Immunophenotypic and Cytogenetic Characteristics of Pediatric Acute Lymphoblastic Leukemia: A Burden Estimation Study from Eastern India.

BACKGROUND: Acute lymphoblastic leukemia (ALL) is the most common childhood cancer. Immunophenotype (IPT) and cytogenetics are essential for diagnosis, risk stratification, and management for ALL. OBJECTIVES: Evaluating the burden of immunophenotypic and cytogenetic profile of pediatric ALL patients. MATERIALS AND METHODS: A descriptive cross-sectional study was conducted on 100 patients of ALL (1-18 completed years) attending a tertiary-care center in Kolkata, Eastern India. RESULTS: Ninety-six percent of patients had B-cell ALL (94.00% pre-B ALL and 2.00% Pro-B ALL) and 4.0% had T-ALL. 60% B-cell ALL were CD19/CD10 positive, 10% were CD79a positive, 9% were only CD19 positive, and 7% were only CD10 positive. Thirty-three percent of T-ALL were CD3+, whereas 22% were positive each for CD4 and CD7. 51.0% of patients had diploid, 46.0% hyperdiploid, and 3.0% hypodiploid karyotype. Among hyperdiploids, 98% had good prednisolone response and 89% had measurable residual disease (MRD) <0.01. CONCLUSION: The most commonly diagnosed ALL by IPT was pre-B ALL. Among the detectable cytogenetic abnormalities, t(12; 21) ETV6-RUNX1 was the most common. ZNF-384 gene arrangement was also detected in our study. t(12;21) ETV6-RUNX1 had a good treatment response, while t(9;22) BCR-ABL, t(1;19) TCF3-PBX1, iAMP-21, MLL gene rearrangement, and ZNF-384 gene arrangement had poor treatment response in terms of MRD.

Spontaneous Bacterial Peritonitis Because of Actinomyces.

Actinomycosis is a rare chronic granulomatous disease that manifests with nonspecific symptoms of abdominal pain, anorexia, and weight loss. The disparity in the presentation of this condition presents a tremendous diagnostic challenge. There are few reports of Actinomyces species causing spontaneous bacterial peritonitis without previous localized masses or abscesses have been published. We provide a case of spontaneous bacterial peritonitis secondary to Actinomyces species in a 46-year-old woman with uterine fibroids and a lack of preceding abscess. Although rare, spontaneous bacterial peritonitis because of Actinomyces should be considered in differential in female patients without pre-existing liver disease presenting with spontaneous bacterial peritonitis.

Unconventional Rocky Mountain Spotted Fever Presentation From Kentucky: A Compelling Case Report and Literature Review.

Rocky Mountain spotted fever (RMSF) is a potentially lethal tick-borne disease caused by Rickettsia rickettsii, known for its tropism for vascular endothelial cells. Its classic symptoms include fever, headaches, and a rash, but atypical presentations can challenge diagnosis. We present the case of a 71-year-old male with fever, weakness, and hiccups, evolving into confusion. Laboratory findings showed severe hyponatremia, leukocytosis, and abnormal blood parameters. Initial management addressed sepsis and hyponatremia, leading to symptom improvement. Later, a fever of 106.5°F prompted ICU transfer, broad-spectrum antibiotics, and testing for tick-borne diseases. The patient reported tick exposure and received prophylactic doxycycline. Follow-up confirmed the RMSF diagnosis based on serological testing and clinical symptoms. This case highlights the diagnostic challenges posed by atypical RMSF presentations and underscores the importance of early detection and treatment to prevent complications.

A Case of Bradycardia, Renal Failure, Atrioventricular Nodal Blockade, Shock, and Hyperkalaemia (BRASH) Syndrome in an Elderly Male and Its Management: A Case Report and Literature Review.

