Hydroxyurea: Pattern of Use, Patient Adherence, and Safety Profile in Patients with Sickle Cell Disease in Oman.

OBJECTIVES: Many barriers contribute to the underutilization of hydroxyurea (HU) in the treatment of sickle cell disease (SCD), and adherence to its use is often reported to be suboptimal. It is important to have information on the safety of HU in patients with SCD. Our study assessed the pattern of use, patients' adherence to medication, discontinuation of use, and safety of HU in patients with SCD. METHODS: This cross-sectional study was conducted in the department of medicine of a referral hospital in Oman over five months and included a review of patient files and patient interview. Approval was obtained from the Regional Research and Ethics Committee of the A'Dakhiliyah Governorate and the hospital administration. The parameters were compared between groups using the chi-square test. RESULTS: Of 298 patients studied, 128 (43.0%) had used HU at some points. The difference in the prevalence of HU use was statistically significant based only on age (p = 0.014), with younger patients more likely to be currently using HU or used HU in the past. The majority of patients were adherent (82.5%) based on self-reported adherence. The prevalence of discontinuation (temporary or permanent) of HU use was high (57.0%), and suspected adverse drug reaction (ADR) was the most common reason. Among those who had never used HU, 33.7% of patients had an indication for the initiation of HU. A quarter of patients who used HU developed a suspected ADR, with blood abnormalities being the commonest. The duration of HU use influenced ADR prevalence (p = 0.015). CONCLUSIONS: Among the current users of HU, the majority of the patients were adherent based on self-reported medication adherence. The prevalence of discontinuation of HU use and instances of non-initiation of HU among those indicated were high. A larger study, ideally of a prospective nature, in various governorates of Oman, would provide a wider picture at the national level.

Colloid cyst of the third ventricle presenting with features of Terson's syndrome.

This report describes a middle-aged man presenting to the ophthalmologist with history of seeing floaters before both eyes since 2-weeks duration. A history of intermittent headache and dizziness of recent onset was elicited on questioning. Ocular examination showed bilateral early papilloedema and mild vitreous hemorrhage. Brain computed tomography (CT) disclosed features suggestive of colloid cyst of the third ventricle in the region of foramen of Monro with moderate hydrocephalus. Emergency craniotomy and excision of the cyst was done, and the patient is doing well for the last 18 months after the surgical intervention. The mechanism of this presentation, importance of early investigations, and timely intervention are highlighted in order to avoid serious neurological sequelae. The literature was extensively reviewed for atypical presentations of intraventricular colloid cyst.

Blurred vision as the sole initial presentation of disseminated lung malignancy.

Symptomatic choroidal metastasis (CM) is a rare presenting feature of disseminated lung malignancy. Detection of the primary malignancy usually precedes ocular findings. We report a rare case of blurred vision secondary to bilateral CM as the sole initial manifestation of disseminated lung malignancy in a female patient. Pulmonary symptoms appeared 2 months later. She received radiotherapy and palliative chemotherapy and survived for 7 months after the initial presentation.

Adult Onset Still's Disease in Oman.

OBJECTIVE: To 1. Highlight the demographic characteristics, clinical features, laboratory investigations and outcome if possible of a relatively rare disease (Adult Onset Still's Disease {AOSD}) and 2. To compare our results with those reported earlier by others. METHOD: A retrospective review of the clinical, laboratory and radiological manifestations in 6 patients admitted with AOSD. Data were collected from clinical summary of each case highlighting the demographic, clinical features and relevant investigation. The diagnosis was made on clinical base and supported by the laboratory and radiological examinations to confirm the diagnosis and/or exclude other differential diagnoses. RESULTS: Mean age of patients (21.6), presence of fever (80.2%) and its pattern, skin rash (80.2%), arthralgia(100%), arthritis(66.65%), and the duration of morning stiffness, all these findings were compatible with earlier results. However young adult females constitute (80.2%) of our small group, which is quite high in comparison with others. Splenomegaly (33.3%), hepatomegaly(16.6%), and lymphadenopathy(16.6%) were less than that reported by others who have more serious intra-abdominal visceral involvement. One patient (16.65%) had a fatal pulmonary embolism, although usually pulmonary involvement is a mild one. None of our patients had neurological, ophthalmological or hearing involvement. The results of the investigations and radiological findings are more or less similar to those in other series. CONCLUSION: The clinical and laboratory characteristics of our small number of patients are more or less consistent with findings of others. It is important to keep in mind that AOSD is an uncommon syndrome with a range of signs and symptoms which are non-specific and may simulate a variety of connective tissue and general medical problems. Differences in the expression of AOSD were found between patients from different locations and the disease can be responsible for life-threatening complications.