Splenomegaly in Children and Adolescents

Suttorp, Meinolf; Classen, Carl Friedrich

doi:10.3389/fped.2021.704635

Cited by 42 publications

(46 citation statements)

References 85 publications

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“…Moreover, the spleen is also involved in the development of autoimmune diseases, as e.g. splenomegaly is common in RA, systemic lupus erythematosus (SLE), and systemic sclerosis [ 17–20 ]. Interestingly, in 1% of RA patients Felty syndrome develops, which is defined by the presence of splenomegaly and neutropenia, wherein splenectomy can be an effective therapy of choice in some cases to improve the neutrophil count [ 21 , 22 ]; however, the potential effect on the arthritis itself was not reported specifically.…”

Section: Introductionmentioning

confidence: 99%

Splenectomy modulates the immune response but does not prevent joint inflammation in a mouse model of RA

Khanfar

Olasz

Gajdócsi

et al. 2022

Clinical and Experimental Immunology

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The spleen is the largest secondary lymphoid organ which is involved in the development of B cells and also in systemic (auto)immune responses. Here, we investigated the significance of the spleen in the autoimmune arthritis induction and pathogenesis using the recombinant human G1 domain-induced arthritis (GIA) model in splenectomized and control BALB/c mice. Splenectomized mice developed GIA with similar clinical picture to the control group. However, we observed significant alterations in the humoral and cellular immune responses in splenectomized mice. In the sera of the splenectomized mice, we found lower pro-inflammatory cytokine and anti-rhG1 IgM levels, but higher IL-4, anti-rhG1 IgG1 and anti-CCP and RF antibodies. The arthritis induction in the splenectomized group was associated with a significant expansion of activated helper T cells and an increase in the proportion of the circulating B1 and marginal zone B cell subsets. Importantly, immunization of the splenectomized mice with the rhG1 induced the formation of germinal centers in the inguinal- and mesenteric lymph nodes (i/mLNs) which showed active immune response to rhG1. Finally, both B- and T cells from the mLNs of the splenectomized mice showed decreased intracellular Ca 2+ signaling than those of the control group. Collectively, these findings indicate that the presence of the spleen is not critical for the induction of GIA, and in its absence the autoimmune arthritis is most likely promoted through the compensatory activity of the i/mLNs. However, our data implies the immunological role of the spleen in arthritis which could be further assessed in human RA.

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Section: Introductionmentioning

confidence: 99%

Splenectomy modulates the immune response but does not prevent joint inflammation in a mouse model of RA

Khanfar

Olasz

Gajdócsi

et al. 2022

Clinical and Experimental Immunology

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“…The thymus is a major immune organ, the index of which is an important index for assessing immunity [ 35 ]. Meanwhile, as another lymphatic organ of the human body, the spleen palys a core role in immune system and is used for assessing immunomodulatory effects of drugs [ 36 ]. TXS treatment lowered the spleen index and enhanced the thymus index.…”

Section: Discussionmentioning

confidence: 99%

Ta-Xi-San Suppresses Atopic Dermatitis Involved in Multitarget Mechanism Using Experimental and Network Pharmacology Analysis

