Background: Pulmonary atresia is a congenital heart defect that results from failure in the formation of the pulmonary valve that leads to right ventricular outflow obstruction, preventing the right heart ejection of blood to the lungs. It is also associated with a high incidence of chromosomal anomalies.

Methods: A retrospective styesyesudy of all infants diagnosed with pulmonary in Bahrain was undertaken. Pulmonary atresia was classified into pulmonary atresia with intact ventricular septum, pulmonary atresia in Tetralogy of Fallot, and pulmonary atresia in complex cyanotic heart defects. Short-term outcomes, survival, and associated genetics were analyzed.

Results: 84 patients were diagnosed. 57 (67.9%) were males and 27 (32.1%) were females (p = 0.204). The median age at diagnosis was 25 days. Patients diagnosed with pulmonary atresia and complex cyanotic heart defects had the highest early survival rate. The early survival rate of patients diagnosed with pulmonary atresia with intact ventricular septum was lowest (p = 0.0179).

Twenty-two patients (26.19%) had chromosomal anomalies. The DiGeorge syndrome was found in 18 patients, and 2 with Down Syndrome and other dysmorphisms in 2. 19 (86.36%) with chromosomal anomalies had pulmonary atresia and Tetralogy of Fallot, and 3(13.64%) with pulmonary atresia and complex cyanotic heart defects (p = 0.027).

Conclusions: Pulmonary atresia is a rare but serious congenital heart defect. The lowest short-term survival is in those with intact intraventricular septum. There is a high incidence of chromosomal deletion of 22q11 leading to DiGeorge syndrome in those with Tetralogy of Fallot with pulmonary atresia. Early catheterization and surgical techniques are effective palliation for short-term survival.

Keywords: Atresia, Genetic, Outcomes, Pulmonary, Valve

Background: Adherence to therapy is one of the basic predilections to successfully treating chronic obstructive pulmonary disease (COPD). Unfortunately, predictors of medication adherence are not well-known in chronic obstructive pulmonary disease (COPD) in Sudan.

Objective: To assess the medication adherence in the patients treated for COPD in ALSha’ab Teaching Hospital.

Methods: An observational, hospital-based cross-sectional study conducted on a sample of COPD outpatients. The following information was obtained: adherence to COPD therapy (Morisky Medication Adherence Scale - 4 items), age, gender, smoking status, treatment regimen for COPD, and COPD medication costs per month paid by the patients. Data was analyzed by using SPSS version 21.0.

Results: Among 100 COPD patients, 73 (73%) were males, and (47%) belonged to the age group 40- 60 years. Regarding adherence, 47 (47%) reported as medium adherent, 38 (38%) as low adherents, and 15 (15%) patients as high adherent. High Adherence to COPD therapy was associated with older age (27%), married (16.5%), literate (23.3%), drugs with less frequent dosing (50%), oral medications (75%), aware of the inhaler usage (22.7%), a smaller number of drugs (41.4%), and affordable drugs (28.8%).

Conclusion: The level of adherence to COPD therapy is low. Understanding factors associated with medication adherence could help enhance health outcomes in COPD. Non-adherence was associated with middle age, unmarried patients, number of respiratory drugs and daily COPD drug doses, Low knowledge and increased difficulties in inhaler technique, and non-affordability of drugs.

Keywords: COPD, Patients, Sudan, Adherence.

Background: Patients with suspected or known coronary artery disease (CAD) can be challenging to evaluate. Our study aims to assess the efficacy and utility of coronary computed tomographic angiography (CCTA) as a diagnostic method in patients with abnormal myocardial perfusion imaging (MPI) who were initially referred for Invasive Coronary Angiography (ICA).

Methods: This is a single-center retrospective descriptive study. We evaluated 210 patients who underwent CCTA instead of ICA. Data of these patients were documented and analyzed accordingly.

Results: Of the 210 patients who underwent CCTA, the procedure was found to be diagnostic in 172 (81.9%) patients. Of these 172 patients, 152 (88.4%) had normal coronaries or minor CAD, and 20 (11.6%) had significant disease requiring subsequent ICA. In 38 (18.1%) patients out of the total cohort, the CCTA was not diagnostic or could not be performed due to technical difficulties, requiring either ICA or another ischemia evaluation diagnostic modality.

Conclusion: CCTA is a viable and reliable diagnostic tool for evaluating patients with suspected CAD referred for ICA following an abnormal MPI test. It is recommended as an initial test to rule out significant coronary stenosis and as it can avoid unnecessary ICA.

