Extensive perineal ecthyma gangrenosum in leukocyte adhesion deficiency type 1 associated with Staphylococcus hominis bacteremia.

Leukocyte adhesion deficiency (LAD), a disorder of neutrophil function, is characterized by a defect in leukocyte adhesion to the endothelium. Recurrent infections in the skin, soft tissue, gingiva, and lungs due to Staphylococcus aureus, Pseudomonas aeruginosa, and Klebsiella sp. are common in these patients. Ecthyma gangrenosum (EG) is an ulcer of skin and subcutaneous tissue with a black eschar and surrounding erythematous halo secondary to a bacterial infection. Here, we report an unusual presentation of LAD type-1 with extensive EG of perineum secondary to Staphylococcus hominis bacteremia treated successfully with combination of granulocyte transfusion and diversion colostomy.

Clinical profile and outcomes of multisystem inflammatory syndrome in children (MIS-C): Hospital-based prospective observational study from a tertiary care hospital in South India.

AIM: To study the clinical profile and outcomes in children with multisystem inflammatory syndrome in children (MIS-C). METHODS: Children aged 1 month to 15 years presenting with MIS-C (May 2020 to November 2021) were enrolled. Clinical, laboratory, echocardiography parameters and outcomes were analysed. RESULTS: Eighty-one children (median age 60 months (24-100)) were enrolled. Median duration of fever was 5 days (3-7). Twenty-nine (35.8%) had shock (severe MIS-C) including 23 (28.3%) requiring inotropes (median duration = 25 h (7.5-33)). Ten required mechanical ventilation, 12 had acute kidney injury and 1 child died. Left ventricular (LV) dysfunction was seen in 38 (46.9%), 16 (19.7%) had coronary artery abnormalities (CAA) and 13 (20%) had macrophage activation syndrome. Sixty-one (75.3%) were SARS CoV-2 positive (10 by RT-PCR and 51 by serology). Sixty-eight (83.9%) received immunomodulators. Younger age was significantly associated with CAA (P value = 0.05). Older age, LV dysfunction, SARS CoV-2 positivity, low platelet count and elevated serum ferritin were significantly associated with severe MIS-C (univariate analysis). Younger age was an independent predictor of CAA (P = 0.05); older age (P = 0.043) and low platelet count (P = 0.032) were independent predictors of severe MIS-C (multivariate logistic regression analysis). CONCLUSION: Our patients had diverse clinical manifestations with a good outcome. Younger age was significantly associated with CAA. Older age, LV dysfunction, low platelet count and elevated serum ferritin were significantly associated with severe MIS-C. Younger age is an independent predictor of CAA. Older age and low platelet count are independent predictors of severe MIS-C.

Outcome and prognostic factors in childhood B non-Hodgkin lymphoma from India: Report by the Indian Pediatric Oncology Group (InPOG-NHL-16-01 study).

The literature on B-non-Hodgkin lymphoma (NHL) in India is restricted to individual hospital data. The study aimed to evaluate the epidemiology and outcome of B-NHL in our country. One hundred and ninety-one patients of B-NHL from 10 centers diagnosed between 2013 and 2016 were analyzed retrospectively. B/T lymphoblastic lymphoma and patients with inadequate data were excluded. The median age was 88 months (IQR: 56, 144) with an M:F ratio of 5.6:1. Undernourishment and stunting were seen in 36.5% and 22%. Primary site was abdomen in 66.5%. Hypoalbuminemia was noted in 82/170 (48.2%). Histological subtypes: Burkitt lymphoma (BL): 69.6%, Burkitt-like: 10.4%, and diffuse large B cell lymphoma (DLBCL): 13.6%, unclassified and others (6.4%). Stage distribution: I/II, 33 (17.3%), III, 114 (59.7%), and IV, 44 (23%). One-eighty-six patients took treatment. Protocols used were LMB and BFM in 160/186 (86%). At a median follow-up of 21.34 (IQR: 4.34, 36.57) months, the disease-free-survival (DFS) was 74.4% and event-free-survival (EFS) was 60.7%. Treatment-related mortality (TRM), relapse/progression and abandonment were 14.3%, 14.5%, and 8.4%, respectively. Bone marrow positivity, stage IV disease, and lactate dehydrogenase (LDH) > 2,000 U/l predicted inferior EFS. Stage IV disease, LDH > 2,000 U/l, bone marrow positivity, tumor lysis syndrome and low albumin predicted TRM; LDH retained significance on multivariate analysis for EFS and TRM [OR: 4.54, 95% CI: 1.14-20, p 0.03; OR 20, 95%CI: 1.69-250, p 0.017]. BL was the main histological subtype. High TRM and relapse/progression are hampering survival. An LDH > 2,000 U/l was adversely prognostic. These data demonstrate a need to develop a national protocol that balances toxicity and potential for cure.

