Osteopoikilosis coexistent with ankylosing spondylitis and familial Mediterranean fever

Osteopoikilosis (OPK) is a rare benign sclerosing disease of the skeleton and inherited as an autosomal dominant trait. OPK is associated with inflammatory rheumatic disorders, such as rheumatoid arthritis, scleroderma, reactive arthritis and familial Mediterranean fever (FMF). We report a rare case of OPK coexistent with ankylosing spondylitis and FMF. The patient presented multiple sclerotic lesions within and around the sacroiliac joints and a series of radiological diagnostic challenges.     

A Synthetic Analogue of 20‐HETE, 5,14‐HEDGE,Reverses Endotoxin‐Induced Hypotension via Increased 20‐HETE Levels Associated with Decreased iNOS Protein Expression and Vasodilator Prostanoid Production in Rats

Abstract: Nitric oxide (NO) produced by inducible NO synthase (iNOS) is responsible for endotoxin (ET)‐induced hypotension and vascular hyporeactivity and plays a major contributory role in the multiorgan failure. Endotoxic shock is also associated with an increase in vasodilator prostanoids as well as a decrease in endothelial NO synthase (eNOS) and cytochrome P450 4A protein expression, and production of a vasoconstrictor arachidonic acid product, 20‐hydroxyeicosatetraenoic acid (20‐HETE). The aim of this study was to investigate the effects of a synthetic analogue of 20‐HETE, N‐[20‐hydroxyeicosa‐5(Z),14(Z)‐dienoyl]glycine (5,14‐HEDGE), on the ET‐induced changes in eNOS, iNOS and heat shock protein 90 (hsp90) expression as well as 20‐HETE and vasodilator prostanoid (6‐keto‐PGF_1α and PGE₂) production. ET‐induced fall in blood pressure and rise in heart rate were associated with an increase in iNOS protein expression and a decrease in eNOS protein expression in heart, thoracic aorta, kidney and superior mesenteric artery. ET did not change hsp90 protein expression in the tissues. ET‐induced changes in eNOS and iNOS protein expression were associated with increased 6‐keto‐PGF_1α and PGE₂ levels and a decrease in 20‐HETE levels, in the serum and kidney. These effects of ET on the iNOS protein expression and 6‐keto‐PGF_1α, PGE₂ and 20‐HETE levels were prevented by 5,14‐HEDGE. Furthermore, a competitive antagonist of vasoconstrictor effects of 20‐HETE, 20‐hydroxyeicosa‐6(Z),15(Z)‐dienoic acid, prevented the effects of 5,14‐HEDGE on the ET‐induced changes in systemic and renal levels of these prostanoids and 20‐HETE. These data are consistent with the view that an increase in systemic and renal 20‐HETE levels associated with a decrease in iNOS protein expression and vasodilator prostanoid production contributes to the effect of 5,14‐HEDGE to prevent the hypotension during rat endotoxemia.     

RalA signaling pathway as a therapeutic target in hepatocellular carcinoma (HCC)

Ral (Ras like) leads an important proto‐oncogenic signaling pathway down‐stream of Ras. In this work, RalA was found to be significantly overactivated in hepatocellular carcinoma (HCC) cells and tissues as compared to non‐malignant samples. Other elements of RalA pathway such as RalBP1 and RalGDS were also expressed at higher levels in malignant samples. Inhibition of RalA by gene‐specific silencing caused a robust decrease in the viability and invasiveness of HCC cells. Additionally, the use of geranyl–geranyl transferase inhibitor (GGTI, an inhibitor of Ral activation) and Aurora kinase inhibitor II resulted in a significant decrease in the proliferation of HCC cells. Furthermore, RalA activation was found to be at a higher level of activation in HCC stem cells that express CD133. Transgenic mouse model for HCC (FXR‐Knockout) also revealed an elevated level of RalA‐GTP in the liver tumors as compared to background animals. Finally, subcutaneous mouse model for HCC confirmed effectiveness of inhibition of aurora kinase/RalA pathway in reducing the tumorigenesis of HCC cells in vivo. In conclusion, RalA overactivation is an important determinant of malignant phenotype in differentiated and stem cells of HCC and can be considered as a target for therapeutic intervention.     

