The Presence of Precursors of Benign Pre-B Lymphoblasts (Hematogones) in the Bone Marrow of a Paediatric Patient with Cytomegalovirus Infection

Hematogones are normal B-lymphoid precursors that multiply in the bone marrow of small children and of adults with ferropenic anaemia, neuroblastoma or idiopathic thrombocytopenic purpura. They are not normally found in peripheral blood, and the immunophenotype is virtually indistinguishable from that of B lymphoblasts. We discuss the case of a 3-month infant with an active cytomegalovirus infection, with hepatitis and pancytopenia associated with 13% hematogones in the bone marrow

Bronchopulmonary dysplasia: clinical aspects and preventive and therapeutic strategies

Abstract Background Bronchopulmonary dysplasia (BPD) is the result of a complex process in which several prenatal and/or postnatal factors interfere with lower respiratory tract development, leading to a severe, lifelong disease. In this review, what is presently known regarding BPD pathogenesis, its impact on long-term pulmonary morbidity and mortality and the available preventive and therapeutic strategies are discussed. Main body Bronchopulmonary dysplasia is associated with persistent lung impairment later in life, significantly impacting health services because subjects with BPD have, in most cases, frequent respiratory diseases and reductions in quality of life and life expectancy. Prematurity per se is associated with an increased risk of long-term lung problems. However, in children with BPD, impairment of pulmonary structures and function is even greater, although the characterization of long-term outcomes of BPD is difficult because the adults presently available to study have received outdated treatment. Prenatal and postnatal preventive measures are extremely important to reduce the risk of BPD. Conclusion Bronchopulmonary dysplasia is a respiratory condition that presently occurs in preterm neonates and can lead to chronic respiratory problems. Although knowledge about BPD pathogenesis has significantly increased in recent years, not all of the mechanisms that lead to lung damage are completely understood, which explains why therapeutic approaches that are theoretically effective have been only partly satisfactory or useless and, in some cases, potentially negative. However, prevention of prematurity, systematic use of nonaggressive ventilator measures, avoiding supraphysiologic oxygen exposure and administration of surfactant, caffeine and vitamin A can significantly reduce the risk of BPD development. Cell therapy is the most fascinating new measure to address the lung damage due to BPD. It is desirable that ongoing studies yield positive results to definitively solve a major clinical, social and economic problem

Hypertension and Biliary Ductopenia in a Patient with Duplication of Exon 6 of the Gene

We describe a neonatal patient with biliary ductopenia featuring duplication of exon 6 of the JAG1 gene. Facial alterations were observed, consisting of a prominent forehead, sunken eyes, upward slanting palpebral fissures, hypertelorism, flat nasal root and prominent chin. From birth, these were accompanied by the development of haematuria and renal failure and by renal Doppler findings indicative of peripheral renal artery stenosis. JAG1 gene mutations on chromosome 20 have been associated with various anomalies, including biliary cholestasis, vertebral abnormalities, eye disorders, heart defects and facial dysmorphia. This syndrome, first described by Alagille, is an infrequent congenital disorder caused by a dominant autosomal inheritance with variable expressivity. Anatomopathological effects include the destruction and disappearance of hepatic bile ducts (ductopenia). The duplication of exon 6 of JAG1 has not previously been described as an alteration related to the Alagille syndrome with peripheral renal artery stenosis

Effectiveness of Vitamin A in the Prevention of Complications of Prematurity

To assess the effectiveness of vitamin A supplementation in very low birth weight (VLBW) infants to prevent complications of prematurity. Methods: This was a retrospective cohort study to determine the effectiveness of vitamin A in preventing complications of prematurity in VLBW infants. Vitamin A was delivered intramuscularly at a dose of 5000 IU, three times weekly during the first 28 days of life. Results: Of the 187 eligible VLBW infants, we excluded from the analysis (due to death or transfer to another hospital), 16 infants weighing <1000 g and 17 weighing 1000–1500 g. Sixty VLBW infants received the vitamin supplement. We observed no differences between the groups in the duration of oxygen therapy or in the risk of bronchopulmonary dysplasia. The risk of sepsis was up to three times higher among the infants who were given the vitamin A supplement. Conclusion: Given the increased risk of sepsis in patients weighing >1000 g, the risk associated with repeated intramuscular injections of vitamin A and the modest clinical results described, we do not believe the universal administration of vitamin A to VLBW infants to be justified as prophylaxis for bronchopulmonary dysplasia

Total antioxidant capacity of plasma in asymptomatic carrier state of Neisseria meningitidis

Reduction of the antioxidant capacity of plasma has been linked with the impairment of an effective immune response and so we hypothesized that the carriage rate of Neisseria meningitidis in asymptomatic subjects might correlate with the levels of antioxidants in plasma. To this end we took pharyngeal swabs from 339 children in Marquesado Basic Health Zone, Granada, Spain and in addition determined the total antioxidant capacity (TAC) in plasma samples from these subjects. The overall prevalence of N. meningitidis carriage was 5·9% (mean age 7·1 years) with rates of 10·3% in children aged ⩽3 years, 3·9% between 4 and 7 years and 2·4% in older subjects. Plasma TAC for the ⩽3-year-olds was 0·13 for carriers and 1·10 for non-carrier controls (P=0·04), 0·13 for carriers aged 4–7 years (controls 0·63) and 0·28 for carriers aged >7 years (controls 0·52). We analysed the association between TAC in plasma (<0·37 – 2 s.d.) and the carrier state of N. meningitidis. In the carrier state, the odds ratio for this association (TAC in plasma <0·25) was 8·44 (95% CI 1·5–48·9). These findings may suggest a reduced immune response in the host favourable to nasopharyngeal persistence of meningococci