Perception and behavior toward neuropsychiatric disorders in Saudi Arabia: A systematic review and quality assessment.

LAY ABSTRACT: In 2010, Saudi Arabia became the first country from the Gulf Cooperation Council states to join the World Mental Health Survey Initiative, which collaborates with Harvard University and has undertaken over 33 countries. The Saudi National Mental Health Survey revealed that 80% of Saudis with severe mental health disorders do not seek treatment. Considering the strong evidence set out in Saudi national study and the recommendations for future research, this systematic literature review was initiated to examine the most studied neurodevelopmental disorders reported in the Kingdom of Saudi Arabia to explore public knowledge, attitudes, and behaviors. Findings have shown that attention deficit hyperactivity disorder and autism spectrum disorder have been the most studied neurodevelopmental disorders in Saudi Arabia since 2010. However, various groups in Saudi society, including healthcare professionals, medical students, and the general public, lacked knowledge about these health conditions, often leading to stigmatized attitudes and behaviors toward people with attention deficit hyperactivity disorder and autism spectrum disorder. However, demographic data showed that most of the studies were carried out in the Central and Western provinces. More research is needed in all regions of the Kingdom of Saudi Arabia to contribute to the knowledge about mental health conditions of attention deficit hyperactivity disorder and autism spectrum disorder children and their parents to increase knowledge about neurodevelopmental disorders and mental health disorders in the Kingdom of Saudi Arabia, thereby enabling people to rethink their attitudes and behavior.

A Saudi Woman with Ceftriaxone Induced Fixed Drug Eruption.

Background: A fixed drug eruption (FDE) is an immunological cutaneous adverse reaction, classified as a cutaneous adverse drug reaction (CADR) and characterized by well-defined lichenoid lesions that occur at the same site each time. Ceftriaxone is a third-generation antibiotic of cephalosporin antibiotics of the beta-lactam antibiotic family, which has typical in vitro activity against many Gram-negative aerobic bacteria. This is the first clinical case from Saudi Arabia and the fifth in the world to document a woman's experience with recurrent FDE after repeated ceftriaxone use. Case Report. A 25-year-old Saudi woman with a known case of sickle cell anemia (SCA) with a history of avascular necrosis of the right hip after replacement was hospitalized with a pain crisis triggered by an upper respiratory tract infection. The patient denied having a history of allergy previously. Due to fever, leukocytosis, and active follicular tonsillitis, ceftriaxone was started. However, a few hours later she developed lip edema and a fixed drug eruption measuring 7 × 11 cm on the left side of her back. The lesion reformed over a hyperpigmented lesion (4 × 8 cm) that the patient did not report upon initial examination. It turned out that this was due to the intravenous administration of ceftriaxone, a year ago in another hospital. An allergy to ceftriaxone was considered, and steroids and antihistamines were started. The case was labeled as ceftriaxone induced FDE. Conclusion: Ceftriaxone induced FDE is an uncommon type of allergic reaction that has been reported infrequently. Understanding this condition and the mechanism by which FDE becomes recurrent with the same previous fixed lesion is of great importance for both academic and future research purposes.

"Familial Multiple Coagulation Factor Deficiencies of FXI and FXII in an Asymptomatic Saudi Woman".

