Combination Of Intravenous Immunoglobulin Research Articles

Chronic dysimmune hypertrophic plexopathy as a variant of atypical chronic inflammatory demyelinating polyneuropathy

The authors report the clinical case of a 7-year-old child with gradual development in left leg weakness considering by the neurologist as the unknown lesion of the sciatic nerve. According to the results of clinical and instrumental examinations, the diagnosis was established as a focal form of chronic inflammatory demyelinating polyneuropathy (CIDP). The dysimmune cause of the disease was confirmed by MRI of the lumbosacral plexus with contrast enhancement of neural structures and response to therapy with high-dose intravenous immunoglobulin in combination with corticosteroids.

Blocked D in RhD hemolytic disease of fetus and newborn

A 3-day-old term neonate was admitted to our hospital for the evaluation of neonatal jaundice. The neonate was born to an RhD-negative multiparous mother who had never received anti-D prophylaxis. The neonate's direct antiglobulin test was 4+. Blood group was B, and Rh D typing was negative using tube technique and positive (2+) using gel card. The mother had anti-D, and anti-D titers were 1:1024. After heat elution, RhD typing of the neonate was positive using tube (2+) and gel technique (4+). The eluate was shown to have anti-D. As the red cells of the neonate were saturated with maternal anti-D, commercial monoclonal anti-D could not bind to D antigen which resulted in false-negative D typing in the neonate. Such blocking phenomenon is rare. The discrepancy was identified while using two different techniques (tube and gel) which aided in early diagnosis and tailored an appropriate treatment. The neonate improved after the initiation of a combination of intravenous immunoglobulin and phototherapy.

Combined Treatment with Zinc Aspartate and Intravenous Immunoglobulins (IVIGs) Ameliorates Experimental Autoimmune Encephalomyelitis (EAE).

Intravenous immunoglobulins (IVIGs) are widely used in replacement therapy of primary and secondary immunodeficiency disorders and in approved autoimmune indications. In addition, IVIG application is used off-label for treatment of other autoimmune diseases, e.g., multiple sclerosis (MS), an inflammatory autoimmune disorder with a clear T cell-mediated immune pathogenesis. The trace element zinc is shown to play a regulatory role in the maintenance of immune functions. Changes of zinc homeostasis affect both the innate and the adaptive immune system. On one hand, therapeutic zinc supplementation can normalize impaired immune functions due to zinc deficiency. On the other hand, therapeutic zinc supplementation is under consideration as a possible option to treat T cell-mediated autoimmune diseases. The aim of the present study was to investigate the influence of IVIG (Octagam®), zinc aspartate (Unizink®), and the combined application of both preparations in the experimental autoimmune encephalomyelitis (EAE), the animal model of MS. Therapeutic intraperitoneal application of zinc aspartate significantly diminished clinical signs during the relapsing-remitting phase of EAE in SJL/J mice. In contrast, IVIG given in a therapeutic manner did not influence the course of EAE. Interestingly, the combined application of both, IVIG and zinc aspartate, significantly reduced the severity of the disease during the acute and the relapsing-remitting phase of the EAE. Our data suggest that the combination of IVIG and zinc aspartate may have beneficial effects in autoimmune diseases, like MS. Further studies should verify the benefit of a controlled immunosuppressive therapy with IVIG and zinc for such diseases.

Retrospective analysis of a single-center clinical experience toward development of curative treatment of 123 pemphigus patients with a long-term follow-up: efficacy and safety of the multidrug protocol combining intravenous immunoglobulin with the cytotoxic immunosuppressor and mitochondrion-protecting drugs.

