Infantile Disseminated Bacille Calmette-Guérin Disease with Hemophagocytosis and Mimicking Juvenile Myelomonocytic Leukemia: A Case Report with Concise Literature Review.

Bacille Calmette-Guérin (BCG) is a live-attenuated vaccine routinely administered to newborns to prevent severe forms of tuberculosis (TB) in TB-endemic countries. Disseminated BCG vaccine disease is a classic feature of children with human immunodeficiency virus (HIV) or primary immunodeficiency disorders (PIDs) and is associated with high mortality. We report a case of a 6-month-old infant with disseminated BCG disease and hemophagocytic lymphohistiocytosis mimicking juvenile myelomonocytic leukemia with no demonstrable features of HIV or PID even after extensive laboratory work-up and succumbed to progressive disease. Disseminated BCG disease is a rare and potentially fatal complication of BCG vaccine, and prompt immunological evaluation complemented by initiation of 4-drug antitubercular therapy and definitive treatment with antiretroviral therapy or hematopoietic stem cell transplant is warranted.

Drug Induced Diabetes Mellitus in Pediatric Acute Lymphoblastic Leukemia: Approach to Diagnosis and Management.

Corticosteroids and l -asparaginase used in the treatment of pediatric acute lymphoblastic leukemia (ALL) can cause drug-induced diabetes mellitus (DIDM). DIDM can lead to dyselectrolytemia, a higher risk of infections including cellulitis, bacteremia, fungemia, and a higher incidence of febrile neutropenia and may have an impact on the outcome of ALL. Literature on the management of DIDM among children with ALL is sparse and the diagnostic criteria for pediatric diabetes should be carefully applied considering the acute and transient nature of DIDM during ALL therapy. Insulin remains the standard of care for DIDM management and the choice of Insulin regimen (stand-alone Neutral Protamine Hagedorn or basal bolus) should be based on the type and dose of steroids used for ALL and the pattern of hyperglycemia. A modest glycemic control (postmeal 140 to 180 mg/dL, premeal <140 mg/dL) to prevent complications of hyperglycemia, as well as hypoglycemia, would be the general approach. This review is intended to suggest evidence-based practical guidance in the diagnosis and management of DIDM during pediatric ALL therapy.

Evaluating the Need for Integrated Pediatric Palliative Care Services in a Pediatric Oncology Setting: A Retrospective Audit.

OBJECTIVES: Early integrated palliative care has shown to improve the quality of life in patients with cancer. During the past decade, pediatric palliative care has become an established area of medical expertise, however due to scant information available regarding the triggers for referral and referral practice very few children receive a formal palliative care consult. MATERIALS AND METHODS: A retrospective audit of medical case records of pediatric oncology patients over a period of 1 year from September 30, 2019, to September 30, 2020, was conducted. Demographic details, diagnosis, staging, clinical parameters, reason for referral, and palliative care plan were captured in a predesigned pro forma. RESULTS: Among 126 children with cancer, 27 (21.4%) patients were referred to palliative care. Majority 21 (77%) referrals were inpatient consults. Symptom management 17 (44.7%) was the most common trigger for referral followed by referrals for psychosocial support 12 (14.4%). Children with solid tumors 16 (59%) were more often referred than hematological malignancies. Among those needing end of life care, 8 (88.8%) out of 9 families preferred home than hospital. CONCLUSION: Low incidence of palliative care referral and presence of symptoms as a trigger for palliative care referral suggests gaps in the integrated approach. The study findings prompt a review of palliative care referral criteria and referral practice in a pediatric oncology setting.

Bilateral massive nephromegaly-A rare presentation of t-cell acute lymphoblastic leukemia.

INTRODUCTION: Renal infiltration by leukemia causing massive bilateral nephromegaly is an extremely rare presentation of T-cell acute lymphoblastic leukemia(T-ALL). CASE REPORT: 18-month-old female toddler presented with fever and progressive abdominal distension of 4-6 weeks duration. Imaging revealed bilateral massively enlarged kidneys with normal excretion. Peripheral blood counts and smear examination was unremarkable and immunophenotypic evaluation of marrow was consistent with T-ALL. Chest imaging was unremarkable. She was started on modified Indian Childhood Collaborative Leukemia Group (ICiCLe) ALL protocol. Even with the best anti-tumor lysis syndrome (TLS) prophylaxis the child required two sessions of hemodialysis. An end-induction morphological remission & end-consolidation negative minimal residual disease (MRD) could be achieved. CONCLUSION: Bilateral massive nephromegaly is an extremely rare presentation of T-ALL. This case emphasizes the unusual presentation, need for prompt remediation of TLS, and most importantly the use of early intensification with four drug anthracycline & dexamethasone-based therapy for the treatment of T-ALL in children.

Vitamin C Deficiency and Oxidant Levels in Children With Transfusion-Dependent b-Thalassemia.

OBJECTIVES: To study vitamin C levels in children with transfusion-dependent b-thalassemia and correlate with age, transfusions received and iron overload; and to study the effect of administering vitamin C on its levels and Malondialdehyde (MDA) in deficient patients. METHODS: This case-control study enrolled 100 children with transfusion-dependent b-thalassemia and 30 healthy controls. MDA levels before and after administration of vitamin C were performed randomly in 36 children with low vitamin C levels. RESULTS: 81/95 (85.3%) study subjects vs none in control group, had low plasma vitamin C levels (P<0.001). Vitamin C levels were low in 64 of 71 (74.7%) subjects with dietary deficiency, while none of the 19 (63.3%) controls with dietary deficiency had low levels (P=0.04). Increasing serum ferritin values correlated with vitamin C deficiency (P=0.02). The mean level of MDA reduced (P<0.001) with vitamin C supplementation. CONCLUSIONS: Low levels of vitamin C are common in children with thalassemia. Dietary counseling along with supplementation with vitamin C, in those with low levels may prevent oxidative stress.