An Unusual Presentation of Recurrent Childhood Kimura Disease: Ileocecal Involvement.

Kimura disease commonly presents as an isolated swelling over the head and neck region. Intestinal involvement by Kimura disease in children is uncommonly reported. We report a 10-year-old boy who had presented with an ileocaecal mass with peripheral blood eosinophilia and elevated immunoglobulin E levels. The histopathologic examination from the ileocaecal mass was suggestive of Kimura disease. He had 2 recurrences once in the left axillary region and once in the bilateral cervical region. Ileocaecal involvement in Kimura disease is an uncommon presentation in childhood. Careful evaluation of complete blood count is critical in making diagnosis and avoiding unnecessary invasive procedures.

Intravenous fosaprepitant for the prevention of chemotherapy-induced vomiting in children: A double-blind, placebo-controlled, phase III randomized trial.

BACKGROUND: Fosaprepitant is a neurokinin-1 receptor antagonist, approved for the prevention of chemotherapy-induced nausea and vomiting. The data on the use of fosaprepitant in children are limited and therefore we conducted a phase III randomized controlled trial. PROCEDURE: Children aged 1-12 years scheduled to receive moderately or highly emetogenic chemotherapy were randomly assigned to arm-A (fosaprepitant) or arm-B (placebo). Children recruited to arm-A received intravenous ondansetron plus dexamethasone followed by fosaprepitant infusion. Children recruited to arm-B received the same drugs as those given to children in arm-A, except that fosaprepitant was substituted with a placebo. Ondansetron and dexamethasone were continued for 48 hours after completion of chemotherapy. The primary end point of the study was to determine the proportion of patients who achieved a complete response (CR), defined as no vomiting, no retching, and no use of rescue medication, during the 24-120 hours (delayed phase) after administration of the last dose of chemotherapy. Secondary end points were the proportion of patients who achieved a CR during the acute phase (0-24 hours) and overall after administration of the last dose of chemotherapy. RESULTS: One-hundred-sixty-three patients were analyzed (81 in the fosaprepitant arm and 82 in the placebo arm). CR rates were significantly higher in the fosaprepitant arm compared to those in the placebo arm during the acute phase (86% vs 60%, P < 0.001), delayed phase (79% vs 51%, P < 0.001), and overall phase (70% vs 41%, P < 0.001). Three (4%) patients in the fosaprepitant arm and sixteen (20%) in the placebo arm required rescue anti-emetics (P = 0.0017). CONCLUSION: Addition of fosaprepitant to ondansetron and dexamethasone improved chemotherapy-induced vomiting control in children treated with moderately or highly emetogenic chemotherapy.

Neuroimaging Abnormalities in Patients With Nontransfusion-dependent Thalassemia.

