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Good?s syndrome is defined by the association of thymoma, hypogammaglobulinemia and recurrent infections especially bronchopulmonary and sinus. We report the case of a 53 years old man , with a history of recurrent respiratory infections, hospitalized in June 2017 for bronchial syndrome dragging with radiographic front chest presence of bilateral alveolar syndrome and lat?rotrach?ale opacity right, supplemented by a thoracic scan, which objectified the chance discovery of a regular meditational tissue mass without locoregional malignancy, with no clinical and electrophysiological signs of myasthenia gravis. The patient was operated for his m?diatisnal mass which histological study was in favor of a thymoma type A, the outcome was favorable. In our patient the association between thymoma and hypogammaglobulinemia and bronchopulmonary infection severe and recurrent led to the diagnosis of Good\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\'s syndrome. The patient was put under appropriate antibiotic therapy combined with weekly infusions of intravenous immunoglobulin, with a positive trend, achieving significant clinical improvement, biological and radiological. Good?s syndrome represents only 5% of parathymiques diseases, but its research is essential because it allows effective treatment of infections, avoiding lung destruction and the use of lung surgery.

The clinical and therapeutic aspects of primary Sjögren syndrome (PSS) in patients with peripheral neuropathy were analyzed and the specifics of individual case studies are discussed.We retrospectively studied six patients (four women, two men; mean age 64.5 years) presenting with PSS with peripheral neurological involvement over a five-year period (2008-2012). All patients had neurological examinations, including nerve conduction studies, somatosensory evoked potentials, and sural nerve biopsies. Treatment regimens included corticosteroids, intravenous gammaglobulin, or immunosuppressive treatment.Peripheral neuropathy was observed in six (7.9 %) of 76 patients with SS as the underlying disease; three were cases of multiple mononeuropathy, two cases had sensory ataxic neuropathy, one of which was autonomic neuropathy, and one case was diagnosed as painful sensory neuropathy without sensory ataxia. Four of the six patients were diagnosed with SS after the onset of neurological symptoms. Individual peripheral neuropathies had distinct neurological, electrophysiological, and pathological characteristics. The effect of steroids and intravenous gammaglobulin differed depending on the case.In PSS patients, a precise diagnosis is important, because the therapeutic strategy and response varies depending on the type of neuropathy. In clinical practice, it is important to consider a diagnosis of SS when patients present with peripheral neuropathy.

3 aplastic patients with acute leukaemia, strongly HLA-immunized and refractory to platelet transfusions, received polyvalent gammaglobulin i.v. infusions (0.4 g/kg/d for 5 or 6 d) in association with daily random platelet transfusions. Platelet recovery was obtained in 2 patients. The 3rd patient did not show any significant rise in platelet count. The ability of gammaglobulin to prolong the life-span of incompatible transfused platelets could facilitate the management of HLA-immunized patients.

12 patients with severe chronic idiopathic thrombocytopenic purpura (ITP) were treated with i.v. polyvalent intact immunoglobulin (0.14-0.4 g/kg body weight for 5 d) because of various bleeding episodes or prior to surgery. In 9 patients a significant rise in platelet counts was noted, starting on d 2 and reaching its maximum between d 4 and 11. Thereafter, platelet counts decreased slowly, approaching pre-treatment values after 20 d in most cases. Response to immunoglobulin was reproducible, when infusions were repeated. Response to high-dose immunoglobulin was independent of age of patients and duration of disease. Platelet-associated IgG decreased in all patients tested. A pepsin-treated immunoglobulin preparation was ineffective in 3 patients.

