Coronavirus disease 2019 convalescent children: outcomes after congenital heart surgery.

BACKGROUND: Children with exposure to coronavirus disease 2019 in recent times (asymptomatic or symptomatic infection) approaching congenital heart surgery programme are in increasing numbers. Understanding outcomes of such children will help risk-stratify and guide optimisation prior to congenital heart surgery. OBJECTIVE: The objective of the present study was to determine whether convalescent coronavirus disease 2019 children undergoing congenital heart surgery have any worse mortality or post-operative outcomes. DESIGN: Consecutive children undergoing congenital heart surgery from Oct 2020 to May 2021 were enrolled after testing for reverse transcription-polymerase chain reaction or rapid antigen test and immunoglobulin G antibody prior to surgery. Convalescent coronavirus disease 2019 was defined in any asymptomatic patient positive for immunoglobulin G antibodies and negative for reverse transcription-polymerase chain reaction or rapid antigen test anytime 6 weeks prior to surgery. Control patients were negative for any of the three tests. Mortality and post-operative outcomes were compared among the groups. RESULTS: One thousand one hundred and twenty-nine consecutive congenital heart surgeries were stratified as convalescence and control. Coronavirus disease 2019 Convalescent (n = 349) and coronavirus disease 2019 control (n = 780) groups were comparable for all demographic and clinical factors except younger and smaller kids in control. Convalescent children had no higher mortality, ventilation duration, ICU and hospital stay, no higher support with extracorporeal membrane oxygenation, high flow nasal cannula, no higher need for re-intubations, re-admissions, and no higher infections as central line-associated bloodstream infection, sternal site infection, and ventilator-associated pneumonia on comparison with coronavirus disease 2019 control children. CONCLUSIONS: Convalescent coronavirus disease 2019 does not have any unfavourable outcomes as compared to coronavirus disease 2019 control children. Positive immunoglobulin G antibody screening prior to surgery is suggestive of convalescence and supports comparable outcomes on par with control peers.

An Update on the Management of Budd-Chiari Syndrome.

Budd-Chiari syndrome (BCS) is an uncommon condition, caused by obstruction to hepatic venous outflow. It is largely underdiagnosed, and a high index of suspicion is required for any patient with unexplained portal hypertension. The understanding of its etiology and pathology is improving with advances in diagnostic techniques. Recent studies reported an identifiable etiology in > 80% of cases. Myeloproliferative neoplasm (MPN) is the most common etiology, and genetic studies help in diagnosing latent MPN. Better cross-sectional imaging helps delineate the site of obstruction accurately. The majority of BCS patients are now treated by endovascular intervention and anticoagulation which have improved survival in this disease. Angioplasty of hepatic veins/inferior vena cava remains under-utilized at present. While surgical porto-systemic shunts are no longer done for BCS, liver transplantation is reserved for select indications. Some of the unresolved issues in the current management of BCS are also discussed in this review.

A new and simplified comprehensive ultrasound protocol of haemophilic joints: the Universal Simplified Ultrasound (US-US) protocol.

AIM: To present a new protocol to optimise ultrasound (US) assessment of haemophilic arthropathy. MATERIALS AND METHODS: Ultrasound of haemophilic arthropathy joints was performed using three different ultrasound protocols, namely, the Toronto-Vellore Comprehensive Ultrasound (TVC-US) protocol, the Haemophilia Early Arthropathy Detection with Ultrasound (HEAD-US), and the newly developed Universal Simplified Ultrasound (US-US) protocol. Synovial hypertrophy, haemosiderin deposition, effusion, erosion, and cartilage loss were evaluated in 20 joints. The reliability and diagnostic efficiency of these protocols was compared using magnetic resonance imaging (MRI). RESULTS: The correlation between the TVC-US and US-US protocols for synovial hypertrophy was excellent: kappa significance (KS) was 1, but was substantial (KS=0.65) with the HEAD-US protocol. For effusion, both the TVC-US and the HEAD-US protocols had substantial correlation with the US-US protocol (KS=0.7 and 0.6 respectively). The correlation for erosion and cartilage loss was excellent between the TVC-US and the US-US with MRI (KS=1), but poor (KS=0) with the HEAD-US protocol. The US-US protocol also had good interobserver agreement (KS=1). CONCLUSION: The accuracy of the US-US protocol is comparable to the TVC-US protocol and MRI and is superior to the HEAD-US protocol in the assessment of haemophilic arthropathy.