Pemphigus vulgaris (PV) is a life-long IgG autoantibody-mediated blistering disease affecting the mucosal surfaces lined by the stratified epithelium (oral, nasal, genital) and sometimes also the skin. While corticosteroid treatment is life saving, the high dose and prolonged courses required for disease control are associated with significant adverse effects, including death. Although introduction of rituximab (RTX) provided for a favorable outcome, the high relapse rate, that is, up to 80%, precludes successful use of RTX as a monotherapy. Intravenous immunoglobulin (IVIg) is being increasingly utilized as off-label therapy for a variety of autoimmune and inflammatory diseases, including PV and pemphigus foliaceus (PF). The goal of pemphigus research is to develop an effective treatment modality that would allow patients to achieve and maintain a stable clinical remission without the need for additional treatments, or cure. This article summarizes clinical outcome of 123 pemphigus patients treated with a combination of IVIg, an immunosuppressive cytotoxic drug (ICD) and mitochondrion-protecting drugs in the Blistering Disease Clinic at the University of California, Irvine from 2007 to 2017. The mean time to disease control was 0.2 months and time to complete remission - 1.7 months. Duration of complete remission on drugs until relapse or end of treatment was 19.3 months. The mean duration of complete remission off drugs until relapse was 15.8 months. That until end of follow up was 48.4 months, with a minimum of 14 and a maximum of 91 months. The overall complete remission rate off all drugs was 100%, with 12% overall relapse rate. Most relapses, 8.1 vs. 3.3%, occurred during the time of treatment, compared to posttreatment. No patients had more than a single relapse. The duration of the posttreatment follow-up ranged from 9 to 97 months with a mean of 64.8 months, or 5.4 years. The total number of IVIg cycles ranged from 26 in patients without a relapse to 37 in patients with a relapse. The clinical outcome in patients that received IVIg with RTX or another ICD were found to be very similar. Thus, the multidrug IVIg regimen allowed to achieve three principal treatment objectives: (i) rapid control of pemphigus symptoms; (ii) stable disease remission; and (iii) overall safety of treatment. While the individualized therapeutic approaches to eradicate the autoreactive B cell clones causing disease in each particular PV or PF patient are being developed, all pemphigus patients can benefit from the treatment protocol described in this study.

Combination of intravenous immunoglobulin and steroid pulse therapy improves outcomes of febrile refractory status epilepticus

BackgroundFebrile infections are an important cause of paediatric refractory status epilepticus, and immune-mediated mechanisms and inflammatory processes have been associated with neurological manifestations in such patients. The aim of this study was to investigate the effects of immunotherapy as adjuvant treatment for febrile refractory status epilepticus. MethodsWe retrospectively reviewed cases of febrile refractory status epilepticus in a paediatric intensive care unit between January 2000 and December 2013 and analysed their clinical characteristics. Patients positive for antineuronal antibodies against surface antigens were excluded. ResultsWe enrolled 63 patients (38 boys), aged 1–18 years, all of whom received multiple antiepileptic drugs. Twenty-nine (46%) of the patients received intravenous immunoglobulin alone, 16 (25.4%) received a combination of intravenous immunoglobulin and methylprednisolone pulse therapy, and 18 (28.6%) did not receive immunotherapy treatment. Overall, 12 (19%) patients died within 1 month. After 6 months, 12 (20%) patients had good neurological outcomes, including two who returned to baseline and 13 (29.5%) who had favourable seizure outcomes. We compared the outcomes of the different treatments, and found that a combination of intravenous immunoglobulin and methylprednisolone pulse therapy had the best neurological and seizure outcomes at 6 months compared to intravenous immunoglobulin alone and no immunotherapy. ConclusionsOur observational study showed that a combination of intravenous immunoglobulin and methylprednisolone pulse therapy as adjuvant treatment for febrile refractory status epilepticus was associated with better neurological and seizure outcomes. Further prospective studies are needed to confirm these findings.

The Epidemiology of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in China.

Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) are life-threatening disease. However, there are only few epidemiologic studies of SJS/TEN from China. To analyze the clinical characteristics, causality, and outcome of treatment for SJS/TEN in China, we reviewed case reports of patients with SJS/TEN from the China National Knowledge Infrastructure (CNKI) and Wanfang database from 2006 to 2016 and patients with SJS/TEN who were admitted to the First Affiliated Hospital of Fujian Medical University during the same period. There were 166 patients enrolled, including 70 SJS, 2 SJS/TEN overlap, and 94 TEN. The most common offending drugs were antibiotics (29.5%) and anticonvulsants (24.1%). Carbamazepine, allopurinol, and penicillins were the most common single offending drugs (17.5%, 9.6%, and 7.2%). Chinese patent medicines accounted for 5.4%. There were 76 (45.8%) patients receiving systemic steroid and intravenous immunoglobulin (IVIG) in combination therapy, especially for TEN (80.3%), and others were treated with systemic steroids alone. Mortality rate of combination treatment comparing with steroid alone in TEN patients had no statistical significance. In conclusion, carbamazepine and allopurinol were the leading causative drugs for SJS/TEN in China. Combination of IVIG and steroids is a common treatment for TEN, but its efficacy in improving mortality needs further investigation.

The clinical features of patients concurrent with Guillain-Barre syndrome and myasthenia gravis.

Objectives:To evaluate all the coincidence cases of Guillain-Barré syndrome (GBS) and myasthenia gravis (MG).Methods:We performed web-based research of the overlapping incidence of GBS and MG in studies occurring from 1982 to 2016 and restricted to the English language.Results:Among 15 cases, an elevated CSF protein level without pleocytosis was found in 10 cases (66.7%); reduced nerve conduction was found in 13 cases (86.6%); a positive repetitive nerve stimulation test occurred in 11 cases (73.3%); anti-AChR antibodies were found in 13 cases (86.6%); anti-GQ1b antibodies were found in 6 cases (40%); a positive edrophonium chloride test was present in 10 cases (66.7%); and a co-occurring thymoma or thymectomy occurred in 4 cases (26.6%). The MG co-occurred with acute inflammatory demyelinating polyneuropathy (AIDP) in 8 cases and with Miller Fisher Syndrome in 5 cases. Treatment in the assessed cases included pyridostigmine (10 cases), prednisolone (7 cases), intravenous immunoglobulin (9 cases), plasmapheresis (3 cases), combined intravenous immunoglobulin and plasmapheresis in one case, and immunosuppressive drugs in 2 cases (azathioprine). Functional outcome was mentioned in 13 patients. The prognosis was favorable in 8 of the 15 recorded patients (Hughes 0-1), and 2 cases resulted in death.Conclusion:Although comorbidity of GBS and MG is extremely rare, early recognition of this combination of inflammation of peripheral nerves and the neuromuscular junction is of great importance for both initial treatment and a better prognosis.

Guillain-Barré Syndrome and Its Variants: Clinical Course and Prognostic Factors.

We aimed to analyze the frequency, clinical characteristics, medical treatment options and final functional status of Guillain-Barré syndrome (GBS) and its variants in a population from a tertiary hospital setting. All medical records of patients with acute inflammatory polyneuropathy between the years of 1998-2013 were retrospectively screened. Demographic, clinical and laboratory information, treatment options and the rate of recovery of the patients were gathered. A total of 183 patients met the study criteria. Subtypes were typical demyelinating form (n=102, 79.1%), acute motor sensory axonal variant (n=11, 8.5%), acute motor axonal variant (n=10, 7.8%), Miller-Fisher syndrome (n=5, 3.9%), and pure sensory subtype (n=1, 0.8%). Remaining patients had the diagnosis of acute-onset chronic inflammatory demyelinating polynuropathy. The data of treatment option were available for 70 patients. Most of the patients received intravenous immunoglobulin (IVIg) treatment or the combination of IVIg and methylprednisolone. One patient died, there was no improvement in eight patients and rest showed improvement with varying degrees. We did not observe major change of recovery between different treatment options, however, most of the patients using methylprednisolone required IVIg because of inadequate response.