BRASH syndrome, characterized by bradycardia, renal dysfunction, atrioventricular (AV) nodal blockage, shock, and hyperkalemia, is a rare but potentially life-threatening condition resulting from the interplay between AV nodal blockers and hyperkalemia. This complex syndrome poses significant challenges in diagnosis and management, with patients often presenting with bradycardia and high potassium levels. This case report highlights the need for increased awareness of BRASH syndrome, especially in an aging population and evolving cardiovascular treatments. Early recognition and a comprehensive, multidisciplinary approach are crucial for improving outcomes in affected patients.

A Rare Case of Non-Small-Cell Lung Carcinoma Complicated by Massive Pericardial Effusion and Cardiac Tamponade: A Case Report.

This case report presents a late middle-aged man with a right infra-hilar lung mass and pericardial effusion (PE). The patient was diagnosed with metastatic small-cell lung carcinoma, with metastases to the liver, pancreas, and cerebellum. The pericardial fluid cytology confirmed the presence of malignant cells most compatible with non-small-cell carcinoma. The patient received carbo/etoposide chemotherapy, and his treatment plan included adding atezolizumab and radiation therapy. Despite the excellent efficacy of immunotherapy, immune-related adverse events (IRAEs), including cardiac toxicity, were noted in some patients. PE related to immune checkpoint inhibitor (ICI) use is rare but potentially severe. This case highlights the importance of vigilant monitoring for cardiovascular symptoms during immunotherapy and the significance of pericardial fluid analysis in diagnosing malignant pericardial disease. Prompt diagnosis and appropriate treatment can lead to improved patient outcomes in cases of lung cancer-associated cardiac complications.

N-Terminal Pro-Brain Natriuretic Peptide Levels in Kawasaki Disease, Sepsis and Other Febrile Illnesses.

OBJECTIVE: To compare the values of N-Terminal Pro-Brain Natriuretic Peptide (NT-proBNP) in acute phase of Kawasaki disease (KD), sepsis and other acute febrile illnesses. METHODS: We conducted a cross-sectional study on 40 KD patients and 40 age- and sex-matched controls with sepsis and other febrile illnesses between January, 2019 and June, 2020. Complete blood count, C-reactive protein (CRP), liver and renal function tests, serum electrolytes, chest X-ray, NT-proBNP level, Bactec blood culture, urine microscopy and culture along with detailed physical examination was done for all cases and control. RESULTS: Mean (SD) values of NT-proBNP levels in acute KD was higher than sepsis/other febrile illnesses (914.9 vs 219 pg/mL; P=0.001). Also, the number of KD patients whose NT-proBNP was elevated were significantly higher compared to controls (70% vs 32.5%; P<0.001). CONCLUSION: NT-proBNP may be useful as a biomarker in the diagnosis of acute phase KD.

Risk factors in IVIG-resistant Kawasaki disease and correlation with Japanese scoring systems - a study from Eastern India.

OBJECTIVES: To assess the risk factors of intravenous immunoglobulin (IVIG)-resistant Kawasaki disease (KD) and to evaluate the performance of the three Japanese risk-scoring systems, namely the Kobayashi, Egami, and Sano scores in predicting IVIG resistance among the Indian patients. METHODS: Prospective observational study on children admitted with KD at Institute of Child Health, Kolkata, over a period of 16 months, from January 2019 to April 2020. The study included 70 KD patients all of whom were treated with IVIG. Clinical parameters, laboratory variables, and risk scores were compared between the IVIG-responsive and the IVIG-resistant groups. RESULTS: A total of 31.4% were IVIG non-responders. Skin rash was found to be significantly associated with IVIG-resistant KD. The IVIG-resistant group had higher total bilirubin, lower albumin, higher CRP levels, and higher ALT and AST levels. High Kobayashi score, high Egami score, and high Sano score were significantly associated with IVIG resistance, individually. Sano score had the highest sensitivity (81.8%) and Kobayashi score had the highest specificity (77.1%) in our cohort. CONCLUSION: The presence of skin rash, high total bilirubin, high CRP, high AST, high ALT, and low albumin were important predictors of IVIG resistance in our population. Among the three scores, Sano score is the most reliable in identifying potential non-responders to IVIG. But Sano score lacked good specificity. Therefore, Indian KD patients may need an exclusive scoring system to predict non-responsiveness to IVIG so that a more aggressive therapy can be instituted at the earliest. Key points • Early prediction of IVIG-resistant KD is necessary to limit cardiac injuries. • Sano score has high sensitivity to predict IVIG resistance in Indian population.