Factor XI deficiency (FXI) is the third most common coagulation factor deficiency after hemophilia A and B, ie, in the hierarchy after factors VIII and IX, taking into account von Willebrand's factor deficiency, as bleeding disorders are higher than in hemophilia C. Factor XII deficiency (FXII) is a congenital condition, inherited in the vast majority of cases in an autosomal recessive manner, more often associated with thromboembolic complications. A combination of both factor deficiencies has been found very rarely, and it can be familial multiple coagulation factor deficiency (FMCFD). This study reports the case of a 39-year-old woman from Saudi Arabia who had the combination of FXI and FXII deficiencies, known to be on treatment for hypothyroidism and was referred to a hematology clinic with an incidental finding of prolonged activated partial thromboplastin time (aPTT). Although there was no history of bleeding tendency, her siblings had a family history of an unknown type of bleeding disorder. On physical examination, the patient did not show any bruising, petechiae, or ecchymosis. The aPTT was 69 seconds (27-38) with normal use of other hemostatic agents and was corrected after a 50:50 mixing study. Intrinsic coagulation factors were evaluated, and they revealed severe FXI and moderate FXII deficiencies. Due to a strong family history, the patient was diagnosed with FMCFD. In conclusion, familial combined multiple clotting factor deficiency (FCMFD) is a rare condition that requires attention and reporting. The management strategy in such cases has not been well studied, especially in the long-term symptomatic patient with severe but asymptomatic combined FXI and FXII deficiencies, which is an area for review and further study.

Impending splenic rupture as indicator of atypical chronic lymphocytic leukemia: A case report from Saudi Arabia.

BACKGROUND: Chronic lymphocytic leukemia (CLL) is one of the hematological malignancies in which the bone marrow overproduces mature, dysfunctional lymphocytes. Affected lymphocytic cells can affect the lymph nodes, spleen, liver, bone marrow, and rarely other organs. Spontaneous rupture of the spleen is a rare health condition, with a few cases caused by CLL. Since the preliminary presentation of either impending or real splenic rupture is unusual and requires recording and reporting, this case report was developed. CASE REPORT: A 55-year-old male patient presented with a significant weight loss of 20 kg, loss of appetite, fatigue, abdominal pain, and early satiety. Clinical examination revealed massive splenomegaly 22 cm below the costal margin, otherwise unremarkable on systemic examination. The size and dimensions of the spleen were confirmed by computed tomography of the body. The patient underwent a bone marrow biopsy, which was suggestive of atypical CLL. Due to the rapid progression of the size of the spleen and the pattern of impending rupture of the spleen, an emergency splenectomy was performed, and the histopathological report confirmed low-grade B-cell non-Hodgkin's lymphoma, mostly typical of CLL. CONCLUSION: Given the rarity of cases, continuous data recording and reporting is required to enrich the scientific literature and share experiences from case to case. This will create a complete picture of this health condition across diverse regions and countries.

A young Saudi female with combined hemophagocytic lympho-histiocytosis and Kikuchi's disease: A case report.

Kikuchi's disease is an idiopathic self-limiting condition first reported in Japan in 1972. However, hemophagocytic lympho-histiocytosis is a condition that occurs due to overstimulation of the immune system. The presence of the two conditions is rare, and the clinical observation of this unusual clinical syndrome is worth reporting. We are reporting an 18-year-old Saudi female patient who presented with high-grade fever and diaphoresis 3 weeks before her presentation. Physical examination showed palpable cervical and axillary lymphadenopathy; laboratory investigation found neutropenia, a high lactate dehydrogenase of 550 U/L, and high ferritin levels. A thoracoabdominal computed tomographic study revealed generalized lymphadenopathy. She was diagnosed with hemophagocytic lympho-histiocytosis based on a bone marrow biopsy finding and Kikuchi's disease based on an excisional cervical lymph node biopsy. She received a high dose of dexamethasone with complete resolution of the condition. In conclusion, hemophagocytic lympho-histiocytosis and Kikuchi's Disease are uncommon conditions. The presence of a combination of such two conditions is extremely rare and worth reporting. Early diagnosis and initiation of the management with high dexamethasone dose could save patient life.

Turnover-attachment motive of Saudi Arabia nursing workforce: A Cross-Sectional study.