Clinical Outcome of Rituximab and Intravenous Immunoglobulin Combination Therapy in Kidney Transplant Recipients with Chronic Active Antibody-Mediated Rejection.

BACKGROUND We previously reported that rituximab (RIT) and intravenous immunoglobulin (IVIg) combination therapy is effective in deterring the progression of chronic active antibody-mediated rejection (CAMR), but that report was based on the assessment of a small number of cases for a short period. MATERIAL AND METHODS Forty-three patients with CAMR were recruited during the study period after 2010. The patients were divided into high (n=17, 39.5%) and low proteinuria groups (n=26, 60.5%) based on spot urine protein-to-creatinine ratio of > or <3.5 g/g. We compared clinical outcomes between the two groups in terms of allograft survival rate, decrease in estimated glomerular filtration rate (ΔeGFR), change in proteinuria level, and infectious complications. We also evaluated the risk factors of allograft failure. RESULTS The 3-year allograft survival rate after combination treatment was 60.5% overall, but was higher in the low proteinuria group than in the high proteinuria group (69.2% versus 47.1%; log rank p<0.05). The combination treatment reduced the eGFR slope in both groups, and this effect was more definite in the low proteinuria group. No significant differences in the amount of proteinuria and infectious complication rate were found between the two groups. Proteinuria and eGFR at treatment were independent predictive factors of allograft failure (p<0.01 and p<0.001, respectively). CONCLUSIONS RIT and IVIg combination therapy was effective in reducing the progression of CAMR, and this effect was more definite in the patients with low proteinuria.

A Prospective Randomized Crossover Comparison Between Piperacillin/Tazobactam and Meropenem As Empirical Antibiotic Therapy for Febrile Neutropenia in Children with Hematological and Malignant Disorders

BackgroundFebrile neutropenia (FN) remains an important cause of morbidity and mortality in pediatric cancer patients. Although monotherapy using broad spectrum antibiotics is now a standard of care, there is currently no consensus on optimal agents. Carbapenems are effective and widely used in the treatment of FN; however, they have several disadvantages such as the risk of the emergence of drug-resistant bacteria. The usefulness of intravenous immunoglobulin (IVIG) for FN remains unclear. This randomized crossover study was designed to assess whether piperacillin/tazobactam (PIPC/TAZ) is as effective as meropenem (MEPM). We also evaluated the effectiveness of IVIG in combination with switched antibiotics as a second-line therapy.METHODSFebrile and neutropenic patients treated with conventional or high-dose chemotherapy at the Department of Pediatrics, Sapporo Hokuyu Hospital between April 2012 and March 2016 were included. As a first-line treatment, FN cases were randomized for treatment with either PIPC/TAZ (337.5 mg/kg/day in 3 portions, 1-hour DIV, maximum 13.5 g/day; arm PM) or MEPM (120 mg/kg/day in 3 portions, 1-hour DIV maximum 3 g/day, arm MP). Success was defined as (1) fever and clinical signs of infection resolving within 120 hours of the initiation of antibiotic therapy; and (2) no recurrence of infection/fever after the end of the treatment. Failure was defined as the persistence of fever and infectious signs more than 120 hours after the initiation of antibiotic therapy, a required change in the initial antibiotic therapy, or deterioration/death due to infection. FN cases judged as failures were registered for a second-line treatment, in which antibiotics were switched to another antibiotic (MEPM (arm PM) or PIPC/TAZ (arm MP)), and further randomized to be treated with or without IVIG at 100 mg/kg/day (maximum 5 g/day) for 3 consecutive days. The effects of the second-line therapy were evaluated 72 hours after its initiation. The Institutional Review Board of the hospital approved our project, and written informed consent was obtained from all patients or their parents.RESULTS A total of 434 febrile episodes in 105 patients (42 females and 63 males) with a median age of 8 years (range, 0-25) were enrolled for the first-line therapy. During the study period, 26 patients underwent hematopoietic stem cell transplantation (HSCT). Blood cultures were positive in 47 out of 434 episodes (10.8%) and the leading cause of bacteremia was a-streptococcus (25.5%). Regarding responses to the first-line treatment, success rates between the PIPC/TAZ and MEPM groups were similar (62.4% vs 65.9%, P=0.484), even if patients were restricted to those with bacteremia (26.1% vs 37.5%, P=0.534). Mortality rates did not significantly differ between the two groups (0.8% vs 0%, P=0.500). A total of 144 febrile episodes in 71 patients (29 females and 42 males) with a median age of 11 years (range, 0-25) were enrolled for the second-line therapy. During the study period, 20 patients underwent HSCT. Success rates did not significantly differ between cases treated with PIPC/TAZ (switched from MEPM, with and without IVIG; arm MP) and with MEPM (switched from PIPC/TAZ, with and without IVIG; arm PM) (55.3% vs 44.3%, P=0.936). Cases treated with IVIG had slightly better success rates than those without IVIG (58.7% vs 41.9%, P= 0.064). When cases were restricted to those with IgG <500 mg/dl at the beginning of the second-line therapy, success rates were significantly better in cases with IVIG than in those without (81.8% vs 38.5%, P=0.047). No significant differences were observed in total success rates between arm PM (PIPC/TAZ followed by MEPM) and arm MP (MEPM followed by PIPC/TAZ) (57.7% vs 63.4%, P=0.174). The rates of adverse events in the first- and second-line therapies were not significantly different between the two groups.DISCUSSIONThis study demonstrated that PIPC/TAZ and MEPM were effective and safe as empirical therapies for FN. Therefore, we concluded that these agents are equally effective. Large numbers of cases with treatment failure after MEPM may be rescued by PIPC/TAZ and vice versa. Carbapenems are now more widely used in the treatment of FN; however, this may increase the prevalence of drug-resistant bacteria, and, thus, the use of PIPC/TAZ for neutropenic children is favorable. In addition, the concomitant use of IVIG needs to be considered for patients with low serum IgG values. DisclosuresNo relevant conflicts of interest to declare.

