Objective – Subglottic infantile haemangioma (SGH) is a rare but potentially life-threatening disease. We describe a case of successful treatment with propranolol of a severely respiratory compromised 2-month-old infant with an obstructing SGH. Case report – A fullterm 2-month-old male infant, weighing 4.8 kg, without cutaneous haemangioma, presented with stridor, dyspnoea, oxygen desaturation and tachycardia. Contrast enhanced neck–chest CT scan was used to diagnose a 5A—7A—7 mm subglottic elliptic lesion, referable to SGH with a free air column of 2 mm wide. Per oral propranolol was started with a therapeutic dosage of 2 mg/kg/day in 3 administrations. On the second day of treatment there was rapid clinical improvement of the infant with withdrawal of respiratory symptoms. After treatment, the control CT showed the regression of SGH. Twenty-one months later the patient is without respiratory symptoms. Conclusion – Propranolol medical treatment should be considered in all subglottic haemangioma, even in acute cases.

We report two new cases of cystic fibroepithelioma of Pinkus together with immunohistochemical features and analyze the presence of cystic changes in a series of 16 classical fibroepitheliomas of Pinkus. Our findings show that the formation of cystic spaces is most probably caused by ischemic degeneration of stromal fenestrations, rather than by central tumor cell necrosis. This finding is supported by lack of CD34 positive blood vessels in edematous and hyalinized stromal fenestrations undergoing transformation into cystic spaces, as opposed to the uninvolved stromal fenestrations. Therefore, it is probably more accurate to refer to this process as pseudocystic stromal degeneration rather than true cyst formation. Also, two out of 16 classical Pinkus fibroepitheliomas exhibited focal pseudocystic changes in 50% and 10% of the tumor, respectively, demonstrating that this degenerative process can be found, rarely and focally, in classical cases as well.

Juvenile scleroderma (JS), represents a rarely seen group of connective tissue disease with multiple organ involvement. Although quite rare in childhood, cardio-vascular and pulmonary involvements are the most important mortality and morbidity factors. Pulmonary arterial hypertension (PAH), the most important sequelae of pulmonary involvement, could be determined by echocardiographic examinations. Early cardio-vascular and pulmonary involvement determination is extremely important in reducing mortality of patients.

This paper deals with on-line motion imitation of a human being by a humanoid robot using inverse kinematics (IK). First, the human observed trajectories are scaled in order to match the robot geometric and kinematic description. Second, a task prioritization process is defined using both equality and minimized constraints in the robot IK model, with four tasks: balance management, end-effectors tracking, joint limits avoidance and staying close to the human joint trajectories. The method was validated using the humanoid robot NAO.

Varicella infection is a highly contagious disease which can have a complicated course especially in immunocompromised children and children receiving immunomodulatory therapy.

Paediatric rheumatic diseases (PRD) form a group of (rare) diseases that can lead to significant morbidity. Evidence-based guidelines are sparse and treatment regimens differ throughout Europe. In 2012, a European initiative called SHARE (Single Hub and Access point for pediatric Rheumatology in Europe) was granted by the European Agency for Health and Consumers (project number 2011 1202) to optimize and disseminate diagnostic and management regimens in Europe for children with PRD.

PFAPA syndrome is the most common autoinflammatory fever disorder in childhood, characterized by recurrent fever, aphthous stomatitis, pharyngitis and adenitis. Mutations in the MEFV and NLRP3 genes are known to cause syndromes with PFAPA overlapping symptoms (Familial Mediterranean Fever and Cryopyrin-Associated Periodic Syndrome), which are rarely reported in patients from Slovenia.

Macrophage activation syndrome (MAS) is a potentially fatal complication of systemic juvenile idiopathic arthritis (sJIA), whose prompt recognition and treatment are critical. However, early diagnosis of MAS is often challenging and none of the current diagnostic criteria is satisfactory. An international project aimed to develop a new set of classification criteria for MAS was recently started.

Results We report the patient characteristics at time point 0, 6 and 12 months of their follow up. We present date on 25 patients. The mean follow up of the patients in the cohort are 3.5 years. No patient died during the follow up. Eighteen of the 25 patients were female. The mean age of the onset of Raynaud symptomatic was 10.4 years, the youngest patient was 2.0 years of age. The mean age at the onset of the non-Raynaud symptomatic were 11.0 years. 19 of the 25 have diffuse subtype, 6 of them have an overlap symptomatic, two of them associated with diffuse subtype. ANA positive were 20, and 8 of them were antiScl 70 positive. None of them was anticentromere positive The mean modified Rodnan Skin Score was at timepoint 0, 6 and 12 month 18.1, 15.1 (n=21) and 15.1. (n=17). Raynaud ́s Phenomen occurred in 22/25 at time point 0 and 16 of 21 at time point 6 months and 12 of 17 at 12 months. 18 of 25 of them had capillary changes already at time point 0. 7 of them had already ulcerations at time point zero, 9 of 21 at month 6 and 4 of 17 at months 12. 15 of them had cardiopulmonary involvement, at time point zero already, 9 of them had interstitial lung disease. 6 of 21 have cardiopulmonary involvement at month 6 and 7 of 17 at month 12 of follow up. Two of them have renal involvement at time point 0 and 3 at time point 6 and 12 months. 9 of 25 had gastrointestinal involvement, and 5 of them oesophageal involvement at time point zero, 3 from 21 at month 6 and 5 of 17 at 12 months. 22 of 25 have musculoskeletal involvement 19 of 21 at month 6 and 16 of 17 at 12 months.

Single nucleotide polymorphisms (SNPs) are common (1%) variations in DNA sequence, that can be the reason for individual variability in drug efficacy and drug safety.

Kawasaki Disease (KD) and Henoch Schonlein Purpura (HSP) are paediatric vasculitides that can lead to significant morbidity. Evidence-based guidelines are sparse and management is mostly based on physician experience. Consequently, treatment regimens differ throughout Europe. In 2012, a European initiative called SHARE (Single Hub and Access point for paediatric Rheumatology in Europe) was launched to optimize and disseminate guidelines for diagnosis and management for children and young adults with paediatric rheumatic diseases (PRD) such as KD and HSP within Europe.

Antiphospholipid syndrome (APS), either primary or secondary to other paediatric rheumatic diseases, is rare in children, but it can lead to significant morbidity. Evidence-based guidelines are sparse and management is mostly based on physician’s experience. Consequently, treatment regimens differ throughout Europe. In 2012, a European initiative called SHARE (Single Hub and Access point for paediatric Rheumatology in Europe) was launched to optimize and disseminate diagnostic and management regimens in Europe for children and young adults with rheumatic diseases such as APS.