Independence from regular transfusions is the hallmark of nontransfusion-dependent thalassemia (NTDT). However, the associated complications need anticipation and screening. One such complication is a hypercoagulable state predisposing to development of thrombosis. We evaluated children with NTDT >10 years of age for prevalence of neuroimaging abnormalities (NIA) and identified associated risk factors. In total, 29 patients were evaluated. Blood counts, serum ferritin, protein C, protein S, antithrombin III, brain magnetic resonance imaging, and angiography was done in all patients. Possible risk factors for thrombosis or cerebrovascular disease were analyzed for association with NIA. The median age was 14 (12 to 15) years. Fifty percent were splenctomized and 31.5% were transfusion naïve. Eleven patients (37.9%) had NIA: 6 with silent cerebral infarction (SCI); 2 with cerebral arteriopathy (CA) and 3 having both CA and SCI. Higher white blood cell (WBC) count was associated with NIA (P=0.034) [silent cerebral infarction (P=0.047) and cerebral arteriopathy (P=0.067)]. Presence of 7 or more risk factors had 4.5 times greater risk of a NIA, especially silent cerebral infarction (SCI) (P=0.03). We conclude that cerebral infarction and arteriopathy seem to start in late childhood. There is a need to develop strategies for preventing neurologic complications in NTDT similar to sickle cell disease.

Prevalence of multi-drug resistant organisms in stool of paediatric patients with acute leukaemia and correlation with blood culture positivity: A single institution experience.

BACKGROUND: Multi-drug resistant (MDR) bacteria are associated with increased morbidity and mortality in children with acute leukaemia. The present study was conducted to assess the prevalence of MDR bacteria in stool cultures of patients with acute leukaemia at presentation to the hospital. The results were then correlated with blood cultures when patients developed septicaemia. PATIENTS AND METHODS: The study involved analysis of case records of patients with newly diagnosed acute leukaemia less than 18 years of age treated at our centre from January 2015 to December 2015. Stool cultures were sent within 72 hr of hospital admission and blood cultures were sent when clinically indicated. MDR was defined as resistance to at least one antibiotic in three or more following antimicrobial groups: cephalosporins, ß-lactam/ß-lactamase inhibitor, carbapenems, fluoroquinolones and aminoglycosides. RESULTS: The analysis included 85 patients with acute leukaemia, among whom 48 of 85 (56%) patients had positive stool cultures and 42 of 85 (50%) patients were positive for MDR bacteria. Blood cultures were positive in 13 of 48 patients (27%, seven MDR and six non-MDR) with positive stool cultures and three of 37 patients (8%, one MDR and two non-MDR) with negative stool cultures (P = 0.01). The concordance between stool and blood culture for similar organism was 61%. There were seven deaths in 48 stool culture positive patients and two deaths in 37 stool culture negative patients. CONCLUSION: This study shows the high prevalence of MDR bacteria in newly diagnosed children with acute leukaemia. Colonisation with MDR bacteria in stools is associated with increased positivity of blood cultures and mortality.

Orbital Mass in a Child With Acute Lymphoblastic Leukemia: A Case Report and Review of the Literature.

Acute lymphoblastic leukemia arising from lymphoid precursor cells of the bone marrow, the lymphoreticular system, and the soft tissue can present with medullary and extramedullary involvement. Extramedullary involvement has the propensity to affect a multitude of organs. Presentation with proptosis secondary to orbital mass in childhood acute lymphoblastic leukemia (ALL) is very rare. We report a child with pre-B cell ALL with an extramedullary soft tissue mass involving both orbits presenting with proptosis, and give a brief overview of the literature about this unusual entity. Rapid investigation and timely initiation of treatment are needed to salvage the eye and the vision. Orbital involvement is considered to confer a poorer prognosis to children with ALL.

Clinical Outcome and its Predictors in Children With Newly Diagnosed Immune Thrombocytopenia.

