Intravenous Immunoglobulin Research Articles

Posttransplant Lymphoproliferative Disorder in Pediatric Solid-Organ Transplant Recipients: A 7-Year Single-Center Analysis.

Posttransplant lymphoproliferative disorder is a consequential complication following solid-organ transplant, particularly associated with the Epstein-Barr virus. We studied a single center's cases of pediatric posttransplant lymphoproliferative disorder for a 7-year period and focused on incidence rates, anatomic sites involved, and correlation with clinical outcomes. We explored clinical features and treatment outcomes in patients with pediatric posttransplant lymphoproliferative disorder, with emphasis on patient survival and associated clinical ramifications. This was a retrospective analysis of medical records from pediatric solid-organ transplant recipients (liver or kidney) at Baskent University Ankara Hospital Organ Transplantation Center between January 1, 2017, and January 1, 2024, approved by the Institutional Review Board (KA24/63). We identified cases based on pathology-confirmed persistent lymphadenopathy or tumorous lesions. Patient categorization distinguished between malignant and benign groups. Early posttransplant lymphoproliferative disorder was defined within the initial year after transplant. Epstein?Barr virus association was determined through in situ hybridization, and patient characteristics were reviewed comprehensively. In 7 years, 10 pediatric patients (9 liver transplants, 1 kidney transplant) were diagnosed with posttransplant lymphoproliferative disorder, with an incidence of 8.7% for pediatric liver transplants. Mean age at diagnosis was 46.4 months, and mean time from transplant to diagnosis was 21.2 months. The most common complaints at diagnosis included fever, lymphadenopathy, hepatosplenomegaly, dyspnea, and diarrhea. Treatment modalities included rituximab, immunosuppression reduction, intravenous immunoglobulin therapy, and chemotherapy (NHL Berlin-Frankfurt-Münster 90 protocols). All patients achieved remission (mean follow-up, 22.9 mo). Early diagnosis of posttransplant lymphoproliferative disorder is important, and rituximab with immunosuppression reduction is effective to achieve complete remission, particularly in early polymorphic cases. Despite challenges, all patients achieved remission, signaling improved outcomes in pediatric posttransplant lymphoproliferative disorder. Active monitoring of Epstein?Barr virus infection may further reduce posttransplant lymphoproliferative disorder complications in pediatric solid-organ transplant; hence, early diagnosis is crucial.

Clinical Features and Electroencephalogram Analysis ofBrain Network Functional Connectivityin Anti-Leucine-RichGlioma-Inactivated1Antibody Encephalitis.

To summarize the clinical manifestations, laboratory findings, and magnetic resonance imaging (MRI) characteristics of anti-leucine-rich glioma-inactivated 1 (LGI1) antibody encephalitis (anti-LGI1 antibody encephalitis) and explore the electroencephalogram (EEG) features. We retrospectively analyzed the medical history of 16 patients diagnosed with anti-LGI1 antibody encephalitis at the First Hospital of Hebei Medical University from 2021 to 2023. EEGs of patients with anti-LGI1 antibody encephalitis and healthy individuals were analyzed. Based on Video-EEG signal analysis of EEG δ, θ, α, β frequency bands, weighted phase lag index values were calculated to form brain network matrices, studying differences in coherence between brain regions of patients with anti-LGI1 antibody encephalitis and healthy individuals. Patients with anti-LGI1 antibody encephalitis often presented with subacute onset seizures and cognitive decline. Routine test of cerebrospinal fluid was mostly normal. Serum testing revealed hyponatremia in 62.50% of patients, along with positive serum antinuclear antibodies, decreased vitamin B12, and abnormal cytokines such as interleukin-6. Head MRI revealed abnormal lesions related to the disease in seven cases (43.75%), mainly located in the unilateral or bilateral frontal and temporal lobes of the hippocampus. The EEG mainly showed generalized and focal slow waves, sometimes with focal discharges. Brain network functional connectivity analysis found a significant weakening of functional connections in the frontal-temporal lobe in the δ and β frequency bands. Intravenous pulse corticosteroids and intravenous immunoglobulin are first-line immunotherapies for anti-LGI1 antibody-related encephalitis. The disease recovery and cognitive decline improved in most patients. Anti-LGI1 antibody encephalitis is characterized by seizures and cognitive dysfunction. Serum may show abnormalities in immune indicators such as cytokines. Head MRI mainly reveals abnormal signals in the frontal-temporal lobes and the hippocampus. EEG brain network connectivity analysis reveals characteristic weakening of functional connections in the frontal-temporal lobe in the δ and β frequency bands.