The Effect of Intravenous Immunoglobulin Combined with Corticosteroid on the Progression of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Meta-Analysis.

BackgroundIntravenous immunoglobulin (IVIG) treatment is commonly used to treat Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) with controversial therapeutic effect.MethodsWe conducted a comprehensive meta-analysis through combining the published eligible studies to evaluate the effectiveness of IVIG on SJS and TEN treatment.ResultsA total of 26 studies were selected from public available databases. The combination of IVIG and corticosteroid markedly reduced the recovery time (by 1.63 days, 95% CI: 0.83–2.43, P < 0.001), compared with solo corticosteroid group. The favorable effects were greater in Asian (2.19, 95% CI: 1.41–2.97, P < 0.001), TEN (2.56, 95% CI: 0.35–4.77, P = 0.023) and high-dose IVIG treated individuals (1.78, 95% CI: 0.42–3.14, P = 0.010). The hospitalization length reduced by 3.19 days (95% CI: 0.08–6.30, P = 0.045), though the outcome was proven to be unstable. We found heterogeneities, which sources were probably regional factors. Besides, IVIG was inclined to decrease SJS/TEN mortality (SMR: 0.84, 95% CI: 0.66–1.08, P = 0.178). This impact was possibly more profound when patients were treated with high dose IVIG (SMR: 0.74, 95% CI: 0.50–1.08, P = 0.116), or when patients were diagnosed as TEN (SMR: 0.68, 95% CI: 0.45–1.01, P = 0.058).ConclusionsOur current meta-analysis suggests that IVIG combined with corticosteroid could reduce recovery time for SJS and TEN. This effect is greater among Asian patients. Whereas, its impact on reducing mortality is not significant.

