Multifocal osteosarcoma: an unusual presentation

PURPOSE: Report the unusual presentation, clinical course, and cytogenetic abnormalities in a child with multifocal osteosarcoma. PATIENTS AND METHODS: A 10-year-old boy had multifocal osteosarcoma involving the entire skeleton, pleura, bone marrow, and lungs. He had marked anemia, thrombocytopenia, and severe hypocalcemia at diagnosis. RESULTS: Despite aggressive chemotherapy, he died from progressive disease 1 month after diagnosis. Cytogenetic analysis of tumor cells within the pleural fluid showed multiple chromosomal abnormalities with amplification of the c-myc oncogene. CONCLUSION: Multifocal osteosarcoma should be considered in the differential diagnosis of a child with pancytopenia and multiple bone lesions. Amplification of the c-myc oncogene may have had a significant role in the pathogenesis, etiology, and rapid progression of this patient's multifocal disease. Additional studies will be needed to determine the biologic significance of c-myc amplification in multifocal osteosarcoma.     

Polyglutamine diseases and transport problems: deadly traffic jams on neuronal highways

The expansion of CAG repeats encoding glutamine (polyQ) causes, to date, 9 late-onset progressive neurodegenerative disorders, including Huntington disease, spinobulbar muscular atrophy, dentatorubral-pallidoluysian atrophy, and spinocerebellar ataxias 1, 2, 3, 6, 7, and 17. Although many studies using both knockout and transgenic mouse models suggest that a toxic gain of function is central to neuronal dysfunction, the exact mechanisms of neurotoxic effects remain elusive. Protein aggregations within neurons seem to be a common manifestation in almost all polyQ diseases, and such accumulations are perhaps major triggers of cellular stress and neuronal death. Recent data lead to the tantalizing proposal that disruption of axonal transport pathways within long, narrow-caliber axons could lead to protein accumulations that can elicit neuronal death, ultimately causing a neuronal dysfunction pathway observed in polyQ expanded diseases. Perhaps perturbations in transport pathways are an early event involved in instigating polyQ disease pathology.     

Increased oxidative stress in younger as well as in older humans

BACKGROUND: The free radical theory of aging is based upon the adverse effects of oxidative stress (OS), and indices of OS generally increase with advancing age. However, since OS may also be a normal physiological response in youth, when reactive oxygen species (ROS) act as signal transducers during normal growth and development, we compared markers of OS in normal humans over a wide spectrum of different ages. METHODS: Fasting breath samples were collected from 102 healthy volunteers (age 9 to 89 years) and volatile organic compounds (VOCs) were assayed by gas chromatography and mass spectroscopy. The intensity of OS in each volunteer was estimated by the breath methylated alkane contour (BMAC), a three-dimensional display of the abundance of C4-C20 alkanes and monomethylated alkanes. The collective abundance of these VOCs in a breath sample was reduced to a single value, the volume under curve (VUC), and correlated with chronological age. RESULTS: Compared to subjects aged 20-40 years, the mean BMAC VUC was significantly increased in subjects aged < 20 (p < 0.0001) and >40 years (p < 0.001). A cubic function correlated BMAC VUC (x) with chronological age (y): y = 33.7 - 3.29x + 0.072x(2) - 0.0004x(3) (r = 0.48). CONCLUSIONS: Breath markers of OS were significantly increased both in younger and in older subjects, compared to those aged 20-40 years. Increased OS in older subjects was consistent with previous reports, but increased OS in younger subjects aged < 20 years is a new observation; this may be a normal physiological response in youth.     

Use of routinely collected past surveillance data in identifying and mapping high-risk areas in a malaria endemic area of Sri Lanka

Stratification of malaria endemic areas on eco-epidemiological criteria is an important step in planning and implementing malaria control programs. The uses of stratification of malaria endemic areas lead to better targeting of control measures such as residual insecticide spraying in countries where unstable malaria transmission occur. In this study, two methods that can be used for stratification of malaria endemic areas in Sri Lanka using routinely collected surveillance data over a period of 9 years are described. In the first method, the median Annual Parasite Incidence (API) was used as the criterion to classify an area as at risk for malaria while in the second method, the API and the Falciparum Rate (FR) were used as the criteria. Risk maps were produced by plotting the results of the analyses on maps generated by EPIMAP. The potential uses of risk maps are discussed.     

