Orthopedic management of the extremities in patients with Morquio A syndrome

Background

Musculoskeletal involvement in Morquio A syndrome (mucopolysaccharidosis IVA; MPS IVA) contributes significantly to morbidity and mortality. While the spinal manifestations of the disorder have received considerable attention in the literature, there have been few reported studies to date to guide the management of the orthopedic problems associated with the lower and upper extremities.

Purpose

The objective was to develop recommendations for the management of the extremities in patients with Morquio A syndrome.

Methods

A group of specialists in orthopedics, pediatrics and genetics with experience in the management of Morquio A patients convened to review and discuss current clinical practices and to develop preliminary recommendations. Evidence from the literature was retrieved. Recommendations were further refined until consensus was reached.

Results and conclusions

This present article provides a detailed review and discussion of the lower and upper extremity deformities in Morquio A syndrome and presents recommendations for the assessment and treatment of these complications. Key issues, including the importance of early diagnosis and the implications of medical therapy, are also addressed. The recommendations herein represent an attempt to develop a uniform and practical approach to managing patients with Morquio A syndrome and improving their outcomes.     

Post-coital Contraceptive Action of Pueraria tuberosaDC. Extract in Rats

Biochemical and physiological alterations have been observed in the ovary and uterus of pregnant rats treated with an extract of Pueraria tuberosa DC. Its administration from day 1 to 5 post-coitally caused a significant increase in the wet weight, protein and glycogen contents. Activity of acid phosphatase and ATPase was depleted sharply on days 4 and 5, but the activity of alkaline phosphatase was elevated on days 4 and 5. Ovaries of treated rats showed an increased number of follicles of different age. Cells of the corpora lutea showed hypertrophy. Uterine histoarchitecture appeared nonreceptive on the day of implantation. No deciduoma was observed on day 5. These changes have been correlated with the antiimplantation action of the butanol extract.     

Antilipid Peroxidative Property of Shilajit

We have investigated the effect of shilajit on lipid peroxidation and glutathione content in rat liver homogenate. It inhibited lipid peroxidation induced by cumene hydroperoxide and ADP/Fe⁺⁺ complex in a dose dependent manner. It also reduced the rate of oxidation of reduced glutathione content and inhibited ongoing lipid peroxidation, induced by these agents immediately after its addition to the incubation system.     

Antifeedant Activity of Ursolic Acid Isolated from Duboisia Myoporoides

Ursolic acid isolated from the leaves and stems of Duboisia myoporoides (Solanaceae) was bioassayed by leaf disc method for feeding deterrence using Spilosoma obliqua and Spodoptera litura as test insects. This compound was proved to be a potent antifeedant under laboratory conditions. Azadirachtin was used as standard. Ursolic acid produced 90.12% and 91.96% inhibition at 5000 ppm concentration, respectively, against S. obliqua and S. litura .     

Protective Action of Butanolic Extract of Pueraria tuberosa DC. Against Carbon Tetrachloride Induced Hepatotoxicity in Adult Rats

Administration of CCl₄ at a dose of 0.1 mL/kg/day for 7 days to normal rats provoked significant alterations in the various biochemical variables. It also caused severe histopathological lesions. Priming with the butanol extract of P. tuberosa or conjoint administration of extract along with CCl₄, provided significant protection against CCl₄ induced injury.     

Comparison of the Seventh and Eighth Edition of American Joint Committee on Cancer (AJCC) Staging for Selected and Nonselected Oropharyngeal Squamous Cell Carcinomas

ObjectivesThe eighth edition American Joint Committee on Cancer (AJCC) Staging incorporates significant changes to the seventh edition in the staging of oropharyngeal squamous cell carcinomas (OPSCC). An important change was the inclusion of OPSCC associated with the human papilloma virus (HPV). Our goal is to compare the performance of both staging systems for patients with HPV-selected and unselected clinical characteristics for OPSCC.MethodsUsing the Surveillance, Epidemiology, and End Results (SEER) database, 2004-2016, we identified patients with likely HPV-associated OPSCC based on surrogate markers (white males aged <65 years old with squamous cell carcinomas of the tonsil and base of tongue), excluding those who underwent surgery. We re-classified these patients using seventh and eighth edition staging for HPV-selected OPSCC and compared the prediction performance of both staging editions for overall survival (OS) and disease-specific survival (DSS). We performed the same analysis for clinically unselected patients with OPSCC.ResultsOur analysis included 9554 patients with a median follow-up of 67 months. Comparing the eighth versus seventh edition for our HPV-selected cohort, clinical staging changed for 92.3% of patients and 10-year OS was 62.2%, 61.2%, 35.3%, and 15.5% for Stage I, II, III, and IV, versus 52.9%, 59.2%, 61.6%, 55.1%, 38.3%, and 15.5% for stage I, II, III, IVA, IVB, and IVC, respectively. A similar pattern was observed for 10-year DSS. The concordance statistics for our HPV-selected cohort were improved for both AJCC 7 (0.6260) and AJCC 8 (0.6846) compared with the unselected cohort, 0.5860 and 0.6457 for AJCC 7 and 8, respectively.ConclusionThe overall performance of discrimination improved from AJCC 7 to AJCC 8 for both clinically selected and unselected patients, but more notably for our HPV-selected cohort. Despite the lack of statistically significant differentiation between Stages I and II in AJCC 8 in either groups, markedly improved discrimination was observed between Stages I/II, III, and IV in the HPV-selected cohort.     

Inferior vena cava syndrome in neonates: An evidence‐based systematic review of the literature

Neonatal inferior vena cava syndrome (IVCS), though uncommon, is associated with significant morbidity and mortality. Information on risk factors, diagnosis, treatment, and outcomes is limited. This review comprised 61 neonates across 33 reports. Thrombosis occurred in 98% and 42% involved a central venous catheter. Diagnosis was mainly established by ultrasound in 82%. Therapeutically, heparin was employed in 36% and thrombolysis in 18% of the cases. The overall mortality was 23%. An algorithm of clinical signs, investigation, and management is presented. Well‐designed prospective studies are needed to establish a concrete investigational approach to neonatal IVCS and institute safe, evidence‐based treatment.