Knowledge and attitudes of primary care nurses towards geriatric depression in Jazan Region, Saudi Arabia.

Depression is a global health concern, particularly in the geriatric population. The increasing number of hospital admissions among older individuals highlights the need for healthcare professionals, particularly nurses, to understand and treat geriatric depression. Nurses play a crucial role in caring for older adults with depressive symptoms or depression. This study aimed to assess knowledge and attitudes regarding geriatric depression among primary care nurses in Jazan Region, Saudi Arabia. A cross-sectional study was conducted among 210 primary healthcare nurses in Jazan City using a validated self-administered questionnaire. Knowledge scores were measured and compared among selected demographic variables as well as attitudes toward geriatric depression. Data obtained were analyzed using the Statistical Package for the Social Sciences, version. 20.0. Chi-square test, fisher's exact test were used for comparison of variables with categorical data. Most primary care nurses were interested in caring for older patients with depression; however, they never attended training courses focused on geriatric depression. Where the study indicated that 38.1% of participants have poor knowledge about geriatric depression while 25.2% have good knowledge. Where the majority had a high understanding of the potential side effects of antidepressant medications, while they had limited knowledge about symptoms, diagnosis, and medications, the majority of participants demonstrated a positive attitude regarding feeling comfortable dealing with depressed patients' needs (56.7%) and considered their profession as a well-placed to assist patients (83.3%) However, 15.2% had a negative attitude citing a lack of self-discipline and willpower.

La dépression est un problème de santé mondial, en particulier dans la population gériatrique. Le nombre croissant d'hospitalisations chez les personnes âgées met en évidence la nécessité pour les professionnels de santé, en particulier les infirmières, de comprendre et de traiter la dépression gériatrique. Les infirmières jouent un rôle crucial dans la prise en charge des personnes âgées présentant des symptômes dépressifs ou une dépression. Cette étude visait à évaluer les connaissances et les attitudes concernant la dépression gériatrique parmi les infirmières de soins primaires de la région de Jazan, en Arabie Saoudite. Une étude transversale a été menée auprès de 210 infirmières de soins primaires de la ville de Jazan à l'aide d'un questionnaire auto-administré validé. Les scores de connaissances ont été mesurés et comparés parmi certaines variables démographiques ainsi que les attitudes à l'égard de la dépression gériatrique. Les données obtenues ont été analysées à l'aide du progiciel statistique pour les sciences sociales, version. 20,0. Le test du Chi carré et le test exact de Fisher ont été utilisés pour comparer les variables avec les données catégorielles. La plupart des infirmières de soins primaires souhaitaient soigner des patients âgés souffrant de dépression ; cependant, ils n'ont jamais suivi de formation axée sur la dépression gériatrique. L'étude indique que 38,1 % des participants ont de mauvaises connaissances sur la dépression gériatrique tandis que 25,2 % ont de bonnes connaissances. Alors que la majorité des participants avaient une bonne compréhension des effets secondaires potentiels des médicaments antidépresseurs, alors qu'ils avaient une connaissance limitée des symptômes, du diagnostic et des médicaments, la majorité des participants ont démontré une attitude positive et se sentaient à l'aise pour répondre aux besoins des patients déprimés (56,7 %). et considéraient leur profession comme bien placée pour assister les patients (83,3 %). Toutefois, 15,2 % avaient une attitude négative citant un manque d'autodiscipline et de volonté.

Home Management and Prevention of Vaso-Occlusive Crisis of Sickle Cell Disease Knowledge among Nursing Students.

Sickle cell crisis, or vaso-occlusive crisis, is a painful complication of sickle cell disease that occurs in adolescents and adults, which is considered the most common reason these patients seek medical attention in an emergency room. Despite the high prevalence of sickle cell disease in the Jazan region, Saudi Arabia, there hasn't been a study looking at nursing students' knowledge about sickle cell disease and home management and prevention of vaso-occlusive crises associated with sickle cell disease. Most of those focused on the investigation of the public, parents of children with sickle cell disease, school students, and patients with sickle cell disease. Therefore, this study aims to assess the level of knowledge about home management and prevention of vaso-occlusive crises among Saudi nursing students at the Aldayer University College, Jazan University, Kingdom of Saudi Arabia. A descriptive cross-sectional design was used in this study that involved 167 nursing students. The study revealed that Aldayer nursing students had adequate knowledge about the home management and prevention of sickle cell disease vaso-occlusive crises.