Background: The clinical significance and efficacy of treating patients who have immunoglobulin (Ig) G subclass deficiency and/or antibody deficiency with Ig-replacement therapy has been debated. There are no clear guidelines to recommend intravenous gammaglobulin (IgIV) in these patients as there are few published studies documenting its efficacy. Methods: We studied in an open-label protocol 10 adult patients with recurrent respiratory infections and IgG subclass and/or antibody deficiency. All patients received monthly IgIV for 12 months and then were observed for 3 months without IgIV infusions. We studied quality of life, incidence of infections, need for antibiotics, frequency of hospitalizations due to infections, IgG subclass and antibody (tetanus and pneumococcal) levels. Innate immunity was evaluated by studying the status of Toll-like receptors and polymorphisms, mannan-binding lectin levels and genotypes. Correction of the immune defects during IgIV therapy was evaluated. Results: Monthly IgIV significantly improved quality of life, decreased the number of infections and the need for antibiotics, and improved IgG subclass and antibody serum levels. No consistent finding of innate immunity could be detected. Conclusions: IgIV could be beneficial in patients with IgG subclass or antibody deficiency.

Either 5 g Sandoglobulin or 5 g Gamma-Venin was administered in short-time intravenous infusions to 14 volunteers. The gamma-globulin preparation was given to some of the volunteers 24 hours after rubella vaccination, in the others 14 days, in three of the Sandoglobulin group 28 days before rubella vaccination. Gamma-Venin in these circumstances did not retard the vaccination-induced rubella infection. In all volunteers the usual rubella titres developed after the vaccination. Sandoglobulin, on the other hand, in all the given conditions either completely inhibited or at least decreased the rubella infection, an effect which is well known from the experimental studies with standard globulin preparations under administration of similar amounts of antibodies.

Intravenous Ig (IVIg) products are used in various medical conditions. Differences in excipients account for most adverse events (AE). Reports of complications including acute myocardial infarction (AMI) and acute renal failure (ARF) have emerged. Herein is described one institution's experience with IVIg-related complications. This study is a retrospective analysis of infusion-related AE that are associated with various IVIg products. Infusion-related AE were monitored during and after the administration of three IVIg products: Gamimune-N 10% (n = 76), Polygam (n = 105), and Carimune (n = 98). AE segregated to specific IVIg products. No patients who received Gamimune-N experienced AMI or ARF. Five (4.7%) patients (P0.01) in the Polygam group experienced AMI. Eight (8.2%) patients (P0.0001) in the Carimune group developed ARF. IVIg was safe to give on hemodialysis. IVIg products differ in osmolality, pH, and sugar and sodium content; this results in specific AE. Polygam resulted in no ARF but an increase in AMI. Carimune products at 9% concentration resulted in an increase in ARF. Gamimune-N 10% and other IVIg products were frequently associated with headaches. Administration of IVIg to patients who are on hemodialysis seems to be safe and effective.

The first therapeutic indication, and still currently the most applied of gamma globulin, is the one which uses its substitutive capacity in patients with antibodies deficiencies. Its immunomodulatory effectiveness with pro and anti-inflammatory activity holds a second place in their indications, in autoimmune and inflammatory illnesses. The first gamma globulin to be used in patients with agammaglobulinemia in the 50’s, were of intramuscular administration, with significant limitations on the volume and the infusion’s amount, as well as being painful . For over 20 years intravenous immunoglobulin (IVIG) has been widely used in human pathology. The first proteolytic enzyme-treated intravenous immunoglobulins allowed large doses infusions, thereby getting higher levels of IgC circulating in plasma, a subclass profile similar to the normal population, as well as a much higher average life expectancy. The pharmaceutical industry efforts brought about new more purified and viral safer immunoglobulins. The Cohn and cold ethanol methods were quickly supplemented with ulterior procedures that strengthened the viral safety of these products. The application of molecular techniques to the screening study of plasma donors, as well as incorporating new viral inactivation procedures during its manufacture, have made of IVIG a safe product whose consumption has exponentially increased in recent years. IVIG’s high cost, the hypothetical plasma supply problems and risks associated to its administration made essential the writing of usage protocols for official agencies, which, besides, hospitals also adopted.

