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The authors describe an Indian child, who presented in the accelerated phase of the Chédiak-Higashi syndrome. The disease usually presents in early childhood with recurrent skin and mucosal infections. This patient had subtle pigmentary abnormalities and no family history of the disease, which made the clinical diagnosis difficult. The cytopenias, hepatosplenomegaly, lymphohistiocytic infiltrate in the bone marrow, and the characteristic granules in the leucocytes clinched the diagnosis. This case underscores the importance of a bone marrow examination in patients with unusual presentations of rare disorders. [ABSTRACT FROM AUTHOR]

Introduction: Long saphenous vein grafts (LSVGs) are pivotal conduits in coronary artery bypass grafting (CABG), yet concerns persist regarding early failure and long-term patency. Endothelial damage, a potent initiator of graft failure, necessitates exploration of factors contributing to endothelial injury during LSVG preparation., Methods: A prospective, single-center study was conducted, assessing the impact of unregulated distension pressure on LSVG endothelium during CABG. Histological and CD31 (cluster of differentiation 31) immunohistochemical analyses were performed on 21 paired vein samples, categorized into non-distended (group A) and distended (group B) groups. Pressure recordings were obtained using different syringe sizes during vein distension., Results: Histological examination revealed a significantly higher percentage of endothelial cell loss in distended veins (31.95% ± 31.31) compared to non-distended veins (11.67% ± 28.65) ( p  = 0.034). CD31 immunohistochemistry corroborated greater endothelial cell loss in distended veins ( p  = 0.001). The pressure recordings with a 20-cc syringe, as opposed to using a 10-cc syringe, were considerably lower (44.5 mmHg vs. 92.75 mmHg) emphasizing the inverse relationship between syringe size and pressure generated. In our study, pre-existing endothelial injury was observed in one-third of diabetic patients (36%), with all instances of such injury exclusively identified in individuals with diabetes., Conclusion: Unregulated distension pressure during LSVG preparation is associated with greater endothelial damage, as evidenced by histological and immunohistochemical analyses. The inverse relationship between syringe size and pressure underscores the importance of controlled distension., Competing Interests: Conflict of interestNone., (© Indian Association of Cardiovascular-Thoracic Surgeons 2024. Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.)

Competing Interests: Conflict of interestNone.

Background: Rheumatic fever in childhood is the most common cause of Mitral Stenosis in developing countries. The disease is characterized by damaged and deformed mitral valves predisposing them to scarring and narrowing (stenosis) that results in left atrial hypertrophy followed by heart failure. Presently, echocardiography is the main imaging technique used to diagnose Mitral Stenosis. Despite the high prevalence and increased morbidity, no biochemical indicators are available for prediction, diagnosis and management of the disease. Adopting a proteomic approach to study Rheumatic Mitral Stenosis may therefore throw some light in this direction. In our study, we undertook plasma proteomics of human subjects suffering from Rheumatic Mitral Stenosis (n = 6) and Control subjects (n = 6). Six plasma samples, three each from the control and patient groups were pooled and subjected to low abundance protein enrichment. Pooled plasma samples (crude and equalized) were then subjected to in-solution trypsin digestion separately. Digests were analyzed using nano LC-MS(E). Data was acquired with the Protein Lynx Global Server v2.5.2 software and searches made against reviewed Homo sapiens database (UniProtKB) for protein identification. Label-free protein quantification was performed in crude plasma only., Results: A total of 130 proteins spanning 9-192 kDa were identified. Of these 83 proteins were common to both groups and 34 were differentially regulated. Functional annotation of overlapping and differential proteins revealed that more than 50% proteins are involved in inflammation and immune response. This was corroborated by findings from pathway analysis and histopathological studies on excised tissue sections of stenotic mitral valves. Verification of selected protein candidates by immunotechniques in crude plasma corroborated our findings from label-free protein quantification., Conclusions: We propose that this protein profile of blood plasma, or any of the individual proteins, could serve as a focal point for future mechanistic studies on Mitral Stenosis. In addition, some of the proteins associated with this disorder may be candidate biomarkers for disease diagnosis and prognosis. Our findings might help to enrich existing knowledge on the molecular mechanisms involved in Mitral Stenosis and improve the current diagnostic tools in the long run.

Piroxicam (chemically 4-hydroxy-2-methyl- N -2-pyridinyl-2 H -1,2-benzothiazine-3-carboxamide), a classical non-steroidal anti-inflammatory drug (NSAID) is orally administered to arthritic patients. Inhibition of prostaglandin E 2 (PGE 2 ) synthesis and subsequent free hydroxyl radical generation in vivo exert gastro-toxic side effects on piroxicam treatment. Leaves of curry plant are rich in antioxidants with prolific free radical scavenging activities. This led us to investigate the efficiency of the use of curry leaves in ameliorating piroxicam induced gastric damage. Piroxicam was orally (30 mg per kg body weight) administered in male albino Wistar rats to generate gastric ulcers. These rats were orally fed with graded doses of aqueous extract of curry or Murraya koenigii leaves (Cu LE) prior to piroxicam administration. Oxidative stress biomarkers, activities of antioxidant and pro-oxidant enzymes, mucin content and nature, PGE 2 level, activities of mitochondrial enzymes and histomorphology of gastric tissues were studied. Piroxicam treatment altered all the above mentioned parameters whereas, curry leaf extract pre-treated animals were protected against piroxicam induced alterations. Hence, the protective action of the antioxidant rich Cu LE was investigated to propose a new combination therapy or dietary management to arthritic patients using piroxicam.

