Avascular necrosis (AVN) of the hip is a common cause of morbidity in sickle cell disease (SCD). Its prevalence increases with age and predisposing factors include coexistent α-thalassemia trait, frequent vaso-occlusive crisis and a high hematocrit (Hct). SCD is relatively mild among Kuwaiti patients because of their elevated Hb F levels, but a subset exists with severe recurrent vaso-occlusive crises. We carried out a prospective magnetic resonance imaging (MRI) study of the hip in a group of patients being followed in the Pediatric Hematology clinics of Al-Mubarak and Al-Amiri Hospitals. The association of AVN with age, frequency of hospitalization, α-thal trait, steady-state Hb, Hct, Hb F, WBC and platelet counts was investigated. MRI was carried out with a 1.5-tesla GE unit with a super-conducting magnet. Thirty patients (19 males, 11 females) (23 SS and 7 SβThal) were studied. Their ages ranged from 6 to 17 years, with a mean of 9.8 ± 3.5 years, and Hb F from 11 to 35% with a mean of 22.8 ± 5.7%. Among the SS patients, 11 (47.8%) had coexistent α-thal trait (–3.7-kb deletion). A total of 8 (26.7%) patients (6 SS and 2 SβThal) had varying degrees of osteonecrosis of the hip. Four (36.4%) of the 11 SS patients with α-thal trait and 2 (16.7%) of those without α-thal trait had osteonecrosis. This difference is, however, not statistically significant (χ2 = 0.3, p = 0.5). While there was also no significant difference in the mean age and hematological parameters (Hb, Hct, Hb F, WBC, platelets), the SS patients with osteonecrosis had a significantly higher number of hospitalizations for vaso-occlusive crisis in the preceding 3 years than those without osteonecrosis.
Overt stroke is rare among sickle cell disease (SCD) patients in Kuwait. However, there are no previous studies of silent cerebral infarcts, which have been described in up to 20% of American children with Hb SS. We have carried out a prospective brain MRI study among otherwise normal SCD patients, who were consecutive patients seen in a 1‐year period to document the prevalence of silent cerebral infarcts in children with sickle cell disease in Kuwait. Any patient with a previous seizure or other neurological abnormality was excluded. MRI was done with a 1.5 Tesla unit with super‐conducting magnet. T1‐ and T2‐weighted sagittal and axial sections and proton density axial images were obtained in 5‐mm thick sections. The study group consisted of 30 (23 SS and 7 Sβ0Thal) patients—19 males and 11 females—whose ages ranged from 6 to 17 (mean of 9.8 ± 3.5) years. Hb F ranged from 11% to 35% with a mean of 22.8% ± 5.7%. Only one patient, a 10‐and‐a‐half‐year‐old boy with Hb SS, showed hyperintense signals in the parietal white matter, consistent with small infarcts, thus giving a prevalence of 3.3%. Silent brain infarcts are uncommon in our patients, and the protective factors remain to be fully elucidated. Am. J. Hematol. 70:228–231, 2002. © 2002 Wiley‐Liss, Inc.
Objective: To report a case with early presentation of acute lymphoblastic leukemia (ALL) as bilateral renal masses and renal failure. Clinical Presentation and Intervention: A 6-year-old boy was admitted with bilaterally enlarged kidneys and severe renal impairment. Magnetic resonance imaging (MRI) showed bilateral renal enlargement with features suggestive of an infiltrative lesion. Accordingly, bone marrow examination was performed, and diagnosis of ALL was made. The patient developed acute renal failure after initiation of chemotherapy, so he received hemodialysis. His renal function normalized and kidney enlargement regressed. Conclusion: This case demonstrates an unusual early renal involvement in ALL in a child. MRI is a valuable imaging modality in the evaluation of renal masses.
Life-threatening arrhythmias have been variably reported among patients hospitalized for COVID-19 infection. Sudden cardiac arrest (SCA) in COVID-19 patients is an alarming concern for clinicians. Multiple factors play an important role in the development of SCA in patients with severe systemic illness. We describe a case of COVID-19 in a New York City hospital in Spring 2020 that rapidly developed SCA and, before discharge, received a single lead transvenous implantable cardioverter defibrillator for secondary prevention. This case highlights the use of an automated implantable cardioverter-defibrillator as a secondary preventive measure irrespective of left ventricular function as a means of preventing recurrence of SCA as a sequela of COVID-19.
The field of Cardio-oncology is rapidly growing with significant advances in research leading to better understanding of the underlying pathogenesis with implications in the diagnosis and management of cancer-related cardiomyopathy. Parallel to advancement in cardio-oncology is an increased awareness of the incidence of congestive heart failure and cardiomyopathy associated with malignancy. While specific cardiotoxic profiles exist for certain chemotherapeutic agents, there is increasing evidence of unexpected cardiotoxic side effects of some therapeutic modalities, combination chemo- and radiotherapy with large analyses identifying a strong association between malignancy and Takotsubo cardiomyopathy. Takotsubo Cardiomyopathy, also known as “broken-heart” syndrome or stress cardiomyopathy, is characterized by transient and reversible, regional or global, myocardial dysfunction without inciting ischemic perfusion defect from obstructive coronary artery disease. While direct causative pathophysiologic mechanisms continue to be investigated, much of the postulated pathways center on the high emotional and physical burdens of cancer and the related emotional stress associated with the diagnosis of cancer as well as the corporal effects of anti-neoplastic therapies, radiation, and oncologic surgery. In this manuscript we review the most current data in this rapidly emerging field highlighting the epidemiology, the postulated pathogenetic mechanisms as well as the current guidelines by major societies addressing malignancy -associated heart failure and cardiomyopathy, a rather complex disease entity with high morbidity and mortality.
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