Rosai-Dorfman Disease as Constrictive Pericarditis: An Unusual Childhood Presentation.

Rosai-Dorfman disease (RDD) is a rare, multisystemic, histiocytic disorder that usually presents with painless cervical lymphadenopathy. We describe a case of constrictive effusive pericarditis with congestive cardiac failure in a six-year-old child as an initial presentation of RDD. The child underwent pericardiectomy and was treated with steroids, following which the symptoms resolved entirely. While tuberculosis accounts for the majority of cases presenting with constrictive pericarditis in developing countries, the presentation of RDD can be easily missed if not kept in mind as a possibility. This case report documents the unique presentation of a rare disease.

Renal Thrombotic Microangiopathy: A Complication of Paediatric Systemic Lupus Erythematosus That Requires Attention.

Systemic lupus erythematosus (SLE) is a multi-system disorder with a variety of clinical presentations. A wide range of renal vascular lesions (VL) is described predominantly in adult patients. The exact prevalence of renal VL in the pediatric SLE (pSLE) population is yet to be determined. A 10-year-old female patient with lupus nephritis (LN) presented with deteriorating kidney function. An exhaustive array of clinical-biochemical and pathological evaluations resulted in a diagnosis of class IV LN with thrombotic microangiopathy (TMA) associated with malignant hypertension and hypocomplementemia. Renal VL is overlooked or underreported in SLE patients, as it is neither accorded much importance in the International Society of Nephrology/Renal Pathology Society (ISN/RPS) classification nor in the activity and chronicity scoring. The TMA lesions in LN patients can be managed following the recently devised PLASMIC score; hence, reporting such VL has therapeutic implications.

Nanostructural Design of ZnO Using an Agro-Waste Extract for a Sustainable Process and Its Photocatalytic Activity.

The use of agro-waste extracts (AWEs) as a sustainable medium for developing cost-effective and ecologically friendly nanomaterials has piqued the interest of current researchers. Herein, waste extracts from papaya barks, banana peels, thumba plants, and snail shells were used for synthesizing ZnO nanostructures via a hydrothermal method, followed by calcination at 400 °C. The crystallinity and pure wurtzite phase formation of ZnO nanostructures were confirmed via X-ray diffraction. ZnO nanostructures with various morphologies such as tight sheet-like, spherical, porous sheet-like, and bracket-shaped, comprising small interconnected particles with a highly catalytically active exposed (0001) facet, were observed via field emission scanning electron microscopy and transmission electron microscopy. The formation mechanism of the various morphologies of the ZnO nanostructures was proposed. Ultraviolet-visible spectra showed different absorption band edges of ZnO nanostructures with a bandgap in the range of 3.17-3.27 eV. Photoluminescence studies showed the presence of various defect states such as oxygen and zinc vacancies and oxygen and zinc interstitials on ZnO nanostructures, which are usually observed in traditionally prepared ZnO. The photocatalytic activity of ZnO nanostructures was evaluated under direct sunlight using rhodamine B (RhB) and Congo red (CR) dyes as probe pollutants. Furthermore, prepared ZnO nanostructures could potentially adsorb anionic dyes (e.g., CR) in the absence of light. Superoxide and hydroxide radicals played a vital role in the photocatalytic activity of ZnO. The photocatalyst could be reused for up to three cycles, indicating its stability. Therefore, this study reports the diverse use of AWEs as cost-effective media for nanomaterial synthesis.

Clinicopathological, Immunological, and Laboratory Parameters of Childhood Lupus Nephritis: A Study from Northeast India.

