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Objective: To study the clinical features and laboratory parameters of neonatal lupus erythematosus (NLE) from India. Patients and methods: We analyzed case records of children diagnosed with NLE in the Pediatric Rheumatology Clinic at tertiary care centre from North India during the period January 1999 - December 2023. Results: Twenty-four babies are diagnosed with NLE during the study period. Median age at diagnosis was 60 days with a female predisposition (Male to female- 1:2). Cutaneous manifestations were reported in 14 (58%) patients. Hepatomegaly was noted in 15 (62.5%), and splenomegaly in 2 (8%). Elevated transaminases were noted in 14 (58%) patients, and cholestatic jaundice in one. Hematological manifestations were noticed in 20 (83.3%), and neurological manifestations were noticed in 8 patients. Most non-cardiac manifestations were self-limiting. Intravenous immunoglobulin was used for phrenic nerve palsy and autoimmune hemolytic anemia. Oral corticosteroid was used in a patient with refractory cytopenia, and four patients required transfusions. Cardiac involvement was reported in 13 (54%) patients – 3rd-degree heart block in 9 patients, and 6 were managed with epicardial pacemaker insertion. One patient required pacemaker reimplantation due to infective endocarditis. Congenital hydrops was seen in 3 patients, and required respiratory support and recovery was uneventful. Conclusion: NLE is associated with significant morbidity. More than half had cardiac manifestation, of which 70% had 3rd-degree heart block requiring pacemaker insertion. Other rare clinical manifestations like phrenic nerve palsy and seizures are noticed in our cohort. [ABSTRACT FROM AUTHOR]

The article presents a 4-year-old patient with unexplained cytopenias, ultimately diagnosed with extrahepatic portal vein obstruction (EHPVO), emphasizing its rare presentation and management. Topics discussed include hypersplenism as a cause of cytopenias, the diagnostic process for portal hypertension, and multidisciplinary approaches to managing EHPVO in children.

Myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML) are heterogeneous neoplasms of hematopoietic stem cells that are challenging to diagnose, differentiate, and prognosticate. Cytogenetic and mutational analyses are useful in humans but unavailable for dogs, where diagnosis and classification still rely largely on hematologic and morphologic assessment. The objectives of this study were to apply a classification scheme to myeloid neoplasms and to assess outcome in relation to predictor variables. Keyword search of a laboratory database, application of sequential exclusion criteria, and consensus from 3 reviewers yielded 70 cases of myeloid neoplasia with hematology results, and cytologic (11), histologic (14), or both (45) types of marrow specimens. Based on blast percentage and morphology, 42 cases were classified as MDS and 28 as AML. Dogs with MDS had significantly lower body weights, hemoglobin concentrations and blood blasts, and higher red blood cell size variability and platelet numbers than dogs with AML. Estimates of median survival using Kaplan-Meier curves for dogs with MDS and AML were 384 and 6 days, respectively (P <.001). The instantaneous risk of death for dogs with MDS was approximately 5× lower than that of dogs with AML. Significant predictor variables of survival were body weight, white blood cell count, platelet count, and percent blood blasts (P <.05). Hazard ratios (HRs) derived from best-fitting Cox regression models were 1.043, 0.998, and 1.061 for increased neutrophils, decreased platelets, and increased blood blasts, respectively. Findings from this study suggest that hematologic and morphologic variables are useful to predict outcomes in myeloid neoplasia. [ABSTRACT FROM AUTHOR]