Paraneoplastic Cerebellar Degeneration Leading to an Early Diagnosis of Peritoneal Serous Papillary Carcinoma.

A 77-year-old female with a subacute progression of ataxia and serum anti-Yo antibodies was suspected to have paraneoplastic cerebellar degeneration (PCD). An examination of an underlying cancer showed no abnormality in the gynecological organs, but the findings did show a mass in the Douglas fossa. The mass was resected and diagnosed as stage IIB peritoneal serous papillary carcinoma (PSPC), a rare gynecologic cancer that is difficult to diagnose in the early stages. PCD was treated with intravenous immunoglobulin (IVIG). For an early diagnosis and treatment, PSPC should be included in the list of malignancies that cause PCD with anti-Yo antibodies.

Clinical insights: Resolving the specter of cardiac sequelae in multisystem inflammatory syndrome in children (MIS-C) – A 24-month follow-up case series

Background: During the COVID-19 pandemic, multisystem inflammatory syndrome (MIS-C) emerged as a novel and severe complication. Objectives: The present prospective study followed patients in Brazil from February 2020 to December 2022, and evaluated children discharged from reference centers with an MIS-C diagnosis. The aim of the study is to describe long-term cardiac findings. Methods: Following a clinical and imaging procedure, children with MIS-C were followed up. Results: Thirty-six children during hospitalization coursed with fever; 61.1 % presented with gastrointestinal symptoms, 77.7 % with cardiocirculatory manifestations, and 28 % with respiratory issues. Shock was observed in 37 % of patients and the average hospital stay was 9 ± 5 days, with 54 % exhibiting severe symptoms requiring intensive care. Elevated inflammatory and cardiac markers were common; 42 % of patients presented elevated troponin and D-dimer levels. During intensive care, echocardiographic abnormalities were found in 56 % of the patients, including coronary dilation and pericardial effusion. All patients were hospitalized and treated with intravenous immunoglobulin, oral steroids, and acetylsalicylic acid, resulting in 86 % recovery. However, 14 % of the patient had persistent (mild cardiac) alterations at discharge. Sex and previous chronic conditions did not affect the persistence of cardiac findings, whereas marginal age differences indicated that older children tend to have more severe symptoms. BMI was identified as a risk factor but must be considered carefully. Six months post-discharge, all patients successfully recovered from cardiac alterations. Conclusion: Only few case series have reported persistent MIS-C findings, and this case series elicits a positive post-discharge prognosis for MIS-C over time.

Parent perceptions of various treatment approaches for PANS and PANDAS

Pediatric autoimmune neuropsychiatric disorder (PANDAS) is characterized by sudden, dramatic onset obsessive-compulsive disorder (OCD) following a Group A Streptococcus infection. Pediatric acute neuropsychiatric syndrome (PANS) refers to sudden, dramatic onset OCD and/or restricted eating triggered by infections and other inflammatory reactions. A variety of treatments have been utilized for PANS/PANDAS; however, there is no “gold standard” intervention protocol. Parental expectations of a given treatment have been found to improve a child's overall treatment experience; however, parent attitudes towards PANS/PANDAS treatments are unknown, which was the purpose of this study. An online survey was distributed to 208 parents of children with self-reported PANS/PANDAS. Treatments were grouped together within overarching categories (i.e., psychotherapy, psychiatric/psychotropic, inflammation/infection mitigation, supplements, lifestyle changes, and surgery). Categorically, parents rated inflammation/infection mitigation interventions and lifestyle changes as most appropriate, and psychiatric/psychotropic interventions as least appropriate. At the individual level, treatments including antibiotics, non-steroidal anti-inflammatory drugs, intravenous immunoglobulin, and family counseling received ratings between “appropriate” and “extremely appropriate” Alternatively, treatments including deep brain stimulation, transcranial magnetic stimulation, antidepressant medications, and exposure and response prevention received ratings between “inappropriate” and “extremely inappropriate.” Study limitations include a lack of gender and race representation in our sample. Findings indicate a need for dissemination of current, relevant research to the parent population as well as further examination of the parent experience throughout onset, diagnosis, and treatment.

Fatal outcome in isolated Pauci-immune pulmonary capillaritis: A case report.