SummaryThree and 5 year follow-up studies of eighty-nine young people with Chronic Fatigue Syndrome who completed a double blind randomised controlled trial of intravenous gammaglobulin has been conducted to determine whether the improvement following the intravenous gammaglobulin was sustained.Initial telephone contact and a questionnaire that assessed functional outcomes including, physical activity, social activities, work/school attendance and work/school workload was used. Strategies and treatment that were found helpful and ways to improve management were also asked.Follow-up data were obtained on 86 of 89 after the study concluded. The 3-year follow-up yielded a 75% response to the questionnaire. A 78% follow up response rate at 5 years was achieved for those enrolled in the study with 84% (n = 74) of those who completed the study being traced. The mean follow-up time from commencement of illness was 56 months (s.d. 25 months), range 15-112 months. There was no persistent deterioration in function ...

Although generally well tolerated there have been a number of reports of acute deterioration in renal function with the use of intravenous immune globulin (IVIG). It is of generally limited duration and is self-limiting. We for the first time have described a patient with multiple myeloma and normal renal function who developed chronic renal failure shortly after IVIG treatment in a patient with no pre-existing renal disease. We would therefore recommend the cautious use of IVIG in patients with normal renal function or with pre-existing renal disease. Renal function should also be assessed both prior to and after application of IVIG.

though intravenous gammaglobulin (IVIG) with aspirin is effective therapy for most children with acute KD, its mechanism of action is unknown, and some children do not respond. In the present era of sophisticated medicine, this conversation, with all its unknowns, is very unsettling both to me and to the family. In addition, when I diagnose acute KD in a child during the first week of fever and promptly administer IVIG and aspirin therapy but the child fails to respond and coronary aneurysms develop and worsen despite additional therapies, I ponder the sobering reality that even those of us who have devoted a great deal of our careers to the study of KD cannot always stop this process. All of this leads directly to the question: what is the cause of this disease? Finding the cause of KD is a pediatric infectious diseases research priority. Identification of the causative agent(s) would be the most promising step toward developing a diagnostic test and specific therapy and, ultimately, preventing the disease. Therefore, a study by Esper et al. [1] from Yale, published in the Journal of Infectious Diseases in February 2005, that implicated an association between the new human coronavirus (HCoV) NL-63 and KD, was met with great interest. Those investigators reported detection of the virus in respiratory samples from 8 of 11 patients with KD and 1 of 22 control subjects by reverse transcription-polymerase chain reaction (RT-PCR). As a result of that article, laboratories around the world performed additional studies to determine whether an association between KD and HCoV NL-63 could be confirmed. This

Guillain-Barré syndrome is an acute inflammatory demyelinating neuropathy characterized by progressive symmetric polyradiculoneuritis, predominantly manifested by weakness and areflexia. In this article, we report our findings in 25 children treated with intravenous immunoglobulin and compare them with the remaining 30 children who received supportive care only. Only supportive care was given to 30 children who were not able to receive intravenous gammaglobulin because of shortcomings in intravenous gammaglobulin availability owing to a poor import during those years. Twenty-five patients were treated with intravenous gammaglobulin; they received intravenous gammaglobulin 0.4 g/kg/day for 5 consecutive days. Seventeen of the intravenous gammaglobulin group had received intravenous gammaglobulin within 10 days after the first symptoms, and eight of them had received intravenous gammaglobulin after the first 10 days. The average time elapsed for the symptoms to reach the maximum level was 6.9 (range 4—12) days in patients receiving intravenous gammaglobulin in the first 10 days, and it was significantly shorter than the time elapsed for the supportive care group (6.9 versus 8.8 days, respectively) (P < .05). Admission to the hospital after the first symptom, disability grade, time to improve in disability grade, the period of hospitalization, and mortality were not different in the intravenous gammaglobulin and supportive care groups (P > .05). Our suggestion for intravenous gammaglobulin treatment in Guillain-Barré syndrome is that if the patient has risk factors for respiratory insufficiency, then the treatment should be started. We more confidently carry out the follow-up of these patients after the results of this study. In conclusion, although it has been reported that intravenous gammaglobulin facilitates improvement in the disease and the decrease in mortality in children with Guillain-Barré syndrome, it has been mentioned in some studies that the intravenous gammaglobulin treatment was not better than supportive care, as in our study. However, further studies are essential to determine when intravenous gammaglobulin should be given to patients having which clinical and laboratory findings. (J Child Neurol 2006;21:972—974; DOI 10.2310/ 7010.2006.00214).

