Case Of Immunoglobulin Research Articles

Combined nutcracker syndrome and glomerulonephritis in pediatric patients: a single-center retrospective case series.

Nutcracker syndrome (NCS) has been reported to coexist with various glomerulonephritis (GN). This study investigated clinical features of NCS combined with GN in a pediatric case series and the possible relationship between these two conditions. Clinical and pathologic findings of 15 children with NCS and biopsy-proven GN were analyzed. NCS was diagnosed with renal Doppler ultrasonography, abdominal computed tomography, and/or magnetic resonance imaging. Glomerular lesions were divided into two pathological categories: minor glomerular abnormalities (MGAs) and definite GN. Mean age of all patients was 11 ± 3.36 years and mean follow-up duration was 53.8 ± 29.3 months. Chief complaint was proteinuria with or without hematuria. During follow-up, five patients developed left kidney enlargement. Abnormal levels in immunological tests were revealed in 10 patients. Extrarenal symptoms including gonadal varicocele, splenic cyst, syncope, and anemia were found in seven patients. On kidney biopsy, seven patients had MGAs and eight children showed definite GN (one case of focal GN, one case of mesangial proliferative GN, one case of focal segmental glomerulosclerosis, two cases of immunoglobulin A [IgA] vasculitis nephritis, and three cases of IgA nephropathy). While the MGA group showed a higher proportion of isolated proteinuria and a lower estimated glomerular filtration rate (eGFR) at the last visit, there were no differences in age, clinical features of NCS, extrarenal symptoms, immunological tests, and eGFR decline rate between the two groups. NCS may be associated with the presence of various GN. The causal relationship between NCS and GN should be further investigated.

Pomegranate seed allergy in a child with multiple tree nut allergies

Pomegranate (Punica granatum) is a fruit-bearing plant of the Lythraceae family that consists of pulp surrounding fleshy seeds. Pomegranate juice and seeds are widely consumed, and rare cases of hypersensitivity reactions to pomegranate have been described elsewhere in the world. In this study, we report what we believe is the first case of immunoglobulin (Ig) E-mediated pomegranate seed allergy in a girl with multiple tree nut allergies in the United States.

Report of a Case of Immunoglobulin A Nephropathy in a Patient Without a Family History of Kidney Disease

Report of a Case of Immunoglobulin A Nephropathy in a Patient Without a Family History of Kidney Disease

Immunoglobulin M (IgM) multiple myeloma versus Waldenstr\xf6m macroglobulinemia: diagnostic challenges and therapeutic options: two case reports

BackgroundImmunoglobulin M multiple myeloma and Waldenström macroglobulinemia are two different hematological diseases with the common finding of an immunoglobulin M monoclonal gammopathy of unknown significance. However, clinical characteristics of the two entities can overlap.Case presentationIn this report, we describe two cases of immunoglobulin M neoplasm with the same histological bone marrow presentation but with different clinical behavior, cytogenetics, and biological assessment. On the basis of comprehensive diagnostic workup, these patients were considered to have different diseases and treated accordingly with different approaches. Patient 1 (Caucasian man) presented with increased serum protein and immunoglobulin M (7665 mg/L) with an M-spike electrophoresis of 4600 mg/L. His bone marrow biopsy revealed a small-cell immunoglobulin M multiple myeloma. The result of testing for the MYD88 L265P mutation was negative, while fluorescence in situ hybridization analysis showed translocation t(11,14). A diagnosis of immunoglobulin M-κ multiple myeloma was made. Patient 1 was a candidate for bortezomib plus thalidomide and dexamethasone, followed by autologous stem cell transplant consolidation. Patient 2 (Caucasian man) showed an M-spike by protein electrophoresis (300 mg/L, 4.9%), with serum immunoglobulin M level of 327 mg/L. His bone marrow biopsy revealed immunoglobulin M-κ multiple myeloma. Computed tomography showed many enlarged lymph nodes and splenomegaly. Patient 2’s clinical features were suggestive of Waldenström macroglobulinemia, in contrast to the bone marrow biopsy results. The result of testing for the MYD88 L265P mutation was positive. Patient 2 was diagnosed with Waldenström macroglobulinemia and received rituximab, cyclophosphamide, and dexamethasone.ConclusionsA correct differential diagnosis between immunoglobulin M multiple myeloma and Waldenström macroglobulinemia is a critical point in the setting of a new immunoglobulin M monoclonal gammopathy onset. These patients should undergo a complete diagnostic workup with pathological, radiological, and serological examinations to establish the diagnosis and plan the most appropriate treatment in order to improve the prognosis.

