Impact of COVID-19 on vasooclusive crisis in patients with sickle cell anaemia.

OBJECTIVES: The study aimed to assess COVID-19 impact on the morbidity and mortality of vasooclusive crisis (VOC) in sickle cell anaemia (SCA) patients. METHODS: A prospective cohort study of 100 SCA patients; 50 with COVID-19 (COVID group) and 50 without (non-COVID group). All patients signed written informed consent. RESULTS: The COVID group had a significantly higher VOC episode median per year; 3 (IQR,1-6) vs 2 (IQR,2-12) (P < 0.05). The need for hospitalisation was similar in both groups. The non-COVID group had more history of culture-proven infection (P = 0.05). The COVID-group had more osteonecrosis (P < 0.05), splenic sequestration, splenomegaly and hepatic crisis (P = 0.05, 0.006, 0.02; respectively) and significantly higher (P < 0.05) symptoms of fever, cough, fatigue, abdominal pain and anosmia. Mean haemoglobin, lymphocyte subset, platelets, and reticulocytes were reduced in both groups, while lactate dehydrogenase and ferritin levels were significantly elevated. In the COVID group, the rise in white blood cell count, reticulocyte percentage, platelets and ferritin was subdued (P < 0.05). Two patients in the COVID group and 3 in the non-COVID group died; there was no statistically significant difference in mortality. CONCLUSIONS: Although COVID-19 may have triggered the onset of VOC, it did not significantly influence VOC-related morbidity or mortality in this SCA cohort.

Cardiovascular function in Omani children with sickle cell anaemia.

Systolic murmurs were detected in 22 (61%) of the 36 children with sickle cell anaemia (SCA) who completed the study. Cardiomegaly was detected in 14 (39%). Mean values of left and right ventricular dimensions were higher in SCA than in controls (p < 0.05). Left atrial chambers and aortic root dimensions followed the same pattern. The dilated cardiac chambers in SCA were not associated with any abnormality in systolic or diastolic left ventricular function nor with significant pulmonary hypertension.

Adenotonsillar hypertrophy: a precipitating factor of cerebrovascular accident in a child with sickle cell anemia.

Cerebrovascular accident is one of the most serious complications of sickle cell anemia. The specific factors that predispose patients with sickle cell anemia to stroke are increased disease severity, higher baseline white blood cell count and lower baseline hematocrits. Likewise the presence of a co-existent alpha thalassemia trait and/or high fetal hemoglobin (HbF%) may reduce the risk. We report a child with sickle cell anemia and marked adenotonsillar hypertrophy resulting in obstructive sleep apnea syndrome. There was no other known risk factor for developing cerebrovascular accident in this child during her hospitalization for adenotonsillectomy.

A prospective study of soft-tissue ultrasonography in sickle cell disease patients with suspected osteomyelitis.

AIM: A prospective study was done to assess the accuracy of soft tissue ultrasonography in patients with sickle cell disease (SCD) presenting with suspected osteomyelitis. MATERIALS AND METHODS: Thirty-one SCD patients had soft tissue ultrasonography on 38 occasions (18 men, 13 women; mean age 8.2 years). The initial ultrasonographic signs and diagnosis were compared with the final clinical diagnosis, which was based on clinical progress and scintigraphy. RESULT: The overall sensitivity of ultrasound in diagnosing osteomyelitis was 74% with a specificity of 63%. The principal ultrasonographic finding of subperiosteal fluid was present in 14 (74%) patients with osteomyelitis and seven (37%) patients without infection. A finding of a subperiosteal fluid depth of 4 mm or more was significantly associated with osteomyelitis (P < 0.01). CONCLUSION: Ultrasonography should be the initial investigation in SCD patients if osteomyelitis is suspected clinically. In such a clinical setting, a finding of 4 mm depth or more of subperiosteal fluid appears to be diagnostic. Previous statements that the presence of any subperiosteal fluid indicates infection are shown to be inaccurate. Patients with less than 4 mm of subperiosteal fluid require further imaging or aspiration to establish the diagnosis of osteomyelitis.

Case report. Successful outcome of invasive nasal sinus zygomycosis in a child with relapsed acute lymphoblastic leukaemia due to liposomal amphotericin B.

We report a case of life-threatening nasal sinus zygomycosis that developed during remission induction therapy for a relapsed acute lymphoblastic leukaemia. The patient was successfully treated with liposomal amphotericin B and granulocyte-colony stimulating factor followed by surgical reconstruction of the resultant cutaneous defect.