Impact of Mannose-Binding Protein Gene Polymorphisms in Omani Sickle Cell Disease Patients.

OBJECTIVES: Our aim was to study mannose-binding protein (MBP) polymorphisms in exonic and promoter region and correlate it with associated infections and vasoocculsive (VOC) episodes in sickle cell disease (SCD) patients since MBP plays an important role in innate immunity by activating the complement system. METHODS: We studied the genetic polymorphisms in the Exon 1 (alleles A/O) and promoter region (alleles Y/X; H/L, P/Q) of the MBL2 gene, in SCD patients as an increased incidence of infections is seen in these patients. A PCR-based, targeted genomic DNA sequencing of MBL2 was used to study 68 SCD Omani patients and 44 controls (healthy voluntary blood donors). RESULTS: In SCD patients, the frequency of the genotype related to the high production of MBL was 0.35 (YA/YA) and for intermediate/low production was 0.65 (YA/XA, XA/XA, YA/YO, XA/YO, YO/YO). The observed frequencies of MBL2 gene promoter polymorphism (-221, Y/X) were 44.4% and 20.5% for the heterozygous genotype Y/X and 3.2% and 2.2% for the homozygous (X/X) respectively between SCD patients and controls. MBL2 Exon1 gene mutations were 29.4% and 50% for the heterozygous genotype A/O and 5.9% and 6.8% respectively for the homozygous (O/O) genotype between SCD patients and controls. The distribution of variant MBL2 gene polymorphisms did not show any correlation in SCD patients with or without VOC attacks (p=0.16; OR -0.486; CI=0.177 -1.33), however, it was correlated with infections (p=0.0162; OR -3.55; CI 1.25-10.04). CONCLUSIONS: Although the frequency of the genotypes and haplotypes of MBL2 in SCD patients did not differ from controls, overall in the SCD patient cohort the increased representation of variant alleles was significantly correlated with infections (p<0.05). However, these variant MBL2 polymorphisms did not seem to play a significant role in the VOC episodes in this SCD cohort.

Thiamine responsive megaloblastic anemia: the puzzling phenotype.

BACKGROUND: Thiamine responsive megaloblastic anemia (TRMA) is characterized by a triad of megaloblastic anemia, non-type 1 diabetes mellitus and sensorineural deafness. Other clinical findings have been described in few cases. The SLC19A2 gene on chromosome 1q 23.3 is implicated in all cases with TRMA. Our aim is to discuss the clinical manifestations of all Omani children diagnosed with TRMA and determine genotype-phenotype relationship. PROCEDURE: Clinical and laboratory data of all patients diagnosed in Oman were retrospectively collected. Mutation analysis of affected families was conducted using two Microsatellite markers. Genotyping was performed with fluorescent-labeled PCR primers. To define the deletion breakpoint region, PCR reactions were carried out using different primer pairs located at the introns 3 and 3'-untranslated region with Expand Long Template PCR kit. RESULTS: A total of six children have been diagnosed with this syndrome. They were five females and one male. They all presented with sensorineural deafness at birth while the age of anemia presentation ranged between 6 weeks to 19 months. They all belong to same family with complex interfamilial marriages and presented with the typical triad. Of interest is the very rare presentation of one patient with Uhl cardiac anomaly (total absence of right ventricular myocardium with apposition of endocardium and pericardium) that has never been described before in patients with TRMA. All patients have a novel large deletion of 5,224 bp involving exons 4, 5, and 6 of SLC19A2. CONCLUSIONS: TRMA is a disease of expanding phenotypic spectrum with poor genotype-phenotype correlation.