Isolated Pauci-immune pulmonary capillaritis (IPIPC) is a rare form of small vessel vasculitis that affects only the lungs, causing inflammation of pulmonary capillaries and potentially leading to severe outcomes like alveolar haemorrhage. A 23-year-old woman with a prior diagnosis of rheumatoid arthritis presented with hemoptysis and respiratory distress, ultimately diagnosed with IPIPC. Despite treatment with high-dose steroids and intravenous immunoglobulin, her condition deteriorated, resulting in respiratory failure and death. IPIPC often lacks systemic symptoms and ANCA positivity, complicating diagnosis and treatment. Imaging, bronchoscopy, and histopathology are key for diagnosis, while management typically involves corticosteroids and possibly immunosuppressives. The case underscores the challenges in identifying and treating IPIPC, highlighting the importance of early intervention to improve prognosis, even though complications can still lead to significant respiratory issues and mortality.

No sex-based differences in odds of starting or time to treatment of generalized myasthenia gravis: A single center cohort study.

Females with generalized myasthenia gravis (gMG) report lower quality of life (QoL) and have more severe disease than males. Sex differences in disease characteristics exist, however whether there are sex differences in the treatment of gMG that may contribute to QoL disparities is unknown. Our objective is to determine whether there are sex differences in the treatment of gMG. We performed a single-center retrospective study of people diagnosed with gMG at the University of Calgary between 1997 and 2021. Primary outcome was proportion starting treatment and secondary outcome was time from diagnosis to treatment initiation. Treatments included pyridostigmine, prednisone, steroid sparing therapies (azathioprine, mycophenolate mofetil [MMF], methotrexate [MTX], or tacrolimus), intravenous immunoglobulin (IVIg), plasmapheresis, rituximab, eculizumab, cyclosporine, stem cell transplantation, and thymectomy. Multivariable logistic and Cox proportional hazards regression models were used to examine treatment associations with sex, adjusted for time from onset to diagnosis, age at diagnosis, presence of thymoma, and antibody status. A total of 179 people with gMG were included (41.9% female). Odds of starting treatment were not statistically associated with sex after adjustment for confounders and correction for multiple testing. Results of the secondary analysis using time to treatment initiation as the outcome were similar. We found no sex differences in odds of starting treatment or time to treatment initiation that might explain previously observed sex-based differences in QoL. Future work should capture physician and patient treatment preferences that may influence disease management.

Refractory Kawasaki Disease With a Giant Aneurysm Successfully Treated With Infliximab and Enoxaparin: A Case Report

Kawasaki disease (KD) is the most common vasculitis in children, and can result in the development of coronary artery aneurysms (CAAs) if not properly managed. While intravenous immunoglobulin (IVIG) and aspirin are standard first-line treatments, refractory KD may develop, increasing the risk of coronary complications. Herein, we report the case of a young girl with KD who initially responded to IVIG, but later developed a giant CAA, despite additional treatments. Infliximab stabilized her condition, and one year later, the CAA remained stable without thrombus formation. This case demonstrates that clinical appearance, particularly fever, may not fully reflect the patient's condition, as fever can subside immediately after treatment, but may relapse days later, underscoring the need for vigilant monitoring.

Acute disseminated encephalomyelitis (ADEM) in a patient with post streptococcal glomerulonephritis (PSGN): A case report.

Concurrent recurrence of acute disseminated encephalomyelitis (ADEM) and poststreptococcal glomerulonephritis (PSGN) in a thalassemia intermedia patient is rare and underscores the complexity of autoimmune disorders. This case emphasizes the importance of considering ADEM in the differential diagnosis of children presenting with PSGN accompanied by neurological symptoms. Post-streptococcal glomerulonephritis (PSGN) is a common group A streptococcal (GAS) infection sequela. The pathophysiology of PSGN involves immune complex deposition, with type 3 hypersensitivity reaction triggered by GAS. Certain neurological conditions may also arise following a GAS infection, possibly due to molecular mimicry in the brain, a pathophysiology similar to rheumatic fever, another common sequel of GAS infection. We present the case of a child with β-thalassemia intermedia who exhibited the classic triad (edema, hypertension, hematuria) of PSGN along with neurological manifestations, including a low glasgow coma scale (GCS) score and seizures. Magnetic resonance imaging (MRI) of the brain indicated changes consistent with acute disseminated encephalomyelitis (ADEM). Initially treated with methylprednisolone, the patient eventually received intravenous immunoglobulin (IVIG) due to lack of response. The patient had a good outcome, with complete resolution of all symptoms and no residual neurological deficits. This case underscores the importance of considering ADEM in the differential diagnosis for patients presenting with neurological signs and symptoms following a recent throat infection with GAS. Furthermore, given the increased risk of infection in thalassemia, patients with thalassemia who have a throat infection and neurological symptoms should be evaluated for the possible presence of ADEM.

