Influence of Lateral Sitting Wedges on the Rasterstereographically Measured Scoliosis Angle in Patients Aged 10-18 Years with Adolescent Idiopathic Scoliosis.

Adolescent idiopathic scoliosis (AIS) is a three-dimensional axial deviation of the spine diagnosed in adolescence. Despite a long daily sitting duration, there are no studies on whether scoliosis can be positively influenced by sitting on a seat wedge. For the prospective study, 99 patients with AIS were measured with the DIERS formetric III 4D average, in a standing position, on a level seat and with three differently inclined seat wedges (3°, 6° and 9°). The rasterstereographic parameters 'scoliosis angle' and 'lateral deviation RMS' were analysed. The side (ipsilateral/contralateral) on which the optimal correcting wedge was located in relation to the lumbar/thoraco-lumbar convexity was investigated. It was found that the greatest possible correction of scoliosis occurred with a clustering in wedges with an elevation on the ipsilateral side of the convexity. This clustering was significantly different from a uniform distribution (p < 0.001; chi-square = 35.697 (scoliosis angle); chi-square = 54.727 (lateral deviation RMS)). It should be taken into account that the effect of lateral seat wedges differs for individual types of scoliosis and degrees of severity. The possibility of having a positive effect on scoliosis while sitting holds great potential, which is worth investigating in follow-up studies.

Continuous cardiac troponin I release in Fabry disease.

BACKGROUND: Fabry disease (FD) is a rare lysosomal storage disorder also affecting the heart. The aims of this study were to determine the frequency of cardiac troponin I (cTNI) elevation, a sensitive parameter reflecting myocardial damage, in a smaller cohort of FD-patients, and to analyze whether persistent cTNI can be a suitable biomarker to assess cardiac dysfunction in FD. METHODS: cTNI values were determined at least twice per year in 14 FD-patients (6 males and 8 females) regularly followed-up in our centre. The data were related to other parameters of heart function including cardiac magnetic resonance imaging (cMRI). RESULTS: Three patients (21%) without specific vascular risk factors other than FD had persistent cTNI-elevations (range 0.05-0.71 ng/ml, normal: <0.01). cMRI disclosed late gadolinium enhancement (LGE) in all three individuals with cTNI values ≥0.01, while none of the 11 patients with cTNI <0.01 showed a pathological enhancement (p<0.01). Two subjects with increased cTNI-values underwent coronary angiography, excluding relevant stenoses. A myocardial biopsy performed in one during this procedure demonstrated substantial accumulation of globotriaosylceramide (Gb3) in cardiomyocytes. CONCLUSION: Continuous cTNI elevation seems to occur in a substantial proportion of patients with FD. The high accordance with LGE, reflecting cardiac dysfunction, suggests that cTNI-elevation can be a useful laboratory parameter for assessing myocardial damage in FD.

Cardiac Troponin I elevation after epileptic seizure.

BACKGROUND: Cardiac troponin-I (cTNI) is highly specific biomarker to prove myocardial damage, e.g. in acute coronary syndrome (ACS). However, it occurs in other conditions as well. We therefore analysed cTNI increase in patients after generalized convulsive seizure. METHODS: Consecutive patients admitted with acute generalized convulsive seizure were included in case of cTNI measurement on admission. Among 898 selected cases, 53 patients were referred secondary to our department; in 845 cases cTNI measurements on admission were available. In case of multiple admissions (81 cases), only the first admission entered our analysis. In 17 patients elevated cTNI was determined due to ACS; in one patient a myocarditis was found. 5 patients suffered of relevant renal insufficiency. Finally 741 patients were included in the analysis. A cTNI cut-off level of ≥ 0.1 ng/ml was considered. Factors associated with a cTNI increase were analysed subsequently. RESULTS: The mean age of the study population (n = 741) was 47.8 years (SD ± 18.6), 40.9% were female. In 50 patients (6.7%) a cTNI elevation of unknown origin was found; no obvious cardiac involvement could be detected in these patients who all remained asymptomatic. A vascular risk profile (including at least hypertension, hypercholesterolemia or diabetes) (OR = 3.62; CI: 1.59 to 8.21; p = 0.001) and elevated creatine kinase on admission (OR = 2.36; CI: 1.26 to 4.39; p = 0.002) were independent factors associated with cTNI release. CONCLUSION: cTNI release occurs in patients with generalized convulsive seizure with predominance in patients with vascular risk profile.

Dynamic hyperinflation during exercise in patients with precapillary pulmonary hypertension.

BACKGROUND: Patients with pulmonary arterial hypertension often present with a mild obstructive lung pattern, however, the functional consequences are not known. METHODS: We analysed flow volume loops during exercise in 61 patients with precapillary pulmonary hypertension (PH) (age 55 ± 14 years) in comparison with 21 patients with COPD (60 ± 12 years), 39 patients with pulmonary fibrosis (58 ± 11 years) and 38 healthy controls (HC) (39 ± 15 years). Inspiratory capacity (IC) was measured at rest, and during maximum exercise (max). RESULTS: HC exhibited a stable IC of 3.0 ± 0.9 l at rest, and at max. A reduction in IC of 2.6 ± 0.8 l at rest to 2.0 ± 0.7 l at max was observed in patients with COPD. Patients with PH exhibited a significant reduction in IC from 2.3 ± 0.6 l at rest to 2.1 ± 0.6 l at max, while patients with pulmonary fibrosis exhibited a stable IC of 1.8 ± 0.6 at rest and 1.7 ± 0.6 l at max. In patients with PH, a weak negative correlation was drawn between the change in IC (%) and peak VO2 (r = -0.29, p = 0.01), as well as with PVR (r = -0.27, p = 0.02). CONCLUSION: Patients with PH demonstrate a characteristic change in IC during exercise, which might contribute to impaired exercise tolerance.

