Glycogen storage disease in pediatric population

Introduction: The pathway of glycogen metabolism is regulated by many hormones such as insulin, glucagon, and corticosteroids. Glycogen storage diseases [GSD] most commonly affect muscles, liver, or both and occur in each 20000 to 43000 live birth. They are classified into 12 subtypes, but types I, II, and IX are the most common

Methodology: We conducted this review using a comprehensive search of MEDLINE, PubMed, and EMBASE, from January 2001, through February 2017. The following search terms were used: glycogen storage diseases, Von Gierke disease, Pompe's disease, Cori Disease, Forbes disease, Andersen's disease, McArdle disease, neonatal hypoglycemia, neonatal hepatomegaly

Aim: In this review, we aim to study the genetic basis, diagnosis, presentation, and different management approach to various common types of glycogen storage diseases prevalent in pediatric population

Conclusion: There are no cures for any type of glycogen storage diseases presently. Most treatments are designed to control signs and symptoms. The overall goals are primarily avoiding hypoglycemia, hyperlactatemia, hyperuricemia, and hyperlipidemia. Liver transplantation should be deliberated for patients with GSD type IV and for other progressive hepatic types of GSDs in order to avoid hepatic failure or malignancy. More research must be carried out to develop newer and more effective ways of management

Glycogen storage disease in pediatric population

Introduction: The pathway of glycogen metabolism is regulated by many hormones such as insulin, glucagon, and corticosteroids. Glycogen storage diseases [GSD] most commonly affect muscles, liver, or both and occur in each 20000 to 43000 live birth. They are classified into 12 subtypes, but types I, II, and IX are the most common

Methodology: We conducted this review using a comprehensive search of MEDLINE, PubMed, and EMBASE, from January 2001, through February 2017. The following search terms were used: glycogen storage diseases, Von Gierke disease, Pompe's disease, Cori Disease, Forbes disease, Andersen's disease, McArdle disease, neonatal hypoglycemia, neonatal hepatomegaly

Aim: In this review, we aim to study the genetic basis, diagnosis, presentation, and different management approach to various common types of glycogen storage diseases prevalent in pediatric population

Conclusion: There are no cures for any type of glycogen storage diseases presently. Most treatments are designed to control signs and symptoms. The overall goals are primarily avoiding hypoglycemia, hyperlactatemia, hyperuricemia, and hyperlipidemia. Liver transplantation should be deliberated for patients with GSD type IV and for other progressive hepatic types of GSDs in order to avoid hepatic failure or malignancy. More research must be carried out to develop newer and more effective ways of management

Prevalence of intake of dietary supplements in the population of Saudi Arabia- Jeddah

Background: several studies have shown micro-nutrient deficiencies in the Middle East region. Prominent defects in vitamin D, folate, and iron were especially noted. There are not many studies currently that tell us about the prevalence of dietary supplement intake in Saudi Arabia

Aim: the aim was to observe the current prevalence of dietary supplement use in Saudi Arabia. We will also attempt to elicit the reason they take the supplements, and how it affects their overall quality of life

Method: cross-sectional study among 489 Saudi patients was without any exclusion criteria using a standard questionnaire. The study was held at the endocrinology department of King Abdul Aziz Hospital and Oncology Center in Jeddah, Saudi Arabia

Results: the overall prevalence of multivitamin, mineral and supplement intake in the Saudi population was found to be 22%. Among them 16% were male and 24% were female

Conclusion: healthcare providers must spread more awareness about the potential benefits of dietary supplements in preventing non-communicable diseases

Frequency of anxiety related to dental procedures among professional students

Objective: To determine the frequency of self-reported anxiety levels related to dental procedures amongst professional students using Revised Corah's Dental Anxiety Scale, [DAS-R]

Study Design: A cross sectional study

Place and Duration: Jinnah Medical and Dental College, Karachi and Isra University, Hyderabad from 1[st] October 2010 to 30[th] April 2011

Methodology: A total of 259 professional students comprised the study sample. All those students who consented to participate were scored for anxiety using revised Corah's Dental Anxiety Scale [DAS-R]

Results: Of the total students, we categorized 20.95% as anxious, owing to their DAS scores above 12. From within them, only 20.7 % were finally classified dental phobic, scoring 15 or above on the same scale. Other findings are detailed subsequently in the full text