Responses of C fiber afferents of the rabbit airways and lungs to changes in extra-vascular fluid volume

Effects of changes in extra-vascular fluid volume produced by pulmonary lymphatic obstruction and plasmapheresis on the activities of bronchial and pulmonary C fiber receptors and rapidly adapting receptors (RARs) were investigated in New Zealand White rabbits. In intact rabbits, pulmonary lymphatic obstruction either alone or in combination with plasmapheresis did not stimulate pulmonary C fiber receptors. Only the combined stimulus activated the bronchial C fiber receptors. Bronchial C fiber receptors were also stimulated by graded increases in left atrial pressure (+5 and +10 mmHg). In contrast, RARs were activated by lymphatic obstruction either alone or in combination with plasmapheresis. These procedures increase the extra-vascular fluid volume in the carina and bronchi but not in the lungs (alveoli). In rabbits with chronic pulmonary venous congestion secondary to mitral valve damage, bronchial C fiber receptors were not stimulated by these increments in left atrial pressure which were insufficient to increase the extra vascular fluid content of the airways. However, both pulmonary and bronchial C fiber receptors were stimulated when the left atrial pressure was raised to 25 mmHg in these animals to cause pulmonary edema.     

Hepatic angiosarcoma in a child: Successful therapy with surgery and adjuvant chemotherapy

We report a 3-year and 11-month-old Caucasian female, who initially presented with an unresectable hepatic angiosarcoma. After three courses of chemotherapy with adriamycin/cisplatin, the tumor decreased in size considerably, allowing complete surgical resection. She also received postoperative chemotherapy with alternating cycles of ifosfamide/etoposide, cisplatinum/adriamycin, and vincristine/actinomycin D/cyclophosphamide for 18 months. She remains disease-free for greater than 44 months from the initial diagnosis. Our experience suggests that total excision of the tumor, together with an aggressive chemotherapy regimen, can improve the disease-free survival for children with this highly malignant vascular tumor of the liver. Med. Pediatr. Oncol. 28:139–143 © 1997 Wiley-Liss, Inc.     

Anthelmintic Prescribing Patterns of a Sample of General Practitioners from Selected Areas in the Colombo District of Sri Lanka

General Practitioners (GPs) provide first contact care of children and pregnant mothers in the community. This study ascertained the prescribing pattern of anthelmintics to children and pregnant women by a sample of GPs from the district of Colombo. Two hundred medical practitioners engaged in full-time General Practice (100 urban and 100 rural), were selected randomly. A pre-tested interviewer-administered questionnaire was used to collect data. A total of 183 GPs aged between 26 and 72 years (median 38) participated with 94 coming from urban areas. Seventy percent of the GPs were male. Almost 13% of GPs from urban areas had a Postgraduate degree in comparison to 4.5% from the rural areas (P < 0.05). Over 50% of GPs had 6-20 years of service and over 30% treated 16-30 patients daily. Seventy-three percent of GPs from rural areas accessed health-related reading material either daily or weekly in contrast to only 40% from urban areas (P < 0.001). All GPs prescribed anthelmintics to children. Pyrantel pamoate was the preferred anthelmintic used for children by both groups. Approximately 55% and 64% of GPs from urban and rural areas, respectively, prescribed anthelmintics during pregnancy. A majority of GPs prescribed drugs after the first trimester. However, 25% from urban areas gave drugs during any trimester (P < 0.001). Regression analysis revealed that GPs with postgraduate qualifications, those having frequent access to health-related material and those seeing more than 30 patients daily, prescribed anthelmintics to pregnant women more often. Although routine de-worming of pregnant women and children should occur through government antenatal and well-baby clinics, and through the schools de-worming programme, it may not happen due to various reasons. Thus, GPs play a vital role in achieving good coverage of anthelmintics among children and pregnant women. Making available clear national guidelines on prescribing anthelmintics in Sri Lanka would improve the prescribing patterns of anthelmintics among GPs.