Cytokine landscape in hospitalized children with multisystem inflammatory syndrome

The etiology of multisystem inflammatory syndrome in children (MIS-C), frequently observed following COVID-19 infection, remains elusive. This study unveils insights derived from cytokine analysis in the sera of MIS-C patients, both before and after the administration of intravenous immunoglobulin (IVIG) and glucocorticosteroids (GCS). In this study, we employed a comprehensive 45-cytokine profile encompassing a spectrum of widely recognized proinflammatory and antiinflammatory cytokines, as well as growth factors, along with other soluble mediators. The analysis delineates three principal cytokine-concentration patterns evident in the patients’ sera. Pattern no.1 predominantly features proinflammatory cytokines (IL-6, IL-15, IL-1ra, granulocyte-macrophage colony-stimulating factor (GM-CSF), tumor necrosis factor α (TNFα), C-X-C motif chemokine ligand 10 (CXCL10/ IP-10), and IL-10) exhibiting elevated concentrations upon admission, swiftly normalizing post-hospital treatment. Pattern no. 2 includes cytokines (IL-17 A, IL-33, IFNγ, vascular endothelial growth factor (VEGF), and programmed death ligand (PD-L1)) with moderately elevated levels at admission, persisting over 7–10 days of hospitalization despite the treatment. Pattern no. 3 comprises cytokines which concentrations escalated after 7–10 days of hospitalization and therapy, including IL-1α, IL-1β, IL-2, IL-13, platelet-derived growth factor AA/BB (PDGF AA/BB). The observed in cytokine profile of MIS-C patients showed a transition from acute inflammation to sustaining inflammation which turned into induction of humoral memory mechanisms and various defense mechanisms, contributing to recovery.

567P A prospective observational study on the effect of intravenous immunoglobulin on small fiber neuropathy after SARS-CoV-2 infection or vaccination

567P A prospective observational study on the effect of intravenous immunoglobulin on small fiber neuropathy after SARS-CoV-2 infection or vaccination

Case report: A novel high-dose intravenous immunoglobulin preparation for the treatment of severe pemphigus vulgaris failing standard therapy.

Pemphigus vulgaris (PV) is a severe autoimmune bullous dermatosis that is characterized by autoantibodies against epidermal adhesion proteins causing painful mucosal and skin blistering. Standard treatments for PV include corticosteroids, steroid-sparing immunosuppressants, or intravenous monoclonal anti-CD20-antibody therapy. The European guidelines suggest high-dose intravenous immunoglobulin (IVIg) therapy as a promising approach for severe or treatment-resistant cases. We report on a 65-year-old woman with severe and recurrent disease who achieved long-term disease stabilization with IVIg treatment. Because of recurrent fatigue and headache, the patient was switched to an alternative IVIg preparation with a novel manufacturing process, thus ensuring high purity and better tolerability. We observed excellent efficacy, yet side effects remained largely unchanged. Further studies are necessary to evaluate the long-term efficacy and tolerability of this new IVIg preparation.

A Unique Presentation of Excitatory Catatonia Alongside AntiNmda Receptor Encephalitis: A Case Report