Correlating clinical and radiological assessment of joints in haemophilia: results of a cross sectional study.

OBJECTIVES: This study was undertaken to determine the correlation between the radiological changes in haemophilic arthropathy [X-ray, Ultrasound (US) and MRI] and clinical assessment as determined by the Hemophilia Joint Health Score (HJHS); and to document the US and MRI changes in joints that appear normal on plain X-ray and clinical evaluation. MATERIALS AND METHODS: Of 55 study joints (22 knees and 33 ankles) in 51 patients with haemophilia/von Willebrand disease, with a median age of 15 years (range: 5-17) were assessed using X-rays (Pettersson score) and clinical examination (HJHS) at two centres (Toronto, Canada; Vellore, India). MRI and ultrasonographic scoring was done through a consensus assessment by imagers at both centres using the IPSG MRI and US scores. RESULTS: The HJHS had a good correlation with the Pettersson score (rs = 0.66). Though the HJHS had moderate correlation with the osteochondral component of the MRI and US scores (rs 0.51, 0.45 respectively), its correlation with the soft tissue component was poor (rs 0.19; 0.26 respectively). Of the 18 joints with a Pettersson score of zero, 88.9% had changes that were detected clinically by the HJHS. Osteochondral abnormalities were identified in 38.9% of these joints by the MRI, while US images of the same joints were deemed abnormal in 83.3% by the current criteria. US identified haemosiderin and other soft tissue changes in all of the joints, while the same changes were noted in 94.4% of these joints on MRI. There were four joints with a HJHS of zero, all of which had soft tissue changes on MRI (score 1-7) and US (score 2-7). Osteochondral changes were detected in three of these joints by US and in 2 by MRI. There were four joints with an MRI score of 0-1 that had significant US scores (3-5) and HJHS scores (0-6). CONCLUSION: US and MRI are able to identify pathological changes in joints with normal X-ray imaging and clinical examination. However, further studies are required to be able to differentiate early abnormalities from normal. Clinical (HJHS) and radiological assessment (US/MRI) provide complimentary information and should be considered conjointly in the assessment of early joint arthropathy.

Pharmacological concentrations of recombinant factor VIIa restore hemostasis independent of tissue factor in antibody-induced hemophilia mice.

UNLABELLED: ESSENTIALS: The role of tissue factor (TF) in recombinant factor VIIa (rFVIIa) therapy in hemophilia is unclear. An acquired mouse hemophilia model with very low or normal levels of human TF was used in the study. rFVIIa is equally effective in correcting the bleeding in mice expressing low or normal levels of TF. Pharmacological doses of rFVIIa restore hemostasis in hemophilia independent of TF. BACKGROUND: Recombinant factor VIIa (rFVIIa) has been used widely for treating hemophilia patients with inhibitory autoantibodies against factor VIII or IX. Its mechanism of action is not entirely known. A majority of in vitro studies suggested that pharmacological concentrations of rFVIIa restore hemostasis in hemophilia in a phospholipid-dependent manner, independent of tissue factor (TF). However, a few studies suggested that a TF-dependent mechanism has a primary role in correction of bleeding by rFVIIa in hemophilia patients. Here, we investigated the potential contribution of TF in rFVIIa-induced hemostasis in hemophilia employing a model system of FVIII antibody-induced hemophilia in TF transgenic mice. METHODS: Mice expressing low levels of human TF (LTF mice), mice expressing relatively high levels of human TF (HTF mice) and wild-type mice (WT mice) had neutralizing anti-FVIII antibodies administered in order to induce hemophilia in these mice. The mice were then treated with varying concentrations of rFVIIa. rFVIIa-induced hemostasis was evaluated with the saphenous vein bleeding model. RESULTS: Administration of FVIII inhibitory antibodies induced the hemophilic bleeding phenotype in all three genotypes. rFVIIa administration rescued the bleeding phenotype in all three genotypes. No significant differences were observed in rFVIIa-induced correction of bleeding between LTF and HTF mice that had FVIII antibodies administered. CONCLUSIONS: Our results provide strong evidence supporting the suggestion that the hemostatic effect of pharmacological doses of rFVIIa stems from a TF-independent mechanism.

