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Pheochromocytomas (PHEOs) and paragangliomas (PGLs) are rare neuroendocrine tumors. About 30% or more of them are thought to be of inherited origin due to germ-line mutations in at least 10 well-characterized genes. There are data linking specific genotypes of these tumors to specific locations, typical biochemical phenotypes or future clinical behaviors. Conversely, clinical features, catecholamine production and immunohistochemistry evaluation can help with the proper order of genetic testing for PHEO and PGL. The identification of a germ-line mutation can lead to an early diagnosis, appropriate treatment, regular surveillance and better prognosis not only for the patient but also for their family members. Moreover, the latest discoveries in molecular pathogenesis of these tumors will provide an important basis for future personalized therapy. [ABSTRACT FROM AUTHOR]

An abstract of the article "The relation between brain natriuretic peptide (BNP) serum level echocardiographic Tei index and the degree of a diastolic function in patients with diastolic heart failure," by D. Karasek and colleagues is presented.

Abstract: More than 40% of renal allografts show chronic transplant nephropathy (CTN) early after renal transplantation. Cardiovascular disease is the leading cause of death in this population. Thus endothelial dysfunction represents an early angiopathy causing CTN and atherosclerosis. We sought to evaluate changes in endothelial dysfunction and vascular wall thickness during the first year posttransplantation as well as their association with HLA nondependent risk factors for CTN. At 3 and 52 weeks after grafting, we studied 25 patients without overt atherosclerotic disease and acute posttransplant complications for von Willebrand factor (vWF), plasminogen activator inhibitor-1 (PAI-1), tissue plasminogen activator (tPA), big endothelin-1 (ET-1), flow-mediated dilatation (FMD), intimal media thickness (IMT), serum total cholesterol (TC), and triglycerides (TAG). FMD and IMT at 52 weeks showed significant correlations (P < .05) with recipient age, and the FMD ratios at 3 and 52 weeks correlated with the time on hemodialysis. Recipient age was significantly correlated with TC and PAI-1 with TAG. vWF was the only parameter that significantly correlated with donor age. There were no significant correlations with creatinine clearance. Decreased TAG approached statistical significance (P = .07) and TC decreased nonsignificantly. The worsening of FMD and ET-1 was not significant. A nonsignificant improvement in IMT was not associated with any analyzed parameters. Our results implied that the time on hemodialysis, the presence of hyperlipoproteinemia, and the recipient age significantly contributed to endothelial dysfunction during the first year after transplantation. [Copyright &y& Elsevier]

Introduction. Thyroid tissue ectopically located in the ovary can be reported accidentally after adnexectomy, but as a primary cause of hyperthyroidism this diagnosis is rare. The clinical search for a functional ectopic thyroid tissue requires intense clinical focus and a multidisciplinary approach. Case Description. This case report demonstrates a patient with a history of Graves' disease who had undergone thyroidectomy combined with postoperative 131I radioablation. Despite the previous treatment, she developed an outburst of hyperthyroidism ten years later. Only very close follow-up enabled us to disclose the right condition. The ovarian source of thyroid hormone production was removed by laparoscopic adnexectomy and a right sided benign ovarian struma was confirmed. Conclusion. Most patients treated by thyroidectomy and radioiodine do not require extended periods of followup or postoperative investigations, but when the clinical or laboratory signs change, clinicians should be prepared to perform the necessary re-evaluation in order to provide the best care. [ABSTRACT FROM AUTHOR]

The skeletal growth in a course of acute lymphoblastic leukemia (ALL) may be affected, and the aim of the longitudinal study was to assess the skeletal status in survivors of (ALL). The studied population consisted of 38 subjects (17 female and 21 male) measured at the age of 13.9 ± 3.8 years (5.7 ± 2.9 years after completion of the therapy, 11.0 ± 14.4 years after diagnosis) and 2 years earlier; compared with 1402 controls (628 female and 774 male). Patients and controls did not differ significantly in regard to age, height or weight. Skeletal status was assessed by quantitative ultrasound (US) measurements at the hand phalanges using the DBM Sonic 1200, which measures amplitude-dependent speed-of-sound, Ad-SoS (m/s). rms CV% was 0.43%. Mean baseline Ad-SoS value in patients was 1990 ± 76 m/s and, at second measurement, 2045 ± 86 m/s (p < 0.000001). In 31 patients, Ad-SoS increased and, in one patient, decreased more than the value of the least significant change. In controls, mean Ad-SoS values were 1973 ± 64 m/s (baseline) and 2016 ± 86 m/s (follow-up) and did not differ significantly vs. baseline values in patients. At second measurement, Ad-SoS in controls was significantly lower than in patients (p < 0.05). In five patients with low baseline Ad-SoS values, bone mineral density (BMD) at the spine using DPX-L was estimated; baseline mean BMD was 0.95 ± 0.11 g/cm2, Z-score was 1.25 ± 0.97 and, at second measurement, 1.16 ± 0.07 g/cm2, Z-score was 0.23 ± 0.43. A significant increase in BMD (p < 0.01) and Z-score (p < 0.05) was noted. In patients, Ad-SoS correlated significantly with age, period after completion of the therapy, body size and Tanner stages (r ranged from 0.43 to 0.83, p ranged from 0.0001 to 0.05). It can be concluded that skeletal status assessed by quantitative US at the hand phalanges in survivors of ALL improved significantly over the period of observation. (E-mail: ) [Copyright &y& Elsevier]

Bone status was assessed in 15 children and adolescents with predialysis chronic renal failure (CRF) and in 25 subjects with end-stage renal failure (ESRF). The mean age in the whole group was 14.6±3.2 years and CRF had been recognized 5.8±4.0 years earlier. The mean age, body size, duration of the disease and Tanner stages did not differ significantly between patients with predialysis CRF and ESRF. The control group consisted of 890 healthy subjects matched with patients for age. Bone mineral density (BMD) was measured by DPX-L (Lunar, Madison, WI) at the spine (s-BMD) and total body (TB-BMD); quantitative ultrasound (QUS) was performed by DBM 1200 (IGEA, Italy) at the hand phalanges (Ad-SoS). Laboratory investigations included the evaluation of intact parathyroid hormone (i-PTH), total and ionized serum calcium, and serum phosphate. In the whole group of patients the following mean values were obtained: Ad-SoS 1952±79 m/s (significantly lower than in controls, who had Ad-SoS 2022±85 m/s, p<0.05; the difference remained significant after adjusting for body mass index), s-BMD 0.87±0.22 g/cm2 (Z-score –1.6), TB-BMD 0.92±0.12 g/cm2 (Z-score –1.44), i-PTH 276±300 pg/ml, total calcium 2.46±0.19 mmol/l, ionized calcium 1.14±0.08 mmol/l, phosphate 1.68±0.61 mmol/l. Skeletal measurements correlated significantly with age, body size and Tanner stages (also after adjusting for age), while significant correlations of these parameters with the duration of CRF and laboratory investigations (except of correlations of i-PTH with Ad-SoS and with TB-BMD in predialysis patients) were not observed. None of the studied variables differed significantly between predialysis and dialysis patients. In conclusion, both predialysis and dialysis children and adolescents showed a decrease in BMD and quantitative ultrasound measurements. The severity of skeletal alterations was similar in the early phase (predialysis patients) and end stage (dialysis patients) of the disease and did not show a tendency to progress with CRF duration. [ABSTRACT FROM AUTHOR]