Secondary PSF/TFE3-associated renal cell carcinoma in a child treated for genitourinary rhabdomyosarcoma

Xp11.2 translocation-associated renal cell carcinoma (RCC) is a rare tumor that accounts for at least one-third of childhood RCC. Different reports have emphasized that previous radio/chemotherapy might be involved in its pathogenesis. We describe a child who developed a t(X;1) (p11.2;p34) associated RCC after previous treatment for genitourinary rhabdomyosarcoma in infancy. The presence of the PSF-TFE3 fusion has only been described in a very limited number of cases. Our report expands the spectrum of tumors in which RCC can arise in the pediatric age group after chemotherapy.

Vitamin A deficiency among Brazilian school-aged children in a healthy child service

Background/Objectives: Vitamin A deficiency (VAD) is a world public health problem contributing to the increase in childhood morbidity and mortality in developing countries and severe deficiency of vitamin A may lead to xerophthalmia and blindness. The objective of this study was to determine the prevalence of VAD among Brazilian school-aged children attended at a primary health unit and to verify if some considered risk factor was associated with VAD in this group. Subjects/Methods: A descriptive prospective transverse study was conducted on 103 randomly selected children. A total of 54 boys and 49 girls aged 5.5-11 years had the relative dose-response (RDR) test performed on. Possible ocular alterations related to vitamin A and the status of anemia, serum zinc, some acute-phase proteins, and anthropometric situation were determinate by an analytic design. Results: No child presented xerophthalmia. Serum retinol values lower than 1.05 and 0.7 mu moll(-1), respectively were found in 26.2 and 5.8% of the children. The prevalence of hypovitaminosis detected by RDR test was 20.4%. The following variables and their relationship with VAD were evaluated: sex (P = 0.33; 95% confidence interval 0.61-4.34), weight and height (P >= 0.5), hemoglobin (P = 0.15), C-reactive protein (P = 0.56; 95% confidence interval 0.75-18.26), alpha-1-acid-glycoprotein (P = 0.56; 95% confidence interval 0.15-15.42) and serum zinc (P = 0.31). None of these variables was related to VAD. Conclusions: In this population, the prevalence of VAD detected could be considered a public health problem. School-aged children can be considered at risk for VAD mainly of a subclinical level, even without some associated risk factors.

Hepatoportal Sclerosis and Extrahepatic Portal Venous Obstruction Associated with Anti-phospholipid Antibody Syndrome in Child

We report a 2-year-old child with extrahepatic portal venous obstruction, hepatoportal sclerosis and pulmonary thromboembolism whose sole hypercoagulability factor was the presence of anti-phospholipid antibodies.

Effect of Acute and Chronic Physical Exercise on Patients with Periodic Leg Movements

ESTEVES, A. M., M. T. DE MELLO, M. PRADELLA-HALLINAN, and S. TUFIK. Effect of Acute and Chronic Physical Exercise on Patients with Periodic Leg Movements. Med. Sci. Sports Exerc., Vol. 41, No. 1,. pp. 237-242, 2009. Purpose: Nonpharmacological interventions may lead to an improvement in sleep quality. The objective of our study was to evaluate the effects of acute intensive exercise and chronic exercise on sleep patterns in patients with periodic leg movements (PLM). Methods: The study involved acute and chronic exercise. The acute intensive exercise group consisted of 22 volunteers who underwent a maximum effort test and a polysomnography (PSG) on the same night. The chronic exercise group included. 11 patients who performed 72 physical training sessions undergoing three PSG studies on the night of sessions 1, 36, and 72. Blood samples were collected from both acute and chronic groups for beta-endorphin dosage. Results: Our results showed that both forms of physical exercise lowered PLM levels. The acute physical exercise increased sleep efficiency, rapid eye movement (REM) sleep, and reduced wake after sleep onset, whereas the chronic physical exercise increased sleep efficiency, REM sleep, and reduced sleep latency. We also found a significant negative correlation between beta-endorphin release after acute intensive exercise and PLM levels (r = -0.63). Conclusion: Physical exercise may improve sleep patterns and reduce PLM levels. The correlation between beta-endorphin release after acute intensive exercise and PLM levels might be associated with the impact physical exercise has on the opiodergic system. We suggest that physical exercise may be a useful nonpharmacological treatment for PLM.

