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Systemic lupus erythematosus (SLE) is a chronic, multisystem, autoimmune disease characterized by periods of increased disease activity caused by inflammation of blood vessels and connective tissue. Pediatric patients with SLE have a more severe clinical course when compared with adults. Patients commonly present with rash, fever, and arthritis, although the presentation may be unpredictable. Hematological findings are more predominant in children than adults. Thirty-nine percent of children with SLE will develop hematological abnormalities, one of the American Rheumatic Association criteria for classifying the disease. In our case series we found varied hematological picture and presentation. We present here four case reports of SLE cases with interesting hematological features. Our first case is a 13 month old female child who was initially diagnosed as Evans syndrome and 2 years later diagnosed as SLE. Second case is a 3 year old male child who had SLE with warm antibody AIHA. Third case is a 6 year old female child who presented with AIHA and was diagnosed with SLE 6 years later. Fourth case is a 6 year old female child diagnosed as SLE with aplastic anemia. Hematological findings should be carefully assessed and treated in order to decrease disease related morbidity.

Leukocyte adhesion deficiency 1 (LAD-1) is a rare autosomal recessive disorder of leukocyte function. LAD-1 affects about 1 per 10 million individuals and is characterized by recurrent bacterial and fungal infections and depressed inflammatory responses despite striking blood neutrophilia. Patients with the severe clinical form of LAD-1 express <0.3% of the normal amount of the β2-integrin molecules, whereas patients with the moderate phenotype may express 2-7%. Skin infection may progress to large chronic ulcers with polymicrobial infection, including anaerobic organisms. The ulcers heal slowly, require months of antibiotic treatment, and often require plastic surgical grafting. The diagnosis of LAD-1 is established most readily by flow cytometric measurements of surface CD11b in stimulated and unstimulated neutrophils using monoclonal antibodies directed against CD11b. Pyoderma gangrenosum (PG) is an uncommon condition characterized by recurrent sterile, inflammatory skin ulcers. Commonly, PG occurs in the context of inflammatory bowel disease or rheumatic, hematologic, or immunologic disorders. Here, we present a 5-year-old female with a long history of PG, which healed with atrophic scarring, who was ultimately diagnosed with leukocyte adhesion deficiency type 1 (LAD1). She had a good response to high-dose prednisone therapy (2 mg/kg) and was discharged after 3 weeks of admission but only to be re-admitted 3 weeks later with severe pneumonia. During hospital stay, she developed pneumothorax and pneumomediastinum and later succumbed to her illness.

A prolonged course of prednisolone therapy for an initial episode of nephrotic syndrome can be considered, as it reduces the rate of relapses without increasing the risk for steroid side effects.

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OBJECTIVE: To determine if initial fluid resuscitation with balanced crystalloid (e.g., multiple electrolytes solution [MES]) or 0.9% saline adversely affects kidney function in children with septic shock. DESIGN: Parallel-group, blinded multicenter trial. SETTING: PICUs of four tertiary care centers in India from 2017 to 2020. PATIENTS: Children up to 15 years of age with septic shock. METHODS: Children were randomized to receive fluid boluses of either MES (PlasmaLyte A) or 0.9% saline at the time of identification of shock. All children were managed as per standard protocols and monitored until discharge/death. The primary outcome was new and/or progressive acute kidney injury (AKI), at any time within the first 7 days of fluid resuscitation. Key secondary outcomes included hyperchloremia, any adverse event (AE), at 24, 48, and 72 hours, and all-cause ICU mortality. INTERVENTIONS: MES solution (n = 351) versus 0.9% saline (n = 357) for bolus fluid resuscitation during the first 7 days. MEASUREMENTS AND MAIN RESULTS: The median age was 5 years (interquartile range, 1.3–9); 302 (43%) were girls. The relative risk (RR) for meeting the criteria for new and/or progressive AKI was 0.62 (95% CI, 0.49–0.80; p < 0.001), favoring the MES (21%) versus the saline (33%) group. The proportions of children with hyperchloremia were lower in the MES versus the saline group at 24, 48, and 72 hours. There was no difference in the ICU mortality (33% in the MES vs 34% in the saline group). There was no difference with regard to infusion-related AEs such as fever, thrombophlebitis, or fluid overload between the groups. CONCLUSIONS: Among children presenting with septic shock, fluid resuscitation with MES (balanced crystalloid) as compared with 0.9% saline resulted in a significantly lower incidence of new and/or progressive AKI during the first 7 days of hospitalization.

Systemic lupus erythematous (SLE) is a multisystem autoimmune disorder characterized by immune dysregulation and formation of autoantibodies. A high index of suspicion is necessary to diagnose SLE. Children have more systemic involvement than adults. Kidney involvement is seen in a significant proportion of children. With advancement of therapy the survival rate of patients with SLE has significantly improved. Even then lupus nephritis is still the most important predictor of morbidity and mortality. Treatment of lupus nephritis is mostly derived from studies in adults as data on children is still lacking. Prednisolone and cyclophosphamide was the mainstay of treatment till now. Recently drugs like mycophenolate mofetil, azathioprine, rituximab are also being used in treatment of lupus nephritis with promising results and without significant adverse effects. In this review we will be discussing lupus nephritis, its diagnosis, pathogenesis, clinical picture and treatment advancements.