AIM: This study aimed to explore the association of turnover-attachment motive with socio-demographic data among Saudi and foreign nurses in a hospital in the Eastern Province of Saudi Arabia. METHODS: A cross-sectional correlation study was conducted among n = 180 registered Saudi and foreign nurses working at a private tertiary hospital in the Eastern Province, Saudi Arabia. The turnover-attachment motive survey was used to evaluate the eight motivational forces. Data were analysed using JASP version 16 statistical software and Orange 3 version 3.26.0. The data were subjected to correlation analysis and multiple linear regression. RESULTS: Only 8% of participants were identified with a high intention to leave driven by alternative, behavioural, normative and constitutive forces. Younger healthcare workers tend to have higher intentions to quit the job; the 29-33 age group has a statistically significant negative effect, increasing intention to leave. It was found that those with 5-6 years of clinical experience at Dr. Sulaiman Al Habib Hospital, Al Khobar and no experience abroad had statistically significantly lower intention to leave. CONCLUSION: This study presented the factors influencing nursing staff to stay or leave work in a hospital in the Eastern Province of Saudi Arabia that can be a basis for reviewing existing policies and procedures to improve nurses' working conditions.

A case report of congenital factor X deficiency in an adult patient.

Factor X deficiency is one of the rarest coagulation disorders representing 10% of all rare bleeding diseases with a frequency of 1:1,000,000. A 39-year-old male patient with no previous medical conditions was admitted to the hospital with a left carpal ganglion for surgical excision. Routine preoperative laboratory examination revealed a high international normalized ratio of 5.4 IU (0.8-1.1) and a prothrombin time of 72.2 s (10.9-13.6), with an isolated factor X level of less than 5%. Genetic testing for congenital factor X deficiency identified a homozygous mutation c.271 > A (p.Glu91). Vitamin K supplementation did not improve his international normalized ratio or increase factor X levels; hence, surgery was delayed. The patient was re-hospitalized to remove a wisdom tooth, during which fresh frozen plasma was administered. An allergic reaction complicated this procedure in the form of a rash on the body. As a result, the tooth was removed without active bleeding. This report presents a unique factor X deficiency case with limited treatment options to improve factor X levels after failed vitamin K administration and an allergic reaction to fresh frozen plasma. A physician's observation and ongoing follow-up were the only reasonable approaches in treating the patient with mild to moderate factor X deficiency due to lack of prothrombin complex concentrates or factor X replacement at the center at the time.

Hereditary factor XII deficiency in an adult patient: A case report.

Factor XII deficiency is a rare autosomal recessive health condition usually discovered incidentally during routine coagulation screening before surgery after investigating a prolongation of the activated partial thromboplastin time. This is a case of a 29-year-old man from Saudi Arabia who was selectively admitted to the surgical department to treat a perianal fistula and found incidentally prolonged activated partial thromboplastin time and factor XII deficiency. Examination of the skin revealed no bruising, petechiae, or ecchymosis. Systemic examination was normal. Laboratory examination showed an activated partial thromboplastin time > 160 s (normal between 27 and 38), which was repeated twice with low factor XII < 5.7% (73-121). Other factors and the work of hemostasis were within the normal range. Surgery was delayed at the request of the patient. One year later, the patient was admitted to the clinic after surgery without bleeding and did not require factor correction before or after surgery. However, treating factor XII-deficient patients specifically for preoperative preparation is challenging. Therefore, this rare case should be recorded and reported the same way as a number of previously rarely reported cases.

Steroid induced hypertriglyceridemia in pregnant waman with immune thrombocytopenia - case report.

