Children with haemophilia often experience limitations in activities of daily life. Recently the Paediatric Haemophilia Activities List (PedHAL) has been developed and tested in Dutch children with intensive replacement therapy. The psychometric properties of the PedHAL in children not receiving intensive replacement therapy are not known. The objective was to gain further insight into the psychometric properties of the PedHAL and to study the functional health status of Romanian children and adolescents with haemophilia. Children attending to the rehabilitation centre of Buzias in Romania were sampled consecutively. Construct validity of the PedHAL was evaluated by concurrent testing with objective and subjective measures of physical function and functional ability. Reproducibility was tested by a 3-day test-retest by intraclass correlation coefficient (ICC) and limits of agreement (LOA). Responsiveness to rehabilitation was assessed by Haemophilia Joint Health Score (HJHS) and PedHAL. Twenty-nine children with severe (n = 25) or moderate (n = 4) haemophilia participated. Mean age was 13.2 years (SD 4.0). Median score of the PedHAL was 83.5 (IQR 47.9-90.5). The PedHAL correlated moderately with HJHS (rho = -0.59), Functional Independence Score in& Haemophilia (rho = 0.65) and Child Health Questionnaire-physical function (rho = 0.40) and not with Child Health Questionnaire-mental health, Child Health Questionnaire-behaviour and 6MWT. Test-retest reliability was good (ICC = 0.95). LOA was 17.4 points for the sum score. HJHS scores improved slightly after rehabilitation, whereas PedHAL scores did not change. In general, construct validity and test-retest reliability were good, test-retest agreement showed some variability. Therefore, currently the PedHAL may be more appropriate for research purposes than for individual patient monitoring in clinical practice.

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Hypoplastic left heart syndrome is a heterogeneous group of congenital cardiac malformations which associates hypoplastic/aplastic left ventricle, mitral and aortic valve, hypoplastic/atresia and severe aortic artery coarctation, and represents a medical-surgical emergency. We present a case of a newborn hospitalised in three clinics (two clinics from Timisoara and one from Vienna), and operated for hypoplastic left heart syndrome, without aortic coarctation, using a mixed technique cardiovascular repair surgery. The initial therapeutic conduct included maintaining the permeability of the arterial canal with prostaglandin E1. At the Vienna General Hospital, at the age of 17 days, bilateral banding of the pulmonary artery was performed and, at the age of 20 days, during the cardiac catheterisation, the Rashkind procedure (balloon atrial septostomy) was performed, with two stents being implanted in the arterial canal. Postoperative complications were postcardiotomy syndrome, pneumonia with Enterococcus faecalis and Stenotrophomonas maltophilia, sepsis with methicillin-resistant Staphylococcus aureus, coagulopathy, mixed anaemia, and metabolic acidosis. The patient died 1 month after the intervention due to cardiorespiratory arrest, bilateral congestive heart failure, left heart hypoplasia with shunt through the arterial canal and pulmonary artery banding, multiorgan failure, and severe secondary haemorrhagic disease. In conclusion, the initial cardiac surgical reconstruction consisted of a mixed technique, and anticoagulant medical treatment with heparin, antibiotics (bacterial endocarditis prophylaxis to be performed throughout life); postintervention hypoxic and infectious complications resulted in multiorgan failure and death.

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