Keywords: CAD, CCTA, CVD, ICA, MPI

Background: Atopic dermatitis is a chronic relapsing-remitting dermatosis commonly presented in primary healthcare facilities. Although there is no cure for atopic dermatitis, early diagnosis, and appropriate treatment are essential in the management of atopic dermatitis and in preventing its complications. Primary healthcare physicians are the first to encounter most patients with atopic dermatitis; hence, correct practices and knowledge of atopic dermatitis are important among primary healthcare physicians. This study aims to assess the knowledge and practice of primary healthcare physicians concerning diagnosing and treating patients with atopic dermatitis.

Methods: A cross-sectional study was conducted among primary health care physicians working at governmental primary health centers in Bahrain to determine the knowledge, attitudes, and practices towards atopic dermatitis. In addition, a self-administered online questionnaire was distributed among the participants using online surveys.

Results: A total of two hundred and eighty (n=280) primary healthcare physicians were recruited (response rate 80%). The average knowledge score (correct answers) was 46.7 ± 14.0 out of 100. Being a general practitioner (P<0.002), having more years of experience (P=0.044), and being of older age (P=0.003) were significantly associated with a lower knowledge score. The study also showed a wide range of varying practices among participants in managing patients with atopic dermatitis.

Conclusion: There were significant variations in the knowledge and practice amongst primary care physicians towards atopic dermatitis that could delay the treatment of the condition and, therefore, possibly decrease treatment adherence. Conducting targeted educational interventions and developing strategies by dermatologists could minimize such gaps in practice and knowledge.

Keywords: Atopic, Dermatitis, Bahrain, Physicians, Primary Care

Background: Patient satisfaction is one of the desired outcomes of health care, as it is not only an indicator of quality of care but also plays a vital role in the management of chronic patients such as Diabetes mellitus because it promotes adherence to advice and treatment, utilization of health care services, and the establishment of the doctor-patient relationship.

Aims and Objectives: This study aims to assess diabetic patients’ level of satisfaction attending the Central Diabetes Clinics in primary health care. Additionally, this study aims to determine whether patient satisfaction is related to individual characteristics such as sociodemographic parameters and disease-related factors.

Materials and Methods: A cross-sectional study was conducted in the Central Diabetes Clinics of Primary Health Care from February 6th to February 17th, 2022. The data was collected via a self-administered questionnaire. It was divided into two sections: the first part contained the patient's characteristics, and the second included 16 items obtained from the short form of the Patient Satisfaction Questionnaire (PSQ-18).

Results: The study included 369 diabetes patients in total. Most participants (85.9%) were Bahrainis between the ages of 51 and 70 (70.5%). The overall satisfaction mean score was 69.4 (86.75%, SD = 8.9). Patients were most satisfied with the interpersonal manners, while they were dissatisfied with the waiting time for emergency services. A significant association was found between overall satisfaction and education level, as well as with the duration of treatment. Lower educational levels and a duration of Diabetes of 5–10 years were associated with higher satisfaction. No significant association was found between overall satisfaction and other factors such as sex, age, nationality, marital status, employment status, monthly income, the presence of other chronic conditions, the presence of diabetes-related complications, or the treatment type.

Conclusion: This study concluded that most diabetic patients who visit Central Diabetes Clinics are generally satisfied with the quality of healthcare services.

Keywords: Diabetes, Diabetes Clinics, Patient Satisfaction, Primary Health Care, Bahrain

Alagille syndrome (AS) commonly presents with cholestasis, much like other liver diseases, making the diagnosis challenging. We report a case of a patient with AS mimicking biliary atresia (BA) with a poor outcome. The infant, a product of a non-consanguineous marriage, presented with jaundice, clay stools, peripheral pulmonary stenosis, atrial septal defect, and butterfly vertebrae. Cholescintigraphy showed an absence of radiotracer excretion. Surgical exploration revealed the presence of a hypoplastic hepatic duct but a normal gallbladder, cystic, and common bile ducts. Intraoperative cholangiogram favored BA, and a Kasai procedure was performed. The liver biopsy demonstrated focal areas of ductular proliferation and periportal ballooning degeneration without bile duct paucity. The patient exhibited worsening cardiac and liver conditions, growth failure, and developmental delay. She died suddenly at home at the age of 34 months. The cholangiographic and histological abnormalities found in our patient were suggestive of BA. At the same time, she displayed four out of five diagnostic criteria for AS. Based on our experience with this case, we suggest expeditious genetic testing should be considered for any case of neonatal cholestasis with diagnostic uncertainty. This may help avoid unwarranted surgical interventions, potentially associated with worse outcomes.

Keywords: Alagille syndrome, Liver, Bile duct paucity, Cholestasis, Kasai procedure