Case 237: Renal Cell Carcinoma with Osseous Metaplasia.

History A 47-year-old man presented with palpitations and decreased exercise tolerance. A peripheral blood smear revealed anemia, thrombocytopenia, and blast cells, and a diagnosis of acute myeloid leukemia was made. Immunohistochemistry revealed positivity for cluster of differentiation (or CD) markers, which have been reported to be associated with an increased risk of extramedullary leukemic involvement. Thus, contrast material-enhanced computed tomography (CT) of the thorax, abdomen, and pelvis was requested to enable exclusion of any extramedullary extension of leukemia. Unenhanced and contrast-enhanced nephrographic phase CT was performed. Follow-up CT 3 months later showed minimal interval change in the lesion (images not shown).

High prevalence of sacroiliitis and early structural changes in the sacroiliac joint in children with enthesitis-related arthritis: findings from a tertiary centre in Hong Kong.

BACKGROUND: Epidemiological studies have demonstrated a wide, unexplained disparity in the prevalence of juvenile idiopathic arthritis (JIA) subtypes depending on geographical location, ethnicity and other factors. Enthesitis-related arthritis (ERA) is more prevalent in Southeast Asia. Axial involvement in ERA patients is increasingly recognised to occur early in the disease course. Inflammation in the sacroiliac joint (SIJ) observed on MRI seems highly predictive of subsequent structural radiographic progression. The resulting structural damage can have significant impacts on both functional status and spinal mobility. This study aimed to evaluate the clinical characteristics of ERA in a tertiary centre in Hong Kong. The primary objective of the study was to provide a comprehensive description of the clinical course and radiological findings of the SIJ among ERA patients. METHOD: Paediatric patients diagnosed with JIA attending the paediatric rheumatology clinic from January 1990 to December 2020 were recruited from our registry based at the Prince of Wales Hospital. RESULTS: In our cohort, 101 children were included. The median age of diagnosis was 11 years, interquartile range (IQR) 8-15 years. The median follow-up duration was 7 years (IQR 2-11.5 years). ERA was the most prevalent subtype (40%), followed by oligoarticular JIA (17%). Axial involvement was frequently reported in our cohort of ERA patients. 78% demonstrated radiological evidence of sacroiliitis. Among those, 81% had bilateral involvement. The median duration from disease onset to confirmation of radiological sacroiliitis was 17 months (IQR 4-62 months). Among the ERA patients, 73% had structural changes of the SIJ. Alarmingly, 70% of these patients had already developed radiological structural changes when sacroiliitis was first detected on imaging (IQR 0-12 months). Erosion was the most common finding (73%), followed by sclerosis (63%), joint space narrowing (23%), ankylosis (7%) and fatty change (3%). The duration from symptom onset to diagnosis was significantly longer in ERA patients with SIJ structural changes (9 vs 2 months, p = 0.009), comparing with those without. CONCLUSION: We found that a high proportion of ERA patients had sacroiliitis and a significant number of them had radiological structural changes during early disease. Our findings illustrate the importance of prompt diagnosis and early treatment in these children.

Focal cerebral arteriopathy-inflammatory type in a child - MR diagnosis using vessel wall imaging technique with review of classification and diagnostic evaluation criteria.

Acute ischemic stroke (AIS) in childhood is defined by a stroke occurring after 28 days of life to 18 years of age. This presents a distinct clinical challenge in terms of both diagnosis and treatment. The overlapping clinical presentations of acute ischemic stroke and its mimics such as migraine with aura, seizure with Todd paresis and encephalitis renders early accurate diagnosis of this time-sensitive condition difficult, with a change in the final diagnosis in up to 40% of patients. Identification of the etiology after establishing the diagnosis of ischemic stroke is paramount for prognostication and treatment decisions. These include cardioembolic, arteriopathy, thrombophilia and inflammatory causes. Magnetic resonance imaging (MRI) plays an indispensable role towards tackling the initial diagnostic dilemma and subsequent evaluation of the underlying etiology, particularly in patients with arteriopathy. Here we present the MRI findings including vessel wall imaging with longitudinal follow-up, which support the diagnosis of focal cerebral arteriopathy-inflammatory type (FCAi) in a pediatric patient.

Predictors of the success and failure of emergency pelvic artery embolisation for primary postpartum haemorrhage: a 12-year review.

INTRODUCTION: This study aimed to identify predictors of the outcome and clinical efficacy of emergency pelvic artery embolisation (PAE) for primary postpartum haemorrhage (PPH) and to assess the post-embolisation fertility of PAE patients in a regional hospital setting. METHODS: A 12-year retrospective study of patients undergoing emergency PAE was conducted at a regional acute general hospital. Clinical and procedural parameters, clinical outcomes and post-embolisation pregnancy success rates were analysed. RESULTS: There were 47,221 deliveries at the hospital during the study period, of which 33 patients required urgent PAE for primary PPH. The technical success rate of embolisation was 97.0% (n = 32). Clinically adequate haemostasis was achieved by a single embolisation procedure in 24 (72.7%) patients; the remaining eight eventually required surgery to achieve cessation of bleeding. Among the parameters studied, multivariate logistic regression analysis showed that pre-embolisation platelet count (p = 0.036) and maternal age (p = 0.019) were the only significant independent predictors of embolisation failure. Only two patients successfully conceived after PAE, although one of them had an ectopic pregnancy. CONCLUSION: Emergency PAE is an effective measure to arrest life-threatening bleeding in patients with primary PPH. As low pre-embolisation platelet count and advanced maternal age are associated with higher odds of embolisation failure, careful post-embolisation monitoring may be required for such patients. Embolisation also allows subsequent pregnancy. However, further studies are required to assess the outcomes of post-embolisation pregnancies.