Oral manifestations of 17 patients affected with mucopolysaccharidosis type VI

Objective

To assess oral manifestations of 17 patients with mucopolysaccharidosis type VI (MPS VI) or Maroteaux-Lamy syndrome.

Methods

We performed comprehensive oral examinations in 17 patients with MPS VI. Panoramic radiographs was performed only in 14 patients. All patients were of Thai, Turkish, and Indian origins. Ten of 17 patients had enzyme replacement therapy (ERT) (Naglazyme). Most Turkish patients (10/11) were on ERT. The Thai and Indian patients have never had ERT.

Results

Oral and radiographic examinations showed that hypoplastic mandibular condyles (93.3 %), malposition of unerupted teeth (92.9 %), large dental follicles (92.3 %), anterior open bite (86.7 %), maxillary constriction (56.3 %), and taurodontism (53.8 %) were common among patients with MPS VI. Newly recognized oral findings found in our study included taurodontism, long tooth roots, abnormal frenum, missing teeth, supernumerary teeth, and microdontia. Two patients who started ERT prior to 3 years old did not develope anterior open bite and one of them had mildly affected mandibular condyles.

Conclusion

Our study provides the most comprehensive study of oral manifestations in patients with MPS VI. Receiving ERT at very young ages may lessen craniofacial malformations including hypoplasic mandibular condyles and anterior open bite. Oral manifestations can be used as diagnostic features for MPS VI prior to assessing leukocyte ARSB activity or urinary excretion of dermatan sulfate.     

Cause-specific mortality in individuals with lymphoplasmacytic lymphoma/Waldenström macroglobulinaemia, 2000–2016

Data on cause-specific mortality after lymphoplasmacytic lymphoma (LPL) and Waldenström macroglobulinaemia (WM) are lacking. We identified causes of death amongst 7289 adults diagnosed with incident first primary LPL (n = 3108) or WM (n = 4181) during 2000–2016 in 17 USA population-based cancer registries. Based on 3132 deaths, 16-year cumulative mortality was 23·2% for lymphomas, 8·4% for non-lymphoma cancers and 14·7% for non-cancer causes for patients aged <65 years at diagnosis of LPL/WM, versus 33·4%, 11·2% and 48·7%, respectively, for those aged ≥75 years. Compared with the general population, patients with LPL/WM had a 20% higher risk of death due to non-cancer causes (n = 1341 deaths, standardised mortality ratio [SMR] 1·2, 95% confidence interval [CI] 1·1–1·2), most commonly from infectious (n = 188; SMR 1·8, 95% CI 1·6–2·1), respiratory (n = 143; SMR 1·2, 95% CI 1·0–1·4), and digestive (n = 80; SMR 1·8, 95% CI 1·4–2·2) diseases, but no excess mortality from cardiovascular diseases (n = 477, SMR 1·1, 95% CI 1·0–1·1). Risks were highest for non-cancer causes within 1 year of diagnosis (n = 239; SMR_<1year 1·3, 95% CI 1·2–1·5), declining thereafter (n = 522; SMR_≥5years 1·1, 95% CI 1·1–1·2). Myelodysplastic syndrome/acute myeloid leukaemia deaths were notably increased (n = 46; SMR 4·4, 95% CI 3·2–5·9), whereas solid neoplasm deaths were only elevated among ≥5-year survivors (n = 145; SMR_≥5years 1·3, 95% CI 1·1–1·5). This work identifies new areas for optimising care and reducing mortality for patients with LPL/WM.     

Pharmacists’ contributions to the delivery of pharmaceutical care to patients with type 2 diabetes in Kuwait

This study was designed to identify pharmacists’ potential contributions to the delivery of pharmaceutical care to patients with type 2 diabetes in Kuwait, and to identify and explore barriers that were preventing them from providing care to this specific group. A pretested self-administered questionnaire was distributed to all pharmacists registered in the Kuwait Pharmaceutical Association’s email registry (N?=?250). Invitations to a focus group interview were then sent to all pharmacists (N?=?50) who had responded to the questionnaire. Seven pharmacists accepted the invitation and participated in the focus group interview. Of the 50 respondents to the questionnaire, 31 (62.0 %; 95 % CI: 47.2–75.4) indicated that they were “comfortable” and “extremely comfortable” in discussing patient’s blood pressure target and annual screening with physicians rather than discussing smoking cessation advice or specific medication-related care issues. More than 75 % of the respondents were “comfortable” and “extremely comfortable” in sharing and verifying the patient’s drug history, blood pressure, cholesterol and stability of blood glucose with the healthcare team, and to maintain a pharmaceutical care plan for patients with diabetes. Overall, pharmacists indicated that they were more comfortable in undertaking clinical activities than discussing care issues with physicians. The focus group interview identified issues related to pharmacist-physician interaction, pharmacists’ confidence, pharmacists’ image by patients and physicians and barriers to implementing pharmaceutical care. This study shows that pharmacists in Kuwait perceive that their contribution to the delivery of pharmaceutical care could develop further with increased partnership between pharmacists and physicians and provision of further education, training and continuing professional development support.     

