secondary to cystic fibrosis of pancreas. Comments: CF may have a clinical presentation as Kwashiorkor with coagulation disturbance caused by vitamin K deficiency. Health professionals should be aware of this possibility in the differential diagnosis of infants with severe malnutrition and edema. 30 1 139 143 Beaudet, A.L., Cystic fibrosis (1989) The Metabolic Basis of Inherited Disorders, pp. 2649-2680. , In: Scriver CR, Beaudet AL, Sly WS, Valle D, editors, 6 th ed. New York: McGraw-Hill Reed, T.E., Caucasian genes in American Negroes (1969) Science, 165, pp. 762-768 Castro, L.V., Martins, M.C., Application of Schwachman's scoring system to patients with cystic fibrosis with focus on the nutritional aspect (2005) Rev Pediatr Ceará, 6, pp. 27-36 Reis, F.J., Damaceno, N., Cystic fibrosis (1998) J Pediatr (Rio J), 74 (SUPPL. 1), pp. S76-S94 Mérelle, M.E., Griffioen, R.W., Dankert-Roelse, L.E., Cystic fibrosis presenting with intracerebral haemorrhage (2001) Lancet, 358, p. 1960 Mei-Zahav, M., Solomon, M., Kawamira, A., Coates, A., Durie, P., Cystic fibrosis presenting as kwashiorkor in a Sri Lankan infant (2003) Arch Dis Child, 88, pp. 724-725 Tanner, J.M., Whitehouse, R.H., Clinical longitudinal standards for height, weight, height velocity, weight velocity, and stages of puberty (1976) Arch Dis Child, 51, pp. 170-179 Borowitz, D., Baker, R.D., Stallings, V., Consensus report on nutrition for pediatric patients with cystic fibrosis (2002) J Pediatr Gastroenterol Nutr, 35, pp. 246-259 Durie, P.R., Vitamin K and the management of patients with cystic fibrosis (1994) CMAJ, 151, pp. 933-936 Verghese, T., Beverley, D., Vitamin K deficient bleeding in cystic fibrosis (2003) Arch Dis Child, 88, p. 553 Rashid, M., Durie, P., Andrew, M., Kalnins, D., Shin, J., Corey, M., Prevalence of vitamin K deficiency in cystic fibrosis (1999) Am J Clin Nutr, 70, pp. 378-382 Sinaasappel, M., Stern, M., Littlewood, J., Wolfe, S., Steinkamp, G., Heijerman, H.G., Nutrition in patients with cystic fibrosis: A European Consensus (2002) J Cyst Fibros, 1, pp. 51-75 Barros, M.D., Kulesza, T.M., Rañna, W., Carneiro-Sampaio, M.M., Papel do leite materno na defesa do lactente contra infecções (1982) Pediatria (S Paulo), 4, pp. 88-102 Field, C.J., The immunological components of human milk and their effect on immune development in infants (2005) J Nutr, 135, pp. 1-4 Yigit, H., Selimoglu, M.A., Altinkaynak, S., Sweat test results in children with primary protein energy malnutrition (2003) J Pediatr Gastoenterol Nutr, 37, pp. 242-245 Gomez, F., Galvan, R.R., Cravioto, J., Frenk, S., Malnutrition in infancy and childhood with special reference to kwashiorkor (1955) Adv Pediatr, 7, pp. 131-169 Damaceno, N., Muramatu, L.H., Alonso, F.H., Kwashiorkor as early clinical manifestation in a baby with cystic fibrosis (1994) J Pediatr (Rio J), 70, pp. 243-246 Bernstein, M.L., McCusker, M.M., Grant-Kels, J.M., Cutaneous manifestations of cystic fibrosis (2008) Pediatr Dermatol, 25, pp. 150-157 Wenk, K.S., Higgins, K.B., Greer, K.E., Cystic fibrosis presenting with dermatitis (2010) Arch Dermatol, 146, pp. 171-174 Gomes, A.P., Silva Filho, L.V., Reis, V.M., Manifestações cutâneas em pacientes com fibrose cística (1995) An Bras Dermatol, 70, pp. 557-559 Brooks, S.E., Golden, M.H., The exocrine pancreas in kwashiorkor and marasmus. Light and electron microscopy (1992) West Indian Med J, 41, pp. 56-60