OBJECTIVE: To determine the predictors for chronic and/or persistent immune thrombocytopenia (ITP) among children with newly diagnosed ITP. METHODS: Ours was a mixed-design study (prospective: January 2020 to March 2022 and retrospective: January 2014 to December 2019), wherein we enrolled children, aged 1 month to 18 years presenting with newly diagnosed ITP. RESULTS: Of the 64 enrolled participants, 58 were followed up for atleast 1-year duration and 6 children were followed up for 3 to 12 months' duration. The median (IQR) age of the cohort was 8 (5, 11) years with a female preponderance (62.5%). Wet bleeding was seen in 56%; 6.25% developed intracranial bleeding. 67.2% (43/64) and 41.4% (24/58) children developed persistent and chronic ITP, respectively. Of the 34 children who achieved complete response at 12-months follow up, 21 (62%) achieved complete response by 3 months and the rest achieved complete response over the next 9 months. Development of overall response (complete or partial) at 3 and 12 months, was associated with a higher absolute lymphocyte count (ALC) at admission. The median ALC (×103/µL) at admission was 3.77 and 2.87 in children who had overall response and no response at 3 months, respectively (P = 0.03). The median ALC (×103/µL) at admission was 3.99 and 2.96 in children who had overall response and no response at 12 months, respectively (P = 0.04). Response rate was lesser in the treated group by approximately 10% compared to the non-treated group. CONCLUSION: The rate of chronicity and intracranial bleeding in our cohort is more than the reported rates in literature. Higher ALC was found to be associated with response.

A rare case of congenital insensitivity to pain with anhidrosis.

A 22-month-old girl of consanguineous parents was admitted with a high-grade fever. She was found to have insensitivity to painful stimuli and an absence of perspiration. She also displayed self-mutilating behaviour and was insensitive to cold/hot water on her body. On examination, there was loss of the tip of the tongue, missing teeth, generalised xerosis, and several ulcers at sites of minor trauma. She also had dysplastic nails and digital ulcers. Sensory examination demonstrated a complete lack of awareness of pain and temperature, vibration and fine touch were intact and lacrimation was normal. Differential diagnoses of hereditary sensory and autonomic neuropathy (HSAN), Lesch-Nyhan syndrome, hypohidrotic ectodermal dysplasia and leprosy were considered. Results of routine blood investigations including serum uric acid were normal. On performing clinical exome sequencing, the diagnosis of congenital insensitivity to pain with anhidrosis (CIPA) of autosomal recessive inheritance was confirmed. A novel, predicted to be pathogenic variant detected at exon 16 of the NTRK1 gene resulting in congenital insensitivity to pain with anhidrosis is reported.Abbreviations: CIPA: congenital Insensitivity to pain with anhidrosis; HSAN: hereditary sensory and autonomic neuropathy; NGF: nerve growth factor; NTRK1: neurotrophic tyrosine kinase receptor 1 gene; TrKA: tropomyosin receptor kinase A.

Longitudinal Echocardiographic Follow-Up of a Pediatric Multisystem Inflammatory Syndrome Cohort.

OBJECTIVE: Significant involvement of the cardiovascular system is known in multisystem inflammatory syndrome in children (MIS-C). This study aimed to examine the recovery of affected cardiovascular parameters over a medium-term follow-up. METHODS: A cohort of 69 children was studied prospectively. Assessments of left ventricular (LV) function and coronary artery abnormalities (CAA) were conducted at admission, 1.5 months, and 3 months. Coronavirus Disease 2019 (COVID-19) antibody titers were assessed at these three time points. Echocardiographic and antibody parameters (rising/decreasing) were analyzed for correlation. Outcomes were assessed using logistic regression. RESULTS: At admission, among the 78.2% of patients who were tested, 88.9% tested positive for Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2). A quarter of the patients had pericardial effusion, and half had valvulitis. Decreased ejection fraction, global circumferential strain (GCS), and global longitudinal strain (GLS) were seen in 54.4%, 68.6%, and 35.8% of patients, respectively. CAAs were observed in 27.78% of patients. Systolic dysfunction was significantly associated with older age. During follow-up, severe LV dysfunction normalized within 6-7 weeks, while mild to moderate dysfunction reached normalcy by two weeks. Both GCS and GLS reached normalcy within a median of two weeks. Diastolic parameters recovered by six weeks. Most small and moderate coronary aneurysms resolved, but a giant aneurysm in an infant remained large even after 15 months. Trends in antibodies and ejection fraction (EF) at three months were significantly correlated. Admission EF, GLS (at 6 weeks) and deceleration time (at 3 months) were significantly associated with intensive care unit (ICU) admission. The median segmental strain of the cohort remained low in certain segments at three months. CONCLUSION: Smaller CAAs resolve, whereas giant CAAs persist. EF and GLS are important predictors of Pediatric Intensive Care Unit (PICU) stay. The residual impairment of median segmental strain and persistent diastolic dysfunction at three months indicate the need for long-term follow-up.