Background Lupus nephrtis in children is associated with high morbidity and mortality. The incidence of childhood systemic lupus erythematosus (SLE) ranges from 3.3 to 8.8/100000 children with a higher Asian preponderance. The predominance of SLE in female pediatric patients increases gradually with age to the values observed in adults. Objectives To assess the clinical, immunological, and histopathological spectrum of childhood lupus nephritis in northeast India and explore the relationship between clinical, biochemical, serological, and histopathological findings. Materials and Methods A retrospective descriptive study was performed over 8 years. Histopathology slides were reviewed by two pathologists, whereas other details were collected from patients' records. Statistical Analysis Statistical analysis was based on the chi-square test and a p -value < 0.05 was considered statistically significant. Results Fifty-three cases of lupus nephritis were included in the study. The patients' age ranged from 5 to 18 years with a mean age of 14.5 years and a female: male ratio of 6.5:1. Edema and hypertension were the commonest clinical presentations, whereas proteinuria was the commonest presenting laboratory parameter. Amongst all the immunological markers, dsDNA was the commonest. Histopathologically, predominantly study population belonged to class IV lupus nephritis. The patients with class IV showed a statistically significant correlation with proteinuria and hematuria at the time of diagnosis. Immunological markers, namely, ANA and anti-ds-DNA positivity were significantly associated with advanced renal histopathology. Conclusion cSLE in northeast India presents mostly as Class IV LN presenting mostly with deranged laboratory parameters and preponderance of various immunological markers and clinical presentations.

Xp11.2 Translocation Renal Cell Carcinoma: A Rare Renal Cell Carcinoma.

Translocation-associated renal cell carcinoma (TRCC) is a group of under-recognized malignant renal neoplasms owing to the unavailability of ancillary diagnostic tools and considering the fact that these tumors may histomorphologically mimic a heterogeneous group of neoplasms ranging from benign to malignant ones. Xp11.2 translocation-associated renal cell carcinoma is a disease of the young with a relatively less known prognosis owing to the rarity of such reported neoplasms. The histological appearance of bulbous tumor cells with abundant, vacuolated cytoplasm and the presence of psammomatoid bodies are clues to the diagnosis but are not entirely specific. The immunohistochemistry (IHC) finding of transcription factor E3 (TFE3) positivity is an important pointer, but the demonstration of Xp11.2 translocation by fluorescence in situ hybridization (FISH) serves as the confirmatory test. In our case report, we highlight the fact that a combined approach involving light microscopy, immunohistochemistry, and fluorescence in situ hybridization is the key to its diagnosis.

Clinicopathological Profile of Immunoglobulin A Nephropathy: A Study from Northeast India.

Immunoglobulin A (IgA) nephropathy is the most common primary glomerulopathy, with wide variation in its prevalence as well as clinical symptoms. Among the laboratory parameters, increased serum creatinine (SCr) levels, mean arterial pressure (MAP), and a decreased estimated glomerular filtration rate (eGFR) point toward poorer renal function. The Oxford 2016 scoring system for IgA nephropathy identified various histopathological variables, which serve as indicators of renal outcomes. There is a paucity of studies on the prevalence as well as the various clinical laboratory parameters correlating with the 2016 Oxford scoring system in northeastern India. The present study showed that IgA nephropathy was more common in the second and third decades, more prevalent in females, and mostly presented with edema. Nephrotic proteinuria, higher SCr, MAP, and decreased eGFR levels at presentation suggested poorer renal function in most subjects. The endocapillary hypercellularity, segmental sclerosis, tubular atrophy, and crescent variables of the 2016 Oxford scoring system showed a statistically significant relationship with various laboratory parameters at presentation.

Immunoglobulin A Nephropathy in a SARS-CoV-2-Positive Patient With Coexistent Metabolic Syndrome.

SARS-CoV-2 viral infection though primarily affects the respiratory system, but concurrent renal involvement is been reported in the medical literature. Acute kidney injury (AKI) is a common finding in SARS-CoV-2-positive patients. An isolated case of IgA nephropathy in a SARS-CoV-2 virus-infected patient has been already reported in the medical literature. Incidence of metabolic syndromes is on the rise considering the change in lifestyle and food habits and the global pandemic of obesity. Renal manifestations of metabolic syndrome are myriad with IgA nephropathy being an occasional manifestation in such patients. We reported a case of IgA nephropathy in a patient in her fourth decade of life diagnosed as metabolic syndrome with concomitant SARS-CoV-2 infection that progressed to chronic kidney damage (CKD) subsequently. In this case report, we postulate that cytokine storm along with hypoxemia secondary to SARS-CoV-2 infection may accelerate the declining renal function however further studies are necessary to confirm this hypothesis considering the rarity of such cases.

High-grade transformation of adenoid cystic carcinoma of parotid gland with isolated intratemporal facial nerve involvement: a case report and literature review.