Trigeminal nerve involvement in T-cell acute lymphoblastic leukemia: value of MR imaging

A 30-year-old male with T-cell acute lymphoblastic leukemia presented with facial numbness. Neurological examination revealed paresthesia of the left trigeminal nerve. Cerebrospinal fluid (CSF) cytology showed no atypical cells. Gadolinium-enhanced magnetic resonance (MR) imaging demonstrated enlargement and enhancement of intracranial portions of the left trigeminal nerve. The abnormal MR imaging findings almost completely resolved after the chemotherapy. Gadolinium-enhanced MR imaging is not only a useful procedure for the early diagnosis of cranial nerve invasion by leukemia but it might be helpful to follow the changes after the treatment.     

Augmented surgical amounts for intermittent exotropia to prevent recurrence

Purpose:

The purpose was to evaluate the results of bilateral lateral rectus (BLR) recession which is based on augmented surgical amounts of classical surgical table of Parks’ for basic and pseudo-divergence excess type intermittent exotropia [X(T)].

Materials and Methods:

Patients with X(T) operated by the same surgeon and followed-up for at least 6 months were included. Patients with prior surgery, neurobehavioral and musculoskeletal conditions, strabismus different from that mentioned above X(T) were excluded. All the patients received BLR only. The amount of the recession was increased by the amount needed to correct 5 prism diopters (PD) more X(T) than what was measured. After the operation, 1^st week, 2^nd and 6 months measurements were recorded. The patients were grouped according to their 1^st week (3–7 days) postoperative examination as: >10 PD esotropia (Group 1), ≤10 PD esotropia (Group 2), exotropia (Group 3), and orthotropic (Group 4), respectively. Final surgical outcomes were classified as “good” (≤10 PD exotropia and ≤5 PD esotropia), “recurrence” (>10 PD exotropia) and “overcorrected” (>5 esotropia).

Results:

Thirty-seven patients were included. The mean age was 6.78 ± 2.87 years (range: 2–12 years). Mean preoperative deviation was 29.72 ± 8.07 PD (range: 15–45 PD) at distance and 20.94 ± 11.65 PD (range: 10–45 PD) at near (P < 0.0001). There were 21 (56.8%) patients in Group 1, 9 (24.3%) patients in Group 2, 1 (2.7%) patient in Group 3 and 6 (16.2%) patients in Group 4. Initial esotropia was achieved in 30 (30/37) of the patients. Twenty-eight of them had good results at the end of the 6 months. Overall “motor surgical” success rate was found to be 89.2% (33/37 patients), with 1 (2.7%) overcorrection and 3 (8.1%) recurrences at the end of the 6 months.

Conclusion:

This study demonstrated that early overcorrection of 10–20 PD after X(T) surgery can achieve acceptable motor outcomes in the first 6 months postoperative period.     

A slip connecting the peroneus longus and tibialis posterior tendons at the forefoot: MRI, anatomic, and histologic findings in a cadaver

The anatomy of the peroneus longus and tibialis posterior tendons is well described in literature from both anatomy and radiology. Though a slip connecting these two structures is described in the anatomic literature, its existence has not been confirmed with magnetic resonance imaging (MRI). In this study in a cadaver, such a connection is documented using high-resolution MRI with anatomic and histologic correlation. This connection can provide support to the Lisfranc joint complex and further stabilize the region of the first and second metatarsal bases.     

Conceptual models for Mental Distress among HIV-infected and uninfected individuals: A contribution to clinical practice and research in primary-health-care centers in Zambia

Background  

Mental distress is common in primary care and overrepresented among Human Immunodeficiency virus (HIV)-infected individuals, but access to effective treatment is limited, particularly in developing countries. Explanatory models (EM) are contextualised explanations of illnesses and treatments framed within a given society and are important in understanding an individual's perspective on the illness. Although individual variations are important in determining help-seeking and treatment behaviour patterns, the ability to cope with an illness and quality of life, the role of explanatory models in shaping treatment preferences is undervalued. The aim was to identify explanatory models employed by HIV-infected and uninfected individuals and to compare them with those employed by local health care providers. Furthermore, we aimed to build a theoretical model linking the perception of mental distress to treatment preferences and coping mechanisms.     

The ethical basis for promoting nutritional health in public schools in the United States

Schools may have an ethical obligation to act in response to the precipitous increase in the incidence of obesity among children. Using a bioethics framework, we present a rationale for school programs to improve the nutritional quality of students' diets. Because children are required to spend half their waking hours in school and because they consume a substantial portion of their daily food there, school is a logical focus for efforts to encourage healthy dietary behaviors to prevent obesity and its consequent individual and collective costs. We suggest that beyond strategic considerations, the concept of the common good justifies actions that may appear to conflict with freedom of choice of children, parents, and school staff, or with the interests of food and beverage companies.