Stemming MRSA-induced pneumonia

Infectious Disease Controversies persist about the use of human intravenous immunoglobulin (IVIG) as an adjunct treatment for severe pneumonia caused by Staphylococcus aureus infections. Diep et al. show that in rabbits, only two specific antibodies contained in IVIG are necessary and sufficient to confer protection against necrotizing pneumonia caused by methicillin-resistant S. aureus (MRSA) strains. These antibodies neutralize the toxic effects of α-toxin and Panton-Valentine leukocidin. Preexposure prophylaxis with IVIG, or postexposure treatment with IVIG in combination with either vancomycin or linezolid, improved survival outcomes. Sci. Transl. Med. 8 , 357ra124 (2016).

Comparing the Outcomes of IVIg with Combination of IVIg and Methylprednisolone in Children with Acute Idiopathic Thrombocytopenia; a Bayesian Logistic Approach

Background This study aimed to evaluate the effectiveness of Intravenous immunoglobulin (IVIg) and combination of IVIg and Methylprednisolone for childhood Idiopathic (autoimmune) Thrombocytopenia (ITP) treatment; in addition investigate the related factors to develop chronic form of under 15 years ITP. Materials and Methods This retrospective study conducted on 88 ITP patients that treated with IVIg or combination of IVIg and Methylpredinosolon. Children were treated with IVIg 2 mg/kg/d or combination of IVIg 2 mg/kg/d and Methylpredinosolon20 mg/kg/dfor maximum 5 days. The numbers of patients with a platelet count > 50,000/μl, after treatment initiation, were the primary outcome. Odds Ratio (OR) as well as 95% Bayesian Credible interval (Crl), were estimated using a Bayesian Logistic regression model. Results The median age of subjects was 3.5+ 4.42 years (Interquartile: 2 8.5). About 13% of patients were discharged from hospitalization in day 2 and day 3. The ITP of 23% of children were progressed to chronic form. The following factors were significantly associated with the development of chronic ITP, combination of IVIg and Methylprednisolone [OR: 3.24, 95% Crl: [1.06 11.11]), and day 2 and 3 of discharge from hospitalization (OR: 7.72, 95% Crl: (1.14 67.16)]. Conclusion The current results, suggest that the both IVIg and combination of IVIg are equally effective in providing a platelet level > 50,000/μl early. In addition patients how received combination drug were more likely to develop to chronic ITP. Therefore, we suggest that this route must be preferentially used in decision making for treatment childhood ITP.

Long-term results of rituximab–intravenous immunoglobulin combination therapy in patients with epidermolysis bullosa acquisita resistant to conventional therapy

Background: Epidermolysis bullosa acquisita (EBA) is a rare subepidermal bullous disease. Long-term remission in this disease is difficult using current treatments, unlike that in patients with other autoimmune bullous diseases.Objective: We retrospectively evaluated the effectiveness and side effects of rituximab–intravenous immunoglobulin (IVIg) combination treatment in five patients with EBA resistant to conventional treatment.Patients and methods: Rituximab (375 mg/m2) was administered for four consecutive weeks to four patients, and their treatment continued with IVIg at a dose of 2 g/kg/month. One patient received two cycles of rituximab for three consecutive weeks, IVIg in the fourth week, followed by monthly IVIg administrations as in the other patients.Results: The total number of IVIg therapy cycles ranged from 10 to 26 (mean 19.4). Mean skin involvement, mucosal involvement, and disease severity scores decreased after a mean follow-up of 22.6 months (range, 10–28 months). In an analysis performed during months 24–28, the number of CD19-positive B cells was found to be below the normal reference range in four patients.Limitations: This was a retrospective study with a limited number of patients.Conclusion: Rituximab–IVIg combination treatment seems to be effective and safe for treating patients with EBA resistant to conventional treatments.