BACKGROUND: High-grade transformation Adenoid cystic carcinoma (HGT-AdCC) of the parotid gland is a rare transformation noted in slow growing low grade AdCC. Perineural invasion and spread is an important feature of this tumor. Temporal bone involvement is rare. A total of only 10 cases of HGT-AdCC in parotid gland has been reported in literature so far predominantly in the elderly with peak incidence in 5th-6th decade. CASE PRESENTATION: We present a young lady of HGT-AdCC of right parotid gland with temporal bone involvement in the form of isolated perineural invasion (PNI) of facial nerve till the tympanic segment. She underwent right radical parotidectomy with modified radical neck dissection with modified lateral temporal bone resection and pectoralis major myocutaneous flap reconstruction. Histopathological examination revealed both low- and high-grade areas. Sections from facial nerve showed tumor invasion. CONCLUSION: The radiological features of isolated perineural spread in intratympanic part of facial nerve can be easily missed if not specifically looked for. Every attempt should be made preoperatively and intraoperatively to determine the complete extent of the tumor for adequate disease clearance. A combined clinico-radiological approach aided by histopathology examination helps in early detection of this carcinoma and in better patient management.

Hepatosplenic T Cell Lymphoma: Diagnostic Conundrum.

Hepatosplenic T cell lymphoma (HSTCL) is a very rare and aggressive peripheral T cell lymphoma that comprises less than 1% of Non-Hodgkin lymphomas (NHL). It is derived from cytotoxic T-cells, usually of γδ T cell receptor type, and is characterized by primary extranodal disease with typical sinusoidal infiltration of the liver, spleen and bone marrow by medium-sized lymphoid cells. HSTCL occurs more frequently in immunocompromised patients, especially in those receiving long-term immunosuppressive therapy. The differential diagnosis is varied, and the clinical course is dismal with a poor response to currently available therapies. Herein we report a case of HSTCL in a 20-year-old immunocompetent male who presented with fever, pallor, weight loss, bicytopenia, hepatomegaly, and massive splenomegaly, highlighting the diagnostic conundrum and pointers towards an accurate diagnosis. The key role for diagnosis was the combination of morphologic finding of atypical lymphoid cells in the bone marrow, typical immunophenotypic profile on flow cytometry and the pattern of involvement of the liver and the spleen, even in the absence of full-fledged diagnostic panels and tools. The report of this case is an endeavor to emphasize the high index of suspicion for timely detection of such a rare entity.

Role of interfacial contact between 2D materials and preselected nanostructures in the degradation of toxic dyes: Multifunctional facets of graphene.

Designing intimate interfacial contact between nanostructures and two-dimensional (2D) materials is highly desirable to influence the movement of generated charge carriers. Nanostructured zinc oxide (ZnO) is a fascinating material with unique optical and electrical properties. 2D reduced graphene oxide (rGO) exhibits semiconductor behaviour with tunable catalytic activity and excellent biocompatibility. Hence, we have designed a hybrid material by selecting nanostructures of an oxide semiconductor (ZnO) with reduced graphene oxide (rGO) using a hard integration technique followed by a low-temperature hydrothermal route. The good encapsulation of rGO over the ZnO nanorods was confirmed by powder X-ray diffraction, scanning electron microscopy, transmission electron microscopy, Fourier transform infrared spectroscopy, and Raman spectroscopy. The photocatalytic activities of ZnO, rGO, and ZnO/rGO were studied under visible-light irradiation using three different toxic dyes, methylene blue (MB), methyl orange (MO), and Congo red (CR). The composite materials exhibited excellent efficiencies of 100, 95, and 90% for the degradation of MB, MO, and CR, respectively. Moreover, the degradation of the dye was found to follow first-order kinetics. The enhanced efficiencies are attributed to the adsorption and efficient charge transfer from rGO to the conduction band of ZnO. The role of the multifunctional facets of graphene was presented to elucidate the visible-light activity of the composite materials for enhanced efficiency. The main reactive species (e-) of the reduction reaction were confirmed through a radical trapping experiment, which showed the generation of highly reactive •OH radicals that decompose the toxic dye. The results provide a perspective for developing graphene-based composite materials with desired preselected nanostructures for solar energy utilisation.