Restoring cigarette smoke-induced impairment of efferocytosis in alveolar macrophages.

Cigarette smoke has been associated with susceptibility to different pulmonary and airway diseases. Impaired alveolar macrophages (AMs) that are major phagocytes in the lung have been associated with patients with airway diseases and active smokers. In the current report, we show that exposure to second-hand cigarette smoke (SHS) significantly reduced efferocytosis in vivo. More importantly, delivery of recombinant granulocyte-macrophage colony-stimulating factor (GM-CSF) to the alveolar space restored and refurbished the efferocytosis capability of AMs. Exposure to SHS significantly reduced expression of CD16/32 on AMs, and treatment with GM-CSF not only restored but also significantly increased the expression of CD16/32 on AMs. GM-CSF treatment increased uptake and digestion/removal of apoptotic cells by AMs. The latter was attributed to increased expression of Rab5 and Rab7. Increased efferocytosis of AMs was also tested in a disease condition. AMs from GM-CSF-treated, influenza-infected, SHS-exposed mice showed significantly better efferocytosis activity, and mice had significantly less morbidity compared with phosphate-buffered saline-treated group. GM-CSF-treated mice had increased amphiregulin levels in the lungs, which in addition to efferocytosis of AMs may have attributed to their protection against influenza. These results will have great implications for developing therapeutic approaches by harnessing mucosal innate immunity to treat lung and airway diseases and protect against pneumonia.

Ultrasound and magnetic resonance imaging of healthy paediatric ankles and knees: a baseline for comparison with haemophilic joints.

The study was undertaken to document cartilage and soft tissue changes/findings in ankles and knees of normal children of different age groups to be used for comparison in the assessment of children with haemophilia. Cartilage thickness and soft tissue changes were recorded at predetermined sites of ankles/knees on both US and MRI in healthy boys in three age groups: 7-9; 10-14; and 15-18 years. To assess the validity of the ultrasound and MRI measurements, an ex vivo study was done using agar phantoms with techniques and scanners similar to those applied in vivo. Twenty (48%) knees and 22 (52%) ankles of 42 boys, were evaluated. There was a reduction in the thickness of joint cartilage with age. A difference in cartilage measurements was noted in most sites between the age groups on both US and MRI (P < 0.05 each), but such difference was not noted for joint fluid in ankles or knees (P = 0.20, P = 0.68 or P = 0.75, P = 0.63 for US, MRI, respectively). Although cartilage measurements were smaller on US than on MRI for both ankles and knees (P < 0.05 each), this observation was not recorded for fluid in knees (P = 0.02). For diminutive measurements (2 mm) mean US measurements were smaller than corresponding phantom's measurements, P = 0.02. Age-related measurements were noted for cartilage thickness on US and MRI in ankles and knees. US measurements were smaller than corresponding MRI measurements at most joint sites, which were supported by results on small-diameter phantoms.

Fine needle aspiration cytology of male breast lesions - a retrospective study over a six year period.