Background: Rheumatic Heart Disease (RHD), a chronic acquired heart disorder results from Acute Rheumatic Fever. It is a major public health concern in developing countries. In RHD, mostly the valves get affected. The present study investigated whether extracellular matrix remodelling in rheumatic valve leads to altered levels of collagen metabolism markers and if such markers can be clinically used to diagnose or monitor disease progression., Methodology: This is a case control study comprising 118 subjects. It included 77 cases and 41 healthy controls. Cases were classified into two groups- Mitral Stenosis (MS) and Mitral Regurgitation (MR). Carboxy-terminal propeptide of type I procollagen (PICP), amino-terminal propeptide of type III procollagen (PIIINP), total Matrix Metalloproteinase-1(MMP-1) and Tissue Inhibitor of Metalloproteinase-1 (TIMP-1) were assessed. Histopathology studies were performed on excised mitral valve leaflets. A p value <0.05 was considered statistically significant., Results: Plasma PICP and PIIINP concentrations increased significantly (p<0.01) in MS and MR subjects compared to controls but decreased gradually over a one year period post mitral valve replacement (p<0.05). In MS, PICP level and MMP-1/TIMP-1 ratio strongly correlated with mitral valve area (r = -0.40; r = 0.49 respectively) and pulmonary artery systolic pressure (r = 0.49; r = -0.49 respectively); while in MR they correlated with left ventricular internal diastolic (r = 0.68; r = -0.48 respectively) and systolic diameters (r = 0.65; r = -0.55 respectively). Receiver operating characteristic curve analysis established PICP as a better marker (AUC = 0.95; 95% CI = 0.91-0.99; p<0.0001). A cut-off >459 ng/mL for PICP provided 91% sensitivity, 90% specificity and a likelihood ratio of 9 in diagnosing RHD. Histopathology analysis revealed inflammation, scarring, neovascularisation and extensive leaflet fibrosis in diseased mitral valve., Conclusions: Levels of collagen metabolism markers correlated with echocardiographic parameters for RHD diagnosis.

Primary angiosarcoma is an extremely rare and aggressive soft-tissue malignancy of endothelial cell origin that occurs most frequently in the skin and subcutaneous tissues of the extremities. Presence of this disease in the intestine as a primary or metastatic deposit is an extremely uncommon incident, and might causes diagnostic confusion with primary colonic neoplasm. We encountered epithelioid angiosarcoma of the sigmoid colon in a 59-year-old male patient who presented with occasional bleeding per rectum and had also had an angiosarcoma arising from the subcutaneous tissue of the right thigh. Retrospective review of histopathology and immunohistochemistry of the two specimens were helpful in the final diagnosis.

An 18-year-old man presenting with paraplegia due to spinal epidural infiltration at D6-D7 underwent laminectomy and spinal decompression followed by near-complete resolution of neurological symptoms; but this resolution was followed by progressively enlarging cervical lymph nodes. Leucocytosis, an elevated erythrocyte sedimentation rate, a marked polyclonal hypergammaglobulinemia and a moderate hepatosplenomegaly were found. The diagnosis of sinus histiocytosis with massive lymphadenopathy (SHML), also known as Rosai-Dorfman disease was established histologically by lymph node biopsy about 2 years after presentation.

Bone marrow necrosis (BMN) is a relatively uncommon clinicopathologic entity with diverse etiology. We describe a case of BMN in a 11 1/2 year old male child with an underlying non Hodgkin's lymphoma T-cell type. With the help of 99m Tc-MDP (methylene diphosphonate) bone scan we were able to find out the etiologic factor in this case.

Objective: To correlate visual cytologic grade with automated nuclear morphometry of carcinoma of the breast., Study Design: We randomly selected 24 histologically proven infiltrating ductal carcinomas of the breast and 10 benign breast lesions (fibroadenoma). Hematoxylin-eosin-stained fine needle aspiration cytology (FNAC) smears were selected for both cytologic grade and automated image morphometry. The same hematoxylin-eosin-stained FNAC smears were studied for area, convex area, standard deviation of nuclear area, diameter, perimeter and convex perimeters of nucleus. At least 100 cells from each case were measured with an image cytometer., Results: Mean nuclear area, standard deviation of nuclear area, nuclear diameter, convex area, convex perimeter and perimeter were significantly increased from benign versus grade 1 carcinomas and grade 1 versus grade 2 and 3 carcinomas (one way ANOVA test). However, there was no significant difference in grade 2 versus grade 3 carcinomas., Conclusion: Automated image cytometry rapidly and successfully measures various nuclear parameters. The measurement of various nuclear parameters would be helpful in future applications of automated diagnosis and grading of breast carcinomas from cytologic material.