Conclusion: We found that self-reported -phobic levels were very close to the statistically calculated levels of anxiety score using the standard DAS scoring criteria. Using the same criteria, the actual frequency of phobia in our study was 4.35%

Restorative material of choice for a moderately sized class i cavity in a lower permanent molar

Objective: To study dentist related factors affecting choice of restorative material in a moderately sized, simple class I cavity on a permanent molar

Study Design: Cross sectional / descriptive study

Place and Duration of the Study: This study was carried out at Jinnah Medical and Dental College between February 2015 to July 2015

Materials and Methods: Dentists from public and private sectors were requested to respond to a self administered questionnaire regarding their choice of restorative material for a moderately sized class I cavity in a permanent molar

Results: 66%, 54% and 16% of the dentists graduating in the years 2011-2015, 2006-2010 and 2001-2005 respectively chose amalgam. Whereas, 31%, 35% and and 73% graduating in the same years preferred composite. 38% of the dentists practicing privately and 69% of dentists working in institutional setup selected amalgam.66% of female dentists selected amalgam and 30% chose composite. In contrast, 43% of male dentist selected composite and 44% chose amalgam

Conclusion: The dependable blend of enduring service of amalgam with its cost effectiveness makes amalgam a better choice for posterior teeth in our settings. [Pakistan]

Learning style preferences of dental students, Karachi

Objective: The prime objective of the study is to determine the learning style preference of dental students in Jinnah Medical and Dental College, Karachi

Study Design: Cross-sectional Descriptive Study

Place and Duration of Study: The study was conducted in Jinnah Medical and Dental College on students of Dentistry, first year through final year. VARK[TM] questionnaire version 7.8[C] was distributed and data was collected between 01 November to 30 November, 2014

Materials and Methods: Total 200 questionnaires were sent out. 160 students who consented to participate in the study were included and those who refused were excluded. Descriptive statistics was used to identify the learning style preferences of the students. The VARK scores were recorded on Excel sheet. Scoring algorithms especially designed for VARK research, available on its website were used for data management and description

Results: 51% of the students [n=82] preferred a uni-modal learning style, of which Aural was the most common. 47% of the dental students [n=75] used all four modes for learning while 2% preferred bi-modal [n=3]. None of the students were tri-modal

Conclusion: In conclusion, majority of students preferred uni-modal followed by the group which preferred all modes of presentation

Anxiety in Dental Patients

Objective: To determine the frequency of self-reported anxiety levels related to dental procedures amongst dental patients using Revised Corah's Dental Anxiety Scale, [DAS-R]. Study Design: A cross sectional descriptive study Place and Duration of Study: This study was carried out at the Medical, Dental and Allied Health Professionals' College from October 2010 and April 201 1. Materials and Methods: 485 patients comprised the study sample. All those patients who consented to participate and were 12-65 years of age were included. They were scored for anxiety using revised Corah's Dental Anxiety Scale [DAS-R]. Results: The mean DAS score for our study population was 9.91 [SD 3.29], while the modal score was 12. Based on the DAS Score, distribution of the respondents was: Non anxious - 63.1% [n=304]; Anxious - 33.2% [n=160]; and Phobic - 3.7% [n=18]. Other findings are detailed subsequently. We found that self-reported-phobic levels were higher in males compared to females, while the DAS scores on the contrary were higher for females than males. Conclusion: We found that DAS score were higher [more anxiety] in females than in males. Interestingly, we also found that self-reported dental phobia was observed more often in males than females

Locating MB2 canal in maxillary first molars with magnification: in vitro study

To determine the ability of locating the second mesiobuccal canals in maxillary first molars with magnification [dental loupes 3.5x]. This was a Cross sectional study. 300 freshly extracted maxillary first molars were stored in 1% thymol solution for 1 week. Rhomboidal access cavities were prepared in these teeth and chamber cleared to look for canals first with naked eye and then with dental loupes [x3.5]. If MB2 was detected two periapical radiographs were exposed with files in MB andMB2 canals. The data was analyzed using chi squared test on SPSS version 10. In 77 out of 300 extracted teethMB2 canal was located with naked eye and with the use of dental loupes the number of located canals increased to 265. There was a statistically significant difference between the two methods of identification of MB2 canals [p value <0.005]. Use of magnification increases the number of MB2 canals located in maxillary first molars compared to those located with naked eye examination alone in vitro