Background: Hypertriglyceridemia is a medical condition defined as fasting triglyceride level more than 150 mg/dl. It could be due to either familial or acquired cause as in obesity, metabolic syndrome, diabetes mellitus type 2, alcohol consumption, decrease exercise or drug affects. Drugs such as corticosteroids rarely induced hypertriglyceridemia, for that we are reporting this case. Case presentation: We are reporting a 35 years old pregnant lady diagnosed with immune thrombocytopenia and started on prednisolone 1mg/kg per oral once a day. Two months later, while on 20 mg of prednisolone, she presented to the emergency department with epigastric pain, nausea and vomiting for 15 days. Physical examination showed dry mucosa, new xanthelasma over both eyelids and epigastric tenderness with palpable suprapubic gravida uterus; otherwise, was unremarkable. Blood samples were highly lipemic, and laboratory investigations showed high triglycerides (TG) of greater than 73 mmol/L, mild diabetic keto acidosis with normal other chemistry including hepatic, renal, and pancreatic panel. She was treated by diet restriction, insulin infusion, Fenofibrate, and Omega 3 as well as rapid tapering down of prednisolone. Conclusion: Corticosteroid-induced hypertriglyceridemia is an uncommon condition and could be fatal, especially in high-risk cases. Unfortunately, no guidelines support a regular screening for lipid profile prior to initiating steroid therapy. However, we are suggesting a further study and creating a recommendation to mandate screening for lipid profile along with fasting blood sugar prior to initiating steroid therapy, especially in high-risk cases as in pregnancy.

Severe thrombocytopenia after Vaxzevria vaccination in a single patient: A case report.

AstraZeneca vaccine became one of the four vaccines that encode different forms of the SARS-CoV-2 spike glycoprotein. We report the case of an 18-year-old medically free female with progressive bruising of the upper and lower extremities for 1 week, beginning 3 days after the first dose of AstraZeneca. Laboratory results showed severe thrombocytopenia of 4.3 × 103/µL with a normal white blood cell count, renal profile, and hemolytic markers. She was treated conservatively with high-dose steroids and intravenous immunoglobulin, which resulted in a full recovery of her platelet count. In our case, the question was raised about the possibility of receiving a second dose of another vaccine product instead of not being vaccinated again, a subject for further research.

Coronavirus disease 2019 (COVID-19) in special groups: A single-center experience in sickle cell disease patients in Saudi Arabia.

BACKGROUND: Sickle cell disease (SCD) is a group of hereditary diseases, inherited as autosomal recessive disorder, which causes mutation in the ß-globin gene. As a result, there is a change in the sixth amino acid from glutamic acid to valine. The affected red blood cell is then prone to polymerization and sickling crisis under conditions of low oxygen tension. One of the major causes of mortality in SCD is acute chest syndrome (ACS). On the other hand, coronavirus disease 2019 (COVID-19) is a pandemic disease that carries significant mortality and morbidity worldwide with unknown outcomes in the affected SCD population. This study was created for that reason. MATERIALS AND METHODS: We report a case series of ten SCD patients who were affected by COVID-19 and required admission between May 1, 2020, and October 30, 2020, at a tertiary care hospital in Dhahran, eastern region of Saudi Arabia. Historical data were obtained retrospectively from electronic records. MS Excel was used for data entry, and SPSS version 23 was used for data analysis. RESULTS: The mean age of the patients involved in the study was 32 years, and the mean duration of symptoms was 5.7 days. None required critical care admission, and there was no mortality. All patients were discharged from hospital in good condition with no requirement of home oxygen. CONCLUSION: Although we expected a fatal outcome of SCD patients affected by COVID-19 infection, our limited case series showed favorable disease behavior and outcome, with a suspicion of underlying unclear protective mechanism from serious complications. However, further studies are required to better understand COVID-19 behavior in SCD patients.

Remsima (a Tumor Necrosis Factor (TNF) -α Inhibitor) induced hemolysis in a patient with Crohn's disease - Case report.

