OBJECTIVES: To study the role of plasma exchange combined with continuous blood purification in the treatment of refractory Kawasaki disease shock syndrome (KDSS). METHODS: A total of 35 children with KDSS who were hospitalized in the Department of Pediatric Intensive Care Unit, Hunan Children's Hospital, from January 2019 to August 2022 were included as subjects. According to whether plasma exchange combined with continuous veno-venous hemofiltration dialysis was performed, they were divided into a purification group with 12 patients and a conventional group with 23 patients. The two groups were compared in terms of clinical data, laboratory markers, and prognosis. RESULTS: Compared with the conventional group, the purification group had significantly shorter time to recovery from shock and length of hospital stay in the pediatric intensive care unit, as well as a significantly lower number of organs involved during the course of the disease (P<0.05). After treatment, the purification group had significant reductions in the levels of interleukin-6, tumor necrosis factor-α, heparin-binding protein, and brain natriuretic peptide (P<0.05), while the conventional group had significant increases in these indices after treatment (P<0.05). After treatment, the children in the purification group tended to have reductions in stroke volume variation, thoracic fluid content, and systemic vascular resistance and an increase in cardiac output over the time of treatment. CONCLUSIONS Plasma exchange combined with continuous veno-venous hemofiltration dialysis for the treatment of KDSS can alleviate inflammation, maintain fluid balance inside and outside blood vessels, and shorten the course of disease, the duration of shock and the length of hospital stay in the pediatric intensive care unit.
Humans ; Child ; Plasma Exchange ; Mucocutaneous Lymph Node Syndrome/therapy* ; Continuous Renal Replacement Therapy ; Renal Dialysis ; Plasmapheresis ; Shock

Artificial liver is one of the effective methods to treat liver failure. Patients with liver failure are critically ill and have great individualized differences. Therefore, the specific program for the treatment of liver failure with artificial liver should be individualized. The commonly used non-biological artificial liver models include simple plasmapheresis, double filtration plasmapheresis, plasma filtration with dialysis, double plasma molecular adsorption system, molecular absorbent recirculating system, hemodiafiltration, continuous venovenous hemodiafiltration, hybrid, etc. The curative effect should be properly judged from patient's symptoms, laboratory test indicators, survival rate and other aspects after artificial liver therapy.
Hemodiafiltration ; Humans ; Judgment ; Liver Failure/therapy* ; Liver, Artificial ; Plasmapheresis

OBJECTIVES: To investigate the efficacy and application value of plasma exchange as an adjuvant therapy in children with hemophagocytic syndrome (HPS). METHODS: A prospective randomized controlled trial was designed. Forty children with severe HPS were enrolled, who were treated in the pediatric intensive care unit (PICU) of Hunan Children's Hospital from October 2018 to October 2020. The children were randomly divided into a plasma exchange group and a conventional treatment group using a random number table, with 20 children in each group. The children in the conventional treatment group received etiological treatment and conventional symptomatic supportive treatment, and those in the plasma exchange group received plasma exchange in addition to the treatment in the conventional treatment group. The two groups were compared in terms of general information, clinical symptoms and signs before and after treatment, main laboratory markers, treatment outcome, and prognosis. RESULTS: Before treatment, there were no significant differences between the two groups in gender, age, course of the disease before admission, etiological composition, pediatric critical illness score, involvement of organ or system functions, and laboratory markers (P>0.05). After 7 days of treatment, both groups had remission and improvement in clinical symptoms and signs. After treatment, the plasma exchange group had significantly lower levels of C-reactive protein, procalcitonin, and serum protein levels than the conventional treatment group (P<0.05). The plasma exchange group also had significantly lower levels of alanine aminotransferase and total bilirubin than the conventional treatment group (P<0.05). The length of stay in the PICU in the plasma exchange group was significantly shorter than that in the conventional treatment group (P<0.05). The plasma exchange group had a significantly higher treatment response rate than the conventional treatment group (P<0.05). There were no significant differences between the two groups in the total length of hospital stay and 3-month mortality rate (P>0.05). CONCLUSIONS Plasma exchange as an adjuvant therapy is effective for children with severe HPS. It can improve clinical symptoms and signs and some laboratory markers and shorten the length of stay in the PICU, and therefore, it may become an optional adjuvant therapy for children with severe HPS.
Child ; Humans ; Intensive Care Units, Pediatric ; Lymphohistiocytosis, Hemophagocytic/therapy* ; Plasma Exchange ; Plasmapheresis ; Prospective Studies

