Ultrasonographic imaging of inflammatory bowel disease in pediatric patients

Inflammatory bowel disease(IBD) is one of the most common chronic gastrointestinal diseases in pediatric patients.Choosing the optimal imaging modality for the assessment of gastrointestinal disease in pediatric patients can be challenging.The invasiveness and patient acceptance,the radiation exposure and the quality performance of the diagnostic test need to be considered.By reviewing the literature regarding imaging in inflammatory bowel disease the value of ultrasound in the clinical management of pediatric patients is highlighted.Transabdominal ultrasound is a useful,noninvasive method for the initial diagnosis of IBD in children;it also provides guidance for therapeutic decisions and helps to characterize and predict the course of the disease in individual patients.Ultrasound techniques including color Doppler imaging and contrast-enhanced ultrasound are promising imaging tools to determine disease activity and complications.Comparative studies between different imaging methods are needed.

Growth Hormone Treatment, Cardiovascular Risk and Autonomic Maturation in Children and Adolescents with Growth Hormone Deficiency or Born Small for Gestational Age

Introduction: The impact of growth hormone therapy in children with short stature on cardiovascular prognosis seems to be unpredictable from big databases. The enhanced cardiovascular risk in this group of patients may be related to adverse autonomic imprinting by early life stress. Autonomic dysfunction and possible effects of growth hormone therapy on the autonomic nervous system can be measured easily by calculating heart rate variability (HRV) from Holter electrocardiogram monitoring. Methods: We performed HRV analysis prior to growth hormone therapy (N = 33), within the first year of growth hormone therapy between 4 and 10 years of age (N = 19), at least a further HRV measurement between 10 and 15 years (N = 30). Additional measurements were performed before and after cessation of growth hormone therapy (N = 14). Data were compared to untreated pediatric patients with short stature and to age matched healthy controls. Results: Untreated patients with short stature due to growth hormone deficiency or intrauterine growth restriction in early childhood have significantly increased heart rates most of all at night and concomitantly reduced global HRV indicated as Standard Deviation of Normal to Normal Intervals (SDNN). Growth hormone treated adolescents and the untreated patients with short stature show significantly elevated mean heart rates and concomitantly reduced vagus activities measured as reduced Route Mean Square Standard Deviation (RMSSD). After cessation of growth hormone treatment SDNN significantly increases and heart rate decreases to normal values in formerly treated patients with catch-up growth. Conclusion: There is a comparable autonomic dysfunction in treated and untreated children with short stature as an indicator for enhanced cardiovascular risk. After cessation of growth hormone therapy, we found a significant improvement of reduced HRV to normal values.

Immunadsorption therapy for end stage heart failure due to Duchenne muscular dystrophy

We report about a successful immunadsorption therapy of a boy with end stage heart failure due to Duchenne muscular dystrophy who has little chance to get cardiac transplantation. Prior to this therapy a medical therapy with an angiotensin converting enzyme inhibitor, a low dose betablocker, an aldosterone antagonist, and diuretics failed. In consent with the patient and his parents immunoadsorption therapy employing a protein A column was performed. Due to clinical improvement the betablocker carvedilol could be titrated from 6.25 mg up to 30 mg. In the following 4 month he improves from NYHA class IV to NYHA class II and NT-Pro-BNP levels fell from 5180 pg/ml to 402 pg/ml. The mean heart rate in Holter ECG decreases from 102/min to 68/min and ejection fraction improved from 25% to 30%. The boy began to walk without any support and was able to visit school. This clinical improvement now holds on for 2 years.

Recurrent pulmonary embolism in a boy with antiphospholipid syndrome

We report a 14-year-old patient with recurrent pulmonary embolism due to catastrophic antiphospholipid syndrome (APS) with severe pulmonary inflammation. We considered elevated antibodies against cardiolipin and anti-beta2-glycoprotein-1, but no clinical nor laboratory manifestations of systemic lupus erythematosus (SLE). Pulmonary embolism had been the first manifestation of catastrophic APS in this patient. We prescribed warfarin and systemic corticosteroids. A second embolism appeared during anticoagulation with warfarin. This event led to a marked decrease of his physical performance due to his obstructive and restrictive lung disease. Anticoagulation was changed from enteral warfarin to subcutaneous enoxaparine. We also prescribed inhaled corticosteroid which led to an improvement of his respiratory symptoms and overall poor physical condition.

Neonatal Marfan Syndrome: Improving the Bad Prognosis with a Strict Conservative Treatment with Carvedilol?

We report about a successful heart failure therapy with carvedilol in two children with neonatal Marfan syndrome (nMFS). As shown in Case 1, double valve replacement in an infant with neonatal Marfan syndrome is feasible but its benefit on long term is uncertain. Excluding our patient, 3 infants with nMFS from the literature died early after cardiac surgery. Our second case is a unique patient who survives nMFS despite diaphragmatic herniae, dilated neonatal cisterna magna and severe atrioventricular valve insufficiencies. Treated with 0.7 mg/kg/day Carvedilol since his seventh month of life, he never developed severe heart failure. However despite his good health status at the age of 9 years, a progressive aortic root dilatation and left conornary aneurysm are still waiting for surgical repair.