Development of a DNA-Based Detection Method for Cocos Nucifera Using TaqMan™ Real-Time PCR.

So far, only a few cases of immunoglobulin E (IgE)-mediated coconut allergies have been described in the literature. Due to a growing consumption of coconut-containing foods in occidental countries, the number of coconut allergies may also increase. As there is no causative immunotherapy in clinical routine, appropriate food labelling is particularly important, also with regard to cross-contamination, to prevent serious health consequences. The purpose of this study was to develop a DNA-based detection method for coconut (Cocos nucifera). Initially, three sets of coconut-specific primers were designed and tested. A TaqMan™ probe was then developed to identify and quantify coconut by real-time PCR assay. With 27 other plant and animal species, the specificity of the primer/probe system was tested and cross reactivity was excluded. In a dilution series, a limit of detection of 1 pg/µL was determined. Thus, the developed real-time PCR assay is a suitable method to detect coconut in food.

Clinical case of immunoglobulin G application with сhemo-­resistant tuberculosis in combination with HIV-infection with severe immunosuppression

Chemo-resistant tuberculosis in combination with HIV infection is one of the most difficult problems of modern phthisiology. Of particular interest is the contingent of patients with MDR TB/HIV with a level of CD 4 in blood plasma below 50 cells/µl, indicating the progression of HIV, and the development of severe immunosuppression in the patient’s body. Such patients are more likely to have severe, generalized forms with multiple organ lesions, extra-pulmonary localizations tuberculosis (TB). We represent a clinical case of a patient V., 27 years old. Clinical diagnosis: В-20 HIV-infection IV clinical stage. A19.0 MDR TB (24.07.2015), both lungs (disseminated) desctuction + (infiltrative phase) tuberculosis of the intrathoracic and intraabdominal lymph nodes, intestinal tuberculosis; mbt + mg + rif + m + c + resist 1 + (HRES) resist 2 + (Et) hist 0 cat 4 (new case) coh 2 (2015). Complications: respiratory failure, type 1. Secondary anemia, severe course. Cachexia. Severe immunosuppression. Polyorganic insufficiency. Concomitant diseases: еxfoliative dermatitis. Thrombocytopenic purpura. Oropharyngeal candidiasis. Primary hypothyroidism, moderate, decompensation. Toxic Hepatitis. Stage 2 of chronic renal disease. Encephalopathy. Neuroretinitis of the viral genesis (CMV, Toxo, Rub). Genital herpes. Amenorrhea. The patient received with the standard treatment the intravenous immunoglobulin G, as a pathogenic effect, at a rate of 4 ml/kg intravenously in two steps. It was introduced slowly (30 drops/ minute) and after 5 pm. Conclusions. As a result of the use of the intravenous immunoglobulin (Ig G) in combination with the standard regimens of second-line anti-TB drugs and antiretroviral therapy, improved clinical features and laboratory data and reduced manifestations of the immune reconstitution inflammatory syndrome.

IgA Vasculitis in Middle Aged Man Followed by Influenza Virus Infection: A Case Report

