Post-dural puncture headache-a single-centre analysis in paediatric patients with and without SMA.

AIM: To gather epidemiologic data on post-dural puncture headache (PDPH) after diagnostic or therapeutic lumbar puncture (LP) in children and adolescents with SMA as well as in a cohort of paediatric patients without SMA. METHODS: We performed a retrospective, single-centre analysis via chart review and questionnaire. Patients were identified using the German procedure classification. Respective charts and SMArtCARE documentation forms (SMA patients) were reviewed concerning documentation of headaches fulfilling criteria of the IHS-classification for PDPH of 2004. Non-SMA patients received additional questionnaires. RESULTS: We identified a total of 218 LPs in 95 patients. Of those 141 were performed in 22 patients with known SMA (mean age SMA patients 9.2 years; non-SMA patients 11.4 years). Following chart review, IHS criteria for PDPH were fulfilled in 6.9% of all procedures (3.5% in SMA patients; 13.0% in non-SMA patients; p = 0.008). Data from questionnaires of non-SMA patients confirmed this result (position dependent headache within 72 h after intervention in 13.0% of procedures). CONCLUSION: The prevalence of PDPH after therapeutic LPs in our cohort of SMA patients was significantly lower than after LPs in the general paediatric cohort. Data of this retrospective analysis show a similar overall prevalence of PDPH in paediatric patients as reported in bigger adult cohorts.

Coagulation disorders in Duchenne muscular dystrophy? Results of a registry-based online survey.

Different complications of hemostasis have been reported in patients with Duchenne Muscular Dystrophy (DMD). These comprise an increased rate of bleeding-symptoms during scoliosis surgery but also thromboembolic complications such as pulmonary embolism, cerebral infarction, deep vein thrombosis or cardiac thrombus. For this cross-sectional study, personalized online survey-links were forwarded to 682 registered patients with a genetically confirmed diagnosis of DMD via the German-Austrian DMD patient registry (www.dmd-register.de). The questionnaire enquired data regarding the degree of mobility, disposition to hematoma, epistaxis and gum bleeding, occurrence of peri- and postsurgical hemorrhage, stroke, deep vein thrombosis, and cardiac thromboembolism. Further data on regular medication and age were recorded. Three-hundred-fifty-one DMD-patients completed the questionnaire (response rate of 51.5%). Of those, 164 (46.7%) were ambulatory and 187 (53.3%) were non-ambulatory. Age distribution was homogeneous. Two participants had a history of thromboembolic events (0.6%). Correlations analysis revealed no coherence with the degree of mobility, age or regular medication. A bleeding tendency was reported by 76 participants (21.7%). No significant correlations with age or degree of mobility were found. We found no association with underlying genetic variants. Results of this patient registry-based survey do not indicate a distinct DMD-specific risk for thromboembolic events that exceeds the risk by typical comorbidities of chronic immobility and cardiac insufficiency in advanced stages of the disease. The results of this survey suggest a mild bleeding tendency in this DMD cohort, whereas a selection bias cannot be excluded.

TGF-beta and fibrosis in different organs - molecular pathway imprints.

The action of transforming-growth-factor (TGF)-beta following inflammatory responses is characterized by increased production of extracellular matrix (ECM) components, as well as mesenchymal cell proliferation, migration, and accumulation. Thus, TGF-beta is important for the induction of fibrosis often associated with chronic phases of inflammatory diseases. This common feature of TGF-related pathologies is observed in many different organs. Therefore, in addition to the description of the common TGF-beta-pathway, this review focuses on TGF-beta-related pathogenetic effects in different pathologies/organs, i. e., arthritis, diabetic nephropathy, colitis/Crohn's disease, radiation-induced fibrosis, and myocarditis (including their similarities and dissimilarities). However, TGF-beta exhibits both exacerbating and ameliorating features, depending on the phase of disease and the site of action. Due to its central role in severe fibrotic diseases, TGF-beta nevertheless remains an attractive therapeutic target, if targeted locally and during the fibrotic phase of disease.