Vidya Krishna

Mendelian susceptibility to mycobacterial disease (MSMD) is a rare group of inborn errors of immunity associated with significant morbidity due to mycobacterial infections. Hematopoietic stem cell transplantation (HSCT) is a curative option; however, persistent infections and high levels of interferon-γ (IFN-γ) create a hostile environment for stem cell proliferation and can mediate graft rejection. We describe the outcome of children with MSMD due to IFN-γ receptor and interleukin-12 (IL-12) defects who underwent HSCT in our unit from December 2015 to December 2023. We collected the data retrospectively from a review of patient charts and analyzed the outcomes. Seven children underwent eight procedures, including four with IFN-γ defects and three with IL-12 defects. The male-to-female ratio was 1.6:1, and the median age at HSCT was 4.5 years. Five received haploidentical HSCT, and two underwent matched family donor transplants; 75% used peripheral blood stem cells. IL-12–deficient cases engrafted well with fludarabine/treosulfan–based conditioning, whereas IFN-γ–deficient cases fared better with fludarabine/treosulfan/thiotepa-based conditioning due to a higher rejection risk. Acute graft-versus-host disease (GVHD) (grade 1–2, skin/gut) occurred in two children: one developed chronic liver GVHD after donor lymphocyte infusion. Cytomegalovirus reactivation occurred in four cases, which responded to ganciclovir or cidofovir. One primary graft failure was rescued with a second HSCT; one secondary graft failure led to death from invasive fungal infection. At a median follow-up of 8 years, the overall survival was 87%. One survivor has ongoing seizures, aggressive behavior, and endocrine dysfunctions requiring supportive care. HSCT is a curative option for children with MSMD. However, we need to understand gene mutation and individualize the approach to preparation and conditioning regimens to help optimize the outcome.

Infections occur frequently in oncological and transplant settings and are complicated due to several factors not limited to the degree of immunosuppression, healthcare exposure, and antimicrobial resistance. In addition to common bacterial pathogens, viruses and fungi and other opportunistic pathogens can cause infections in these patients. A strong understanding of the host susceptibility along with prudent diagnostic workup and management is required to improve the outcomes without compromising on antimicrobial stewardship. In this review article, we discuss approach to febrile neutropenia in oncology patients.

Due to increasing carbapenem usage, resistance to gram negative bacteria is steadily going up in India. Resistance is often enzyme-mediated and transmissible horizontally by plasmids. Use of invasive lines, procedures, prior antibiotic exposure, recent hospitalization, ICU stay, colonization and severity of illness are important risk factors for infections with these bacteria. Before starting therapy, it is important to distinguish colonization from true infection. Early targeted therapy using rapid diagnostic methods is important to ensure optimal outcomes. A growing body of literature recommends the use of novel beta lactam-beta lactamase inhibitors over polymyxins in the management of these infections. Treatment strategies for carbapenem resistant Enterobacterales differs from non-Enterobacterales and is discussed in detail in this review. Infection control and antimicrobial stewardship are crucial preventive measures to curb the emergence of further resistance.

We present the case of a young man with a giant midesophageal ulcer as the primary manifestation of HIV. We highlight the importance of accurate HIV testing, as his diagnosis was much delayed before his presentation here.

Background: Though the risk of tuberculosis is expected to increase during adolescence, the exact burden of the disease in this age group is often underrepresented and understudied in literature. Methods: We did a retrospective analysis of hospital records of adolescents aged 10-19 years admitted with tuberculosis at Apollo Children’s Hospital, Chennai between January 2014 and March 2022, after ethical board clearance. Results: Out of 54 cases, 42 were in the young adolescent group and 12 belonged to the older adolescent group. Sex distribution showed slightly higher incidence in females (55.6% vs 44.4%). Fever was the most common presenting complaint (72.2%) followed by loss of weight (51.9%) and cough (48.1%). Out of total 54 cases, 16 were pulmonary TB, 28 were extrapulmonary and 16 were disseminated. Consolidation was the predominant radiological feature (31.48%) followed by mediastinal lymphadenopathy (25.92%) and cavity (16.66%). Cartridge based nucleic acid amplification test was positive in 68.5% of the cases while smear was positive only in 9 cases (16.7%) and histopathology positive in 5 cases (9.2%). Conclusions: Adolescent tuberculosis occurs mostly in the young adolescent group. This group should be recognized as a key risk period and not clubbed in with childhood.

COVID-19-associated mucormycosis (CAM) emerged as an epidemic in certain parts of the world amidst the global COVID-19 pandemic. While rhino–orbital mucormycosis was well reported during the pandemic, in the absence of routine diagnostic facilities including lower airway sampling, pulmonary mucormycosis was probably under-recognized. In this review, we have focused on the epidemiology and management of COVID-19-associated pulmonary mucormycosis (CAPM). CAPM is a deadly disease and mortality can be as high as 80% in the absence of early clinical suspicion and treatment. While histopathological examination of tissue for angio-invasion and cultures have remained gold standard for diagnosis, there is an increasing interest in molecular and serological methods to facilitate diagnosis in critically ill patients and often, immune-suppressed hosts who cannot readily undergo invasive sampling. Combined medical and surgical treatment offers more promise than standalone medical therapy. Maintaining adequate glycemic control and prudent use of steroids which can be a double-edged sword in COVID-19 patients are the key preventative measures. We would like to emphasize the urgent need for the development and validation of reliable biomarkers and molecular diagnostics to facilitate early diagnosis.