Case of cerebral venous thrombosis on a patient with background of nephrotic syndrome.

Hypercoagulability is a well-described feature of nephrotic syndrome. The risk of developing a venous thrombus is higher at the time of diagnosis or shortly after. The resulting deep vein thrombosis involves the pulmonary, the deep veins of the lower limbs and renal veins, as described in the literature. We present a case of a man in his 20s with background of nephrotic syndrome, diagnosed at an age of 3 years old, with multiple relapses and on maintenance immunosuppression which is unusual, in two respects: First, the site of thrombosis was in the cerebral venous sinus and second, the onset of the thrombotic episode was years after the initial diagnosis. This case report also focuses on the perspective of the patient, who experienced a rare complication after more than two decades of living with the condition. In a literature search with the search words of 'nephrotic syndrome' AND 'cerebral venous thrombosis in adults', written in English and published from 1970 to 2/2021, we could only find a review article including 5 cases and 10 individual case reports, of which there were only 16 number of cerebral sinus venous thrombosis reported.

Volumetric Imaging of Lung Tissue at Micrometer Resolution: Clinical Applications of Micro-CT for the Diagnosis of Pulmonary Diseases.

Micro-computed tomography (micro-CT) is a promising novel medical imaging modality that allows for non-destructive volumetric imaging of surgical tissue specimens at high spatial resolution. The aim of this study is to provide a comprehensive assessment of the clinical applications of micro-CT for the tissue-based diagnosis of lung diseases. This scoping review was conducted in accordance with the PRISMA Extension for Scoping Reviews, aiming to include every clinical study reporting on micro-CT imaging of human lung tissues. A literature search yielded 570 candidate articles, out of which 37 were finally included in the review. Of the selected studies, 9 studies explored via micro-CT imaging the morphology and anatomy of normal human lung tissue; 21 studies investigated microanatomic pulmonary alterations due to obstructive or restrictive lung diseases, such as chronic obstructive pulmonary disease, idiopathic pulmonary fibrosis, and cystic fibrosis; and 7 studies examined the utility of micro-CT imaging in assessing lung cancer lesions (n = 4) or in transplantation-related pulmonary alterations (n = 3). The selected studies reported that micro-CT could successfully detect several lung diseases providing three-dimensional images of greater detail and resolution than routine optical slide microscopy, and could additionally provide valuable volumetric insight in both restrictive and obstructive lung diseases. In conclusion, micro-CT-based volumetric measurements and qualitative evaluations of pulmonary tissue structures can be utilized for the clinical management of a variety of lung diseases. With micro-CT devices becoming more accessible, the technology has the potential to establish itself as a core diagnostic imaging modality in pathology and to enable integrated histopathologic and radiologic assessment of lung cancer and other lung diseases.

Langerhans cell histiocytosis with spinal, pulmonary and pituitary involvement: What about ACTH deficiency without diabetes insipidus? A propos of a case.

Langerhans cell histiocytosis (LCH) is disease process characterized by clonal proliferation of CD1a+ dendritic cells within an inflammatory infiltrate of hematopoietic derived cells. LCH can manifest with a broad spectrum of symptoms and can involve single organs or have a multisystem distribution. Central nervous system (CNS) involvement of LCH can manifest as granulomatous parenchymal or pituitary mass lesions. Focal, space-occupying lesions, such as masses in the meninges, choroid plexus, and brain parenchyma may contain CD1a+ LCH cells, lymphocytes, and macrophages with histology similar to that of extracranial lesions. Here, we describe a rare case of multisystem LCH in an adult patient presenting with spinal lesions and isolated adrenocorticotropic (ACTH) deficiency without diabetes insipidus (DI). In addition, we review the literature summarizing the few reports of hypopituitarism in LCH in the absence of DI.

Magnetic resonance spectroscopy assessment of brain injury after moderate hypothermia in neonatal encephalopathy: a prospective multicentre cohort study.