Introduction: Immunoglobulin A vasculitis (formerly known as Henoch Schonlein Purpura) is the most common pediatric vasculitis, which occurs typically at the age of 3-15 years. Mononucleosis, group A Streptococcus, Campylobacter and Mycoplasma are some of the common infectious causes of immunoglobulin A vasculitis. Immunoglobulin A vasculitis is a clinically diagnosed disease. Most common clinical features include nonthrombocytopenic nonpruritic palpable purpura, gastrointestinal involvement, arthritis or arthralgia and renal involvement. Biopsy of skin and/or gastrointestinal lesions con rm the diagnosis, although this is not necessary. Immunoglobulin A vasculitis is a self-limiting disease, which resolves spontaneously. Steroids can be used for the treatment of moderate to severe disease and for the prevention of renal complications. Prognosis relies upon various factors among which involvement of kidneys dictates poor prognosis and requires close follow up. Case Presentation: We are presenting a case of immunoglobulin A vasculitis in a 39-year-old German male following in uenza virus infection. The infectious agent that cause immunoglobulin A vasculitis in our patient was In uenza A virus. Biopsy of the skin lesion con rmed the diagnosis of immunoglobulin A vasculitis in our patient. The patient responded to steroids and his skin and GI ndings resolved. Conclusion: Our case report adds to the literature of medicine by describing in uenza virus as a cause of immunoglobulin A vasculitis in young adult patients. Considering immunoglobulin A vasculitis after in uenza and outside of the typical age of 3-15 years, can help to make earlier diagnosis and prevent complications.

A case of immunoglobulin M nephropathy with worsened proteinuria associated with type IV allergic reaction to hydroxypropyl cellulose

症例は7 歳女児。IgM 腎症に対しプレドニゾロン(prednisolone: PSL)内服を開始したが，尿蛋白は増加傾向となり治療前尿蛋白Cr 比1.1 g/gCr から最大4.3 g/gCr まで増悪した。血清Alb も低下したため，PSL を漸減し計8 週間の治療後に無効と判断し中止した。PSL 中止後，速やかに治療前の尿蛋白量まで回復したためPSLによる薬剤性腎障害と診断した。薬剤リンパ球刺激試験では使用したプレドニン錠® で陽性だったがメドロール錠® では陰性であり，プレドニン錠® のみに含まれる添加物であるヒドロキシプロピルセルロース(hydroxypropyl cellulose: HPC)で陽性の結果を得たためHPC が原因物質と判断した。PSL 開始後に尿蛋白が増加した場合には，治療無効と判断する前にステロイド剤自体の薬剤性腎障害も考慮するべきで，他の薬剤選択のためにも添加物の評価も行う必要がある。今回原因物質と同定したHPC は多くの薬剤に含まれているため留意すべき添加物である。

Modern classification, progression factors, treatment and outcomes of primary mesangial proliferative glomerulonephritis

The choice of treatment of mesangial proliferative glomerulonephritis (post-infection, immunoglobulin A, G and M nephritis) is performed taking into account the remission achievement, slowing of progression and reduction of the risk of recurrences of glomerulonephritis. The efficiency of etiologic factor removing is debatable: glomerulonephritis associated with infections usually resolves after their elimination; individual patients achieve immunoglobulin A nephritis remission with persistent antimicrobial treatment of focal infection, but surgical removal of the focus (tonsillectomy) does not affect the long-term prognosis, therefore it is not recommended. Treatment of immunoglobulin A nephritis with oral prednisone for up to 4 months, sometimes in combination with cyclophosphamide (cyclophosphane), reduces the likelihood of its relapse. At low risk of progression of immunoglobulin A nephritis with proteinuria less than 1 g/day, long-term therapy with angiotensin-converting enzyme inhibitors or angiotensin receptor blockers is indicated with administration of maximum tolerated doses for proteinuria more than 1 g/day. Also fish oil 3 g/day is administered for up to 2 years. If proteinuria more than 1 g/day persists for 3-6 months, corticosteroids are recommended for 6 months. With mild renal dysfunction, corticosteroids are prescribed orally or in pulse-therapy with high doses intravenously and maintenance therapy with low doses orally. Immunosuppressive therapy - cyclophosphamide, cyclosporine, azathioprine, mycophenolate mofetil - in combination with corticosteroids is indicated in rapid loss of renal function or massive/moderate proteinuria. In minimal proteinuria immunosuppressive therapy is considered to be unreasonable. Use of intravenous immunoglobulin for immunoglobulin A nephritis from the point of view of lesser toxic effect is possible only as the induction therapy. Currently, there are no clinical recommendations for the treatment of immunoglobulin M nephritis, in case of nephrotic syndrome, corticosteroids are the drugs of the 1st line. There are isolated studies of the use of cyclophosphamide, mycophenolate mofetil, and cyclosporine, with the achievement of remission in frequent relapses of nephrotic syndrome or steroid drug resistance. The cases of immunoglobulin M nephritis treates with retuximab with a positive effect are described. The effectiveness of immunoglobulin G nephritis treatment is less studied, the choice of treatment is similar to that of immunoglobulin A and M nephritis.

