Aagesensalazar9334

Objective Pulmonary hypertension related to congenital heart disease (PH-CHD) is a devastating disease caused by hemodynamic disorders. Previous hemodynamic research in PH-CHD mainly focused on wall shear stress (WSS). However, energy loss (EL) is a vital parameter in evaluation of hemodynamic status. We investigated if EL of the pulmonary artery (PA) is a potential biomechanical marker for comprehensive assessment of PH-CHD. Materials and Methods Ten PH-CHD patients and 10 age-matched controls were enrolled. Subject-specific 3-D PA models were reconstructed based on computed tomography. Transient flow, WSS, and EL in the PA were calculated using non-invasive computational fluid dynamics. The relationship between body surface area (BSA)-normalized EL ( E . ) and PA morphology and PA flow were analyzed. Results Morphologic analysis indicated that the BSA-normalized main PA (MPA) diameter (DMPAnorm), MPA/aorta diameter ratio (DMPA/DAO), and MPA/(left PA + right PA) [DMPA/D(LPA+RPA)] diameter ratio were significantly larger in PH-CHD patients. Hemodynamic results showed that the velocity of the PA branches was higher in PH-CHD patients, in whom PA flow rate usually increased. WSS in the MPA was lower and E . was higher in PH-CHD patients. E . was positively correlated with DMPAnorm, DMPA/DAO, and DMPA/D(LPA+RPA) ratios and the flow rate in the PA. E . was a sensitive index for the diagnosis of PH-CHD. Conclusion E . is a potential biomechanical marker for PH-CHD assessment. This hemodynamic parameter may lead to new directions for revealing the potential pathophysiologic mechanism of PH-CHD.Background Kawasaki disease (KD) is the most common cause of acquired heart disease. A proportion of patients were resistant to intravenous immunoglobulin (IVIG), the primary treatment of KD, and the mechanism of IVIG resistance remains unclear. The accuracy of current models predictive of IVIG resistance is insufficient and doesn't meet the clinical expectations. Objectives To develop a scoring model predicting IVIG resistance of patients with KD. Methods We recruited 330 KD patients (50 IVIG non-responders, 280 IVIG responders) and 105 healthy children to explore the susceptibility loci of IVIG resistance in Kawasaki disease. A next generation sequencing technology that focused on 4 immune-related pathways and 472 single nucleotide polymorphisms (SNPs) was performed. An R package SNPassoc was used to identify the risk loci, and student's t-test was used to identify risk factors associated with IVIG resistance. A random forest-based scoring model of IVIG resistance was built based on the identified specific SNP loci with the laboratory data. Results A total of 544 significant risk loci were found associated with IVIG resistance, including 27 previous published SNPs. Laboratory test variables, including erythrocyte sedimentation rate (ESR), platelet (PLT), and C reactive protein, were found significantly different between IVIG responders and non-responders. A scoring model was built using the top 9 SNPs and clinical features achieving an area under the ROC curve of 0.974. Conclusions It is the first study that focused on immune system in KD using high-throughput sequencing technology. Our findings provided a prediction of the IVIG resistance by integrating the genotype and clinical variables. It also suggested a new perspective on the pathogenesis of IVIG resistance.Graft versus host disease (GVHD) is a unique entity wherein the donated marrow cells (graft) view the hosts as foreign and attack various body organs. Skin is the most frequently affected organ followed by mucosa, eyes, gastrointestinal, respiratory, musculoskeletal system, and other organs. The incidence of GVHD varies from 25 to 80%. Cutaneous involvement can present as exanthem, epidermolysis, lichenoid eruptions, erythroderma, ichthyosis, pityriasis rubra pilaris like lesions, psoriasiform lesions or just pruritus. Asymptomatic truncal follicular eruptions as the major presentation is rare. We report a case of aplastic anemia that developed extensive truncal folliculocentric papules 10 months following an allogeneic hematopoietic stem cell transplantation. Histopathological examination of the follicular lesions revealed perifollicular inflammatory infiltrate comprising of lymphocytes, plasma cells and histiocytes at the dermo-epidermal junction. Basal cell vacuolization, pigment incontinence in the upper dermis and few apoptotic keratinocytes in the follicular epidermis were also seen. The patient responded satisfactorily to tapering doses of steroids.Giant porokeratosis (PK) is a rare entity. Many consider it as a variant of PK of Mibelli, while others describe it as a separate variant. The diameter may range between 10 and 12 cm and the wall of hyperkeratotic ridge may be upto 1 cm. High tropical temperature and long duration of outdoor activities without adequate clothing are known influencing factors of PK. To the best of our knowledge, only five cases of solitary giant PK and a case of disseminated giant PK have been documented from India. We report three cases of giant PK for their rarity.Wells syndrome or eosinophilic cellulitis is an idiopathic inflammatory dermatitis characterized by a benign but recurrent evolution. It often causes edematous urticarial plaques on the extremities. Herein, we report two rare cases of Wells syndrome with marked swelling in the hands. Both cases exhibited various clinical features. Case 1 was Wells syndrome with collagen disease-like sclerotic edema in the fingers and annular erythema on the trunk. Case 2 was Wells syndrome with diffuse plate-like hardening of the forearm mimicking cellulitis, which required fasciotomy due to suspected compartment syndrome at the emergency room. GSH nmr Wells syndrome should be included in the differential diagnosis of patients presenting with marked diffuse swelling in the hands.Phaeohyphomycosis encompasses many clinical syndromes occurring due to a wide variety of dematiaceous fungi. It can manifest as superficial, cutaneous, subcutaneous, or systemic forms involving the skin, subcutis, paranasal sinuses, or the central nervous system. Subcutaneous phaeohyphomycosis is the most common subtype and occurs due to wound contamination or traumatic inoculation of the saprophytic fungus from soil and vegetation. Multiple cases of subcutaneous phaeohyphomycosis involving the extremities in the form of cystic lesions and abscesses have been reported. However, involvement of the face in the form of a progressive ulcerative and disfiguring lesion in an immunocompetent person is extremely rare. We report a rare case of subcutaneous phaeohyphomycosis presenting as a slowly progressive disfiguring lesion of face.