Since the 1999 US Food and Drug Administration (FDA) warning of renal failure/dysfunction associated with intravenous gammaglobulin (IVIg), there has been a movement towards developing safer, more convenient formulations. Until now, the scope of renal failure associated with IVIg, has not been well described. The FDA Adverse Event Reporting System (AERS) from 2004 through 2009 was examined for renal impairment reactions due to IVIg and associated demographic features, comorbidities and indications. Anaphylaxis cases associated with IVIg administration were used as a comparison group. There were 90 renal impairment cases associated with IVIg administration. Neuromuscular disorders (33/37%) and hematologic disorders (32/36%) were the predominant treatment indications. When reported anaphylaxis versus renal impairment due to IVIg was examined as a binary outcome in logistic regression modeling, renal impairment was predicted by sucrose presence, increasing age and non-hypogammaglobulinemic indications. Of the 34 hemodialysis cases, the excipient was known in 28 and all but 1 consisted of sucrose. IVIg containing sucrose was used in 33 of 48 nonhemodialysis cases. More hemodialysis cases also had diabetes mellitus. When the interval between renal impairment and the first IVIg infusion was determined, the renal impairment was reported by the second day in 43.3% of cases, and between 3 and 5 days in 41.7% of cases. Despite an evolution in clinical usage and formulations, renal impairment after IVIg administration continues to be reported. Sucrose as the excipient in IVIg plays a major role, but other factors are also important. These findings have implications in the management of patients treated with IVIg.

Guillain-Barre syndrome (GBS) is an eponym for a heterogeneous group of immune-mediated peripheral neuropathies. We describe a case of GBS in a patient who recieved intravenous streptokinase therapy for acute anterior myocardial infarction. Clinical symptoms are thought to result from streptokinase-antibody complex mediated damage to the local blood-nerve barrier. Patient was treated with 5-days course of intravenous gammaglobulin and his outcome was good.

ABSIRACT. Pietz J., Kiefel V., Sontheimer D., Kobialka B., Linderkamp O and Mueller-Eckhardt C. (Division of Neonatology, Children's Hospital, University of Heidelberg, and Institute of Clinical Immunology and Transfusion Medicine, University of Giessen, FRG). High-dose intravenous gammaglobulin for neonatal alloimmune thrombocytopenia in twins. Acta Paediatr Scand 80: 129, 1991. We report the successful treatment of neonatal alloimmune thrombocytopenia with repeated infusions of high-dose immunoglobulin G (400 mg/kg/d for 5 days) in twins. Platelet counts increased within 3 days from less than 20×109/1 to more than 70 × 109/1. The first twin survived without neurological or other sequelae. The second twin had probably developed intracranial hemorrhage (ICH) in utero. This infant developed long-term neurological sequelae with blindness, cerebral palsy and infantile spasms. Implications of the therapeutic approach and prevention of severe complications in pregnancies with known risk for neonatal alloimmune thrombocytopenia are discussed.

A 4-year-old boy with an atypical course of haemolytic uremic syndrome (HUS), who developed microangiopathic antiphospholipid-associated syndrome (MAPS) with signs of multiple organ failure during the course of his disease, is reported. Early and aggressive treatment with intravenous gammaglobulin, pulse methylprednisolone and plasmapheresis resulted in an excellent clinical recovery. Our patient showed a concomitant presence of multiple factors that could precipitate atypical HUS, including positive antiphospholipid antibodies, decreased level of factor H and positive anti-ADAMTS-13 antibodies. We suggest that, along with infections, autoimmune conditions or defined genetic abnormalities of complement regulatory genes, MAPS should be considered among the pathogenic mechanisms in patients with atypical HUS.