First line treatment of pemphigus vulgaris with a novel protocol in patients with contraindications to systemic corticosteroids and immunosuppressive agents: Preliminary retrospective study with a seven year follow-up

BackgroundConventional therapy for pemphigus vulgaris (PV) consists of high-dose systemic corticosteroids (CS) and immunosuppressive agents (ISA). This combination may be ineffective, cause serious adverse events or relapses in some patients. ObjectiveTo determine if the combination of intravenous immunoglobulin (IVIg) therapy and rituximab (RTX) can be used as first-line therapy in PV patients in whom systemic CS and ISA are contraindicated and evaluate its ability to produce long-term sustained remissions. MethodThis a retrospective study of five male and five female patients (mean age 47.87years). RTX was administered once weekly for eight consecutive weeks, followed by once monthly for four months (dose 375mg/m2). Since CD20+ B cells were undetectable, IVIg was infused until they reached normal levels (dose 2g/kg/cycle). IVIg was then continued according to published protocol. ResultsInitial clinical response and complete disease resolution occurred in a mean of 3.2weeks and 7.4weeks, respectively. Mean duration of rituximab therapy was 6.09months and 33.7months for IVIg therapy. Mean duration of follow-up after the last dose of rituximab was 86.08months, during which all patients remained in complete remission. Mean length of total follow-up was 103.99months. No relapses, infections, or hospitalizations were reported. ConclusionsWhen systemic CS and ISA are contraindicated in PV patients, combination RTX and IVIg therapy can produce a prolonged, sustained remission without additional systemic therapy. This positive clinical outcome could be the consequence of pathogenic B cell depletion and restoration of immune regulation.

Short-Term Prognosis of Mechanically Ventilated Patients With Guillain–Barré Syndrome Is Worsened by Corticosteroids as an Add-On Therapy

Intravenous immunoglobulin (IVIg) has been proven most effective in treating Guillain–Barré syndrome (GBS). Corticosteroids as an add-on therapy have been prescribed in severe GBS cases. However, the efficacy of intravenous corticosteroids combined with IVIg in dealing with severe GBS remains unclear. We explored the therapeutic effects of different therapeutic regimens on the short-term prognosis of GBS patients, especially the severe cases.We retrospectively analyzed the clinical data of 527 adult patients with GBS who were prescribed to different treatments from 2003 to 2014. The therapeutic effect of a treatment was evaluated by the improvement of Hughes Functional Grading Scale (HFGS) and Medical Research Council (MRC) sum score.With comparable incidence of infectious complications (P > 0.05), more mechanically ventilated patients were found improvement after IVIg treatment than combination IVIg with intravenous corticosteroids (MRC: 97% vs. 72.4%, P < 0.05; HFGS: 97% vs. 72.4%, P < 0.05). As to bedridden patients without mechanical ventilation, incidence of infectious complications (P > 0.05) and ratio of patients who were improved after IVIg were insignificantly different from the combination therapy (MRC: 89.6% vs. 86.5%; HFGS: 69.6% vs. 61.5%; both P > 0.05), even if the intravenous corticosteroids were initiated within 7 days after onset (P > 0.05). In addition, supportive treatment was sufficient for patients who were able to walk with help (HFGS = 3) and mildly affected (HFGS < 3) when compared with IVIg and intravenous corticosteroids.IVIg is sufficient to GBS patients who are unable to walk (HFGS > 3), while corticosteroids are detrimental for short-term prognosis in mechanically ventilated patients when used in combination with IVIg. Further prospective and randomized studies are warranted to validate this finding.

