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Table of Contents
May-June 2018
Volume 10 | Issue 3
Page Nos. 71-103
Online since Monday, June 4, 2018
Accessed 23,744 times.
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VIEW POINT
Why medical journals in developing countries have no obituary pages?
p. 71
Salem A Beshyah
DOI
:10.4103/ijmbs.ijmbs_35_18
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ORIGINAL ARTICLES
Clinical and genetic profile of a cohort of pyridoxamine 5-Phosphate oxidase deficiency – A single-center experience
p. 73
Waseem Mahmoud Fathalla, Noora Al Menhali, Syed Arif Hosain, Fatima Hashim Ibrahim
DOI
:10.4103/ijmbs.ijmbs_17_18
Background:
Pyridoxamine 5-phosphate oxidase deficiency (PNPOD) is a rare treatable neonatal epileptic encephalopathy. It is important to raise awareness about this condition to enable early treatment.
Methodology:
This is a retrospective chart review of PNPOD cases followed at Mafraq Hospital during 2011–September 2017. The inclusion criteria include confirmed homozygous or compound heterozygous mutation in pyridox(am)ine-5-phosphate oxidase (PNPO) gene.
Results:
Seven cases were identified, all Emiratis from two tribes. Six cases from Tribe A had homozygous genetic variant C.674G>T: P. Arg255 Leu (one is presumed to have the same mutation based on confirmed proband sibling and carrier state of the parents of this sibship). One patient from Tribe B was tested abroad and has a confirmed homozygous pathogenic variant in PNPO gene (details not available). All six patients with the identical mutation are from one Emirati tribe suggest a founder effect. Two neonates treated in the first few days of life had the best clinical outcome of seizure control and neurodevelopment. One mortality (the deceased sibling of a normally developing child with this disease) highlights the great importance of early treatment. The remaining four patients had incomplete seizure control with neurobehavioral delay. Patients with intractable epilepsy and poor neurodevelopment never received pyridoxal 5-phosphate in the 1
st
days of life, although they received pyridoxine and other anti-seizure medications.
Conclusion:
PNPOD is a treatable neonatal epileptic encephalopathy; however, early treatment is essential for optimal outcomes. A management algorithm for intractable neonatal seizures emphasizing the crucial “treat before you diagnose” approach is critical for such cases.
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Well-being of residents in training programs of Abu Dhabi health services
p. 77
Bushra Al Ghailani, Mouza Ali Al Nuaimi, Asma Al Mazrouei, Eiman Al Shehhi, Maha Al Fahim, Ebtihal Darwish
DOI
:10.4103/ijmbs.ijmbs_12_18
Background:
Residency is a stressful period adversely affecting the health and lifestyle of resident physicians. In September 2001, the American Council of Graduate Medical Education released requirements that limit resident working hours in an effort to decrease resident fatigue.
Aims:
We aimed to measure the prevalence of stress, burnout, and depression among residents working in Abu Dhabi Health Services and to determine the percentage of residents with a healthy lifestyle and to compare the well-being of residents from different specialties.
Setting and Design:
A cross-sectional survey was conducted in the year 2016 among resident doctors in Abu Dhabi. The study population comprised 458 residents representing 20 training programs at six institutions.
Subjects and Methods:
All the residents were provided self-administered questionnaires.
Results:
Response rate was 81%. The prevalence of perceived stress among residents was 86.4%. Stress was significantly associated with workload (
P
< 0.01) and night shifts (
P
< 0.001) among residents from most specialties. Regarding burnout, 65.7% of the residents felt emotionally exhausted and 50.8% felt depressed at some point during their residency. Furthermore, 58.8% of the residents with chronic diseases had an uncontrolled disease status.
Conclusion:
Majority of the residents of Abu Dhabi Health Services experienced high rates of burnout, depression, and stress along with poor lifestyle and uncontrolled status of chronic diseases.
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Anti-Hepatitis B Antibody status in children with coeliac disease
p. 83
Areej Abdussalam Hweta, Asma Ali Shagleb, Mohamed Omer Elgadi, Abulraouf Mohamed Zaghdani, Fauzi Abdalla Sagher
DOI
:10.4103/ijmbs.ijmbs_25_18
Objective:
This study aimed to compare the antibodies response to hepatitis B virus (HBV) vaccine between Libyan children with coeliac disease (CD) and healthy control.
Subjects and Methods:
A total of 66 children with CD on a gluten-free diet (GFD) and 31 randomly allocated healthy children who received HBV vaccination according to the standard immunization schedule were included. Hepatitis B virus surface antigen (HBsAg) and the production of specific anti-HBs antibodies were evaluated in all patients and control participants using standard techniques. Patients with <10 IU/L anti-HBs antibodies were considered nonresponses to the vaccination.
Results:
None of the participants were HBsAg reactive. Forty-one of the 66 patients (62%) were female, and 25 (37.8%) were male. The mean age of the CD patients was 8.2 years (range, 22 months-15 years). Anti-HBs titers were positive in 40 (60.6%) patients and negative in 26 (39.3%) patients, whereas they were positive in 19 (61.2%) of the children in the control group and negative in 12 (38.7%).There was no significant difference in response to vaccine between the two groups (
P
= 0.83), the study revealed a statistically significant relation between negative anti-HBs titers and duration from the last dose of HBV vaccine (
P
= 0.004).