BACKGROUND: Fine needle aspiration cytology (FNAC) has a well-established role in the management of palpable breast lumps. However breast masses in males are rarely aspirated and hence there is limited cytopathologic experience. The aim of our study was to determine the efficacy of FNAC in the diagnosis of male breast lesions and also we attempted to describe the cytomorphological features of some of these lesions. MATERIALS AND METHODS: Data on male breast FNAC done between 2008 to 2013 were retrieved from the records of the cytopathology laboratory. FNAC diagnosis were categorized as benign, malignant, suspicious for malignancy and inadequate or unsatisfactory. Cytohistologic correlation was done with data from histopathology records. Sensitivity, specificity and diagnostic accuracy were calculated using standard statistical methods. RESULTS: Forty out of 1098 patients undergoing breast FNAC were males. Histopathology was available in 8 (20%) out of 40 cases. There were no false positive or false negative diagnoses. FNAC had a sensitivity, specificity and diagnostic accuracy of 100% for male breast lesions. CONCLUSION: FNAC is a very accurate tool for the diagnosis of male breast lesions. It is highly sensitive and specific with good cytohistologic correlation. To reduce the high rate of surgical biopsies of benign male breast masses, we conclude that FNAC should be performed as a standard procedure in the clinical evaluation of male breast lesions.

Aortic coarctation associated with an absent segment of the proximal right subclavian artery.

Coarctation of the aorta is a congenital anomaly of the thoracic aorta with many known associations. We describe the case of a young man referred for management of subarachnoid hemorrhage, in whom subsequent work-up revealed the previously undescribed combination of severe postductal aortic coarctation and an absent segment of the proximal right subclavian artery.

Trans-caval trans-jugular liver biopsy--a technical modification of trans-jugular liver biopsy.

OBJECTIVE: To (a) describe the technical modification of trans-caval TJLB and (b) review our series of nine cases. METHODS: We performed a retrospective review of all trans-caval TJLBs performed; we assessed indications for the procedure, technical success, complications, adequacy of specimen and histological positivity. RESULTS: The technical success rate of the procedure was 9/9 (100%); the minor complication rate was 1/9 (11%), adequate specimen was obtained in all cases and a histological diagnosis was achieved in 8/9 (89%) cases. CONCLUSION: This preliminary report suggests that trans-caval modification of TJLB is a relatively safe procedure that may be useful in cases where conventional TJLB is infeasible. ADVANCES IN KNOWLEDGE: (a) We describe the technique of trans-caval TJLBs and report our findings in the largest series of published cases. (b) Trans-caval TJLB is relatively safe and can be used to increase the success rates of conventional TJLB.

Factor VIIa binding to endothelial cell protein C receptor protects vascular barrier integrity in vivo.

BACKGROUND: Recent studies have shown that factor VIIa binds to endothelial cell protein C receptor(EPCR), a cellular receptor for protein C and activated protein C. At present, the physiologic significance of FVIIa interaction with EPCR in vivo remains unclear. OBJECTIVE: To investigate whether exogenously administered FVIIa, by binding to EPCR, induces a barrier protective effect in vivo. METHODS: Lipopolysaccharide(LPS)-induced vascular leakage in the lung and kidney,and vascular endothelial growth factor (VEGF)-induced vascular leakage in the skin, were used to evaluate the FVIIa-induced barrier protective effect. Wild-type, EPCR-deficient, EPCR-overexpressing and hemophilia A mice were used in the studies. RESULTS: Administration ofFVIIa reduced LPS-induced vascular leakage in the lung and kidney; the FVIIa-induced barrier protective effect was attenuated in EPCR-deficient mice. The extent of VEGF-induced vascular leakage in the skin was highly dependent on EPCR expression levels. Therapeutic concentrations of FVIIa attenuated VEGF-induced vascular leakage in control mice but not in EPCR-deficient mice.Blockade of FVIIa binding to EPCR with a blocking mAb completely attenuated the FVIIa-induced barrier protective effect. Similarly, administration of protease activated receptor 1 antagonist blocked the FVIIa induced barrier protective effect. Hemophilic mice showed increased vascular permeability, and administration of therapeutic concentrations of FVIIa improved barrier integrity in these mice. CONCLUSIONS: This is the first study to demonstrate that FVIIa binding to EPCR leads to a barrier protective effect in vivo. This finding may have clinical relevance, as it indicates additional advantages of using FVIIa in treating hemophilic patients.

Evaluation of normal abdominal aortic diameters in the Indian population using computed tomography.