Synthesis, characterization, and photocatalytic activity of ZnO nanoparticles using water extract of waste coconut husk.

The present work reports the use of natural alkaline extract from coconut husk ash as a precipitating agent for metal oxide nanoparticles synthesis. The abundance of K2O and K2CO3 in it makes the extract highly basic and could be the alternative source of basic media in the laboratory. In this study, highly photoactive zinc oxide nanoparticles have been synthesized using water extract of waste coconut husk ash in a green approach which is considered as replacement of homogeneous base like NaOH and KOH. The formation of zinc oxide nanoparticles at different pH of the solution of coconut husk ash was confirmed through powder XRD, BET, SEM-EDX, UV-Vis, FTIR, and photoluminescence spectroscopy. The photocatalytic performance of the samples was evaluated through the degradation of methylene blue (MB) and methyl orange (MO) under solar irradiation which undergo degradation around 97% and 68% within 120 min, respectively. The high photocatalytic activity and rate constant could be attributed to the large surface area due to small particle size that could provide quicker photon absorption and reduction of charge carrier recombination. This current work introduces a new method to reduce energy consumption for the synthesis of highly photoactive low-cost zinc oxide nanoparticles.

Hemophagocytic Lymphohistiocytosis Secondary to Adult-Onset Still's Disease.

Hemophagocytic lymphohistiocytosis (HLH) is a rare hematological condition resulting from dysregulation of the immune system. This unusual clinical syndrome is characterized by fever, cytopenia, liver dysfunction, increased ferritin level, and evidence of hemophagocytosis in the bone marrow. We report a case of a 21-year-old female who presented with recurrent high-grade fever, transient rash, and polyarthritis who was subsequently diagnosed with adult-onset Still's disease (AOSD) with secondary HLH. The patient improved with aggressive management. Our case highlights HLH as a life-threatening and underdiagnosed complication of AOSD.

Secondary Hemophagocytic Lymphohistiocytosis in Post-COVID-19 Patients: A Report of Two Cases.

Hemophagocytic lymphohistiocytosis (HLH) is a disease that can affect both children and adults. HLH can be categorized as primary or secondary. Secondary HLH (sHLH) may be secondary to various viral infections. Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) virus infection is a pandemic with multi-system involvement. HLH in COVID-19 positive patients is a recognized entity. However, in post-COVID-19 patients who have recovered and are negative by serological tests and reverse transcription-polymerase chain reaction test may present with sHLH due to dysregulation of the immune system. We highlight this unusual finding of post-COVID-19 sHLH in two cases, who were diagnosed by the new revised H-score.

Tumour Behaviour of Low-Grade Papillary Urothelial Carcinoma: A Single-Centre Retrospective Study.

Background and objective Carcinoma of the urinary bladder is the most common urological cancer, and it accounts for 3.9% of all cancer cases in men. Patients with the subset of noninvasive low-grade papillary urothelial carcinoma (LG-UrCa) are at higher risk for tumour recurrence. In this study, we aimed to analyse the histopathological features of LG-UrCa and to correlate those with recurrence potential as well as disease stage and grade progression. Materials and methods We conducted a retrospective study from January 2016 to December 2018. All cases with presenting biopsy initially reported as LG-UrCa were included in the study. All cases with initial biopsy reported as high-grade papillary urothelial carcinoma (HG-UrCa) were excluded from the study. We used the 2016 World Health Organization/International Society of Urological Pathology (WHO/ISUP) guidelines for the classification of papillary urothelial neoplasm. Results A total of 48 initially diagnosed cases of LG-UrCa were identified. Two out of 48 cases were reclassified as high-grade urothelial carcinoma and were excluded from the study. The mean age of patients at presentation was 56.7 years. The mean duration of follow-up was 19.8 months. The mean size of initial tumours was 3.4 cm. Tumour recurrence was encountered in 14 (30.4%) of 46 patients. Out of the four patients who had high-grade progression (8.7%), two also developed TNM stage progression. These two patients eventually underwent radical cystectomy. Patients with larger initial tumour sizes were found to have an increased tumour recurrence rate (p=0.009). Patients with multiple lesions at initial diagnosis had a significantly higher tumour recurrence rate than those with a single tumour (p=0.02). There was no significant difference with regard to intravesical Bacillus Calmette-Guérin (BCG) and tumour recurrence (p=0.065). None of the clinicopathological parameters were significantly associated with the grade and/or stage progression. Conclusion Based on our findings, patients with larger initial tumour size and tumour multiplicity at presentation had an increased tumour recurrence rate.