INTRODUCTION: Crohn's disease (CD) is an idiopathic inflammatory disorder of unknown etiology with genetic, immunologic, and environmental influences. Infliximab is a treatment modality for fistulated Crohn's disease. Infliximab induced hemolysis is rare and very few cases reported before in Ulcerative colitis (UC) but not in Crohn's disease. CASE PRESENTATION: We are reporting a 63 years old gentleman who was diagnosed as Crohn's disease and started on Tumor necrosis factor Inhibitor (TNF) -α i.e. (infliximab - Remsima) infusion. The course was complicated by Coomb's negative hemolytic anemia which is suggestive of non-immune drug induced hemolysis. Our patient was treated with steroid and conservative measures. Upon following up, his hemoglobin level as well as all hemolytic markers showed dramatic improvement. Adalimumab was used to treat this patient as an alternative choice without relapse of hemolysis. CLINICAL DISCUSSION: Drug induced Hemolysis is not a well-known complication post receiving Tumor necrosis factor Inhibitor (TNF) -α infusion in patients with Crohn's disease. Coombs negative hemolysis keeps in favor of non-immune drug induced rather than other differentials in our case scenario. CONCLUSION: Although cross-reactivity is expected between one biological agent to another, in our case the use of Adalimumab as alternative choice post Tumor necrosis factor Inhibitor (TNF) -α (Remsima infliximab) induced hemolysis did not cause hemolysis or any type of reaction.

Palms and soles itchiness as a side effect of COVID-19 vaccination.

BACKGROUND: COVID-19 is a pandemic disease that has no definite treatment or preventive medication until late 2020 when vaccines were developed. Vaccines are a very useful new tool against COVID-19 that stimulate the immune system after recognition of viruses or their parts. Complications post vaccination could happen and they depend on the types, vaccine mechanism of action as well as some other patients' factors. CASE REPORT: We are reporting a 39 years old lady who is known as allergic to a strawberry and kiwi, otherwise medically free. She presented to the outpatient unit with a ten-days history of palms of the hands and soles of the feet itchiness that is associated with occasional redness after receiving the first dose of COVID-19 vaccination. There were no skin rashes or pruritis at any other sites. The patient was treated conservatively by antihistamine and the symptoms gradually resolved within five days. CONCLUSION: Allergic reaction is one of the expected complications post any COVID-19 or non-COVID-19 vaccination. Although the type, distributions and severity of allergic reactions are variable from person to another, yet isolated itching to palms and soles could be a rare side effect post first dose of COVID-19 vaccination (Pfizer-BioNTech) which is worth reporting. This presentation could be a type of allergic reaction which may require holding further doses, debate is there for further case reporting, research or evaluation. Learning points.

Isolated ten-eleven translocation 2 positive in triple negative essential thrombocythemia: Case report and literature review.

Essential thrombocythemia is one of the famous diseases under the category of myeloproliferative disorder. It is an end result of a genetic mutation of one or more of the most frequent oncogenes such as Janos kinase 2 (JAK2), MPL proto-oncogene, thrombopoietin receptor (MPL), and calreticulin (CALR). However, negative genetic markers, so-called (triple negative disease), can happen in the presence of other uncommon types of mutation. TET2 (ten-eleven translocation 2) positive as isolated genetic marker in triple negative essential thrombocythemia is uncommon genetic presentation. For that, we are reporting a 22-year-old lady who presented with a feature of dyspepsia and accidentally found to have persistently high platelet count, even after treating her mild iron deficiency anemia with no other secondary causes. Further investigations and bone marrow biopsy supported the diagnosis of isolated TET2 positive in triple negative essential thrombocythemia. We treated her conservatively with good hydration and low dose of aspirin. In conclusion, isolated TET2 positive in triple negative essential thrombocythemia at presentation is uncommon with no clear management or risk stratification guideline. However, it is hypothesized that TET2 mutation precedes JAK2; therefore, the detection of isolated TET2 in a triple negative essential thrombocythemia case should be closely followed for clonal evolution in long term. Further study and guidelines required in this area.

ABO in Correlation to the Requirement of Mechanical Ventilation and Mortality in Critically Ill Patients With COVID-19.

