An evaluation of children with Kawasaki disease in Istanbul: a retrospective follow-up study

BACKGROUND: Kawasaki disease (KD) is an acute, self-limiting vasculitis of unknown etiology. The incidence of KD is increasing world wide. However, the epidemiological data for KD in Turkey has not been well described. OBJECTIVE: To describe the demographic, clinical, and laboratory features of children with KD who were diagnosed and managed in the American Hospital, Istanbul, Turkey. METHOD: Patients with KD were retrospectively identified from the hospital discharge records between 2002 and 2010. Atypical cases of KD were excluded. A standardized form was used to collect demographic data, clinical information, echocardiography and laboratory results. RESULTS: Thirty-five patients with KD, with a mean age of 2.5 + 1.9 years, were identified. Eighty-five point seven per cent of patients were under 5 years of age. A seasonal pattern favouring the winter months was noticed. In addition to fever and bilateral conjunctival injection, changes in the oral cavity and lips were the most commonly detected clinical signs in our cases. Coronary artery abnormalities were detected in nine patients. The majority of our patients had started treatment with intravenous immunoglobulin in the first 10 days of the onset of fever, and only one patient required systemic steroids for intravenous immunoglobulin-resistant KD. The coronary artery abnormalities resolved in all nine patients within 8 months. CONCLUSION: This study is the most comprehensive series of children from Turkey with KD included in Medline. As adult-onset ischemic heart disease may be due to KD in childhood, further prospective clinical investigations are needed to understand the epidemiology, management and long-term follow-up of the disease.

Early onset conjugated hyperbilirubinemia in newborn infants.

OBJECTIVES: To determine the causes and related outcomes of early onset conjugated hyperbilirubinemia in a group of newborn infants and to determine the incidence of sepsis in these neonates. METHODS: The charts of 42 babies with conjugated hyperbilirubinemia were retrospectively reviewed. RESULTS: The mean gestational age was 37 weeks and the mean postnatal age at presentation was 10 days. Culture-proven sepsis was identified in 15 babies (35.7% of total). Gram-negative bacteria were isolated in 10 cases and E. coli was the most common of these agents (7 cases). Perinatal hypoxia-ischemia was the second most frequent etiology (7 patients; 16.7% of total). The other diagnoses were blood group incompatibility (n=5), Down syndrome (n=3), cholestasis associated with parenteral nutrition (n=3), neonatal hepatitis (n=2), metabolic liver disease (n=1), biliary atresia (n=1), portal venous thrombosis (n=1) and unknown (n=4). Thirteen babies with sepsis recovered completely with treatment, whereas the prognosis for those with perinatal hypoxia-ischemia was grave (six of seven died). CONCLUSIONS: The findings suggest that early onset cholestatic jaundice in newborn infants is more commonly from non-hepatic causes, so it is reasonable to monitor these infants carefully for a period of time before undertaking time-consuming or invasive investigations towards a primary liver disease.

Relationship between serum bilirubin and coagulation test results in 1-month-old infants.

OBJECTIVE: Although the connection between cholestasis and conjugated hyperbilirubinemia is well known, mild hepatic dysfunction or cholestasis may also be associated with unconjugated hyperbilirubinemia in some infants with prolonged jaundice. The aim of this study was to investigate the relationship between serum bilirubin levels and alanine aminotransferase levels, aspartate aminotransferase levels, prothrombin time, activated partial thromboplastin time, and international normalization ratio findings in a group of infants. METHODS: The study included 77 healthy, term, breast-fed infants with jaundice and 56 age-matched, healthy, term, non-jaundiced controls. The 133 babies were divided into three subgroups according to their total bilirubin levels [group I (controls) < 50 micromol/L, group II = 50-100 micromol/L, and group III > 100 micromol/L, and the findings for the noted parameters were compared]. RESULTS: The mean conjugated bilirubin level was significantly higher, and the mean activated partial thromboplastin time significantly longer in group III than in group I. A significant positive correlation was found between bilirubin levels and PT and APTT results. CONCLUSION: Clinical vitamin K deficiency appeared unlikely to develop in this group of infants with prolonged unconjugated hyperbilirubinemia. However, a significant positive correlation between bilirubin levels and PT and APTT suggest that a higher bilirubin load to the liver may cause some degree of vitamin K deficiency due to mild cholestasis. The importance of this finding, and the possible benefits of vitamin K supplementation in 1-month-old breast-fed infants with bilirubin levels higher than 100 micromol/L require further investigation.