Recent reports have described cases of septicaemia caused by coagulase-negative staphylococci in preterm neonates, mainly due to the use of artificial intravenous devices. It was of interest to examine if intravenous immunoglobulin therapy, known to be effective in group B streptococcal infections of neonates, had a similar beneficial effect in coagulase-negative staphylococcal infections. Opsonophagocytosis of coagulase-negative staphylococci by normal polymorphonuclear leukocytes in the presence of cord blood serum supplemented with the commercial IgG preparation ‘Sandoglobulin’ was investigated, using luminol-dependent chemiluminescence. It was found that with two different coagulase-negative staphylococcal strains, Sandoglobulin had a concentration-dependent enhancing effect on the chemiluminescent response. This effect was demonstrated in the presence of native as well as inactivated cord blood serum and in the presence of sera from preterm infants (28-33 weeks). It is concluded that intravenous Sandoglobulin therapy may be effective in the treatment of preterm infants with severe coagulase-negative staphylococcal infections.

Objective - To investigate the effect of Rhesus anti-D immunoglobulin (anti-D) in patients with an autoimmune demyelinating neuropathy. Material and methods - Three patients with an autoimmune mediated neuropathy received 1000 IU anti-D weekly for 2 months. Results - Two patients worsened gradually during the treatment and 1 remained unchanged. Conclusion - Rhesus anti-D immunoglobulin has no beneficial effect in patients with autoimmune neuropathy.

Fetal alloimmune thrombocytopenia (FAITP) is a condition associated with significant infant morbidity and mortality. We report on the West of Scotland experience of 30 pregnancies complicated by FAITP over a 17-year period (1982-98). Management options included serial cordocentesis together with platelet transfusion, and maternal intravenous gammaglobulin (IVIgG) therapy. Of those pregnancies managed by serial cordocentesis all had poor outcomes. Weekly IVIgG was administered to the remaining pregnancies, all of which had a good outcome although four infants were thrombocytopenic at birth. None of these cases had previously been complicated by intracranial haemorrhage. In the milder end of the spectrum of FAITP we would suggest that IVIgG is an alternative treatment option.

While established therapies are successful in many patients with systemic lupus erythematosus (SLE), there is still a great need for more effective and safer treatments, particularly with respect to the long-term outcomes and quality of life. New data continues to emerge regarding unproven but widely employed therapies, such as intravenous gammaglobulin and plasmapheresis, while several immunosuppressive and immunomodulatory agents originally developed for use in other diseases are being evaluated for their potential in SLE, in particular, the agent mycophenolate mofetil that in one study showed promising results in the treatment of lupus nephritis. Appropriately, a large number of entirely new therapies for SLE are currently being tested in animal models and in human clinical trials. These include the hormonal agent dehydroepiandrosterone, which has gone through extensive clinical trials with positive results. A number of biological agents, such as monoclonal antibodies targeting B lymphocytes (rituximab), various “second signal” inhibitors (including CTLA4-Ig), and anti-IL10 have also been tested in humans, with varying results. The B-cell toleragen LJP394 is being evaluated for its ability to prevent nephritis flares. In addition, a range of “alternative” treatments including herbs and nutritional supplements are available to the general public. Thus, while the proper evaluation of new treatments for SLE remains a formidable challenge, the prospects are excellent for more successful treatments in the not too distant future.

Rheumatic carditis is a major manifestation of acute rheumatic fever. Conventional therapy includes the use of salicylates and steroids. To date, however, such therapy has not been proven to have a clear benefit in reducing valvar heart disease. We report the use of high-dose intravenous immunoglobulin in two chidlren with acute rheumatic carditis in whom we have been able to document the beneficial effect.

We report a case of complete fetal heart block in a 35-year-old Chinese woman known to be positive for anti-SSA/Ro and anti-SSB/La antibodies. She had fetal hydrops leading to intrauterine death in her first pregnancy Prophylactic intravenous immunoglobulin, given at 14 and 18 weeks' gestation, as well as oral dexamethasone, commenced at 24 weeks' gestation, allowed continuation of the pregnancy until 34 completed weeks of gestation. An external pacemaker was inserted in the baby on the first day of life. Two-and-a-half months later, a permanent pacemaker was inserted.