Case of immunoglobulin G4‐related skin disease: Possible immunoglobulin G4‐related skin disease cases in cutaneous pseudolymphoma only by immunohistochemical analysis

Immunoglobulin G4-related disease (IgG4-RD) is a newly recognized disease characterized by elevated serum IgG4 levels, tissue infiltration rich in IgG4(+) plasma cells. We report on a case which was first considered as pseudolymphoma from the histopathological analysis, but finally diagnosed as IgG4-related skin lesions. As the morphological features of cutaneous involvement of IgG4-RD are consistent with those of cutaneous pseudolymphoma, we tried immunostaining past potential cases of IgG4-RD. Thirty-two skin specimens (15 men and 17 women; mean age, 53 years) diagnosed as having pseudolymphoma were retrieved from the archives to conduct hematoxylin-eosin, IgG and IgG4 staining. Out of the 32 cases of cutaneous pseudolymphoma, germinal center formation was seen in 22 cases, and moderate-severe fibrosis was seen in seven cases. Eleven cases showed more than 10 IgG4(+) plasma cell infiltration/high-power field, and among these 11 cases, seven cases (22%) showed A ratio of IgG4(+)/IgG(+) cells of more than 40%. Thus, out of the 32 cases of cutaneous pseudolymphomas, two cases (6.3%) satisfied IgG4-RD histopathological diagnostic criteria. As clinical presentations and histopathological features of skin involvement of IgG4-RD are analogous to cutaneous B-cell pseudolymphoma, careful identification is required through systemic examination, serum IgG4 measurement and other means.

A case of immunoglobulin A nephropathy treated successfully with tonsillectomy and steroid pulse therapy 20years after onset.

A 46-year-old male was found to have proteinuria on a routine medical examination in 1985 at the age of 22 years and was diagnosed with immunoglobulin A (IgA) nephropathy by renal biopsy. He regularly visited a hospital, but 3 years later made the decision to stop. In 2000, his serum creatinine level was 1.3 mg/dl. His renal function then deteriorated, with persistent proteinuria and hematuria, following which he visited our hospital in December 2008. A further renal biopsy was performed. Active and chronic IgA nephropathy was confirmed histologically, with sclerosing lesions also being found. He was treated with three courses of steroid pulse therapy in February and tonsillectomy in April 2009. During the follow-up period at the outpatient clinic, results for proteinuria and hematuria were negative, suggesting that progression of renal impairment had been prevented. The efficacy of tonsillectomy plus steroid pulse therapy for early IgA nephropathy has been demonstrated, and this treatment was effective in our patient 20 years after the onset of the disease.

A Case of Immunoglobulin A Nephropathy in a Patient with Kimura's Disease

Kimura's disease is an angiolymphoid-proliferative disorder that manifests with benign subcutaneous swelling predominantly in the head and the neck. Kidney involvement, including proteinuria, occurs in 12- 16% of patients with the disease, and 60-78% of such cases is nephrotic syndrome. Reported etiologies of nephrotic syndrome in Kimura's disease include membranous glomerulonephritis, mesangial proliferative glomerulonephritis, minimal-change disease, focal segmental glomerulosclerosis, diffuse proliferative glome- rulonephritis and immunoglobulin A (IgA) nephropathy. There have been only two case reports of IgA nephr- opathy in Kimura's disease, in 1998. In this report, we present a third case of IgA nephropathy associated with Kimura's disease.

