Paediatric chronic liver diseases: how to investigate and follow up? Role of imaging in the diagnosis of fibrosis

Chronic liver diseases are rare in children, but encompass a wide spectrum of disorders that may all be complicated by liver fibrosis and therefore by portal hypertension. They may be classified according to the level of portal flow obstruction: prehepatic, intrahepatic or suprahepatic. Most of them, except presinusoidal diseases, may progress to cirrhosis that carries additional risks of impaired liver function and development of hepatocellular carcinoma. Imaging plays an important role in guiding the diagnosis and biopsy and for follow-up during treatment. US, with high-frequency transducers and Doppler, is the first modality of choice, directs the rest of the investigations and guides interventional radiology. MDCT has made great progress and has replaced angiography for diagnostic purposes. MRI is indicated for parenchyma and nodule characterization and for biliary tract evaluation. To avoid liver biopsy, several elasticity imaging techniques have been developed and have to be evaluated for accuracy and convenience in children. The role of each modality with main imaging findings is described in extrahepatic portal vein obstruction, hepatoportal sclerosis, congenital hepatic fibrosis, cirrhosis and Budd-Chiari syndrome.     

Movements and postures of infants aged 3 to 5 months: to what extent is their optimality related to perinatal events and to the neurological outcome?

Background

The quality of spontaneous general movements (GMs), assessed in the individual infant, has emerged as one of the most reliable and valid predictors especially of severe neurological impairments.

Aims

To implement a more detailed assessment of GMs and co-existing movements and postural patterns in a rehabilitation clinic, and to examine to what extend is the optimality of movements and postures of infants aged 3 to 5 months related to perinatal events and the neurological outcome.

Study design

Prospective study of 41 infants (15 boys and 26 girls; 11 infants born preterm) admitted to the Department of Paediatric Neurology and Rehabilitation of the St. Joseph's Hospital in Kyoto (Japan).

Outcome measures

Clinical, neurological and psychological status at age 5.

Results

Motor optimality at age 3 to 5 months correlated positively with neonatal optimality (r = 0.48, p < 0.01), especially regarding factors associated with hypoxic events. A non-optimal motor performance (lowest possible scores) predicted cerebral palsy with 100% accuracy. Other adverse outcomes such as developmental delays, developmental coordination disorders, pervasive developmental disorder or attention deficit hyperactivity disorder turned out not to be associated with early motor performance. In 13% of cases absence of fidgety movements proved to be false positives, but their normal appearance along with a smooth concurrent motor performance was solely found in infants with a normal neurological development.

Conclusion

Assessing the quality of motor performance at age 3 to 5 months considerably improves our ability to identify infants at risk for maldevelopment.     

Crosstalk between cytokine profile,redox, and iron status in β-Thalassemia: relation to frequency/duration of blood transfusion

β-Thalassemia, a blood disease instigated by mutations in β-globin genes or their regulatory regions. Infectious complications, immune abnormalities and iron overload related organ damage are the chief causes of morbidity and mortality. The role of inflammatory cytokines in the pathophysiology of β-thalassemia is still indistinct, so the present study aimed to evaluate immune-inflammatory and redox status in β-thalassemia and their relation to iron status, frequency/duration of blood transfusion. Seventy β-Thalassemia patients (35 β-thalassemia major; 35 β-thalassemia intermedia) and twenty age and sex-matched healthy controls were included. Interleukin4 (IL4), Interleukin10 (IL10), Interleukin 8 (IL8), and Interleukin 13 (IL13) levels were immunoassayed in addition to iron, hematological and redox status assessment. Significantly increased iron, ferritin, IL8, IL13 levels (p value ?0.001), meanwhile, decreased IL10 level, total anti-oxidant (TAC) and iron binding capacities were observed in β-thalassemia major compared with β-thalassemia intermedia and control groups (p value ?0.001) . IL4 level was decreased in patients group compared to control. Positive correlation was found between malondialdehyde (MDA), IL8, IL13 and iron load, transfusion frequency/duration, total leucocyte count and lymphocyte (%). Meanwhile, negative correlation was found with IL4, IL10, and TAC. Multiple blood transfusions increased iron stores which may critically impair the immune-regulatory balance with disturbed redox status and cytokine profile.     

A multilevel analysis of the relation of socioeconomic status to adolescent depressive symptoms: does school context matter?