Introduction: Anti-NMDA receptor encephalitis, first described in 2007, is a prominent cause of autoimmune encephalitis, predominantly affecting females. Its clinical presentation includes a prodromal phase with flu-like symptoms, followed by psychosis, unresponsiveness, hyperkinesis, and recovery [1]. Catatonia can coexist sounds better than cooccur in patients with anti-NMDA receptor encephalitis, complicating diagnosis and effective treatment. Case Summary: This case report discusses a 41-year-old African American female with a history of seizure disorder, schizoaffective disorder with catatonia, developmental delay, Catatonia in itself is not the diagnosis as per dsm 5. We could use it as a specifier with other diagnosis, be it schizoaffective disorder or seizure disorder here. So we could mention as schizoaffective disorder with catatonia. and recent bereavement. Her symptoms included variable motor hyperactivity, alternating between restlessness and sedation, with an initial Busch Francis Rating Scale score of 18. Initially diagnosed with excitatory catatonia secondary to schizoaffective disorder and treated with benzodiazepines, her condition failed to improve, prompting further investigation. Despite additional treatment for a urinary tract infection and medication adjustment, which included adding valproic acid and amantadine, her condition remained uncontrolled. Subsequent lumbar puncture revealed inflammatory markers, and her anti-NMDA receptor IgG antibody assay was positive at 1:1280, confirming the diagnosis of anti-NMDA receptor encephalitis. Treatment was escalated to include methylprednisolone and intravenous immunoglobulin, alongside six sessions of ECT for management of her catatonia. The patient showed significant improvement, achieving functional recovery and independence in daily activities before discharge. Conclusion: This case emphasizes the importance of considering anti-NMDA receptor encephalitis in patients with unexplained, refractory neuropsychiatric symptoms, such as catatonia, and highlights the value of a multidisciplinary approach in managing and rehabilitating affected individuals. Prompt diagnosis and treatment are crucial to improving outcomes and reducing the risk of persistent cognitive deficits.

A rare presentation of stiff-person syndrome with a nonspecific focal myositis: A case report

Introduction. The stiff-person syndrome (SPS) is a rare disease whose incidence is estimated at approximately 1 in a million individuals in the general population. Diagnosis relies on a combination of clinical, immunological, and electromyographic items. We present the case of a patient diagnosed with the idiopathic SPS, initially misdiagnosed as focal paravertebral myositis. Case presentation. A 36-year-old patient was referred to us for the investigation of subacute dorsal myalgias. The patient reported a severe torticollis seven months ago, which resolved spontaneously after one month, and some episodes of back stiffness. On examination, the patient was afebrile, had hyperlordosis with a paravertebral contracture and tenderness. Neurological and cognitive examinations were normal. C-reactive Protein was at 137 mg/l. The rest of laboratory investigations, including creatine phosphokinase (CPK), were within normal range. Spinal MRI revealed T2 hyperintensity in the semispinalis muscle, erector spinae muscle, trapezius muscle, as well as the intervertebral muscles. Therefore, focal paravertebral myositis was suspected. The electromyogram (EMG) revealed the presence of a continuous motor unit activity in agonist and antagonist muscles, suggestive of stiff-person syndrome. Antinuclear antibodies, anti-glutamic acid decarboxylase 65 and onconeuronal antibodies were negative. Analysis of the cerebrospinal fluid, including anti-glutamic acid decarboxylase 65 test, was normal. We noticed that the paravertebral contracture became less noticeable when the patient was commenced on diazepam. Diagnosis of SPS was established according to Dalakas criteria. Investigations for an underlying neoplasm, were normal. Associated autoimmune disorders have been ruled out. The MRI description was rather explained by the continuous contraction of the affected muscles. Treatment included diazepam, baclofen, intravenous immunoglobulin and corticosteroids. The patient showed important signs of improvement. Conclusion. SPS is a rare condition whose diagnosis can be delayed. Recognizing and managing SPS as early as possible is crucial. It is based on clinical reasoning, imaging, biological features and EMG.

A rare case of hemophagocytic lymphohistiocytosis (HLH) secondary to Salmonella infection

HLH (Hemophagocytic lymphohistiocytosis) is a severe hyperinflammatory disease which could be caused by a range of pathogenic organisms, involving Salmonella. This unique case of bacterial-induced HLH tells the story of a 24y/o male who has been admitted to the hospital and is experiencing the severe symptoms of HLH that developed after an enteric fever caused by Salmonella. Despite earlier hospitalization and intravenous antibiotic treatment, he presented with a high temperature, excessive sweating, yellowish staining of urine and eyes, shortness of breath, and significant systemic inflammation. Laboratory studies revealed severe anemia, bicytopenia, and increased markers such as hyperferritinemia and hypertriglyceridemia, resulting in the diagnosis of HLH. The treatment method includes intensive care with intravenous immunoglobulins (IVIG), corticosteroids, and broad-spectrum antibiotics to address both the underlying infection and the inflammatory condition. Despite intense care, his condition was complex and created substantial complications. This instance emphasizes the significance of early detection as well as treatment of HLH, especially when accompanied by bacterial infections, which are not generally thought to produce this strong immunological response. The example emphasizes the requirement to include HLH in the individuals differential diagnosis with severe illness and systemic inflammatory reactions that do not respond to standard treatment. It also emphasizes the necessity of a comprehensive therapeutic approach, customized to the underlying cause and specific clinical presentations of the disease, in improving outcomes in critically sick patients.