直腸癌を併存したIgG4関連リンパ節症の1例

直腸癌と術前診断し手術を施行したが，小腸間膜リンパ節にIgG4関連リンパ節症を併存していた1例を経験したので報告する．症例は69歳，女性．下血を主訴に近医を受診し，精査にて直腸癌の診断で当院紹介となった．血液検査では，軽度の炎症反応，IgEの上昇，腫瘍マーカーの上昇（CEA 7.1ng/ml，CA19-9 69U/ml）を認めた．CT検査で小腸間膜に数個の結節を認め，腹膜播種の可能性も考え，試験開腹し小腸間膜の結節を迅速病理に提出したところ，IgG4関連リンパ節症の診断であった．低位前方切除術と小腸間膜リンパ節切除術を施行した．術後採血で血清IgG4の上昇（146mg/dl）を認めた．小腸間膜リンパ節の病理組織学的所見では，直腸癌を併存したIgG4関連リンパ節症が最も考えられた．術後はステロイド内服を開始し経過良好である．若干の文献的考察を加えて報告する．

Spontaneous Remission of IgA Nephropathy Associated With Resolution of Hepatitis A

Although most cases of immunoglobulin A (IgA) nephropathy are idiopathic, several diseases are associated with IgA nephropathy. Of these, chronic liver disease resulting from hepatitis B or C virus infection has been reported as a secondary cause of IgA nephropathy. Recently, hepatitis A virus (HAV)-associated kidney disease has received attention because acute kidney injury can occur as a complication of HAV infection, generally caused by acute tubular necrosis or interstitial nephritis. However, unlike IgA nephropathy related to hepatitis B or C, HAV-associated IgA nephropathy is extremely rare and long-term outcomes have not been reported yet. We describe a case of spontaneous remission of IgA nephropathy associated with serologically documented HAV infection. The patient presented with microhematuria and moderate proteinuria, but acute kidney injury did not occur during active hepatic injury. Kidney biopsy specimens clearly showed mesangial IgA deposits with intact tubules and interstitium. Serum liver enzyme levels returned to reference values 1 month after the onset of acute hepatitis, but urinary protein excretion remained increased. Approximately 1 year later, urinary abnormalities were resolved and a second biopsy showed no mesangial IgA deposits. These findings suggest that IgA nephropathy can transiently accompany HAV infection, but may not progress to chronic glomerulonephritis after recovery from HAV.

The compared analysis of Multiple Myeloma of Uygur and Han people in Urumqi Region

Objective To analyze contrastly the typical features of Multiple Myeloma of Uygur and Han people in Urumqi region in Xin Jiang.Methods We have detected the serum of 60 cases by serum protein electrophoresis,immunofixation electrophoresis,Bence Jones protein electrophoresis,and flow cytometry(FCM).Results Monoclone protein peak was found in 31 out of 60 cases tested (18 cases were Uygur people and 13 cases were Han people),it located mainly γ border.The cases of Immunoglobulin G,Immunoglobulin A,Immunoglobulin M,free light chain was found in Uygur people and Han people were ( 8,16 ),( 6,9 ),( 1,5 ),( 11,4 ),1 respectively.Bence Jones protein electrophoresis detected 17 cases were positive ( 11 cases were Uygur and 6 cases were Han people).Compared with the Multiple Myeloma of Han people,the percentage of the CD3,CD4,Bcell,CD4/CD8 were reduced(P ＜0.05 )and the percentage of the CD8 and NK increased in male Uygur patients.While all parameters except CD8 cells which was reduced in the female Multiple Myeloma of Uygur,and the percentage of the CD8 increased obviously ( P ＜ 0.05 ).Conclusion It was found that characteristics of Multiple Myeloma in Urumqi,including nationality,category,and immunophenotype. Key words: Multiple Myeloma; Immunofixation electrophoresis; Immunophenotype; Flow cytometry

Severe Anaphylaxis after Albumin Infusion in a Patient with Ahaptoglobinemia

WE report a patient who developed severe anaphylactic shock and life-threatening ventricular fibrillation immediately after starting infusion of 5% human plasma protein fractions (Albuminar-5%; ZLB Behring, Tokyo, Japan) during general anesthesia. He was diagnosed postoperatively with ahaptoglobinemia associated with haptoglobin gene deletion. We postulated that this event may have occurred as a result of allergic reaction to a precipitating antibody to haptoglobin associated with use of plasma protein fractions.

