Outcomes and Prognostic Factors for Non-metastatic Osteosarcoma of the Extremity

α1-antitrypsin deficiency (α1-ATD) is a genetic disease which is featured with accumulation of a1-antitrypsin mutant Z proteins (A1AT-Z) in the Endoplasmic Reticulum (ER) of liver [1-4]. It is caused by a substitution of lysine for glutamate at residue 342 and occurs in 1 out 1800-2000 live births in the North America [1]. The mutant Z protein is prone to adopt polymerized conformation and aggregates in ER of hepatocytes (insoluble aggregates) rather than secreted appropriately into the serum and body fluids (soluble forms), leading to chronic liver damage, fibrosis and cirrhosis, and even Hepatocellular Carcinoma (HCC). Transplantation is the only curative therapy for the patients with severe liver disease. It has been reported that hepatocytes cope with the burden of accumulated intracellular protein by activating proteasomal degradation pathways (ERAD) and macroautophagy (called autophagy hereafter).

Background: Non-Communicable Diseases (NCDs) have become quite prevalent worldwide. In Ghana as in other developing countries, NCDs are major public health concern. Even though modern health services have been sought by patients suffering from NCDs, poor attitude to those suffering from them exist in Ghanaian society because of traditional beliefs about the causes of such diseases. This paper examines parents and care givers’ perceived attitudes of Ghanaians towards NCDs among children.

Methods: The study was conducted among 700 participants in three regions (Greater Accra, Ashanti and Volta) of Ghana in both rural and urban areas, using a quantitative research instrument. All the major ethnic groups of Ghana- Akan, Ewe, Ga-Dagme and Mole-Dagbani- were captured in the three regions. The dependent variables are: 1) perceived embarrassment of having a child with non-communicable disease, 2) discrimination from close associates and 3) difficulty of taking a child with non-communicable to social gatherings. The independent variable is residential status (rural and urban) and the control variables are socio-demographic characteristics of the respondents.

Results: The findings indicate that the association between the background characteristics with perception of discrimination against families with children who have NCDs are significant according to the results of the Chi-Square test place of residence ( =10.678; p=0.005); level of education ( =19.389; p=0.036), and ethnic background ( =35.267; p=0.000). The results of the multinomial regression model show that those in rural areas have higher likelihood (92.0%) of perceiving that the family of a child with NCD will experience difficulty in going to social functions with the child compared with those in urban areas. Again, the rural dwellers are almost twice as likely (1.82%) to perceive that the family of a child with NCDs will feel embarrassed.

Conclusion: Perceived attitude of society towards children with NCDs is generally poor in the study population. Perceived discrimination against families of such children suggests that the children may not be availed for treatment even as it is indicated that the parents/care givers will have difficulty in taking them out to public places. Rural residents have higher likelihood of such attitudes Health policies need to address such attitudes in Ghanaian society and must target rural residents while not neglecting urban dwellers.

Inflammation is recognized as a pathophysiologic mechanism in obesity and associated chronic diseases such as asthma, diabetes, cardiovascular diseases. There are potential mechanisms for obesity-asthma link. A significant association between hepatitis B and asthma is detected. A synergistic interaction exists between obesity and hepatitis virus infection. Hepatotropic viruses and obesity, which is prevalent among patients with hepatitis affect lung function and respiratory system [1-5].

Hand hygiene is the single most important and preventable method to avoid nosocomial infections [1]. Hospital staffs are the major source in the transmission of nosocomial infections via organisms of their hands [2]. Out of 4 million neonatal deaths in world, one-third are associated with infections [1,2]. While in countries with high neonatal mortality (>45 per 1000 births), 50% of these deaths are attributed to infections [2]. Around 1.4 million people worldwide suffers from serious nosocomial infection [3]. In developing countries nosocomial infection rate varies from 6% to 27% [4].

Developments in empiric antibiotic therapy in recent decade shave led to a decrease in idiopathic primary peritonitis in neonates. Furthermore, intraperitoneal abscesses are extremely rare in neonates. We report a case of an intraperitoneal abscess without an apparent cause, such as necrotizing enterocolitis or surgery, in a preterm infant. The main contribution of our report is a reminder that intra-abdominal abscesses, although rare, can and do occur in neonates, and that diagnosing them can be difficult.

Angiotensin converting enzyme inhibitors are being increasingly used in those with functionally univentricular hearts with minimal evidence of their efficacy in this population. Additional medications, such as angiotensin converting enzyme inhibitors can be a practical inconvenience to patients, increase the cost of care, and may even be detrimental to these patients in some respects. This short communication reviews the current evidence for angiotensin converting enzyme inhibition in patients with functionally univentricular hearts.

Paediatricians with child protection responsibilities evaluate allegations of physical harm and sexual abuse which are subject to both child protection and criminal investigations. This evaluation requires the production of an interim medico-legal report which contributes to interim decisions regarding the safety of the child whilst investigations are still in progress. A second report may be needed at a later time once investigations are considered complete. The interim medico-legal report contributes an expert opinion about injury causation including direct harm or harm through neglect or substandard care which has relevance to mitigating future risk of harm and understanding the support needs of a family. This paper defines key principles in relation to the structure and content of an interim medico-legal report, which must conform to forensic standards of practice. A classification of injury causation for interim medico-legal reports is proposed which can be useful to systematically define outcomes for data collection, audit and research purposes.

Vitamin B 12 has important consequences for hematopoiesis and the central nervous system, its deficiency leads to clinical hematological, neurological and psychiatric manifestations. We report the case of a ten months old male infant, exclusively breastfed, with a significant delay in the developmental and communicative reactions. Physical examination confirmed the pallor, the axial hypotonia and revealed that the child was below the 2nd percentile for weight and the 3rd percentile for length. He had a hemoglobin level of 6.4g/dL with an MCV of 100.2fL, His serum vitamin B12 level was less than 50pg/mL and serum folate level 20.4ng/mL. No intrinsic factor antibodies were found. Hormonal and nutritional screening revealed no other deficiency. Thalassemia screening resulted negative. Maternal investigations showed anemia and maternal vitamin B12 deficiency. The research for anti-parietal antibodies of the stomach returns very positive. Vitamin B12 supplementation permitted with weight gain and rapid improvement of interactions and muscle tone.

Staphylococcal Scalded Skin Syndrome (SSSS) is an uncommon toxin-mediated disease which causes blistering and desquamation of the skin and induced by Staphylococcus aureus (S. aureus). SSSS in neonates is a rare condition and most commonly seen in children aged 6 months to 5 years, with the highest probability of disease being between 2-3 years. This report presents a 35-day-old infant who developed exfoliation and peeling of the skin after purulent conjunctivitis.

Reverse Shapiro syndrome consists of the association of unexplained hyperthermia and corpus callosum agenesis. The genetic basis is still unidentified; yet, neurochemical abnormalities as well as hypothalamic dysfunction have been proposed as possible etiologies. Only four cases of reverse Shapiro Syndrome have been reported to date and many therapeutic managements have been proposed without any radical success. Here we report a new case of a 6 months old male patient, with controlled cryptogenic epilepsy who is also presenting with a reverse Shapiro syndrome. The patient presented with recurrent episodes of hyperthermia that responded completely to a treatment by cyproheptadine.