Knowledge and attitude on childhood cancer survivorship among healthcare trainees: a multicentre study from India.

BACKGROUND: The proportion of childhood cancer survivors (CCS) in low/middle-income countries (LMICs) is rising. CCS often develop several physical and psycho-social long-term adverse effects, with unique healthcare needs. Primary healthcare providers (primary care physicians (PCPs)), especially in LMICs, are often not equipped to handle survivorship care. This study aimed to assess knowledge, and attitude among trainee healthcare providers concerning major issues of paediatric survivorship care. METHODS: A multi-centre, cross-sectional, questionnaire-based study was conducted among nursing and medical undergraduate students, and postgraduate medical residents across three tertiary-care teaching hospitals in India-All India Institute of Medical Sciences, New Delhi; Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry; and Maulana Azad Medical College, New Delhi. A questionnaire with total of 24 questions (14 knowledge-based and 10 attitude-based) was finalised after validation by expert review and piloting. The major domains covered in the questionnaire included knowledge and attitude regarding long-term adverse effects and psychosocial, employment-related issues faced by the survivors. It was administered to the study participants electronically. The knowledge-based questions had true/false responses (scored as 0 or 1 if incorrect or correct, respectively). Attitude-based questions were scored as 5-point Likert scale. RESULTS: Total 898 responses were collected (median age: 21 years, 64% (576/898) female). Among the respondents, 44% were undergraduate medical students, 42% were nursing students and 14% were postgraduate medical residents. The mean (SD) of knowledge score was 8.72 (2.04) (out of 14). On multivariable analysis, only discipline of training predicted knowledge scores regarding survivorship care. Postgraduate medical residents (9.08) as well as undergraduate medical students (8.85), had significantly higher mean knowledge scores than nursing students (8.47) (p=0.004).Two questions were answered incorrectly by the majority; children and siblings of CCS need additional genetic screening (79% incorrectly answered true), and CCS face intimacy issues in relation to normal sexual functioning (59% incorrectly answered false).Nearly half (48%) of respondents believed that their knowledge of cancer survivorship issues was inadequate. Majority of respondents (84%) suggested that oncologists should handle long-term survivorship care rather than PCPs. CONCLUSION: Trainee healthcare providers in India reported inadequate knowledge regarding survivorship care. Improving awareness by incorporating survivorship in teaching curriculum is imperative to equip future PCPs to provide survivorship care across the country.

Neutrophil-Lymphocyte Ratio for Predicting Coronary Artery Lesions in Children With Kawasaki Disease.

BACKGROUND: Coronary artery lesions (CAL) are a specific feature of Kawasaki disease (KD), and develop during the second week of illness. This study was conducted to determine whether Neutrophil: Lymphocyte Ratio (NLR), assessed between the fourth and sixth day of fever onset in children with KD, can predict coronary artery lesion (CAL) development. METHODS: In this review of hospital records, data of patients with KD admitted at our center between January, 2016 and January, 2020 was retrieved. The patients were divided into two groups based on the presence of CAL, and clinical characteristics of patients were compared between the two groups. RESULTS: Out of the 79 patients enrolled, CAL was found in 40 (50.6%) patients and intravenous immunoglobulin (IVIg) resistance was seen in 13 (16.5%) patients. Multivariate logistic regression revealed NLR as an independent predictor of CAL [OR (95% CI) 2.0 (1.2-3.1); P<0.001], and erythrocyte sedimentation rate (ESR) [OR (95% CI) 1.03 (1.001-1.1) P=0.04], as an independent predictor of IVIg resistance. NLR ≥2.08 was 82% sensitive and 80% specific in predicting CAL. ESR ≥88 mm/h was 85% sensitive and 64% specific in predicting IVIg resistance. CONCLUSIONS: NLR is an independent predictor of CAL in KD. NLR ³2.08 done between the fourth and sixth day of fever onset may identify children with KD at risk of CAL.