Diagnosis of thrombotic microangiopathy (TMA) is challenging due to its close association with other forms of microangiopathic hemolytic anemia, such as malignant hypertension and disseminated intravascular coagulation, and because other manifestations including cytopenia and acute kidney injury are manifestations of other medical comorbidities. Further challenges for accurate diagnosis include distinguishing between primary and secondary TMA, as well as between hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP). TTP is typically differentiated from HUS by the presence of more severe thrombocytopenia, along with a higher frequency of altered mental status with relatively preserved renal function. However, the clinical course can vary among patients, requiring polymerase chain reaction testing of patient stools for enterohemorrhagic Escherichia coli and a disintegrin and metalloproteinase with thrombospondin type 1 motif 13 (ADAMTS13) assay. To reduce the mortality rate, prompt initiation of plasmapheresis is important in cases where TPP cannot be excluded. Future advances enabling more rapid testing for ADAMTS13 levels will reduce the need for unnecessary plasmapheresis, so that treatment strategy can be more optimized.
Acute Kidney Injury ; Anemia, Hemolytic ; Comorbidity ; Diagnosis ; Diagnosis, Differential ; Disseminated Intravascular Coagulation ; Enterohemorrhagic Escherichia coli ; Hemolytic-Uremic Syndrome ; Humans ; Hypertension, Malignant ; Mortality ; Plasma Exchange ; Plasmapheresis ; Polymerase Chain Reaction ; Purpura, Thrombotic Thrombocytopenic ; Thrombocytopenia ; Thrombospondins ; Thrombotic Microangiopathies

The catastrophic variant of antiphospholipid syndrome (APS) is a very rare and life-threatening condition of APS. This condition is characterized by thrombosis in multiple organs within a short period of time in the presence of positive antiphospholipid antibodies (aPL). Over the past few decades, considerable progress has been made in the treatment of patients with catastrophic APS; however, the mortality rate still remains very high. Although some cases of rituximab treatment in patients with catastrophic APS have been reported, there is no clear treatment protocol. A 14-year-old girl with systemic lupus erythematosus was diagnosed with catastrophic APS. She received several medications: corticosteroids, intravenous immunoglobulin, and plasmapheresis with anticoagulants. Unfortunately, she did not improve, and rituximab was started with four courses. After the rituximab treatment, she did not experience further thrombotic events during the follow up. This paper reports a pediatric case of catastrophic APS treated successfully with rituximab in Korea.
Adolescent ; Adrenal Cortex Hormones ; Antibodies, Antiphospholipid ; Anticoagulants ; Antiphospholipid Syndrome* ; Clinical Protocols ; Female ; Follow-Up Studies ; Humans ; Immunoglobulins ; Korea ; Lupus Erythematosus, Systemic* ; Mortality ; Plasmapheresis ; Rituximab* ; Thrombosis

In terminally ill patients, organ transplantation could be recommended as the treatment of choice. In Korea, living donor liver or kidney transplantation is much more frequent than deceased donor transplantation due to organ shortages from deceased donors. ABO or HLA incompatibility in transplantation can be a major barrier in living donor transplantation. Currently, the rate of ABO incompatible organ transplantation accompanied by desensitization is 20~25% of living donor transplantation, and the blood bank laboratory plays an active role by plasmapheresis. The desensitization of HLA incompatible transplantation in highly sensitized patients is more difficult than that of ABO incompatible transplantation. The HLA antibody is not easy to remove and it is difficult to prevent sensitization. In addition, setting the target treatment goals and predicting the treatment outcomes based on the HLA antibody results are problematic. Therefore, a range of desensitization protocols have been attempted and various therapeutic goals have been introduced. This article reviews the various desensitization methods for antibody removal focusing on HLA incompatible kidney transplantation, and discusses the prognosis of desensitization methods for antibody removal based on the literature.
Blood Banks ; Humans ; Kidney Transplantation ; Korea ; Liver ; Living Donors ; Organ Transplantation ; Plasmapheresis ; Prognosis ; Terminally Ill ; Tissue Donors ; Transplantation ; Transplants

OBJECTIVE: To study the clinical effect and safety of double filtration plasmapheresis (DFPP) combined with double pulse therapy with methylprednisolone (MP) and cyclophosphamide (CTX) in the treatment of children with severe Henoch-Schönlein purpura nephritis (HSPN). METHODS: A total of 60 children with severe HSPN who were admitted to the hospital from January 2014 to March 2018 were enrolled and were randomly divided into an observation group and a control group (n=30 each). In addition to routine treatment, the children in the control group were given MP+CTX pulse therapy. Those in the observation group were given DFPP treatment in addition to the treatment in the control group, with three courses of treatment in total. After three courses of treatment, the two groups were compared in terms of 24-hour urinary protein, urinary microproteins, renal function parameters, adverse reactions, and clinical outcome. RESULTS: After three courses of treatment, the observation group had significantly greater reductions in 24-hour urinary protein, urinary albumin, urinary immunoglobulin G, urinary β2-microglobulin, serum creatinine, and blood urea nitrogen than the control group (P<0.05). After the treatment ended, the observation group had a significantly shorter time to achieve remission than the control group (P<0.05). No serious adverse reactions, such as hemorrhagic cystitis, thrombocytopenia, and hemolysis, were observed, and there was no significant difference in the overall incidence rate of adverse reactions between the two groups (P>0.05). CONCLUSIONS Compared with MP+CTX pulse therapy alone in the treatment of severe HSPN in children, DFPP combined with MP+CTX pulse therapy can further alleviate renal injury and improve clinical outcome and does not increase the incidence rate of adverse reactions.
Child ; Glucocorticoids ; Humans ; Immunosuppressive Agents ; Nephritis ; Plasmapheresis ; Purpura, Schoenlein-Henoch