De novo chronic lymphocytic leukemia/prolymphocytic leukemia or B-cell prolymphocytic leukemia? The importance of integrating clinico-morphological and immunophenotypic findings in distinguishing chronic lymphoproliferative diseases with circulating phase.

B-cell prolymphocytic leukemia (B-PLL) is an extremely rare disease, accounting for approximately 1% of the lymphocytic leukemias. B-PLL generally occurs in older people. It is characterized by the presence of more than 55% prolymphocytes in the peripheral blood (PB), no or minimal lymphadenopathy, massive splenomegaly, and very high white blood cell counts. The prognosis of B-PLL patients is generally poor, with a median survival of 3 years, although a subset of patients may show a prolonged survival. Herein, we report a case of a 70-year-old male with weakness, generalized lymphadenopathy, and moderate splenomegaly at the initial presentation. Hematologic examination revealed lymphocytic leukocytosis, favoring a chronic lymphoproliferative disorder (CLPD). The key to decoding the precise CLPD was a combination of the clinical profile, morphologic findings on the peripheral blood and the bone marrow, immunophenotypic analysis, and cytogenetic study. The best diagnosis proffered was a de novo chronic lymphocytic leukemia/prolymphocytic leukemia. There was no prior history of lymphoproliferative disorder or lymphocytic leukocytosis. Discriminating this entity from other lymphoproliferative disorders is crucial as the treatment and prognosis are varied compared to the other lymphoproliferative disorders. The diagnostic conundrum encountered and the incredible utility of ancillary studies in such a scenario are highlighted in this study.

Association of Co-dominant Immunoglobulin G Deposit in Immunoglobulin A Nephropathy With Poor Clinicopathological and Laboratory Parameters.

INTRODUCTION: Immunoglobulin A (IgA) nephropathy is the commonest primary glomerulonephritis with a wide range of clinical presentations and laboratory findings. There is a paucity of studies regarding the significance of co-dominant immunoglobulin G (IgG) deposition in IgA nephropathy. METHODS: The study included retrospective and prospective biopsy-proven cases of IgA nephropathy from 2013-2020 with a minimum of eight glomeruli. Clinical and laboratory parameters were analysed for the IgA and IgG co-dominant cases as compared to those of the non-IgG group. RESULTS: A total of 58 cases of IgA nephropathy were included in the study out of which 25 biopsies (43.1%) were categorized as IgA plus IgG, and the rest 33 biopsies (56.8%) as the non-IgG group. A significant correlation was noted amongst the IgA plus IgG group with respect to the elevated mean arterial pressure (MAP) (p=0.038) and proteinuria (p=0.002) as compared to the non-IgG group. Amongst the MEST-C (mesangial hypercellularity, endocapillary hypercellularity, segmental sclerosis, tubular atrophy/interstitial fibrosis, crescents) variables, endocapillary hypercellularity correlated with elevated MAP (p=0.04), raised serum creatinine (p=0.005), and decreased estimated glomerular filtration rate (eGFR) (p=0.002). CONCLUSION: Co-dominant IgG deposit serves as an adverse marker pointing towards a deranged renal function in IgA nephropathy.

