Clinical and familial study of arrhythmogenic right ventricular cardiomyopathy

Objective　To explore the characteristics of arrhythmogenic right ventricular cardiomyopathy (ARVC). Methods　Seven patients with arrhythmogenic right ventricular cardiomyopathy and 34 members of three families were studied. All patients and family members underwent history collection, clinical examination, electrocardiogram (ECG), two-dimensional echocardiography (2-DE) and a signal averaging electrocardiogram. Programmed ventricular stimulation was performed in five patients. Results　All patients and family members had normal morphologic characteristics and normal function of the left ventricular by 2-DE. Fourteen persons had abnormal findings indicating ARVC. Five had enlargement of the right ventricular with diffused hypocontractility, eight had thin and systolic bulging in the focal anterior wall with hypokinesia and one had bulging of the inferior wall. Twenty-five persons (seven patients and 18 family members) had abnormal findings in ECG. Positive ventricular late potential was recorded in 13 persons (six patients). Two to three monomorphic ventricular tachycardia (VT) with left bundle branch block (LBBB) configurations were induced in five patients. Ventricular fibrillation was induced in two patients during the electrophysiologic study (EPS). Five patients had very high pacing threshold and/or ineffective pacing in one or many regions of the right ventricle. Two members of one family died suddenly. One member was a dwarf with ARVC. Spontaneous VT with a left bundle branch block (LBBB) configuration was recorded in five patients, polymorphic VT with extremely short coupling interval in one, and premature ventricular complexes with LBBB configuration in 12 (six patients). Conclusion　Our familial study strongly suggests that ARVC may be a hereditary disease and it is helpful in the diagnosis and detection of ARVC. The most common manifestations were abnormal structure and function of the right ventricle and abnormal ECG of repolarization and ventricular arrhythmia which originates from the right ventricle.     

兔口服炔诺酮肟和炔诺酮的药代动力学

本文用高效液相色谱分离、液闪测定放射性的方法,测定了兔口服炔诺酮肟(NETO)和炔诺酮(NET)的血浓,并比较了二者的药代动力学参数。结果表明:二者吸收迅速,从血中的消除均呈快慢两个时相。NETO在兔体内一部分迅速转变为NET,另一部分则以原药形式存在,24 h内NETO与其代谢产物NET在血清中的浓度大致各占一半。兔口服NETO与NET后,血浓—时间曲线符合二室模型,NETO的达峰时间比NET短,二者有显著差异(P<0.05),其它动力学参数无明显差异(P>0.05)。     

EMT, EndMT, PMT – Mechanismen der interstitiellen Fibrose

Interstitial fibrosis (IF) is a long-term result of inflammatory changes occurring in chronic organ tissue disorders. The replacement of inherent tissue structure with fibrotic tissue is accompanied by increasing loss of function. Only in recent years attention has been focussed on the molecular mechanisms of IF. Traditionally, intrinsic fibroblasts were viewed as the main mediators of renal fibrosis. Stem cells from the bone marrow, having migrated to the mesangium or insterstitium, were thought to participate in the development of such fibrosis. New studies however implicate tubular epithelial, endothelial cells and pericytes (perivascular fibroblasts) in fibrotic development. This article gives an overview of these three recently identified mechanisms of IF in the kidney.     

Postoperative altered plasma growth hormone levels--a predictor for postoperative complications? A case report.

We compared the onset of predictors for postoperative complications (lactate, total T2 (tT2), total T4 (tT4) and cortisone) retrospectively with the onset of altered growth hormone (GH) concentration in a patient who had had a lethal postoperative outcome and in 13 patients who were without postoperative complications for a period of 24 hours postoperatively. Compared with the values of the patients without postoperative complications, GH values were elevated (68-fold) 1 h after surgery to 103 ng/ml and lactate was increased (12-fold) to 12.7 mmol/l at 6 h postoperatively in the patient with the lethal outcome. The other parameters measured (tT3, tT4 and cortisone) showed no rapid alteration during the first hours postoperatively. This case report suggests that the rapid postoperative onset of raised GH concentration in plasma may be an earlier marker for postoperative complications than the 'established' predictors.     

Verapamil metabolism in distinct regions of the heart and in cultures of cardiomyocytes of adult rats.

A substantial number of drugs act either directly or indirectly on the heart, but surprisingly, little is known about drug oxidation in the heart. We therefore investigated the metabolism of the calcium antagonist verapamil in microsomal fractions isolated from the left and right ventricle of heart muscle and in primary cultures of cardiomyocytes of adult rats. Metabolism of verapamil proceeded predominantly with microsomal fractions isolated from the right ventricle of rat heart, and in liquid chromatographic-tandem mass spectrometry (LC-MS/MS) and LC-MS(3) experiments four metabolites (M1-M4) could be identified. Furthermore, the intermediate biotransformation products M5 to M8 could additionally be identified in cultures of primary cardiomyocytes, thus providing new insight into the mechanisms of the N-dealkylation and O-demethylation pathway of verapamil. We show metabolism of verapamil to be predominant in the right ventricle of the heart, and the data reported herein may explain metabolic inactivation and/or adverse drug reactions of certain cardiovascular drugs on the basis of tissue specific metabolism.     