Single dose darbepoetin alfa is useful in reducing red cell transfusions in leukemic children receiving chemotherapy.

The role of erythropoiesis-stimulating agents (ESAs) in the management of chemotherapy-induced anemia (CIA) is becoming increasingly recognized in the field of medical oncology, with paucity of data in pediatrics. We evaluated the efficacy and tolerability of a single-dose darbepoetin alfa, a long-acting ESA, given to 35 pediatric acute lymphoblastic leukemia (ALL) children during induction chemotherapy. Compared to a retrospective control group, the studied patients have required significantly less units of packed red blood cells (0.88 units/patient in the studied group versus 2.04 units in controls), with no major side effects. We recommend further prospective double-blinded studies with more tailored dosing regimens in pediatric ALL cases and solid tumors.

First reported case of compound heterozygosity for HbA2-Yialousa (HBD: c.82 G>C) and HbA2-Wrens (HBD: c.295 G>A) in Oman.

We report the presence of two different dglobin gene mutations causing d?-thalassemia in association with homozygous (-a3.7/-a3.7) genotype for the first time in an Omani child with a low hemoglobin A2 (HbA2) of 0.8 %. Direct nucleotide sequencing revealed compound heterozygote mutations in the patient's d-globin genes: HbA2-Yialousa (HBD: c.82G[C) and HbA2- Wrens (HBD: c.295G[A). In Oman, where a and b-thalassemia and HbS are prevalent, an awareness of the presence of different d-globin gene mutations is important as complex interactions between these hemoglobinopathies can lead to the misdiagnosis of b-thalassemia carriers.

Comparative assessment of deferiprone and deferasirox in thalassemia major patients in the first two decades-single centre experience.

Iron overload is mainly responsible for the morbidity and mortality in patients with beta thalassemia major (TM). Our aim was to compare treatment outcomes with oral iron chelators, deferiprone (DFP), and deferasirox (DFX) in the first two decades on therapy. Seventy patients with TM (mean age ± SD, 7.9 ± 4.2; range 1.5-17 years) attending the pediatric day care unit for regular transfusional support were enrolled in this cross-sectional cohort study. The patients were treated either with DFP at the dose of 75-100 mg/kg/d in three divided doses after food or DFX at the dose of 25-40 mg/kg/d as single dose before food. Mean serum ferritin (±SD) was lower in patients below 10 years (n = 44) at 1283 (±600) ng/mL when compared with patients ≥10 years (n = 19) at 1546 (±589) ng/mL. There was no significant difference in mean serum ferritin (±SD) level in patients receiving DFP (1360 ± 589) versus DFX (1260 ± 641) in this cohort, P > 0.05. 67% of the patients had Vitamin D deficiency (<50 umol/L). Our results show comparable efficacy of DFP and DFX with regards to iron chelation as estimated by serial serum ferritin levels; however, MRI T2* values were higher in the DFP-treated patients compared to DFX treatment.

Transcranial Doppler ultrasonography in sickle cell disease: a study in Omani patients.

Changes on Transcranial Doppler (TCD) ultrasonography have been proposed as significant predictors of cerebrovascular complications in sickle cell disease (SCD). However, consensus with regards to the TCD criteria to recognize abnormalities in cerebral vasculature is lacking. We studied the TCD characteristics of cerebral arteries among Omani patients with SCD and correlated them with cerebrovascular events. TCD was performed through the temporal and suboccipital windows using a 2 MHz probe (DWL). Thirty-three of 59 patients (56%) with SCD had neurological symptoms including stroke--12 (20%) and epilepsy--7. Fifteen patients (25%) had significant TCD abnormalities including: markedly increased velocities--11 (3 with stroke); turbulent flow--2; and reversal of flow--2. No patient had a time averaged maximum mean velocity of >200 cm/s in anterior circulation. On applying a modified definition of "abnormal TCD" to anterior and posterior circulation studies, increased TCD velocities in posterior circulation correlated with history of stroke (P < 0.05). TCD velocities in the 18 adult patients ( older than 15 y) were significantly lower than in children. Logistic regression analysis revealed abnormal TCD in the left posterior cerebral artery to be an independent predictor of stroke in this cohort (P = 0.035).

