Case report: Suspected transfusion-related acute lung injury type II in a child with refractory systemic juvenile idiopathic arthritis complicated by macrophage activation syndrome.

Introduction: Transfusion-related acute lung injury is a rare but potentially fatal complication, which may appear during or post-transfusion of blood products. Patients with macrophage activation syndrome, a serious life-threatening complication associated with systemic juvenile idiopathic arthritis, often require transfusion or administration of blood products for correction of cytopenia, coagulopathy and hypofibrinogenemia. Case report: A 6-year-old girl with a past medical history of systemic juvenile idiopathic arthritis had the first relapse of the disease during which she developed macrophage activation syndrome. During this life-threatening complication, she received a second dose of whole blood derived filtered and irradiated platelets from a single male donor due to profound thrombocytopenia. Approximately one hour post-infusion, the patient developed progressive dyspnea, hypoxemia and bilateral pulmonary edema. She was promptly intubated and placed on mechanical ventilation for 40 h. Clinical, laboratory and radiological findings, as well as the success of supportive ventilation therapy were highly suggestive of transfusion-related acute lung injury, a life-threatening complication that occurs within six hours of blood component transfusion. Blood immunology showed no presence of anti-human neutrophil antigen and anti-leukocyte antigen class I and class II antibodies in the donor's or patient's plasma. Conclusion: To the best of our knowledge, we report the first case of a child with systemic juvenile idiopathic arthritis complicated with macrophage activation syndrome who developed type II transfusion-related acute lung injury following platelet transfusion. It is important to consider transfusion-related acute lung injury in transfusion settings in these children and apply critical and restrictive approach for platelet transfusion.

Visual impairment in children with cerebral palsy: Croatian population-based study for birth years 2003-2008.

AIM: To evaluate visual impairment (VI) in children with cerebral palsy (CP). METHODS: This population-based study included 419 children from the Surveillance of Cerebral Palsy in Europe (SCPE) C28 RCP-HR - Register of Cerebral Palsy of Croatia born 2003-2008. Vision in children with CP (according to SCPE) was classified as normal or impaired, with the subcategory of severe VI. The proportion of children with VI was assessed in groups with different CP type/subtype, gross and fine motor function, and gestational age (GA). RESULTS: A total of 266 children had some degree of VI (266/400; 66.5%), 134 had normal vision, and data on VI were unknown for 19 children. Severe VI was present in 44 children (44/400; 11%). The proportion of children with VI and severe VI increased with the Gross Motor Function Classification System and Bimanual Fine Motor Function levels. Children with bilateral spastic CP had the highest frequency of severe VI (14.9%). The percentage of severe VI in children with bilateral spastic CP was 53.8% in the group born <28 weeks of GA, 13.3% in the group born 28-31 weeks of GA, 11.1% in the group born 32-36 weeks of GA, and 24.4% in the group born >36 weeks of GA (λ2=4.95; df=6; P<0.001). CONCLUSION: Children with CP have a high prevalence of VI and severe VI, which is increasing with the level of motor impairment. Severe VI is significantly more common in children with bilateral spastic CP, especially among extremely premature infants.

DO ORTHOPTIC EXERCISES HAVE ANY INFLUENCE ON CHILDREN AND ADOLESCENTS DIAGNOSED WITH CONVERGENCE INSUFFICIENCY AND ATTENTION DEFICIT/HYPERACTIVITY DISORDER?

The aim was to determine whether improvement of near point of convergence (NPC) and binocular vision after orthoptic exercises had any impact on children and adolescents diagnosed with attention deficit/hyperactivity disorder (ADHD) and convergence insufficiency (CI). In this clinical trial, 50 children and adolescents aged 6 to 18 years diagnosed with ADHD and CI received orthoptic therapy that included home-based exercises (pencil push-ups and stereograms) and office-based therapy on synoptophore. Binocular vision and NPC were measured before, during and after therapy. Study subjects showed significant improvement (p<0.05) in NPC and binocular vision after orthoptic exercises. We found statistically significant correlation between stereovision improvement (Lang I) and near point of convergence, suggesting that improvement of binocular function is possible in children with ADHD and CI. Our results showed that NPC enhancement improved stereovision in patients with ADHD. Since progress of binocular function has positive effect on near work and diminishes visual symptoms in children and adolescents with ADHD and CI, it might be reasonable to suppose that orthoptic therapy helps these children improve concentration as well. Further studies are needed to determine whether it might have positive impact on attention.

Anatomical study of the facial nerve canal in comparison to the site of the lesion in Bell's palsy.

The term Bell's palsy is used for the peripheral paresis of the facial nerve and is of unknown origin. Many studies have been performed to find the cause of the disease, but none has given certain evidence of the etiology. However, the majority of investigators agree that the pathophysiology of the palsy starts with the edema of the facial nerve and consequent entrapment of the nerve in the narrow facial canal in the temporal bone. In this study the authors wanted to find why the majority of the paresis are suprastapedial, i.e. why the entrapment of the nerve mainly occurs in the proximal part of the canal. For this reason they carried out anatomical measurements of the facial canal diameter in 12 temporal bones. By use of a computer program which measures the cross-sectional area from the diameter, they proved that the width of the canal is smaller at its proximal part. Since the nerve is thicker at that point because it contains more nerve fibers, the authors conclude that the discrepancy between the nerve diameter and the surrounding bony walls in the suprastapedial part of the of the canal would, in cases of a swollen nerve after inflammation, cause the facial palsy.