Polyorchidism: case report and review of literature

Polyorchidism is a rare anomaly with approximately 70 cases reported in the literature. It may result from transverse division of the urogenital ridge, a hypothesis that best explains the anatomical features of the supernumerary testicle. The primary accompanying disorders and anomalies include maldescended testis, inguinal hernia and torsion. Malignancy has been reported in 3 cases. In the absence of any concomitant disorder and if testicular tumor can be ruled out by magnetic resonance imaging and ultrasonography, surgical exploration with biopsy is unnecessary.     

Zukunftsperspektiven der myokardialen Regeneration

Kardiovaskuläre Erkrankungen, v. a. koronare Herzerkrankungen, zählen zu den weltweit häufigsten Todesursachen. Trotz verbesserter medizinischer Versorgung sind Behandlungserfolge bis heute moderat, und die Sterblichkeitsrate ist, speziell im ersten Jahr nach einem Myokardinfarkt, sehr hoch. Da die Mehrzahl der Herzerkrankungen alterskorreliert ist und die Lebenserwartung in unserer Gesellschaft stetig steigt, ist in den nächsten Jahrzehnten mit einem weiteren drastischen Anstieg der Inzidenz koronarer Herzerkrankungen zu rechnen. Dies verlangt, dass die Anstrengungen zur Erforschung neuer, unkonventioneller Therapieansätze deutlich forciert werden müssen. Hauptgrund des Herzversagens ist ein massiver Verlust funktioneller Kardiomyozyten, z. B. bedingt durch Infarkte oder chronisch erhöhte Druckbelastung des Herzens. Da Kardiomyozyten jedoch nur über ein extrem niedriges Regenerationspotenzial verfügen, kann dieser Verlust nicht kompensiert werden. Trotz intensiver Forschung auf dem Gebiet der regenerativen Medizin mit dem Ziel, die endogene Herzregeneration anzuregen oder verlorenes Herzgewebe durch Applikation von Stammzellen und Kardiomyozyten auszugleichen, ist bis zum heutigen Tag eine Herztransplantation die einzige nachhaltige Option, Kardiomyozyten zu ersetzen. Dieser Übersichtsbeitrag präsentiert den derzeitigen Stand regenerativer Strategien und diskutiert deren Vorteile, Einschränkungen und Zukunftsperspektiven im Vergleich zu konventionellen Behandlungsmethoden.     

Non‐invasive assessment of kidney allograft fibrosis with shear wave elastography: A radiological‐pathological correlation analysis

Objectives

To evaluate the use of shear wave elastography in assessment of kidney allograft tubulointerstitial fibrosis.

Methods

Shear wave elastography assessment was carried out by two independent operators in kidney transplant recipients who underwent allograft biopsy for clinical indications (i.e. rising creatinine >15% or proteinuria >1 g/day). Allograft biopsies were interpreted by the same pathologist according to the 2013 Banff Classification.

Results

A total of 40 elastography scans were carried out (median creatinine 172.5 μmol/L [interquartile range 133.8–281.8 μmol/L]). Median tissue stiffness at the cortex (22.6 kPa [interquartile range 18.8–25.7 kPa] vs 22.3 kPa [interquartile range 19.0–26.5 kPa], P = 0.70) and medulla (15.0 kPa [interquartile range 13.7–18.0 kPa] vs 15.6 kPa [interquartile range 14.4–18.2 kPa]) showed no significant differences between the two observers. Interobserver agreement was satisfactory (intraclass correlation coefficient of the cortex 0.84, 95% CI 0.70–0.92 and intraclass correlation coefficient of the medulla 0.88, 95% CI 0.78–0.94). The areas under the receiver operating characteristic curves for detection of tubulointerstitial fibrosis were estimated to be 0.75 (95% CI 0.61–0.89), 0.85 (95% CI 0.75–0.95) and 0.65 (95% CI 0.53–0.78) for cortical, medullary tissue stiffness and serum creatinine, respectively.

Conclusions

Shear wave elastography can be used as a non‐invasive tool to evaluate kidney allograft fibrosis with reasonable interobserver agreement and superior test performance to serum creatinine in detecting early tubulointerstitial fibrosis.     

A useful alternative surgical technique to reconstructing large defects following excision of recurrent pilonidal sinus disease in the intergluteal region: An operative approach for the transverse lumbar artery perforator flap

The reconstruction of defects in the intergluteal region following pilonidal sinus excision is challenging due to its anatomical location, close proximity to the anus, and being a high‐tension area prone to wound‐healing problems. Excision and primary closure is known to carry a higher risk of recurrence and subsequent complications compared with using nearby local healthy tissue, such as a flap, to reconstruct defect. Extra due diligence should be given to patient selection and flap choice when deciding the reconstruction of a defect. The senior author, who has briefly reviewed complication rates in previous published literature, prefers the transverse lumbar artery perforator (TLAP) flap for reconstruction following pilonidal sinus excision in the intergluteal region. This paper illustrates the operative approach used by the senior author when raising a TLAP flap.