Zhi

Zhang

et al. 2022

Evidence-Based Complementary and Alternative Medicine

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Atopic dermatitis (AD) is a relapsing and chronic skin inflammation with a common incidence worldwide. Ta-Xi-San (TXS) is a Chinese herbal formula usually used for atopic dermatitis in clinic; however, its active compounds and mechanisms of action are still unclear. Our study was designed to reveal the pharmacological activities, the active compounds, and the pharmacological mechanisms of TXS for atopic dermatitis. Mice were induced by 2,4-dinitrocluorobenzene (DNCB) to build atopic dermatitis model. The pathological evaluation, enzyme-linked immunosorbent assay (ELISA), and hematoxylin and eosin (H&E) assay were performed. The UPLC-Q-Exactive-MSE and network pharmacology analysis were performed to explore active ingredients and therapeutic mechanisms of TXS. TXS treatment decreased levels of immunoglobulin E (IgE), interleukin-4 (IL-4), and tumor necrosis factor-α (TNF-α) in serum induced by DNCB. TXS reduced scratching behavior and alleviated inflammatory pathology of skin and ear. Meanwhile, TXS decreased the spleen index and increased spleen index. The UPLC-Q-Exactive-MSE results showed that 65 compounds of TXS were detected and 337 targets were fished. We collected 1371 AD disease targets, and the compound-target gene network reveled that the top 3 active ingredients were (−)-epigallocatechin gallate, apigenin, and esculetin, and the core target genes were PTGS2, PTGS1, and HSP90AA1. The KEGG pathway and GO analysis showed that TXS remedied atopic dermatitis via PI3K-Akt signaling pathway, mitogen-activated protein kinase (MAPK) signaling pathway, and Toll-like receptor (TLR) signaling pathway with the regulation of inflammatory response and transcription. Further, we found that the targets of PTGS2 and HSP90AA1 were both elevated in ears and skin of AD model mouse; however, TXS decreased the elevated expressions of PTGS2 and HSP90AA1. Our study revealed that TXS ameliorated AD based on (−)-epigallocatechin gallate, apigenin, and esculetin via targeting PTGS2 and HSP90AA1.

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“…The splenomegaly observed in the index patient could have resulted from the associated acute infection suggested by leucocytosis and pneumonic changes on the chest radiograph. [15] The diagnosis of MS is made from the typical clinical features and exclusion of other differential diagnoses. Serum levels of antinuclear antibodies, anti-smooth muscle antibodies, antimitochondrial antibodies and antineutrophil cytoplasmic antibodies are used to exclude autoimmune hepatitis.…”

Section: Discussionmentioning

confidence: 99%

Mauriac Syndrome in a Nigerian child with Type 1 Diabetes Mellitus: A Case Report

Oyenusi¹,

Ezeani²

2021

Ann Health Res

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A 14-year-old boy with Type 1 Diabetes mellitus (diagnosed at eight years of age) presented with complaints of fever, weight loss, growth failure, pubertal delay, abdominal swelling and discomfort. He was on Premixed insulin (70/30) with inadequate follow-up and poor diabetic control. Examination revealed cachexia, generalised lymphadenopathy, a protuberant abdomen and hepatosplenomegaly. Anthropometry showed a bodyweight of 19.6kg, a height of 116cm and a BMI of 14.1kg/m2, all markedly below the 3rd centile. He had no secondary sexual characteristics: axillary hair stage 1, pubic hair stage 1, penile length of 4.9cm and prepubertal testicular volumes of 3mls bilaterally. At presentation, his random blood glucose was 400mg/dl, and glycosylated haemoglobin was 11.6%. Screening for tuberculosis, human immunodeficiency virus, hepatitis and lymphoproliferative disorders were negative. Other blood investigation findings included leucocytosis, erythrocyte sedimentation rate of 30mm/hr, normal liver function tests, normal serum electrolytes, urea and creatinine. His haemoglobin genotype was AS. Chest radiograph showed features of bronchopneumonia. A presumptive diagnosis of Mauriac Syndrome was made. With the optimisation of glycaemic control, he improved clinically with a weight gain of 5.7kg over four months and resolution of hepatosplenomegaly.

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Splenomegaly in Children and Adolescents

Cited by 42 publications

References 85 publications

Splenectomy modulates the immune response but does not prevent joint inflammation in a mouse model of RA

Splenectomy modulates the immune response but does not prevent joint inflammation in a mouse model of RA

Ta-Xi-San Suppresses Atopic Dermatitis Involved in Multitarget Mechanism Using Experimental and Network Pharmacology Analysis

Mauriac Syndrome in a Nigerian child with Type 1 Diabetes Mellitus: A Case Report

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