Background
In Kawasaki disease (KD), a vasculitis of unknown etiology, the most serious complication is the development of coronary artery aneurysm (CAA). To date, the exact pathomechanism of KD is unknown. Both environmental and genetic factors seem to be associated with the development of the disease.
Methods
Data on KD patients recruited from the population-based German Pediatric Surveillance Study during 2012–2014 were used to evaluate the impact of various factors from the perinatal and infancy period on the development of KD. The study design was a matched case-control study with respect to age, sex and place of residence (
n
= 308 KD cases,
n
= 326 controls). All KD patients were individually re-evaluated; all fulfilled the international diagnostic KD criteria. A standardized questionnaire was used to review breastfeeding practices, vitamin D supplementation and birth characteristics. Logistic regression analyses were performed to obtain odds ratios (OR) for various risk factors among the case-control pairs. Simple measures of association were used to assess the impact of these factors on the clinical course.
Results
There was no difference in lengths of gestation, birth weight or parturition between KD patients and controls, but independently from each other vitamin D supplementation and breastfeeding were negatively associated with KD, even when adjusted for age, place of residence and sex. The duration of vitamin D was significantly shorter among children with KD than among children without KD (
p
= 0.039, OR = 0.964, 95% CI: 0.931–0.998), as was the duration of breastfeeding (
p
= 0.013, OR = 0.471, 95% CI: 0.260–0.853). Comparing KD patients with and without breastfeeding and/or vitamin D supplementation, there were no differences regarding developing CAA, being refractory to intravenous immunoglobulin treatment, age at onset of the disease and levels of inflammatory laboratory values.
Conclusion
Our findings indicate breastfeeding and vitamin D supplementation to have protective effects in association with KD in our study population; however, these seem not to influence the natural course of the disease. Although the overall effects were relatively small, they nevertheless underline the overall benefit of both interventions.
Trial registration
Clinical Trial Registration: German clinical trial registration,
http://apps.who.int/trialsearch/Trial2.aspx?TrialID=DRKS00010071
. Date of registration was 26. February 2016. The trial was registered retrospectively.
Electronic supplementary material
The online version of this article (10.1186/s12887-019-1438-2) contains supplementary material, which is available to authorized users.
This report describes the first clinical experience with the new Occlutech Atrial Flow Regulator (AFR®) device for implementation of a late Fontan fenestration. The AFR® device secured a defined diameter of the fenestration without spontaneous re-occlusion of this extra-anatomic perforation. This ensured a permanent clinical improvement of our failing Fontan patient.
Background: Interventional percutaneous pulmonary valve implantation (PPVI) was first reported in 2000. Today, two different valves are certified for this procedure [i.e. Medtronic Melody 1 valve (Medtronic, Dublin, Ireland) and Edwards Sapien TM valve (Edwards Lifesciences, Irvine, CA, USA)]. For a decade, studies have reported an increasing risk of infective endocarditis (IE) after PPVI; as patients for PPVI are usually younger, even a low annual incidence of IE is important. However, the overall incidence and potential differences between the valves remain unclear. Methods: A systematic literature search was performed in the databases Medline, Cochrane Library, and Embase including the clinical trials register. The aim was to summarize and compare the cumulative incidence of IE after PPVI. Using a sensitivity analysis we set the incidence rates of the two valve types in ratio with a normal population. Results: A total of 967 publications were identified searching for "pulmonary valve implantation," "PPVI," and 47 publications were used for final analysis. A total 3616 patients with Melody 1 valves and 501 with Sapien TM valves were included. IE after PPVI occurred in 214 patients with Melody 1 valves and in 5 patients only with Sapien TM valves. The pooled incidence for Melody 1 and Sapien TM valves was 4.9% (95% CI: 3.6-6.2) and 1.3% (95% CI: 0.3-2.3), respectively. Chi-square test was significant. The sensitivity analysis showed that the incidence rate ratio was 252.1 (95% CI: 187.6-338.6) for Melody 1 valves and for Sapien TM valves 2.7 (95% CI: 0.8-9.2). Conclusions: At present, there is an important difference for the risk of IE after PPVI. To reduce the risk of post PPVI endocarditis, a careful valve selection in favor of the Sapien TM valves seems to be beneficial.
ObjectiveThe aim of the current study is to measure long‐term executive function, motor outcome, and QoL in children, adolescents, and young adults after VAD and Htx.MethodsPatients were examined during routine follow‐up. Investigation tools were used as follows: Examination for MND of motor outcomes, Epitrack® for attention and executive functioning, and Kidscreen‐52 and EQ‐5D‐5L questionnaires for QoL. Additional data were retrospectively obtained by an analysis of patient medical records.ResultsOut of 145 heart transplant recipients at the department of pediatric cardiology of the University Hospital Munich, 39 were implanted with a VAD between 1992 and 2016. Seventeen (43.6%) patients died before or after Htx; 22 (56.4%) patients were included in our study. Mean age at transplant was 9.52 years (range: 0.58‐24.39 years, median 9), and the mean follow‐up time after Htx was 6.18 years (range: 0.05‐14.60 years, median 5.82). MND examination could be performed in 13 patients (normal MND: n = 11, simple MND: n = 1, complex MND: n = 1). Executive functioning was tested in 15 patients. Two (13.3%) patients had good results, six (40%) average results, three (20%) borderline results, and four (26.7%) impaired results. QoL (Kidscreen n = 7, EQ‐5D‐5L n = 8) was similar to a healthy German population.ConclusionMotor outcome, executive functioning and QoL in survivors of VAD bridging therapy and Htx can be good, though underlying diseases and therapies are associated with a high risk of cerebral ischemic or hemorrhagic complications.
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