Features of Kawasaki disease in early childhood

Kawasaki disease causes diagnostic difficulties due to insufficient knowledge of doctors, and therefore often goes under the «disguise» of other diseases and therefore is under-recognized in Russia so far. Laboratory tests reveal leukocytosis, increased erythrocyte sedimentation rate, hypochromic anemia and thrombocytosis, accompanied by hypercoagulation. Late diagnosis and treatment of Kawasaki disease increase the risk for thrombosis and coronary arteries aneurysms, myocardial infarction at a young age, death. The results of the original research targeted to analyze the clinical course and outcomes of Kawasaki disease in 15 infants treated at the department of children under one year of age of the Nizhny Novgorod Regional Pediatric Clinical Hospital during the last 6 years, are presented. The cardiovascular conditions included pericarditis in 4 patients, coronary arteritis in 2 patients, coronary arteritis with dilation of the coronary arteries entrance and thickening of the walls - in 4, myocarditis - in 1 patient, 1 patient had no signs of cardiovascular involvement. All patients without coronary artery aneurysms were treated with intravenous immunoglobulin (1-2 g/kg per course), acetylsalicylic acid (80-100 mg/kg daily) up to fever reduction, followed by 3-5 mg/kg per day up to the normal values of acute inflammation markers and platelet counts, and aggregation inhibitors. In 2 patients, Kawasaki disease led to death, thus in both cases the diagnosis and, consequently, treatment initiation were late. Early diagnosis and rational treatment of Kawasaki disease (high doses of intravenous immunoglobulin in combination with acetylsalicylic acid) is the key to a favorable outcome. Fever of unknown origin, which lasts 7 days or more in a child of an early age, is an absolute indication for echocardiography with mandatory visualization of the coronary arteries. Tactics of following up patients, who underwent Kawasaki disease, is determined by the degree of coronary lesions and the risk of myocardial ischemia.

Chronic inflammatory demyelinating polyneuropathy in children: a report of four patients with variable relapsing courses.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronically progressive or relapsing symmetric sensorimotor disorder presumed to occur because of immunologic antibody-mediated reactions. To understand the clinical courses of CIDP, we report variable CIDP courses in children with respect to initial presentation, responsiveness to medical treatment, and recurrence interval. Four patients who were diagnosed with acute-onset and relapsing CIDP courses at Severance Children's Hospital, Seoul, Korea, were enrolled in this retrospective study. We diagnosed each patient on the basis of the CIDP diagnostic criteria developed in 2010 by the European Federation of Neurological Societies/Peripheral Nerve Society Guidelines. We present the cases of four pediatric patients diagnosed with CIDP to understand the variable clinical course of the disease in children. Our four patients were all between 8 and 12 years of age. Patients 1 and 2 were diagnosed with acute cerebellar ataxia or Guillain-Barré syndrome as initial symptoms. While patients 1 and 4 were given only intravenous dexamethasone (0.3 mg/kg/day) for 5 days at the first episode, Patients 2 and 3 were given a combination of intravenous immunoglobulin (2 g/kg) and dexamethasone (0.3 mg/kg/day). All patients were maintained with oral prednisolone at 30 mg/day, but their clinical courses were variable in both relapse intervals and severity. We experienced variable clinical courses of CIDP in children with respect to initial presentation, responsiveness to medical treatment, and recurrence interval.

Treatment of Presumptive BK Nephropathy with Ciprofloxain in Kidney Transplant Recipients: Three Case Reports

BK virus nephropathy has emerged as an important cause of renal allograft dysfunction. Reduction in immunosuppression is the mainstay of BK virus nephropathy treatment. However, decreasing immunosuppressive medications is not sufficient for treatment of BK virus nephropathy. Therefore, there is a need for other treatment strategies such as cidofovir, leflunomide, and intravenous immunoglobulin in combination with immunosuppression reduction. Ciprofloxacin has recently been reported to have antiviral activity and decrease BK viral load in kidney transplant recipients. These findings suggest that the use of ciprofloxacin represents a valuable treatment strategy in patients with BK virus nephropathy. Here, we report on our experience with three patients who developed presumptive BK virus nephropathy after kidney transplantation, who, after 2 months of ciprofloxacin treatment, showed disappearance of BK viremia and improvement in the estimated glomerular filtration rate. Ciprofloxacin may be consid- ered an effective treatment option for BK viremia in kidney transplant recipients.

Intravenous immunoglobulin G in the treatment of autoimmune bullous disease.

Intravenous immunoglobulin G in the treatment of autoimmune bullous disease.