Clinical characteristics analysis of 24 cases of pediatric MOG antibody-associated diseases

ObjectiveTo investigate the clinical characteristics of children with myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). MethodsA retrospective analysis was conducted on the clinical data, antibody tests, imaging, and factors associated with recurrence in 24 children diagnosed with MOGAD at Wuxi Children's Hospital from December 2017 to December 2023. ResultsAmong the 24 included children, the clinical characteristics at the onset of the first episode included fever (12 cases), headache (8), decreased vision (7), drowsiness (6), convulsions (5), ataxia (3), paralysis of both lower limbs (2), urinary and fecal incontinence (2), and central facial palsy (1). Among them, one case started with paralysis of both lower limbs and urinary retention, and electromyography suggested the involvement of peripheral nerves, leading to the diagnosis of MOG antibody-associated central and peripheral demyelinating syndrome (MOGAD-CCPD). Cranial MRI abnormalities were observed in 20 children, and spinal MRI abnormalities were noted in 6 children. All children responded well to corticosteroids and intravenous immunoglobulin, but 7 children experienced a relapse. Among them, 3 children achieved disease control after the addition of mycophenolate mofetil (CellCept), with no further relapses observed during follow-up. ConclusionThe disease course of MOGAD can be monophasic or relapsing. Most children have a good response to acute phase treatments. For those who relapse, immunosuppressants can be added as maintenance therapy, and the clinical prognosis is generally good. This article reports the first highly rare case in China of MOGAD-CCPD in childhood, suggesting that MOG IgG may serve as a potential biomarker associated with CCPD.

Clinical Manifestations of Wiskott-Aldrich Syndrome in an Iranian Patient

Wiskott-Aldrich Syndrome (WAS) is an immunodeficiency disorder resulting from genetic mutations in the WAS protein (WASP) gene in the X chromosome, characterized by thrombocytopenia, eczema, and infections. This case report focused on a 12-year-old Iranian male with WAS with a history of Crohn’s disease, meningitis, and bilateral hernia. His WAS was diagnosed at age six with a hemizygous c.777+1 G>A mutation in the WASP gene. The patient was referred to our clinic with symptoms including fever, abdominal pain, thrombocytopenia, and elevated ESR. Clinical Imaging revealed a significant lung nodule align bronchiectasis, mild ascites, bilateral epididymitis, and lymphadenopathy. Nephrotic syndrome with proteinuria and low levels of albumin have been observed. After six months of receiving intravenous immunoglobulin (IVIG) therapy in addition to antibiotics and antivirals, the patient suffered from arthritis, edema, and fever. Our WAS patient presented the late comorbidity of renal involvement, which highlights the monitoring of this patient, such as those involved in chronic infections. Therefore, a precise treatment approach is needed to manage either the primary immunodeficiency or the late-discovered diseases.

Serum salicylic acid levels in children with Kawasaki disease

BackgroundThis study aimed to clarify serum salicylic acid (SA) levels in patients with Kawasaki disease (KD) after the administration of moderate-dose acetylsalicylic acid (ASA) and their relationship with the therapeutic effect.MethodsWe retrospectively analyzed the clinical data of 142 children with KD. We measured serum SA trough levels during the acute and recovery periods and determined their relationship with clinical and laboratory parameters.ResultsThe median age of patients was 2.4 years. Thirty-one patients had incomplete KD, 29 were intravenous immunoglobulin (IVIG) non-responders, and one patient had coronary artery lesions. The median ASA dose was 49.7 mg/kg/day. The median serum SA level was 22 µg/mL in the acute period and 15 µg/mL in the recovery period, with 45 (33%) in the acute period and 60 (44%) in the recovery period below the limit of measurement (< 10 µg/mL). Serum SA levels during the recovery period were significantly lower in patients who received steroids. There were no significant differences in IVIG responsiveness based on serum SA levels.ConclusionsSerum SA trough levels in KD patients treated with moderate-dose ASA were highly variable and did not reach sufficient levels. Serum SA levels were not associated with IVIG responsiveness.