The effect of aerobic physical training on cardiac autonomic control of rats submitted to ovariectomy

Objective: To investigate the effect of aerobic physical training on cardiovascular autonomic control in ovariectomized rats using different approaches. Design: Female Wistar rats were divided into four groups: sedentary sham rats (group SSR), trained sham rats (group TSR), sedentary ovariectomized rats (group SOR), and trained ovariectomized rats (group TOR). Animals from the trained groups were submitted to a physical training protocol (swimming) for 12 weeks. Results: Pharmacological evaluation showed that animals from group TSR had an increase in their cardiac vagal tonus compared with the animals from groups SSR and SOR. The analysis of heart rate variability (HRV) showed that groups TSR and SOR had fewer low-frequency oscillations (0.20-0.75 Hz) compared with groups SSR and TOR. When groups TSR and SOR were compared, the former was found to have fewer oscillations. With regard to high-frequency oscillations (0.75-2.5 Hz), group SSR had a reduction compared with the other groups, whereas group TSR had the greatest oscillation compared with groups SOR and TOR, with all values expressed in normalized units. Analysis of HRV was performed after pharmacological blockade, and low-frequency oscillations were found to be predominantly sympathetic in sedentary animals, whereas there was no predominance in trained animals. Conclusion: Ovariectomy did not change the tonic autonomic control of the heart and, in addition, reduced the participation of sympathetic component in cardiac modulation. Physical training, on the other hand, increased the participation of parasympathetic modulation on the HRV, including ovariectomized rats.

The effect of ovariectomy on cardiac autonomic control in rats submitted to aerobic physical training

We have investigated the ovariectomy effects on the cardiovascular autonomic adaptations induced by aerobic physical training and the role played by nitric oxide (NO). Female Wistar rats (n =70) were divided into five groups: Sedentary Sham (SS): Trained Sham (TS); Trained Hypertensive Sham treated with N(C)-nitro-L-arginine methyl ester (L-NAME) (THS): Trained Ovariectomized (TO); and Trained Hypertensive Ovariectomized treated with L-NAME (THO). Trained groups were submitted to a physical training during 10 weeks. The cardiovascular autonomic control was investigated in all groups using different approaches: 1) pharmacological evaluation of autonomic tonus with methylatropine and propranolol; 2) analysis of heart rate (HR) and systolic arterial pressure (AP) variability; 3) spontaneous baroreflex sensitivity (BRS) evaluation. Hypertension was observed in THS and THO groups. Pharmacological analysis showed that TS group had increased predominance of autonomic vagal tonus compared to SS group. HR and intrinsic HR were found to be reduced in all trained animals. TS group, compared to other groups, showed a reduction in LF oscillations (LF=0.2-0.75 Hz) of pulse interval in both absolute and normalized units as well as an increase in HF oscillations (HF=0.75-2.50 Hz) in normalized unit. FIRS analysis showed that alpha-index was different between all groups. TS group presented the greatest value, followed by the TO, SS. THO and THS groups. Ovariectomy has negative effects on cardiac autonomic modulation in trained rats, which is characterized by an increase in the sympathetic autonomic modulation. These negative effects suggest NO deficiency. In contrast, the ovariectomy seems to have no effect on AP variability. (C) 2008 Elsevier B.V. All rights reserved.

Illicit Opiate Abuse

Illicit opiate use, especially injected drugs, contributes to premature mortality and morbidity in many developed and developing societies. The economic costs of illicit drug use are substantial. Fatal overdoses and HIV/AIDS resulting from sharing dirty needles and injecting equipment are major contributors to mortality and morbidity. Illicit opioid use accounted for 0.7 percent of global disability–adjusted life years in 2000. An estimated 15.3 million people, or 0.4 percent of the world population ages 15 to 64, used illicit opioids in 2002, with more than half using heroin and the rest using opium or diverted pharmaceuticals such as buprenorphine, methadone, or morphine. The most popular interventions for illicit opioid dependence in many developed societies have been law enforcement efforts to interdict the drug supply and enforce legal sanctions against drug use. One consequence has been that illicit opioid users have been exposed to the least effective intervention: imprisonment for drug or property offenses. The most effective intervention to reduce blood–borne virus infection resulting from illicit drug injections is provision of clean injecting equipment to users. This intervention has been widely supported in developed countries, but less so in developing countries. In addition, vaccinations are effective against hepatitis B. In treatment settings, the most popular interventions have been detoxification and drug–free treatment, which has proven the least productive in retaining opioid–dependent people in treatment. Opioid agonists have a niche role in treatment of opioid dependence, especially if their efficacy improves with development of long–acting injectable forms of the drug.

Low-grade astrocytoma in a child with encephalocraniocutaneous lipomatosis

Encephalocutaneous lipomatosis (ECCL), or Haberland syndrome, is an uncommon congenital disorder with unique cutaneous, ocular and neurological features. In the present article, we describe a 3-year-old boy with ECCL who developed an extensive and recurring intraventricular low-grade glioma with atypical pathological features and elevated mitotic index. Cytogenetic analysis from tumor sample was also performed. This is the first report of a low-grade astrocytoma occurring in a child with ECCL. Whether or not the origin of the tumor is associated to the pathogenesis of the underlying syndrome is a matter for further investigation.