Transient Myeloproliferative Disorder and Down Syndrome: Is there a link?

An extremely premature male neonate presented with an unusual multisystem dysfunction within the first 24 to 48 hours of life. The unfolding of clinical events and investigations revealed a transient myeloproliferative disorder (TMD). TMD was the main indication for karyotyping of this premature infant without clinical symptoms of Down syndrome. The awareness of TMD in a newborn warrants karyotype analysis to look for trisomy 21 and a close surveillance because of its potential progression to true leukaemia.

Mutation of FA2H underlies a complicated form of hereditary spastic paraplegia (SPG35).

Hereditary spastic paraplegia (HSP) describes a heterogeneous group of inherited neurodegenerative disorders in which the cardinal pathological feature is upper motor neurone degeneration leading to progressive spasticity and weakness of the lower limbs. Using samples from a large Omani family we recently mapped a gene for a novel autosomal recessive form of HSP (SPG35) in which the spastic paraplegia was associated with intellectual disability and seizures. Magnetic resonance imaging of the brain of SPG35 patients showed white matter abnormalities suggestive of a leukodystrophy. Here we report homozygous mutations in the fatty acid 2-hydroxylase gene (FA2H) in the original family used to define the SPG35 locus (p.Arg235Cys) as well as in a previously unreported Pakistani family with a similar phenotype (p.Arg53_Ile58del). Measurement of enzyme activity in vitro revealed significantly reduced enzymatic function of FA2H associated with these mutations. These results demonstrate that mutations in FA2H are associated with SPG35, and that abnormal hydroxylation of myelin galactocerebroside lipid components can lead to a severe progressive phenotype, with a clinical presentation of complicated HSP and radiological features of leukodystrophy. (c) 2010 Wiley-Liss, Inc.

Cytogenetic profile of childhood acute lymphoblastic leukemia in Oman.

BACKGROUND: Chromosomal abnormalities have important diagnostic and prognostic significance in acute lymphoblastic leukemia (ALL). The purpose of this study was to define and classify the frequency and type of chromosomal abnormalities among newly diagnosed children with ALL and compare the results with those reported from other geographical regions of the world. METHODS: Bone marrow chromosomal studies with GTG banding were performed in untreated ALL pediatric patients aged from 7 days to 14 years. RESULTS: Among Omani children examined with ALL, 47 (81%) patients yielded results, with 26 (55.3%) showing an abnormal karyotype [10 (21.3%) pseudodiploid, 2 (4.3%) hypodiploid and 14 (29.7%) hyperdiploidy] and 21 (44.6%) had normal diploidy. Structural abnormalities were observed in 16 (34%), of which 11 (23.4%) cases were translocations, the most frequent being t(9;22) observed in three (6.4%) of our patients. Uncommon translocations such as t(9;15)(p11;q10), t(3;6)(p12;q11), t(1;6)(?31;?q23), t(1;19)(q12;q12), der(18)t(12;18)(q11;p11), and other structural aberrations add(2)(q22), add(6)(q16), add(18)(q22), add(14)(q32) along with deletions del(10)(q22), del(12)(p11), del(12)(p12), del(18)(q11) were also observed. CONCLUSIONS: The study showed a good correlation and concordance between the ploidy distribution by cytogenetics and flow cytometry. The patterns of chromosomal anomalies in our patients showed some variations in the frequency of aberrations reported. It is therefore necessary that newer techniques like fluorescence in situ hybridization (FISH) along with reverse transcriptase polymerase chain reaction (RT-PCR) and spectral karyotyping will help us identify chromosomal aberrations not detected by conventional cytogenetic methods in the near future. To our knowledge, this is the first report from the Middle East of a cytogenetic study on childhood ALL.