Forced-eruption time for palatally impacted canines treated with and without ostectomy-decortication technique

Objectives:To compare forced-eruption times for palatally impacted canines treated with and without the ostectomy-decortication technique and to assess the influence of palatally impacted canine pretreatment position and angle on forced-eruption time.Materials and Methods:The sample was composed of 118 patient-subjects with 151 palatally impacted canines treated with the ostectomy-decortication technique (n = 72) and without (n = 79). The orthopantomogram radiographs (OPGs) were analyzed for palatally impacted canine angle and horizontal and vertical position. Recovery time was measured from the start of forced eruption until the canine was within ±1 mm of final dental arch position.Results:The time of forced canine eruption with ostectomy-decortication technique was significantly shorter than without (6.6 vs 21.0 months). Pretreatment canine position significantly increased forced-eruption time in the ostectomy-decortication group but not in the control sample.Conclusions:Forced-eruption time of palatally impacted canines using the ostectomy-decortication technique was 3.2 times more rapid than without. Forced-eruption time increased significantly as a function of pretreatment palatally impacted canine position severity in the ostectomy-decortication group but not in the control.     

A study on nitric oxide,beta-adrenergic receptors and antioxidant status in the polymorphonuclear leukocytes from the patients of depression

BACKGROUND: alterations in the polymorphonuclear leukocyte (PMNs) receptors, second messenger system and in their responses have been found associated with depression. Recently role of tetrahydrobiopterin and nitric oxide has also been reported in the depressive disorders. It was therefore considered worthwhile to investigate the NOS activity in the PMNs, which like neurons, also express neuronal NOS (nNOS), antioxidant enzyme levels [superoxide dismutase (SOD), catalase and glutathione peroxidase (Gpx)] and beta-adrenergic receptors in the patients of depression. METHODS: patients were diagnosed according to the DSM-IV and were medication free, while healthy age-matched controls were also included in the study to estimate nitrite content, beta-adrenergic receptors and antioxidant enzymes in the PMNs according to the standard methodologies. RESULTS: an analysis of 66 cases of depression and 114 controls revealed 73% decrease in nitrite content and 71% decrease in beta-adrenergic receptor binding in the patients as compared to the healthy controls. However, activities of SOD, catalase and Gpx were not significantly altered in the patients. CONCLUSION: the results of the present study for the first time indicate alterations the NOS activity in PMNs obtained form the patients of affective disorders.     

Idiopathic perforation of the biliary tract in infancy and childhood

The case of a 25-day-old Saudi boy with spontaneous perforation of the biliary tract is reported. The mode of presentation and treatment are described and the literature on the subject reviewed.     

Enhancement of Anticorrosive Performance of Cardanol Based Polyurethane Coatings by Incorporating Magnetic Hydroxyapatite Nanoparticles

The present investigation demonstrates renewable cardanol-based polyol for the formulation of nanocomposite polyurethane (PU) coatings. The functional and structural features of cardanol polyol and nanoparticles were studied using FT-IR and 1H NMR spectroscopic techniques. The magnetic hydroxyapatite nanoparticles (MHAPs) were dispersed 1–5% in PU formulations to develop nanocomposite anticorrosive coatings. An increase in the strength of MHAP increased the anticorrosive performance as examined by immersion and electrochemical methods. The nanocomposite PU coatings showed good coating properties, viz., gloss, pencil hardness, flexibility, cross-cut adhesion, and chemical resistance. Additionally, the coatings were also studied for surface morphology, wetting, and thermal properties by scanning electron microscope (SEM), contact angle, and thermogravimetric analysis (TGA), respectively. The hydrophobic nature of PU coatings increased by the addition of MHAP, and an optimum result (105°) was observed in 3% loading. The developed coatings revealed its hydrophobic nature with excellent anticorrosive performance.