OBJECTIVES: The aim of this study was to establish normal diameters for the suprarenal and infrarenal abdominal aorta measured at T12 and L3 vertebral levels in the Indian population and to study the variation in aortic diameters with age, sex, height, weight, body mass index (BMI), and body surface area (BSA). MATERIALS AND METHODS: One hundred and forty-two patients who underwent helical contrast-enhanced computed tomography (CT) scans of the abdomen for non-cardiovascular reasons were recruited.. The mean internal diameters of the suprarenal and infrarenal abdominal aorta (maximum anteroposterior and transverse diameter) were measured at T12 and L3 vertebral levels and tabulated according to various age groups for both men and women. Pearson correlation coefficient was used to evaluate the correlation between aortic diameters, height, weight, BSA, and BMI. RESULTS: The mean diameters of the suprarenal and infrarenal abdominal aorta measured at T12 and L3 vertebral levels, in men were 19.0 ± 2.3 and 13.8 ± 1.9 mm and in women 17.1 ± 2.3 and 12.0 ± 1.6 mm, respectively. The aortic diameter progressively increased in caliber with increasing age of the patients and was smaller in women than men. A significant positive correlation was found in men between the suprarenal and infrarenal aortic diameters and weight, BSA, and BMI. In women, this correlation was significant in the infrarenal aorta but not in the suprarenal aorta. CONCLUSION: We obtained a set of normal values for the abdominal aorta in the Indian population. The aortic diameters correlated with age, gender, and body size of the patients as seen with previously published data in the Western population. A brief comparison of data between Indian and Western population showed that the values obtained were less than published elsewhere and hence, this should be considered while formulating intervention protocols.

MRI in patients with tuberculous spondylitis presenting as vertebra plana: a retrospective analysis and review of literature.

AIM: To present the magnetic resonance imaging (MRI) findings of 10 patients with histopathologically proven tuberculous spondylitis (TS) presenting as vertebra plana (VP) on radiographs. MATERIAL AND METHODS: Radiographs of 451 adult TS patients were reviewed. In this consecutive series, there were 11 patients who presented as VP. MRI of 10 of these patients was available for review. RESULTS: VP-like collapse of a single vertebral body of the dorsal spine with preserved endplates and disc was seen in all cases. Epidural, pre- and para-vertebral soft tissue was found in all patients. Epidural soft tissue presenting on sagittal images as a convexity of the posterior longitudinal ligament was also found in all the signal of which was different from the involved vertebra on axial images. All patients showed posterior element involvement, which was characterized by preserved cortical outline without expansion. CONCLUSION: TS presenting with VP-like collapse of the bone is rare, accounting for 2.4% of the cases in the present series. MRI may show a collapsed vertebra with preserved endplates and disc. MRI findings that are suggestive of TS include: (1) signal intensity of the epidural soft-tissue mass on axial images, which is different from the vertebral body; (2) presence of a thin, T2-weighted hypointense capsule of the para-vertebral soft tissue; (3) posterior element involvement characterized by intact hypointense cortical outline without expansion; and (4) involvement of the costovertebral joint.

An MRI scale for assessment of haemophilic arthropathy from the International Prophylaxis Study Group.

Evaluation of prophylactic treatment of haemophilia requires sensitive methods. To design and test a new magnetic resonance imaging (MRI) scale for haemophilic arthropathy, two scales of a combined MRI scoring scheme were merged into a single scale which includes soft tissue and osteochondral subscores. Sixty-one joint MRI's of 46 patients with haemophilia were evaluated by four radiologists using the new and older scales. Forty-six of the joints were evaluated using two X-ray scales. For all MRI scores, interreader agreement and correlations with X-ray scores and lifetime number of haemarthroses were analysed. The interreader agreement intraclass correlation coefficient was 0.82, 0.89 and 0.88 for the soft tissue and osteochondral subscores and the total score, as evaluated according to the new MRI scale, compared to 0.80 and 0.89 as for the older scales. The total score and osteochondral subscore according to the new scale, as well as scores according to the older scales were correlated (P < 0.01) with number of haemarthroses (Spearman correlation 0.35-0.68) and with the X-ray scores (Spearman correlation 0.40-0.76), but no correlation (P > 0.05) was found between the soft tissue subscore of the new MRI scale and the X-ray scores. The new MRI scale is simpler to apply than the older and has similar reader reliability and correlation with lifetime number of haemarthroses, and by separating soft tissue and osteochondral changes it gives additional information. The new scale is useful for analyses of early and moderate stages of arthropathy, and may help to evaluate prophylactic haemophilia treatment.

