Mara Ferrara

Iron deficiency in childhood and adolescence: Retrospective review

Abstract Two hundred and thirty-eight subjects of both sexes, age range 7.5 months–16 years, with iron deficiency (ID), were included in a retrospective review of ID causes, to determine the best treatment. Inadequate iron intake was the cause of ID or iron deficiency anemia (IDA) in 59 subjects from the first months of life to adolescence. Blood loss linked to cows milk intolerance was the cause of ID or IDA in 37 younger children. Meckels diverticulum (MD) (6 cases), reflux esophagitis (RE) (10 cases), some drugs such as acetyl salicylic acid (11 cases) induced bleeding with ID or IDA in children and adolescents. In pubertal females with ID or IDA, polymenorrhea was observed in 16 cases. Coelic disease (CD) (37 cases), Helicobacter pylori infection (HPI) (39 cases), association of HPI and CD (8 cases), enteromonas infection (15 cases), determining particularly malabsorption, were causes of ID or IDA in patients of a wide age range, unresponsive to iron therapy. Our findings show that iron replacement therapy was not always required and should not be prescribed until the diagnosis is certain.

Effect of Helicobacter pylori eradication on platelet count in children with chronic idiopathic thrombocytopenic purpura

Abstract Recent reports have suggested, particularly in adults, an association between Helicobacter pylori infection (HPI) and chronic idiopatic thrombocytopenic purpura (cITP) with improvement of platelet count after eradication therapy. We investigated the association of HPI and cITP and the effect of HP eradication therapy on thrombocytopenia in a population of 24 children of both sexes mean age 8·0±0·28 years (range 5·4–10·7 years), affected by cITP (PLT ≤50 × 109/l) lasting more than 6 months. HPI was investigated by Helicobacter pylori stool antigens (HpSA). In eight out 24 patients (33·3%) HP infection was identified positive and bacterial eradication was successful following 7 days of triple therapy (amoxicillin, clarithromicin and proton pump inibitors). A follow-up of platelet count was performed for 1 year after HpSA detection. Six out eight patients (75%) had total recovery of platelet count during the first year after bacterial eradication (PLT before therapy 32·5±3·5 × 109/l; after 1 year 275±106·06 × 109/l) (P<0·05), two patients (25%) had partial recovery (PLT before therapy 30 × 109/l, after 1 year 103·5 × 109/l) although not reaching statistical significance (P>0·05). Non-significant differences were found in platelet count between infected and uninfected patients before eradication treatment (PLT 33·0±2·8 × 109/l versus 34·0±5·75 × 109/l) (P>0·05), while significant differences were observed after eradication therapy (PLT 315·0±7·07 × 109/l versus 43·5±2·12 × 109/l) (P<0·05). HP assessment should be performed in all cITP patients and eradication therapy should be attempted in positive cases.

Effect of VDR polymorphisms on growth and bone mineral density in homozygous beta thalassaemia

Summary. We examined the effect of vitamin D receptor (VDR) polymorphisms at exon 2 (FokI) and intron 8 (BsmI) on the stature and bone mineral density at femoral neck (FBMD) and lumbar spine (LBMD) in 108 prepubertal and pubertal homozygous β thalassaemic patients, regularly treated. We found significantly shorter stature and lower LBMD and FBMD in all patients with CC VDR genotype, and significant shorter height and lower LBMD in prepubertal and pubertal female patients with BB VDR genotype. Because homozygous CC and BB VDR genotypes influence Vitamin D activity, they can be considered additional risk factors for bone disease in β thalassaemia.

Diet and iron deficiency in the first year of life: a retrospective study.

Abstract In an observational cohort of 385 infants, we have investigated the relationship between hemoglobin (Hb) levels and iron stores and the type of milk feeding [breast milk (BM), formula milk (FM), cows milk (CM), age and type of weaning, and socioeconomic status] at 8 and 12 months of age. Levels of Hb<11 g/dl and iron stores <15 ng/ml were significantly more frequent in BM and CM groups than in FM group (P<0·05). Significant differences of Hb mean were observed among the three groups, while ferritin mean were lower in BM and CM groups than in FM group (P<0·05). Socioeconomic factors also influenced Hb levels and iron stores through differences in diet. Deprived infants were 23·1% of cohort and many of them received BM (43·5% at 8 months versus 38·5% at 12 months) or CM (42·6% at 8 months versus 47% at 12 months), while >50% were weaned before 6th month of age. FM feeding and weaning <6 months of age are related with better Hb levels and iron stores. Analysis of the impact of weaning on Hb levels and iron stores showed that infants weaned <6 months of life had, regardless of milk feeding, higher Hb and ferritin levels than weaned >6 months.