BACKGROUND: Coronavirus disease 2019 (COVID-19) is caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) that was first discovered in December 2019 and turned to be pandemic in early March 2020. We aimed to describe the dominant ABO group and outcomes of critically ill patients (respiratory failure requiring mechanical ventilation and mortality) in a Saudi Arabian setting. METHODS: We conducted an observational, analytic cross-sectional, retrospective study in a tertiary care hospital. Around 90 candidates tested positive for COVID-19 were enrolled in this study during admission to critical care unit between May 2020 and September 2020. Blood group was detected in all patients included in the study during admission to critical care unit. RESULTS: In this study, data of 90 patients with COVID-19 admitted to critical care unit were collected. Some prevalent medical conditions were collected, in which hypertension (64.2%) and diabetes mellitus (58.9%) were the most reported comorbidities among patients and there was no significant difference between groups. Most of the sample had blood group of O (45.6%), while the least group was AB (5.6%). Patients with blood group of A/AB showed the highest mortality vs. group O/B (32% vs. 18.5%) with significant P value of 0.001. Patients of groups A/AB had higher risk for intubation than O/B groups (52.0% and 49.2%, respectively; confidence interval of 0.44 - 2.8 with insignificant P value of 0.055). Length of stay in critical care unit was significantly higher in group A/AB with a mean course of 18.20 days in comparison to group O/B with a mean course of 12.63 days (P = 0.033). CONCLUSION: Our data indicate that critically ill patients with COVID-19 with blood group A/AB are at increased risk of mortality and length of stay in critical care unit, with insignificant requirement of mechanical ventilation when compared with patients with blood group O/B. Future larger studies are needed to validate and understand the underlying mechanisms.

Acute Soft Head Syndrome (Subgaleal Haematoma) with Periorbital Oedema as a Rare Presentation in Sickle Cell Disease.

BACKGROUND: Sickle cell disease is a genetic condition frequently found in Africa and the Arabian Peninsula. Uncommon complications include subgaleal haematoma (soft head syndrome) and periorbital oedema. CASE PRESENTATION: A 17-year-old male patient presented with body aches and progressive right parieto-temporal and frontal head swelling. Physical examination revealed puffiness of the right eye that progressed rapidly to reddish periorbital oedema sparing the extraocular muscle and pupil response to light. CT and MRI of the brain suggested multiple subgaleal haematomas (soft head syndrome) and right periorbital oedema. CONCLUSION: Subgaleal haematoma (soft head syndrome) and periorbital oedema are uncommon complications of sickle cell disease. Management is conservative rather than surgical. LEARNING POINTS: Subgaleal haematoma concurrently with periorbital oedema is a rare presentation of sickle cell disease.There are no guidelines on treatment, but the conditions in our patient resolved with conservative management.

Critical Care COVID-19 Patient with a Picture of Thrombotic Thrombocytopenic Purpura.

BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is an uncommon haematological disease which can occur at any age and may present with COVID-19. This case describes a COVID-19 complication associated with a presentation resembling TTP. CASE DESCRIPTION: A 51-year-old man who had received a kidney transplant and was on immunosuppressant medication, was admitted to a critical care unit with severe COVID-19 pneumonia/acute respiratory distress syndrome (ARDS) which required intubation, mechanical ventilation and inotropic support. The course was complicated by the classic pentad of thrombocytopenia, intravascular haemolysis, acute kidney injury, neurological symptoms and fever, which prompted the diagnosis of probable TTP. After five sessions of therapeutic plasma exchange, the patient's general status improved, he was weaned off mechanical ventilation and his renal panel and haemolytic markers normalized. CONCLUSION: TTP is a life-threatening condition which requires urgent management with therapeutic plasma exchange. This case highlights some possible complications of COVID-19 generally and in immunocompromised patients specifically. The potential role of plasma exchange in COVID-19 patients without a positive diagnosis of TTP (the so-called 'TTP resembling presentation') is an area of further research. LEARNING POINTS: COVID-19 can manifest as a picture of thrombotic thrombocytopenic purpura (TTP) which requires therapeutic plasma exchange as in other cases of TTP.Further research is required on the use of therapeutic plasma exchange in severe COVID-19 with cytokine storm.