Splenectomy remains the only effective therapeutic modality for children with cITP, although it is associated with transient recurrence and rarely with post-splenectomy sepsis, which could be fatal. Nonetheless, splenectomy should be the last treatment option for the cITP patient, after all available therapeutic modalities have been exhausted and the child still remains profoundly thrombocytopenic and symptomatic.

We retrospectively analyzed the data of 24 children (whereof 11 neonates), with non-central venous line-related and nonmalignancy-related venous thromboembolism (VTE) at uncommon sites, referred to our Unit from January 1999 to January 2012. Thirty patients who also suffered deep vein thrombosis, but in upper/low extremities, were not included in the analysis. The location of rare site VTE was: portal (n=7), mesenteric (n=2) and left facial vein (n=1), spleen (n=3), lung (n=3), whereas 10 neonates developed renal venous thrombosis. The majority of patients (91.7%) had at least 1 risk factor for thrombosis. Identified thrombophilic factors were: antiphospholipid antibodies (n=2), FV Leiden heterozygosity (n=6), MTHFR C677T homozygosity (n=4), protein S deficiency (n=2), whereas all neonates had age-related low levels of protein C and protein S. All but 6 patients received low-molecular-weight heparin, followed by warfarin in 55% of cases, for 3 to 6 months. Prolonged anticoagulation was applied in selected cases. During a median follow-up period of 6 years, the clinical outcome was: full recovery in 15 patients, evolution to both chronic portal hypertension and esophageal varices in 2 children, and progression to renal failure in 7 of 10 neonates. Neonates are greatly vulnerable to complications after VTE at uncommon sites, particularly renal. Future multicentre long-term studies on neonatal and pediatric VTE at unusual sites are considered worthwhile.

INTRODUCTION: Idiopathic thrombocytopenic purpura (ITP) is usually a benign disease that remits within weeks to years. The literature on recurrent ITP (rITP) is limited. OBJECTIVE: The aim of this study was to retrospectively review patients with rITP who were followed up during the period of 1975–2004. METHODS: We reviewed the outcome of 790 children with rITP RESULTS: Among 790 children with ITP, 47 (5.2%) presented with >1 episode of thrombocytopenia. The median age of the children at onset of the disease was 55.9 ± 35.3 months and at final remission was 94.4 ± 58.9 months. The majority of patients (76.6%) had 1 recurrence, whereas the rest of them had >1 recurrence (up to 4); the total number of recurrences was 63. The interval between 2 episodes was <6 months in 25% of the episodes, 6 to 12 months in 29%, 12 to 24 months in 24%, 24 to 36 months in 8%, and >3 years in 14%. Almost half the patients demonstrated bleeding manifestations at diagnosis, whereas only a minority (5) showed bleeding symptoms during the first recurrence. Hemorrhages occurred at times of severe thrombocytopenia and were, in general, mild; however, 1 patient suffered intracranial hemorrhage. Of the episodes, 28.6% necessitated hospitalization and 17.5% required therapeutic intervention with corticosteroids or/and intravenous immunoglobulin. The long-term outcome of all patients was excellent, and none of them needed splenectomy. CONCLUSIONS: rITP occurs mostly in young children, has a good outcome after >1 to 5 episodes, and a median age that ranges from months to years. The course is more often benign; however, life-threatening hemorrhage may occur in a severely thrombocytopenic patient.

INTRODUCTION: Juvenile dermatomyositis/juvenile polymyositis (JDM/JPM), juvenile systemic scleroderma (JSCL-SYST), and juvenile mixed connective tissue disease (JMCTD) are rare in childhood. OBJECTIVE: The objective of this study was to evaluate the prognosis of the rare connective tissue diseases (RCTDs) in children, METHODS: We reviewed the medical charts of children with a diagnosis of RCTD since 1989 and a minimum follow-up of 5 years. RESULTS: Twenty-four (16 female, 8 male) children with JDM/JPM, JSCL-SYST, and JMCTD were studied. The age at disease onset ranged from 4 to 13 years. The follow-up duration was 5 to 12 years. Sixteen children had JDM, and 2 had JPM. Four had JSCL-SYST, and 2 had JMCTD. Until now, 13 children have reached clinical remission, lasting >3 years after stopping drug therapy. Twelve children had JDM/JPM, and 1 had JMCTD. Persistent disease activity was noted in 11 children: 4 with JSCL-SYST, 6 with JDM/JPM, and 1 with JMCTD. Severe pulmonary disease developed in 3 children: 2 with JSCL-SYST and 1 with JMCTD. None of the children with JDM had pulmonary disease. Pulmonary hypertension (PH) was found in 2 children with JMCTD or JSCL-SYST. The child with JSCL-SYST and PH died. Persistent scleroderma pattern by wide-field capillaroscopy was noted in 4 children who had JDM and had had skin ulcerations and have developed subcutaneous calcifications. One of them has also had marked muscle atrophy and severe contractures. CONCLUSIONS: Persistent activity and/or severe pulmonary involvement may be present during the clinical course of RCTD. The presence of PH indicates very poor prognosis in JSCL-SYST/JMCTD cases. Capillaroscopy may identify children who have JDM and are candidates for aggressive therapy.

Objective: Secondhand smoke exposure in children is a recognized risk factor for asthma/wheezing. Even brief exposure can be harmful. Adult smoking prevalence in Greece is of the highest worldwide and pediatric asthma rates are raising. Our objective was to investigate parental smoking habits in homes of children with and without bronchial asthma/wheezing disorders. Methods: This is a case-control study that included 90 children aged 1, 5-17 years old subdivided into two groups: 51 children with asthma/wheezing and 39 healthy peers. SHS exposure was estimated by both questionnaires on smoking habits reported by parents and urinary nicotine and cotinine levels in children. Results: Based on both questionnaires and urinary biomarkers, smoking habits between two groups were not significant (p=0.291). Urinary cotinine assays unveild more children to be exposed to SHS (98% in cases and 89.4% in controls) than reported by their parents (62.75% in cases and 66.6% in controls). More cases were heavier exposed to SHS than controls (>10 ng/ml). Home smoking restrictions between two groups didn't reach statistical significance though they were found to affect urinary cotinine levels in children. Conclusions: Parental smoking habits do not differ between families of children with and without asthma/ wheezing disorders. Parents, especially those nurturing a child with respiratory tract disease, tend to underreport SHS exposure.