Evaluating the performance of egami, kobayashi and sano scores in predicting IVIG resistance in infant kawasaki disease

BackgroundThis study aimed to evaluate the effectiveness of Egami, Kobayashi and Sano scores in predicting intravenous immunoglobulin (IVIG) resistance in infant Kawasaki disease (KD), considering its unique clinical presentation.MethodsWe retrospectively analysed 143 infants aged < 12 months and diagnosed with KD at a single centre from 2019 to 2023. Patients were divided into IVIG-resistant and IVIG-responsive groups. Demographic, clinical and laboratory data were compared between the groups. The diagnostic performance of Egami, Kobayashi and Sano scores in predicting IVIG resistance was evaluated using sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) and area under the receiver operating characteristic curve (AUC). Additionally, we developed a new scoring system based on significant predictors identified in our cohort.ResultsAmong 143 infants, 45 (31.5%) showed IVIG resistance. The IVIG-resistant group had a significantly higher rate of coronary artery lesions (15.6% vs. 5.1%, p = 0.036). Incomplete KD was observed in 61.5% of cases. Egami, Kobayashi and Sano scores exhibited low sensitivity (35.6%, 55.6% and 20%, respectively) and moderate specificity (77.6%, 63.3% and 95.9%, respectively) in predicting IVIG resistance. The AUC ranged from 0.583 to 0.674, indicating poor to fair discriminative ability. Our newly developed scoring system, based on total bilirubin and albumin levels, showed similar performance (AUC 0.633) to existing scores.ConclusionsExisting Japanese risk scoring systems and our newly developed score showed limited effectiveness in predicting IVIG resistance in infant KD. The high proportion of incomplete presentation and IVIG resistance in infants highlights the need for age-specific risk assessment and management. Further research is necessary to develop more sophisticated, dedicated prediction model for IVIG resistance in infants with KD.

Chronic lymphoproliferative disorder of natural killer cells-related neurolymphomatosis with severe autonomic dysfunction: a case report

BackgroundChronic lymphoproliferative disorder of natural killer cells (CLPD-NK) is a rare disease characterized by a persistent increase in NK cells in peripheral blood and is generally asymptomatic. If present, symptoms may include fatigue, B symptoms (fever, night sweats, and unintentional weight loss), autoimmune-associated diseases, splenomegaly, and infection due to neutropenia. Peripheral neuropathy, however, is uncommon with an incidence of 3%. Neurolymphomatosis is a neurological manifestation of non-Hodgkin lymphoma and leukemia in which neurotropic neoplastic cells infiltrate the nerves. Moreover, neurolymphomatosis caused by CLPD-NK is extremely rare, with even fewer cases of autonomic dysfunction. We report a case of neurolymphomatosis associated with CLPD-NK and developed autonomic dysfunction, including orthostatic hypotension and gastrointestinal symptoms.Case presentationThe patient was a 61-year-old male who was referred to our hospital for leukocytosis. He was diagnosed with CLPD-NK; however, was untreated since he had no hepatosplenomegaly, and other systemic symptoms. He later developed numbness in his lower extremities. Cerebral spinal fluid examination revealed a markedly elevated protein level of 140 mg/dL, and contrast-enhanced magnetic resonance imaging showed bilateral L4 and 5 nerve roots with enlargement and contrast effect. An immune-mediated polyradiculoneuropathy was suspected, and he was treated with intravenous methylprednisolone and immunoglobulin followed by oral prednisolone and cyclosporine. Although his symptoms were relieved by the immunotherapy, significant autonomic dysfunction, including intractable diarrhea, decreased sweating, and orthostatic hypotension, appeared. Additionally, tests for onconeuronal antibodies, ganglionic nicotinic acetylcholine receptor (gAChR) antibody, NF155, CNTN1, Caspr1 antibody, and anti-ganglioside antibodies were all negative. A sural nerve biopsy revealed lymphocytic infiltration, and immunohistochemical staining of lymphocytes confirmed the infiltration of NK and T cells. Therefore, a diagnosis of neurolymphomatosis caused by CLPD-NK was made, and chemotherapy led to partial symptom improvement.ConclusionsWe experienced a case of pathologically diagnosed neurolymphomatosis with autonomic dysfunction associated with CLPD-NK. In cases of subacute to chronic autonomic dysfunction, paraneoplastic neuropathy, amyloidosis, and autoimmune autonomic ganglionopathy are considered; however neurolymphomatosis caused by CLPD-NK, an important cause of autonomic dysfunction, is not. In difficult to make diagnosis, aggressive nerve biopsy is required.