De novo chronic lymphocytic leukemia/prolymphocytic leukemia or B-cell prolymphocytic leukemia? The importance of integrating clinico-morphological and immunophenotypic findings in distinguishing chronic lymphoproliferative diseases with circulating phase

B-cell prolymphocytic leukemia (B-PLL) is an extremely rare disease, accounting for approximately 1% of the lymphocytic leukemias. B-PLL generally occurs in older people. It is characterized by the presence of more than 55% prolymphocytes in the peripheral blood (PB), no or minimal lymphadenopathy, massive splenomegaly, and very high white blood cell counts. The prognosis of B-PLL patients is generally poor, with a median survival of 3 years, although a subset of patients may show a prolonged survival. Herein, we report a case of a 70-year-old male with weakness, generalized lymphadenopathy, and moderate splenomegaly at the initial presentation. Hematologic examination revealed lymphocytic leukocytosis, favoring a chronic lymphoproliferative disorder (CLPD). The key to decoding the precise CLPD was a combination of the clinical profile, morphologic findings on the peripheral blood and the bone marrow, immunophenotypic analysis, and cytogenetic study. The best diagnosis proffered was a de novo chronic lymphocytic leukemia/prolymphocytic leukemia. There was no prior history of lymphoproliferative disorder or lymphocytic leukocytosis. Discriminating this entity from other lymphoproliferative disorders is crucial as the treatment and prognosis are varied compared to the other lymphoproliferative disorders. The diagnostic conundrum encountered and the incredible utility of ancillary studies in such a scenario are highlighted in this study.

Analysis of aminoacids pattern in receptor tyrosine kinase using Boolean association rule.

Cancers are characterized by unrestricted cell division and independency of growth factor and other external signal responsiveness. Eukaryotic parental cells of tumors, on the other hand, constitute tissues and other higher structures like organs and systems and are capable of performing various functions in a highly co-ordinated fashion. Hence, cancer cells may be considered as entities capable of incessant growth and cell division but lacking any evolutionarily advanced intracellular or intercellular regulation. Since receptor tyrosine kinases are highly altered and exist in deregulated/constitutively active forms in cancer cells - achieved through various epigenetic mechanisms - we hypothesize the functional RTKs in cancer cells to resemble their counterparts in more primitive species. Analysis of RTK sequences of various species and of cancer is, therefore, expected to prove this hypothesis. Association rule in data mining can reveal the hidden biological information. This study utilizes the Boolean association rule to mine the occurrence pattern of glycine, arginine and alanine in receptor tyrosine kinases (RTKs) of invertebrates, vertebrates and cancer related vertebrate RTKs based on protein sequence informations. The results reveal that vertebrate cancer RTKs resembles prokaryotes and invertebrate RTKs showing an increasing trend of glycine, alanine and decreasing trend in arginine composition. The aminoacid compositions of vertebrates: invertebrates: prokaryotes: vertebrate cancer with respect to Glycine (>=6.1) were 42.86: 50.0: 85.71: 100%, Alanine (>=6.2) were 10.72: 66.67: 85.71: 100%, whereas Arginine (>=5.9) were 21.43: 16.67: 14.29: 0%, respectively. In conclusion, results from this study supports our hypothesis that cancer cells may resemble lower organisms since functionally cancer cells are unresponsive to external signals and various regulatory mechanisms typically found in higher eukaryotes are largely absent.

Synthesis of superacid-functionalized mesoporous nanocages with tunable pore diameters and their application in the synthesis of coumarins.

Here we demonstrate for the first time the preparation of a triflic acid (TFA)-functionalized mesoporous nanocage with tunable pore diameters by the wet impregnation method. The obtained materials have been unambiguously characterized by XRD, N(2) adsorption, FTIR spectroscopy, and NH(3) temperature-programmed desorption (TPD). From the characterization results, it has been found that the TFA molecules are firmly anchored on the surface of the mesoporous supports without affecting their acidity. We also demonstrate the effect of the pore and cage diameter of the KIT-5 supports on the loading of TFA molecules inside the pore channels. It has been found that the total acidity of the materials increases with an increase in the TFA loading on the support, whereas the acidity of the materials decreases with an increase in the pore diameter of the support. The acidity of the TFA-functionalized mesoporous nanocages is much higher than that of the zeolites and metal-substituted mesoporous acidic catalysts. The TFA-functionalized materials have also been employed as the catalysts for the synthesis of 7-hydroxy-4-methylcoumarin by means of the Pechmann reaction under solvent-free conditions. It has been found that the catalytic activity of the TFA-functionalized KIT-5 is much higher than that of zeolites and metal-substituted mesoporous catalytic materials in the synthesis of coumarin derivatives. The stability of the catalyst is extremely good and can be reused several times without much loss of activity in the above reaction.