IMRT implementation and patient specific dose verification with film and ion chamber array detectors.

Implementation of Intensity Modulation Radiotherapy (IMRT) and patient dose verification was carried out with film and I'mariXX using linear accelerator with 120-leaf Millennium dynamic multileaf collimator (dMLC). The basic mechanical and electrical commissioning and quality assurance tests of linear accelerator were carried out. The leaf position accuracy and leaf position repeatability checks were performed for static MLC positions. Picket fence test and garden fence test were performed to check the stability of the dMLC and the reproducibility of the gap between leaves. The radiation checks were performed to verify the position accuracy of MLCs in the collimator system. The dMLC dosimetric checks like output stability, average leaf transmission and dosimetric leaf separation were also investigated. The variation of output with gravitation at different gantry angles was found to be within 0.9 %. The measured average leaf transmission for 6 MV was 1.6 % and 1.8% for 18 MV beam. The dosimetric leaf separation was found to be 2.2 mm and 2.3 mm for 6 MV and 18 MV beams. In order to check the consistency of the stability and the precision of the dMLC, it is necessary to carryout regular weekly and monthly checks. The dynalog files analysis for Garden fence, leaf gap width and step wedge test patterns carried out weekly were in good agreement. Pretreatment verification was performed for 50 patients with ion chamber and I'mariXX device. The variations of calculated absolute dose for all treatment fields with the ion chamber measurement were within the acceptable criterion. Treatment Planning System (TPS) calculated dose distribution pattern was comparable with the I'mariXX measured dose distribution pattern. Out of 50 patients for which the comparison was made, 36 patients were agreed with the gamma pixel match of >95% and 14 patients were with the gamma pixel match of 90-95% with the criteria of 3% delta dose (DD) and 3 mm distance-to-agreement (DTA). Commissioning and quality assurance of dMLC for IMRT application requires considerable time and effort. Many dosimetric characteristics need to be assessed carefully failing which the delivered dose will be significantly different from the planned dose. In addition to the issues discussed above we feel that individual MU check is necessary before the treatment is delivered.

Jejunal access loop cholangiogram and intervention using image guided access.

Jejunal access loop is fashioned in patients who undergo Roux en Y hepaticojejunostomy and biliary intervention is anticipated on follow up. Post-operative study of the biliary tree through the access loop is usually done under fluoroscopic guidance. We present a series of 20 access loop cholangiograms performed in our institution between August 2004 and November 2008. We aimed to evaluate the safety and efficacy of the procedure and to highlight the role of CT guidance in procuring access. Access loop was accessed using CT (n = 13), ultrasound (n = 3) or fluoroscopic guidance (n = 4). Fluoroscopy was used for performing cholangiograms and interventions. Twelve studies had balloon plasty of the stricture at anastomotic site or high up in the hepatic ducts. Seven studies showed normal cholangiogram. Plasty was unsuccessful in one study. Technical success in accessing the jejunal access loop was 100%; in cannulation of anastomotic site and balloon plasty it was 95%. One case required two attempts. Procedure-related complications were not seen. All patients who underwent balloon plasty of the stricture were doing well for variable lengths of time. Access loop cholangiogram and interventions are safe and effective. CT guidance in locating/procuring the access loop is a good technique.

Dual arterial supply to normal lung: within the sequestration spectrum.

We report a rare case of dual arterial supply to an otherwise normal left lower lobe. In addition to normal pulmonary arterial supply, the lower lobe of the left lung also received systemic arterial supply from the coeliac axis. The relevant anatomy and literature are reviewed. To the best of our knowledge, there are no other reported cases of anomalous systemic arterial supply from the coeliac axis to the basal segments of the left lower lobe with normal bronchial branching and pulmonary arterial supply.