Search

Adenosine is produced in the inflammed and damaged lung where it plays roles in the regulation of inflammation and tissue remodeling. Adenosine deaminase (ADA) is an enzyme responsible for the degradation of adenosine. Our aim was to compare the levels of ADA between infants with and without respiratory distress syndrome (RDS) and to determine the relationship between plasma ADA levels and bronchopulmonary dysplasia (BPD). One-hundred and twenty-five premature infants who were admitted to our neonatal intensive care unit were included in the study. Eighty-one of these infants with RDS were study group and the other 44 infants without RDS served as controls. Blood collection was made in the first day of life at the end of 24th-h and was used for laboratory testing. In the RDS group, mean ADA level was 25.5 (±±4.5) U/l, and in controls it was 26.3 (±±5.7) U/l. There was no statistically significant difference ( p == 0.326) in these groups although there was a statistically difference of ADA levels between BPD (34.5 ±± 5.2 U/l) and non-BPD (24.6 ±± 4.1) patients ( p == 0.001). There was also a positive relationship between ADA levels and severity of BPD ( r == ++ 0.845, p == 0.01). Perinatal inflammation is the key mechanism of BPD. ADA level in early postnatal life is elevated in infants with BPD and may be related with perinatal inflammation. [ABSTRACT FROM AUTHOR]

Background: Isolated non-compaction of the ventricular myocardium (INVM) is characterized by prominent and excessive trabeculations in one or more ventricular wall segments, with deep intertrabecular recesses communicating directly with the ventricular cavities. Diagnosis of the disease is based on the 2-D echocardiographic features. Despite the fact that many cases of INVM have been reported in children and adults since the initial description of the disease, it has been diagnosed in only a few fetuses up to now. Therefore, failure to diagnose on fetal echocardiography is probably more common. There are different opinions about diagnosis of INVM on fetal echocardiography. Method: In the present study, six cases of fetal INVM diagnosed between 1999 and 2004 were evaluated for prenatal diagnosis, clinical features and natural course. Results: In four cases, diagnosis was made in the prenatal period, and in the other two cases it was done on postnatal echocardiography at the first day of life. Careful reevaluation of these two patient’s fetal echocardiographic recordings confirmed the postnatal diagnosis retrospectively. Conclusions: The examiner should be aware of the existence of this rare anomaly. If echocardiographic examination shows ventricular hypertrophy and/or poor ventricular function, INVM should be considered and in order to see recesses, ventricle walls should be investigated by zooming in and comparing frame by frame. [ABSTRACT FROM AUTHOR]

There is little information about the iron overload caused by hemolysis in fetuses affected with rhesus hemolytic disease (RHD). The authors therefore studied the iron load in RHD by measuring cord blood ferritin levels in babies affected with RHD and gestational age- and weight-matched controls. Cord blood ferritin levels were higher in babies with RHD. Intrauterine transfusions did not affect the ferritin status of the babies with RHD and there was no correlation between hemoglobin and ferritin levels. The results indicate that there is an increased intrauterine iron load in babies with RHD, independent from intrauterine transfusions and rate of hemolysis. [ABSTRACT FROM AUTHOR]

Congenital dyserythropoietic anemias (CDAs) area group of hereditary refractory anemias characterized by ineffective erythropoiesis, typical morphological abnormalities of erythroblasts, a low or no reticulocyte response, hyperbilirubinemia, and splenomegaly. A massive hydropic newborn born with a very severe anemia (Hb 4.8 g/dL), diffuse edema, hepatosplenomegaly, ascites, pulmonary edema and respiratory distress, and shortness and hallux varus deformity of the great toe of the right foot was diagnosed to have congenital dyserythropoietic anemia on the basis of the hematological (macrocytosis, anisopoikilocytosis, fragmented red cells and erythroblastosis in the peripheral blood, and erythroid hyperplasia with erythroblastosis and erythroblasts with double nuclei and thin chromatin bridges connecting these nuclei in the bone marrow) and serological (negative acidified serum lysis test and no agglutination with anti-i antibodies) findings. In this article the seventh case of neonatal congenital dyserythropoietic anemia presenting with a very severe(lethal) form of hydrops fetalis and a new (hallux varus) deformity of the great toe of the right foot is presented. Congenital dyserythropoietic anemia should be considered in the differential diagnosis of hydrops fetalis presenting with a very severe anemia and a skeletal abnormality of the great toe. [ABSTRACT FROM AUTHOR]

Cystatin C (CysC) is a low-molecular-mass protein (13,343 dalton, 120 amino acids) belonging to the cystatin superfamily of reversible inhibitors of cysteine proteases. CysC appears to be eliminated from the circulation almost exclusively by glomerular filtration, which makes it a promising endogenous marker of renal function. CysC has been demonstrated to reflect glomerular filtration rate better than other low-molecular-weight proteins, including creatinine (Cr). We established reference values for serum CysC and compared them with Cr in 108 preterm infants by particle-enhanced nephelometric immunoassay. On the first day, serum CysC values ranged from 1.25 to 2.84 mg/L, significantly decreasing after 3 days of life. Cr levels determined simultaneously on the first day ranged from 0.05 to 1.12 mg/dl and were also significantly different from day 3 levels. Both CysC and Cr levels were independent of gender, birth weight, hemoglobin levels, and hydration state. Cr correlated negatively with gestational age ( r = −0.25, p = 0.009), but not CysC. A significant correlation was found between CysC and Cr on day 1 ( r = 0.21, p = 0.031), but no correlation was found according to day 3 blood samples ( r = 0.19, p = 0.053). CysC is regarded as an alternative for assessing renal function in preterm neonates, but its advantages over Cr are not yet proven. [ABSTRACT FROM AUTHOR]

Aim: To determine lipid peroxide concentrations in the first 10 days of life.Methods: Malondialdehyde concentrations were investigated in neonates with or without hyperbilirubinaemia during the first 10 days of life.Results: Serum malondialdehyde concentrations were higher in infants with hyperbilirubinaemia than in controls. A positive correlation was found between malondialdehyde and bilirubin concentrations in the study group. When the study group was categorised according to the presence of haemolysis, a significant correlation was found between malondialdehyde and bilirubin concentrations in those infants with hyperbilirubinaemia due to haemolysis. There was no such correlation in those without haemolysis.Conclusion: Exchange transfusion rapidly produces variable changes in pro-oxidant and antioxidant plasma concentrations in neonates, which may be responsible for free radical metabolism. The fall in malondialdehyde concentration is probably directly related to its exogenous removal by exchange transfusion. [ABSTRACT FROM AUTHOR]

A full-term infant girl who had bilateral, symmetrical, sharply defined, triangular-shaped ulcers on both sides of the umbilicus is described. A punch biopsy specimen showed histologic findings of aplasia cutis. Echocardiographic examination revealed primum-type atrial septal defect. To our knowledge, this association is the first of its kind to be reported in the literature. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented discussing lipoid pneumonia caused by intranasal butter application in a newborn infant.

A 41-day-old infant who had severe metabolic acidosis, anemia, bleeding, hypoglycemia, and proximal tubulopathy was diagnosed with Pearson syndrome. Fibrosis in the liver, severe iron deposition in hepatocytes, and multiple renal cortical cysts were found on postmortem examination. Southern blot analysis of mitochondrial DNA obtained from peripheral blood revealed a heteroplasmic deletion of approximately 3.5 kilobases. [ABSTRACT FROM AUTHOR]