BACKGROUND: In neonatal encephalopathy, the clinical manifestations of injury can only be reliably assessed several years after an intervention, complicating early prognostication and rendering trials of promising neuroprotectants slow and expensive. We aimed to determine the accuracy of thalamic proton magnetic resonance (MR) spectroscopy (MRS) biomarkers as early predictors of the neurodevelopmental abnormalities observed years after neonatal encephalopathy. METHODS: We did a prospective multicentre cohort study across eight neonatal intensive care units in the UK and USA, recruiting term and near-term neonates who received therapeutic hypothermia for neonatal encephalopathy. We excluded infants with life-threatening congenital malformations, syndromic disorders, neurometabolic diseases, or any alternative diagnoses for encephalopathy that were apparent within 6 h of birth. We obtained T1-weighted, T2-weighted, and diffusion-weighted MRI and thalamic proton MRS 4-14 days after birth. Clinical neurodevelopmental tests were done 18-24 months later. The primary outcome was the association between MR biomarkers and an adverse neurodevelopmental outcome, defined as death or moderate or severe disability, measured using a multivariable prognostic model. We used receiver operating characteristic (ROC) curves to examine the prognostic accuracy of the individual biomarkers. This trial is registered with ClinicalTrials.gov, number NCT01309711. FINDINGS: Between Jan 29, 2013, and June 25, 2016, we recruited 223 infants who all underwent MRI and MRS at a median age of 7 days (IQR 5-10), with 190 (85%) followed up for neurological examination at a median age of 23 months (20-25). Of those followed up, 31 (16%) had moderate or severe disability, including one death. Multiple logistic regression analysis could not be done because thalamic N-acetylaspartate (NAA) concentration alone accurately predicted an adverse neurodevelopmental outcome (area under the curve [AUC] of 0·99 [95% CI 0·94-1·00]; sensitivity 100% [74-100]; specificity 97% [90-100]; n=82); the models would not converge when any additional variable was examined. The AUC (95% CI) of clinical examination at 6 h (n=190) and at discharge (n=167) were 0·72 (0·65-0·78) and 0·60 (0·53-0·68), respectively, and the AUC of abnormal amplitude integrated EEG at 6 h (n=169) was 0·73 (0·65-0·79). On conventional MRI (n=190), cortical injury had an AUC of 0·67 (0·60-0·73), basal ganglia or thalamic injury had an AUC of 0·81 (0·75-0·87), and abnormal signal in the posterior limb of internal capsule (PLIC) had an AUC of 0·82 (0·76-0·87). Fractional anisotropy of PLIC (n=65) had an AUC of 0·82 (0·76-0·87). MRS metabolite peak-area ratios (n=160) of NAA-creatine (<1·29) had an AUC of 0·79 (0·72-0·85), of NAA-choline had an AUC of 0·74 (0·66-0·80), and of lactate-NAA (>0·22) had an AUC of 0·94 (0·89-0·97). INTERPRETATION: Thalamic proton MRS measures acquired soon after birth in neonatal encephalopathy had the highest accuracy to predict neurdevelopment 2 years later. These methods could be applied to increase the power of neuroprotection trials while reducing their duration. FUNDING: National Institute for Health Research UK.

Multidetector CT angiography characterisation of Type 4 dual left anterior descending coronary artery.

Duplication of the left anterior descending (LAD) coronary artery is a rare anomaly (incidence 1%) characterized by a short LAD that terminates high in the anterior interventricular groove and a long LAD that has a proximal course outside the anterior interventricular groove and returns to the groove in its distal course. The Type 4 variant of this anomaly is extremely rare whereby a long LAD arises from the right coronary sinus with a short LAD arising from the left mainstem. We present a case of Type 4 dual LAD which was characterised with multi-detector computed tomography (CT).

Cardiac inflammatory myofibroblastic tumor: evaluation with dual-source CT.

We present a case of inflammatory myofibroblastic tumor that was evaluated with retrospectively gated dual-source cardiac CT. Imaging features included a broad-based attachment, lobulated contour, and patchy areas of contrast medium enhancement that persisted on delayed imaging. Inflammatory myofibroblastic tumor is an extremely rare slow-growing but locally invasive neoplasm. Cardiac CT has an emerging role in the evaluation of cardiac masses, permitting detailed assessment of lesion extent and relationships. Delayed-enhancement imaging can help with tissue characterization.