Recurrent and De Novo Glomerular Immune-Complex Deposits in Renal Transplant Biopsies

Recurrent and de novo glomerulonephritis is an important cause of renal allograft failure, but estimates of its prevalence vary widely. One reason for such variability is the inconsistency with which electron microscopy and immunofluorescence are used in assessing renal allograft biopsies. To determine the prevalence of immune-complex deposits in all renal allograft biopsies performed during a 1-year period and to correlate their presence with clinical data. Our center accessioned a total of 118 renal allograft biopsies during 1 year from 88 patients. All biopsies were examined by both electron microscopy and immunofluorescence in addition to conventional light microscopy. Patient and donor characteristics were obtained as well as follow-up data for a minimum of 26 months after the index biopsy. Eight cases of immunoglobulin (Ig) A nephropathy were found (recurrent in 7 and de novo in 1). There were 9 instances of what we designate "IgM-positive immune deposits" without specific features of a recognized glomerulonephritis. To the best of our knowledge, the latter has not hitherto been described and may be part of a heterogeneous group of glomerulopathies. Other unexpected findings included de novo fibrillary glomerulonephritis and de novo membranous glomerulonephritis, the latter occurring at 3 months after engraftment. A high proportion (19.5%) of unselected renal allograft biopsies show immune-complex deposits both with and without a recognized glomerulopathy. These require both electron microscopy and immunofluorescence for detection. IgM-positive deposits of uncertain etiology are relatively frequent.

A case of immunoglobulin A nephropathy with a long history (32 years) in the relatively poor prognosis group

We report herein an immunoglobulin A nephropathy patient with a long history of 32 years in the relatively poor prognosis group according to pathological findings. In general, such cases in the relatively poor prognosis group develop end-stage renal failure, and undergo dialysis within 5 to 20 years. However, the period from the appearance of proteinuria to hemodialysis was very long in this patient. His mean blood pressure remained at approximately 100 mm Hg with the administration of some antihypertensive agent. The genotype of the angiotensin converting enzyme was II. Although normal renal function continued for the duration, he had severe acute tonsillitis at 45 and 49 years of age. After these infections, proteinuria and/or hematuria were exacerbated and he gradually progressed to end-stage renal failure. The good prognosis in this patient might be attributable to good control of blood pressure, whereas the decline in renal function might be attributable to severe recurrent upper respiratory tract infection. It seems that control of blood pressure and prevention of recurrent upper respiratory tract infection are important factors in the prognosis of patients with immunoglobulin A nephropathy. Although the association between angiotensin converting enzyme genotype and prognosis of immunoglobulin A nephropathy is controversial, it is postulated that angiotensin converting enzyme genotype II might be correlated with the good prognosis in this case.

Recurrence of immunoglobulin A-κ crystalline deposition disease after kidney transplantation

Cases of immunoglobulin A heavy chain and kappa light chain deposition disease are rare and their clinical presentations vary. We report one patient with histopathologic and clinical findings of a microangiopathic glomerulonephritis due to immunoglobulin A-κ deposition. Ultrastructural studies revealed highly ordered deposits in the capillary lumen, mesangium, and basement membrane. The disease recurred at 2.5 years after a cadaveric kidney transplantation. Pulse steroid therapy was repeatedly effective in retarding further progression of renal deterioration in this patient.

A case of immunoglobulin G conjugated with lactate dehydrogenase, producing both loss of enzyme activity and an abnormal isoenzyme pattern.

We present a patient with an inhibitor of lactate dehydrogenase (LD; EC 1.1.1.27) in serum, who had pericarditis and myocardial hypertrophy. The patient's LD activity in serum was low and the LD isoenzyme pattern was abnormal. The relative molecular mass of this abnormal LD-IgG complex was approximately 490 000. The patient's IgG reacted with all five LD isoenzymes.