Maleewan Kitcharoensakkul

The use of electroconvulsive therapy in a patient with juvenile systemic lupus erythematosus and catatonia

Catatonia is a rare manifestation in patients with systemic lupus erythematosus (SLE). As catatonia can be associated with both psychiatric and organic conditions, this could create a diagnostic dilemma once this occurs in SLE patients. The report describes a 15-year-old female with SLE who developed catatonia three days after the diagnosis of SLE was made. Her catatonia was refractory to the treatment with immunosuppressive therapy, which included pulse methylprednisolone, intravenous cyclophosphamide, rituximab, intravenous immunoglobulin (IVIG) and plasmapheresis. Given her persistent catatonia, electroconvulsive therapy (ECT) was initiated three months after the onset of her symptoms. After the third ECT treatment, her mental status dramatically improved and returned nearly to baseline while she was continued on the immunosuppression. This is the first report of a successful ECT therapy in catatonic lupus in children.

Scurvy revealed by difficulty walking: three cases in young children.

Scurvy is rare in developed countries but is known to cause lower-extremity pain and refusal to ambulate in children. Since the discovery of the link between scurvy and dietary deficiency of ascorbic acid, there has been a substantial decrease in its prevalence and recognition. Here we describe 3 cases of scurvy in young children presenting with difficulty walking. Only 1 of 3 patients had gingival lesions at the initial presentation. Two cases underwent an extensive evaluation for hematologic and rheumatologic diseases before the diagnosis of scurvy was made. Dietary histories eventually revealed that all 3 patients had sharply limited intake of fruits and vegetables secondary to oral aversion, and 1 patient had autism. Radiographic changes of long bones were observed in all patients. Interestingly, all patients had concomitant vitamin D deficiency. After replacement with vitamin C, all patients recovered and started to walk again with improved leg pain. These clinical manifestations and radiologic findings highlight the importance for rheumatologists to have a higher index of suspicion for scurvy in nonambulatory children.

Temporal biological variability in dendritic cells and regulatory T cells in peripheral blood of healthy adults

BACKGROUND Studies evaluating circulating dendritic cells (DCs) and natural and induced regulatory T cells (nTregs, iTregs) are often obtained at a single time point and difficult to interpret without understanding their intrinsic day-to-day biologic variability. METHODS We investigated the day-to-day variability in quantifying DCs, nTregs (FoxP3(+)CD25(+)CD4(+)) and cytokine production by iTregs (granzyme B-GZB, Th1/2 cytokines following CD3 plus CD46 in vitro activation) from peripheral blood mononuclear cells (PBMCs) collected on three consecutive days in healthy adults. Intraclass correlation coefficients (ICCs) were used to evaluate intra-individual variability. RESULTS In 10 healthy adults, the %PBMCs of plasmacytoid (pDC) and myeloid (mDC1 and mDC2) were 0.27 ± 0.12, 0.22 ± 0.10, and 0.02 ± 0.02, with ICC 0.91, 0.90, and 0.17 respectively. Natural Tregs (3.27 ± 1.27% CD4(+) cells) had an ICC of 0.86. Inducible Tregs (GZB-positive, 35.3 ± 17.7% CD4(+) cells) had an ICC of 0.77. The ICCs for IL-10, TNF-α, IFN-γ, IL-4, and IL-5 production by iTregs were 0.49, 0.63, 0.68, 0.74, and 0.82, respectively. There were no significant changes in ICC (<0.1) after adjusting for age, gender and atopy except for IL-4. Substantial variability for iTregs was determined for the control condition (PBS with IL-2). CONCLUSIONS No meaningful day-to-day biologic variability was observed for the quantification of nTregs, pDC and mDC1 in normal adults; however, there was substantial variability in measuring mDC2 proportions and iTreg production of IL-10. These results suggest obtaining an average of several measurements over time to determine the most representative value of these biologic measures.

The Heart and Pediatric Rheumatology

Recent advances in Kawasaki disease have included attempts to define genes involved in its pathogenesis. There have been recent advances in the studies of rheumatic carditis, leading to a better understanding of the mechanism of the disease. Histologic evaluation of patients with neonatal lupus erythematosus has revealed fibrosis with collagen deposition and calcification of the atrioventricular node. Therapy for cardiac involvement in systemic juvenile idiopathic arthritis should involve treatment of the underlying disease and systemic inflammatory state, and typically includes nonsteroidal antiinflammatory drugs, corticosteroids, disease-modifying drugs, and biologic therapies targeting tumor necrosis factor-alpha, interleukin-1, and interleukin-6.

ATS Core Curriculum 2017: Part II. Pediatric pulmonary medicine series editor: Jason T. Poston Part II Editors: Paul E. Moore and Jessica Pittman

Paul E. Moore, Jason T. Poston, Debra Boyer, Emily Barsky, Jonathan Gaffin, Kathleen B. Boyne, Kristie R. Ross, Laura Beth Mann Dosier, Timothy J. Vece, Alicia M. Casey, Sebastian K. Welsh, J. Wells Logan, Edward G. Shepherd, Pelton A. Phinzy, Howard B. Panitch, Christina M. Papantonakis, Eric D. Austin, Amir B. Orandi, Maleewan Kitcharoensakkul, Mark K. Abe, Amjad Horani, Jordan S. Rettig, and Jessica Pittman Department of Pediatrics, Vanderbilt University School of Medicine, Nashville, Tennessee; Section of Pulmonary and Critical Care Medicine, Department of Medicine, and Section of Critical Care, Department of Pediatrics, University of Chicago, Chicago, Illinois; Division of Respiratory Diseases and Division of Critical Care Medicine, Department of Anesthesiology, Perioperative, and Pain Medicine, Boston Children’s Hospital, Harvard Medical School, Boston, Massachusetts; Rainbow Babies and Children’s Hospital, Case Western Reserve University, Cleveland, Ohio; Department of Pediatrics, Duke University School of Medicine, Durham, North Carolina; Division of Pediatric Pulmonology, University of North Carolina–Chapel Hill, Chapel Hill, North Carolina; Division of Neonatology, Nationwide Children’s Hospital, Columbus, Ohio; Division of Pulmonary Medicine, The Children’s Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania; and Department of Pediatrics, Washington University School of Medicine, St. Louis, Missouri