Persistent increase in cardiac troponin I in Fabry disease: a case report.

BACKGROUND: Hypertrophic cardiomyopathy is a frequent manifestation in Fabry disease (FD) - an X-linked lysosomal storage disorder caused by reduced activity of the enzyme α-galactosidase A. In FD an elevation of specific cardiac biomarkers, such as cardiac troponin I (cTNI) has been reported in case of clinical manifestation suggestive of myocardial ischemia. In diagnosing acute myocardial infarction cTNI is considered the most reliable parameter. CASE PRESENTATION: In the referred case we present a 59 years old female patient with the diagnosis of FD presenting with persistently increased cTNI level (lowest value 0.46 ng/ml, highest value 0.69 ng/ml; normal range <0.05 ng/ml) over a period of 5 months lacking cardiac clinical signs. Since renal insufficiency did not explain the degree of cTNI elevation, this was interpreted as a result of cardiac involvement in FD. Cardiac MRI showed marked left ventricular hypertrophy and focal late Gadolinium enhancement. CONCLUSIONS: Our case report demonstrates a persistent cTNI release in FD with cardiac involvement. Proving the persistence in a symptom free interval, it might be related to a direct damage of myocytes. In FD cTNI could serve as a beneficial long term parameter providing new perspectives for screening strategies.

Mycophenolate mofetil-based immunosuppression and cytokine genotypes: effects on monokine secretion and antigen presentation in long-term renal transplant recipients.

BACKGROUND: It has been suggested that increased monocyte responses might play a role in chronic allograft rejection. METHODS: We investigated in vitro monokine responses in 112 patients with long-term stable kidney graft function (ST patients; n=80, non-mycophenolate mofetil [MMF]; n=32, MMF) and 25 patients with chronic renal transplant rejection (CR patients; non-MMF). Interleukin 10 and tumor necrosis factor (TNF)-alpha promoter gene polymorphisms were tested by polymerase chain reaction and sequence-specific primers; antigen-presenting capacity (AC) of monocytes was tested by incubation with staphylococcal superantigens (SEA, SEE, SED). RESULTS: Although non-MMF-based immunosuppression in ST patients did not result in compromised AC or lipopolysaccharide (LPS)-stimulated monokine responses compared with healthy controls, we found MMF therapy to be associated with significantly reduced TNF-R1 expression on monocytes (P<0.001), suppressed AC (P<0.02, SED), and suppressed LPS-stimulated IL-1 beta, IL-10, and TNF-alpha secretion (P<0.01). Coinciding with a significantly higher steroid dosage in CR patients, IL-6 receptor and TNF-R1 expression on monocytes were down-regulated (P< or =0.02) and AC was suppressed in CR compared with ST (non-MMF) patients (P<0.01, SED; P<0.05, SEE). However, LPS-stimulated monokine secretion was not decreased or even enhanced (IL-6, granulocyte-macrophage colony-stimulating factor [GM-CSF]; P<0.05). Enhanced in vitro IL-10 responses (>500 pg/mL) were found predominantly in non-MMF-treated patients with the IL-10 genotype GCC (GCC: 23/62 [37%], non-GCC: 2/27 [7%], P<0.005; GCC and non-MMF: 22/47 [47%], GCC and MMF: 1/15 [7%], P<0.005]. CONCLUSION: Steroids and azathioprine did not sufficiently suppress monokine responses, whereas MMF treatment might inhibit chronic graft rejection because of suppression of TNF-R1 expression and vigorous inhibition of monokine secretion. MMF treatment may especially be indicated in patients with the IL-10 "high-producer" genotype GCC.

The effect of ATG on cytokine and cytotoxic T-lymphocyte gene expression in renal allograft recipients during the early post-transplant period.

BACKGROUND: Despite the long history of ATG use, the exact in vivo mechanism of action remains unclear. In the present study, we analyzed the effect of ATG-induction therapy on expression of 10 immunologically relevant genes in the early post-transplant period. METHODS: Eight renal allograft recipients received post-transplant prophylactic ATG treatment on 10 consecutive days and an additional three patients received treatment on 5, 6, or 7 consecutive days, respectively. Gene expression was measured at the beginning and the end of therapy and normalized to a control gene using Taqman real-time PCR methodology. Results were compared with those of matched control patients. No patients were diagnosed with rejection. RESULTS: ATG-treated patients showed decreases in the expression of cytotoxic T cell genes perforin (-56%, p = 0.03) and granzyme B (-45%, p = 0.01) and cytokine gene IFN-gamma (-75%, p = 0.005), and significant increases in the expression of cytokine genes IL-7 (550%, p = 0.04), IL-10 (275%, p = 0.01), IL-15 (417%, p = 0.03), TNF-alpha (615%, p = 0.01), and TGF-beta (235%, p = 0.02). No significant changes were observed in the control group, with the exception of a decrease in IL-10 expression (-42%, p = 0.01). There were no significant changes in IL-12 or Fas-L expression in either group. CONCLUSION: ATG-induced decreases in the expression of IFN-gamma, perforin, and granzyme B and increases in IL-10 and TGF-beta might be considered beneficial to the recipient, whereas increases in the expression of IL-7, IL-15, and TNF-alpha genes might be involved in immunological processes not effected by ATG that may harm the transplant in the long term.