D-penicillamine has been reported to cause antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis presenting as rapidly progressive glomerulonephritis or pulmonary-renal syndrome mostly in adults. We report a pediatric case of D-penicillamine induced ANCA-associated vasculitis that manifests as a pulmonary-renal syndrome with a mild renal manifestation. A 13-year-old girl who has been taking D-penicillamine for five years under the diagnosis of Wilson disease visited the emergency room because of hemoptysis and dyspnea. She had diffuse pulmonary hemorrhage, microscopic hematuria, and proteinuria. Myeloperoxidase ANCA was positive, and a renal biopsy revealed pauci-immune crescentic glomerulonephritis. Under the diagnosis of D-penicillamine-induced ANCA-associated vasculitis, D-penicillamine was switched to trientine, and the patient was treated with plasmapheresis, glucocorticoid, cyclophosphamide, and mycophenolate mofetil. Pulmonary hemorrhage improved rapidly followed by the disappearance of the hematuria and proteinuria five months later.
Adolescent ; Adult ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis ; Antibodies, Antineutrophil Cytoplasmic ; Biopsy ; Child ; Cyclophosphamide ; Diagnosis ; Dyspnea ; Emergency Service, Hospital ; Female ; Glomerulonephritis ; Hematuria ; Hemoptysis ; Hemorrhage ; Hepatolenticular Degeneration ; Humans ; Penicillamine ; Peroxidase ; Plasmapheresis ; Proteinuria ; Trientine ; Vasculitis

Guillain-Barré syndrome (GBS) is a representative form of post-infectious autoimmune neuropathy with heterogenous manifestations. It was originally considered as an ascending demyelinating polyneuropathy in Western countries. However, the discovery of anti-ganglioside antibodies on the basis of molecular mimicry theory could help us better understand various kinds of focal and regional variants as well as axonal type of GBS those were frequently found from Asian countries. Recent development of new techniques about anti-ganglioside complex antibodies is making more detailed descriptions for specific or unusual clinical manifestations. It has been regarded that GBS has good prognosis if treated properly as early as possible, but it still shows high mortality and morbidity rate with frequent long term neurologic and medical complications. Unfortunately, there are only two options for medical treatment, intravenous immunoglobulin and plasmapheresis, for the last 100 years. Several clinical studies on new immunotherapy targeting complement activating system with background of molecular mimicry using animal model are underway. We hope that these new treatments will be helpful for the future patients.
Antibodies ; Asian Continental Ancestry Group ; Axons ; Complement System Proteins ; Gangliosides ; Guillain-Barre Syndrome ; Hope ; Humans ; Immunoglobulins ; Immunotherapy ; Miller Fisher Syndrome ; Models, Animal ; Molecular Mimicry ; Mortality ; Plasmapheresis ; Polyneuropathies ; Prognosis

Hemolytic uremic syndrome (HUS) is often encountered in children with acute kidney injury. Besides the well-known shiga toxin-producing Escherichia coli-associated HUS, atypical HUS (aHUS) caused by genetic complement dysregulation has been studied recently. aHUS is a rare, chronic, and devastating disorder that progressively damages systemic organs, resulting in stroke, end-stage renal disease, and death. The traditional treatment for aHUS is mainly plasmapheresis or plasma infusion; however, many children with aHUS will progress to chronic kidney disease despite plasma therapy. Eculizumab is a newly developed biologic that blocks the terminal complement pathway and has been successfully used in the treatment of aHUS. Currently, several guidelines for aHUS, including the Korean guideline, recommend eculizumab as the first-line therapy in children with aHUS. Moreover, life-long eculizumab therapy is generally recommended. Further studies on discontinuation of eculizumab are needed.
Acute Kidney Injury ; Atypical Hemolytic Uremic Syndrome ; Child ; Complement System Proteins ; Escherichia ; Hemolytic-Uremic Syndrome ; Humans ; Kidney Failure, Chronic ; Plasma ; Plasmapheresis ; Renal Insufficiency, Chronic ; Stroke