Conclusion:
In the present study, the response rate in Libyan children with CD on GFD was not different from healthy control. However, not all children need booster dose; only nonresponders need an intradermal test-booster dose to reassess the state of their memory cell before considering revaccination.
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Malnutrition and risk factors in tunisian patients with colorectal cancer
p. 88
Rym Ben Othman, Imen Ksira, Amal Smida, Olfa Berriche, Feten Mahjoub, Sabrine Mansour, Jihed Belghith, Henda Jamoussi
DOI
:10.4103/ijmbs.ijmbs_79_17
Background and Objectives:
Colorectal cancer (CRC) is the most frequent digestive cancer. Its occurrence is associated with many factors including nutrition and lifestyle. CRC is often associated to malnutrition which worsens its prognosis. The objectives of the study were to evaluate the nutritional status of patients with CRC.
Patients and Methods:
A retrospective study about fifty patients with CRC recruited in the Department of Gastroenterology and Surgery of “La Rabta” Hospital in Tunis as well as fifty random controls. The groups were matched for age and sex. The nutritional status of patients was assessed by anthropometric measurements, laboratory tests (albumin), and three nutritional scores. Dietary intakes were quantified by a frequency questionnaire and 24-h recall.
Results:
Significant risk factors were obesity (
P
= 0.02), menopause (
P
= 0.006) and the high consumption of red meat (
P
= 0.002), processed meat (
P
= 0.002), fried foods (
P
= 0.0001), and sugar (
P
= 0.0001). The consumption of green tea (
P
= 0.003), fruit (
P
= 0.001), and cereals (
P
= 0.0001) was higher in controls. Malnutrition was common measured by the body mass index, the percentage of weight loss (42%), albumin (60%), and different nutritional scores. The energy and protein intakes of patients were below the recommended requirements in 94% and 92% of patients, respectively. Deficiencies in minerals, vitamins, and trace elements were found.
Conclusions:
Malnutrition was very common in patients with CRC with multiple vitamin and mineral deficits.
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Primary gastric lymphoma: A study of morphology, immunophenotype, and prevalence of
Helicobacter pylori
infection
p. 94
Mohamed Elmoaket, Fatma Emaetig, Firas Abdulmalik, Fairouz Torjman, Nabil Enattah, Adam ElZagheid
DOI
:10.4103/ijmbs.ijmbs_62_17
Objectives:
We aimed to study the pattern of primary gastric lymphoma in two geographically different locations, namely, Tripoli Medical Center (TMC) in Libya and Antwerp University Hospital (Universitair Ziekenhuis Antwerpen [UZA]) in Belgium.
Materials and Methods:
Twenty-four cases of primary gastric lymphoma diagnosed during an 8-year period at TMC and 20 cases diagnosed during 11-year period in UZA were studied. Immunohistochemistry lymphoma panel CD3, CD5, CD20, CD10, CD79a, CyclinD1, KI67, and pancytokeratin were applied in all cases.
Results:
Primary gastric lymphoma in UZA occurs in a slightly older age group with marked male predominance while in TMC occurs at a slightly younger age with marginal male predominance. Two-thirds of the TMC cases were of high-grade lymphoma (HGL) and one-third were low-grade lymphoma (LGL). UZA cases included nine cases of HGL with (45%) and 11 of LGL (55%). Of the TMC primary gastric lymphoma cases, 12 were infected with
Helicobacter pylori
(50%) and eight cases were mucosa-associated lymphoid tissue lymphoma (MALT-L) of which three cases had
H. pylori
infection. In the UZA cases,
H. pylori
infection was evident in seven of 20 primary gastric lymphoma cases (35%) and three of six MALT-L had
H. pylori
-infected cases (50%).
Conclusions:
This comparative study of primary gastric lymphoma in TMC (Libyan) and UZA (Belgian) studied cases showed no marked differences between the two patient populations based on the histological features and immunohistochemical phenotype and genotype and the clinical features.
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CASE REPORT
Unexplained fever and arthritis in a teenager with Type 1 diabetes
p. 99
Touseef Azhar Kazmi, Saira Bano Abbas, Khadija Ahmed Hafidh
DOI
:10.4103/ijmbs.ijmbs_30_18
Brucellosis is a relatively prevalent zoonotic infection in the Mediterranean region and the Arabian Peninsula. Due to the multi-system involvement and nonspecific nature of the complaints, making a diagnosis can sometimes be particularly challenging. We present the case of a Libyan-American adolescent with type 1 diabetes mellitus (T1DM) residing in the United Arab Emirates who presented with fever, flu-like symptoms, and arthritis. When his initial septic workup did not reveal a focus of infection, and he continued to be febrile with the development of multiple painful and swollen joints, a diagnosis of autoimmune arthritis was entertained given his background history of autoimmune T1DM. However, on further probing, we ascertained a history of raw camel cheese ingestion. This redirected us to look for and subsequently confirm the diagnosis of brucellosis. Appropriate antibiotic treatment led to rapid recovery of the patient.
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IN MEMORIAM
Bashir Allaghi (1951–2018)
p. 102
Elmahdi A Elkhammas
DOI
:10.4103/ijmbs.ijmbs_29_18
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ERRATUM
Research and ramadan fasting: Not too much of a good thing! (Correction)
p. 103
DOI
:10.4103/1947-489X.233764
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