Side effects of corticosteroid therapy in children with chronic idiopathic thrombocytopenic purpura

Abstract In 29 symptomatic children of both sexes with chronic idiopathic thrombocytopenic purpura (CITP) with platelet counts ≤30,000/mm3, of mean age 7.2 ± 1.3 years, the side effects of long-term glucocorticoid therapy were evaluated. Patients were divided into three groups based on the different glucocorticoid protocols they were receiving. Baseline measurements (t0) of height (H) as standard deviation score (SDS), body mass index (BMI) as kg/m2 and bone mineral density (BMD) at the femoral neck (FBMD) and lumbar spine L 2–L 4 (LBMD) by a dual energy X-ray absorption technique, expressed as Z score, with follow-up measurements at 1 (t1) and 2 (t2) years were assessed. Group I patients (10 pts) treated with oral prednisone (2 mg/kg/die-for one month for 2 cycles) showed significantly different HSDS, BMI, FBMD and LBMD at t1 and t2 (P < 0.005) than other groups treated respectively with pulsed high doses of dexamethasone (24 mg/m2 over 4 days/months for 6 cycles) and methylprednisolone (9 mg/kg/die for 5 days for 3–4 months). These findings suggest that pulsed high doses of glucocorticoid lead to fewer side effects than oral prednisone therapy.

Thrombophilia and varicella zoster in children

Abstract From 2005 to 2011, 25 children of both sexes (13 boys and 12 girls, mean age 7.8 ± 2.5 years, 5–12.4 years) with acute varicella zoster virus (VZV) infection were selected. Five patients showed venous thromboembolism characterized by deep venous thrombosis (DVT). Comparison of activated partial thromboplastin time, antithrombin III, D-dimer, lupus anticoagulant, free S protein (PS), C protein, and antiphospholipid and PS antibodies was performed on children with acute VZV and DVT (group I), acute uncomplicated VZV (group II), and 30 healthy controls of both sexes (15 boys and 15 girls, mean age 7.5 ± 2.6 years, group III). Genetic thrombophilic mutations (Factor V Leiden, MTHFR C677T, and Prothrombin G20210A) were evaluated. Coagulation disorders and PS antibody were found in children with acute VZV (groups I and II). Significant differences were shown among the three groups (P < 0.05). Acute VZV infection could be associated with coagulation disorders and production of inhibitory PS antibodies in many uncomplicated cases.

Influence of Helicobacter pylori infection associated with iron deficiency anaemia on growth in pre-adolescent children

Abstract In 102 children of both genders aged between 10 and 12 years affected by iron deficiency anaemia (IDA) from various causes, a retrospective study from 1999 to 2007 has been performed to investigate possible relationships between IDA and physical growth. Patients were divided into two groups according to gender. Hb, MCV, ferritin levels, HSDS and body mass index (BMI) were recorded to confirm the diagnosis and the height standard deviation scores were also evaluated. The causes of IDA were investigated. At study entry, both groups of patients, regardless of IDA etiology, had microcytic hypochromic anaemia with low ferritin level. Anthropometric parameters were also reduced. Significant differences of anthropometric parameters were found in both groups in relationship to various causes of IDA (p<0·05) (ANOVA). We conclude that IDA may influence physical growth and etiological factors play an important role on growth delay.

HEMATOLOGICAL AND MOLECULAR ANALYSIS OF β-THALASSEMIA AND Hb LEPORE IN CAMPANIA, ITALY

This epidemiological study was based on a hematological and a molecular analysis of 310 heterozygous β thalassemic and 75 carriers of Hb Lepore out of 3,000 microcythemic subjects from the Campania region of Italy. The molecular analysis of β chains and the δβ hybrid gene has shown different β chain defects, but only the Hb Lepore-Boston-Washington type in association with haplotypes I and V. The prevalence and distribution of these molecular defects in Campania show that they are linked to historical events and to the geographical characteristics of this region.

Effect on haematological and anthropometric parameters of iron supplementation in the first 2 years of life. Risks and benefits

Abstract Effects of iron supplementation (IS) on haematological and anthropometric parameters in a cohort of 121 healthy children, followed from 6 to 24 months of life, in the Paediatric Department, Second University of Naples, were evaluated. Children were randomly segregated in four groups: (1) exclusively breast feeding (BF) weaned with non-iron fortified (NIF) foods, (2) BF and iron fortified formulas (IFF) and foods, (3) exclusively IFF and foods, (4) BF and oral iron supplementation (OIS). Haematological parameters (Hb, MCV, Serum ferritin and transferrin saturation) in addition to anthropometric measurements (length and weight) were obtained. Results from the study at 6, 12, 18 and 24 months showed significantly lower values of haematological parameters in BF infants than other groups and in IFF infants than in those with OIS. In contrast children with OIS showed significant lower length. It appears that IS may be of limited or no benefit for growth in non-iron deficient children.

Chronic immune thrombocytopenic purpura in childhood: pathogenetic mechanisms and management

Abstract A population of 26 children of both sexes mean age 8.5 ± 5.8 years with thrombocytopaenic purpura, disease duration at least 7 months (2.5 ± 1.8 years), platelet count 22 000 ± 12 000/mm3 was studied. Patients were divided into three age groups; I: 2–6 years (8 children); II: 7–10 years (10 children); III: 11–16 years (8 patients). Careful history, physical examination, complete blood count with blood smear, platelet autoantibodies, bone marrow aspirate, and response to intravenous immunoglobulins (IV Igs) were evaluated. Statistical analysis was performed by χ2 test. Platelet count, duration of disease, megakaryocytic reduction, need of splenectomy were significantly lower in younger children than older children of III group (P < 0.05). All patients were responsive to IV Ig. No significant differences of presence of platelet autoantibodies, were found among the groups. Relapse after splenectomy was observed in four older patients among whom three had Evans syndrome: complete remission was obtained with rituximab. Disease duration appears to be associated to megakaryocytic alterations and patient age.