Highly efficient, processive and multifunctional recombinant endoglucanase RfGH5_4 from Ruminococcus flavefaciens FD-1 v3 for recycling lignocellulosic plant biomasses.

Gene encoding endoglucanase, RfGH5_4 from R. flavefaciens FD-1 v3 was cloned, expressed in Escherichia coli BL21(DE3) cells and purified. RfGH5_4 showed molecular size 41 kDa and maximum activity at pH 5.5 and 55 °C. It was stable between pH 5.0-8.0, retaining 85% activity and between 5 °C-45 °C, retaining 75% activity, after 60 min. RfGH5_4 displayed maximum activity (U/mg) against barley ß-D-glucan (665) followed by carboxymethyl cellulose (450), xyloglucan (343), konjac glucomannan (285), phosphoric acid swollen cellulose (86), beechwood xylan (21.7) and carob galactomannan (16), thereby displaying the multi-functionality. Catalytic efficiency (mL.mg-1 s-1) of RfGH5_4 against carboxymethyl cellulose (146) and konjac glucomannan (529) was significantly high. TLC and MALDI-TOF-MS analyses of RfGH5_4 treated hydrolysates of cellulosic and hemicellulosic polysaccharides displayed oligosaccharides of degree of polymerization (DP) between DP2-DP11. TLC, HPLC and Processivity-Index analyses revealed RfGH5_4 to be a processive endoglucanase as initially, for 30 min it hydrolysed cellulose to cellotetraose followed by persistent release of cellotriose and cellobiose. RfGH5_4 yielded sufficiently high Total Reducing Sugar (TRS, mg/g) from saccharification of alkali pre-treated sorghum (72), finger millet (62), sugarcane bagasse (38) and cotton (27) in a 48 h saccharification reaction. Thus, RfGH5_4 can be considered as a potential endoglucanase for renewable energy applications.

Efficacy of empiric esophageal dilation in patients with non-obstructive dysphagia: systematic review and meta-analysis.

BACKGROUND AND AIMS: Empiric esophageal dilation is frequently performed for non-obstructive dysphagia. Studies evaluating its efficacy have reported conflicting results. In this meta-analysis, we have evaluated the efficacy of esophageal dilation in the management of non-obstructive dysphagia. METHODS: We reviewed several databases from inception to 26 May 2021 to identify randomized controlled trials (RCTs) and observational studies that evaluated the role of empiric esophageal dilation for non-obstructive dysphagia. Our outcomes of interest were clinical success (improvement in dysphagia after dilation) and difference in post-operative dysphagia score between groups. For categorical variables, we calculated pooled odds ratios (OR) with 95% confidence intervals (CI); for continuous variables, we calculated standardized mean difference (SMD) with 95% CI. Data were analyzed using a random effects model. We used GRADE framework to ascertain the quality of evidence. RESULTS: We included 4 studies (3 RCTs and one observational) with 243 patients; there were 133 treated with empiric dilation and 110 controls. We found no significant difference in clinical success (OR (95% CI) 1.91 (0.89, 4.08)) or post-procedure dysphagia score between groups (SMD (95% CI) 0.38 (-0.37, 1.14)). Our findings remained consistent on subgroup analysis including RCTs only. Quality of evidence ranged from low to very low based on GRADE framework. CONCLUSIONS: Our meta-analysis does not support the use of empiric esophageal dilation in patients with non-obstructive dysphagia. More studies are required to confirm these findings.