OBJECTIVE: To determine whether the socioeconomic context of the school environment is associated with adolescent depressive symptoms independent of individual household income.Study design Data were drawn from a 1995 nationally representative study of 7th to 12th grade students. Multivariable linear regression at the school and individual levels assessed the relation between income and depressive symptoms. Multilevel modeling techniques were then used to understand how these factors are jointly associated with adolescent depressive symptoms. PARTICIPANTS: Adolescents (n=13,235) in grades 7 through 12 from 132 schools whose parent provided income information. RESULTS: Linear regression analyses indicated that lower household income, average school income, and increasing school-level income inequality were significantly (P<.001) associated with depressive symptoms. Further examination of these relations through multilevel modeling indicated that both household income (P<.01) and average school income (P<.05) were significantly related to depressive symptoms after adjusting for covariates, with evidence for an interaction between the two. The impact of lower household income on depressive symptoms was approximately 2-fold greater for students attending a poor versus a rich school. CONCLUSIONS: School context is associated with adolescents' depressive symptoms, even after adjusting for individual-level factors. The school environment may partially buffer the adverse influence of lower household income on adolescent depressive symptoms.     

Maternal perceptions of her child’s body weight in infancy and early childhood and their relation to body weight status at age 7

Introduction Maternal perception of her child’s weight status has been hypothesised to affect a child’s weight development.Objective The aim of this analysis was to determine in how far the maternal weight perception of her child’s weight at different ages is related to its future body weight status.Materials and methods Longitudinal data on body weight, height and skinfolds from the Dortmund Nutritional and Anthropometric Longitudinally Designed Study were used to determine the risk of being overweight at age 7. Complete data on anthropometry, maternal weight perception and confounding variables were available for 253 children.Results and discussion Maternal weight perception assessments at age 6 months, 12 months, 2 years and 4 years of age were related to body weight status as well as changes in body mass index standard deviation score (BMI-SDS) and skinfold SDS between age at assessment and age 7. With respect to the risk of being overweight at age 7, no independent effect of maternal perception was found. When changes in anthropometric measures were considered, it could be observed that children whose weight was considered too low gained more weight until age 7, and those who were considered to be too heavy lost more weight as compared to the children whose weight was considered to be just right. Among infants aged 6 months who were above the 85th percentile, maternal misperception appeared to promote an unfavourable weight development. These latter results support the hypothesis that maternal weight perception might affect a child’s weight development.Conclusion As our data suggest, this effect might begin to operate already in infancy.     

From puberty to poverty — the health problems and needs of adolescents

‘Oh yes — teenage — that's the time I had between puberty and poverty’ 19-year-old student at University.     

Epidemiological characteristics of pertussis in Estonia,Lithuania, Romania,the Czech Republic,Poland and Turkey—1945 to 2005

Pertussis epidemiology was examined in selected Central and Eastern European countries andTurkey (CEEs) from 1945 to 2005. Epidemiology and immunisation coverage data were collected fromNational Health Departments and Epidemiology Institutes. Pertussis diagnosis was made by the World Health Organization (WHO) clinical criteria, laboratory confirmation and/or epidemiological link, except for Romania (WHO clinical case definition used). In the pre-vaccine era, pertussis incidence (except Turkey) exceeded 200/100,000 (range180–651/100,000), with 60-70% of cases occurring in pre-school children. Until 2007, a second-year booster was givenin Estonia, Lithuania and Turkey, and an additional pre-school booster elsewhere. During 1995–2005, immunisation coverage by the age of 2 years exceeded 80% (range 80–98%) and, excluding Estonia, pertussis incidence was <3/100,000. Age-specific incidence rates rose in 5–14 year olds in Poland, Estonia and the Czech Republic. Incidence rates in children <1 year of age remained unchanged. There were two age distribution patterns. In the Czech Republic and Estonia, 16% of cases occurred in pre-school children and 17% and 22% in children >15 years of age, respectively; in Romania, Turkey and Lithuania, 51%, 71% and 73%, respectively, occurred in pre-school children and <7% in children aged >15 years.Pertussis infection persists, despite high immunisation coverage. Compared with the pre-vaccine era, the age distribution changed differentially in CEEs, with an apparent shift towards older children. Sources of support  This review of data was produced with the financial support of GlaxoSmithKline Biologicals.     

Crohn’s disease,autoimmune thyroiditis,and beta-thalassemia trait in an adolescent: an unusual combination of diseases

An adolescent with complaints of fatigue, tachycardia, abdominal discomfort, and blood-stained diarrhea is presented. Clinical and laboratory evaluation revealed a microcytemic anemia with iron deficiency, beta thalassemia, and thyrotoxicosis with thyroid antibodies. Crohn’s disease was confirmed on endoscopy. A rapid normalization of clinical and laboratory parameters was observed following the initiation of therapy and further exacerbation of her illness was prevented. Although the simultaneous occurrence of Crohn’s disease, autoimmune thyroiditis, and a beta-thalassemia trait is likely to be coincidental, the combination of an autoimmune thyroid disease and Crohn’s disease is rare in pediatrics. Several issues of importance in the treatment of these conditions are discussed. Rectal blood loss associated with Crohn’s disease may lead to severe iron deficiency, especially in patients with preexistent beta-thalassemia trait, and those with thyroiditis are prone to developing hypothyroidism following treatment, requiring that they be monitored closely.