Hemorrhagic disease in an infant fed on a vitamin K-deficient soya formula.

Abstract

Listen

Translate article

Hemorrhagic disease of the newborn typically occurs in the first 3 days of life, with gastrointestinal and umbilical bleeding, ecchymoses, hematuria, and cerebral hemorrhage. The disorder can be prevented by a single intramuscular injection of 1 mg of vitamin K. Therefore, it is recommended that this dose be administrated prophylactically to all new-born infants (1). Single oral prophylaxis appears to be less effective than parenteral administration (2). A single administration after birth, however, may not be completely protective against late neonatal hemorrhagic disease; therefore, vitamin K-deficiency occurs more frequently after 1 month of age, especially in infants with malabsorption syndromes, obstructive jaundice, and cystic fibrosis. In the absence of the risk factors mentioned, the disease is virtually confined to breast-fed infants (3). Here we report one case of late hemorrhagic neonatal disease in an infant fed on a vitamin K-deficient soya formula. CASE REPORT The patient was admitted to our hospital at the age of 40 days. The delivery had been spontaneous, performed at the 40th week and the birth weight was 3.350 kg. Oral prophylactic administration of vitamin K was regularly performed. Because of a family history of atopy, the infant was fed on a soya formula. Physical development in the first month of life was satisfactory. A few days before our observation, the mother described evacuation of tarry stools, and in the following days, progressive pallor and epistaxis. Physical examination showed several ecchymoses on the back, tachycardia, remarkable pallor, and muscular hypotonia. Laboratory studies showed the following blood chemistry values: hemoglobin 4.6 g/dL, platelet count 585 × 109 L, hematocrit 12.6%. Prothrombin time and partial thromboplastin time were undetectable and evaluation of vitamin K-dependent factors confirmed the diagnosis (factor II 1%, factor VII 5%, factor IX 0%, factor X 5% with normal serum levels of factor V and VIII). The patient had normal albumin, glucose, creatinine, eletrolytes, aspartate aminotransferase, and alanine aminotransferase serum levels. Echoencephalography and abdomen ultrasound did not show bleeding. Transfusion with packed red cells, infusion of fresh plasma, and intravenous administration of vitamin K led to immediate clinical improvement and normalization of hematologic values in less than 24 h. After a few days the patient was discharged home with a proteic hydrolysate prescription. The follow-up at the age of 3, 8, and 15 months, respectively, confirmed normal values of coagulation tests and the absence of other signs of disease. Personal history, physical examination, and further laboratory tests, such as sweat test, antigliadin antibodies, and d-xylose absorption, excluded secondary causes of vitamin K deficiency and, in particular, malabsorption and cystic fibrosis. DISCUSSION Suggested intake of vitamin K in a newborn is between 10 and 15 μg (4). The vitamin K content in human milk (15 mcg/L) is significantly lower than in cow's milk (60 mcg/L); thus vitamin K deficiency usually occurs in breast-fed infants, especially when they do not receive prophylaxis at birth. Although weekly oral administration of vitamin K has been proposed as a useful protection (5), breast-fed infants usually show only biochemical markers of vitamin K deficiency in the absence of clinical manifestation (6). Therefore, a minimal dietetic intake, in association with prophylaxis at birth, is probably sufficient to prevent the hemorrhagic disease in most, but not in all, breast-fed infants. However, there is no evidence for a prolonged effect of prophylaxis at birth when the diet is completely deficient in vitamin K. The transplacental passage of vitamin K is limited (7) and its production by intestinal flora is insufficient in the newborn. These considerations agree with recommendations of the Committee on Nutrition (8) about the need of vitamin K supplementation in infant formulas. The possible occurrence of late hemorrhagic disease in infants fed on vitamin K-deficient soya formulas has been previously reported (9,10). However, these cases are very rare, at least in the United States, where vitamin K content in infant formulas has been recognized as satisfactory since 1974 (11). The situation in Italy appears to be quite different, as far as the composition of the most common adapted formulas, follow-on formulas, soya formulas, proteic hydrolysates, and other infant formulas is concerned. The results, shown in Table 1, confirm the possibility of a symptomatic vitamin K deficiency in infants fed on artificial foods and suggest that idiopathic cases of late hemorrhagic disease may sometimes be nutritional in origin. Therefore, we recommend establishing quantitatively the vitamin K intake in all patients with hemorrhagic disease. Our case also suggests the need to investigate the vitamin K content of infant formulas in other countries and confirms the need of verify the composition of infant formulas before medical prescription to add, when necessary, the suitable supplementations.