PURPOSE: Selective immunoglobulin A deficiency (IgAD) is the most common primary immunodeficiency disease. The aim of the present study is to analyze clinical manifestations and immunological characteristics of selective IgAD in children. METHODS: Twenty-one children aged 1-14 years with the serum IgA level of 2 standard deviation (2 SD) below normal for age who had recurrent infection were enrolled in this study at Uijeongbu St. Mary's Hospital of the Catholic University of Korea, between January 2006 and August 2007. Recurrent infection was defined as the presence of at least 6 febrile infection episodes per year before this study. Among IgA deficient patients, we excluded panhypogammaglobulinemic patients. RESULTS: The average frequency of admission was 2.2+/-2.0 per year. The most common causative disease at admission was lower respiratory infection (53.6%). Immunoglobulin G (IgG) subclass deficiency was corcurrent in 9 patients (42.9%): deficiency of IgG2 in 1, IgG3 in 5, IgG4 in 1 and IgG3+IgG4 in 2. Five patients (23.8%) had a decrease in CD3+ cells and 8 patients (38%) had a decrease in CD4+ cells. One patient progressed to common variable immunodeficiency (CVID). Serum IgA levels of 76.5% of the patients increased to 2 SD above normal for age after 15.9+/-4.5 months. CONCLUSION: The patients with IgAD in this study showed frequent respiratory and gastrointestinal infections and combined with other immunodeficiencies including IgG subclass deficiency. Our data suggest that it is necessary to examine the immunological status including IgA level in patients with frequent infections and that those with IgAD should be observed for the potential progression to CVID.
Aged ; Child ; Common Variable Immunodeficiency ; Humans ; IgA Deficiency ; Immunoglobulin A ; Immunoglobulin G ; Korea

PURPOSE: Although an increasing number of children with cancer survive as a result of more intense chemotherapy with advanced supportive care, they may be immunosuppressed because of the anticancer chemotherapy with radiotherapy and are susceptible to severe infections. We surveyed the pattern of immunoglobulin deficiency after chemotherapy in childhood acute leukemia with severe infections. METHODS: We reviewed the medical records and laboratory reports of 11 acute leukemia patients who developed immunoglobulin deficiency after chemotherapy at the Department of Pediatrics, Kyungpook National University Hospital in Daegu, Korea from January 1995 to September 2003. RESULTS: Among 11 acute leukemia patients, the median time interval from the diagnosis of leukemia to that of immunoglobulin deficiency was 21 (2~44) months. At the diagnosis of leukemia, 10 patients had normal levels of IgG, IgA, and IgM. At the diagnosis of immunoglobulin deficiency, 9 patients had low level of IgG (median, 339 mg/dL), IgA (median, 14.1 mg/dL), IgM (median, 24.4 mg/dL), and the other 2 patients had isolated IgG deficiency and isolated IgA deficiency, respectively. They were treated with antibiotics and high dose intravenous immunoglobulin G. CONCLUSION: Acquired immunoglobulin deficiency is one of the causes of frequent and serious infections which develops in childhood acute leukemia with chemotherapy. We suggest that periodic monitoring of immunoglobulin levels is important for early detection and treatment.
Anti-Bacterial Agents ; Child ; Daegu ; Diagnosis ; Drug Therapy* ; Gyeongsangbuk-do ; Humans ; IgA Deficiency ; IgG Deficiency ; Immunoglobulin A ; Immunoglobulin G ; Immunoglobulin M ; Immunoglobulins* ; Korea ; Leukemia* ; Medical Records ; Pediatrics ; Radiotherapy

OBJECTIVETo study the distribution pattern of TCM Syndrome type and its relationship with renal pathology in patients with IgA nephropathy.

METHODSApopting multicenter coordinated method, patients' TCM Syndrome type was differentiated according to their clinical manifestations, data concerning laboratory examination and renal pathology were collected to establish a database for analyzing the relationship between TCM Syndrome type and renal pathology in 286 patients.

RESULTSPatients of Pi-Fei Qi-deficiency type (type 1) and both Qi-Yin deficiency type (type 2) showed rather milder pathological changes, by Lee classification, most of them belonged to grade I-III (72.3%, 70.2%); patients of Gan-Shen Yin deficiency type (type 3) had severe pathological change and majority of them belonged to grade III-IV (84.6%); and the most severe pathological change was shown in patients of Pi-Shen Yang-deficiency type (type 4), and the Lee's grade IV-V was dominant (88.0%) in them. Syndrome type of patients was significantly correlated with their Lee's grade (r = 0.26, P < 0.01). Percentage of glomerular sclerosis in patients of type 4 was higher than that in patients of the other three types. Semi-quantitative scoring upon pathological changes showed that the total, glomerular, tubulo-interstitial and vascular scores were significantly higher in patients of type 4 than in those of the other three types; those scores were higher in patients of type 3 than in type 1; the total, glomerular and vascular scores were higher in patients of type 3 than in type 2; and scores in patients of type 1 and type 2 showed insignificant difference.

CONCLUSIONMulticentric prospective study proves that the TCM Syndrome typeof patients with IgA nephropathy is significantly correlated with the grade and severity of their renal pathological changes, thus, the TCM Syndrome typing shows definite referential importance to conclude the severity of renal pathological change in patients with IgA nephropathy.

Adolescent ; Adult ; Diagnosis, Differential ; Female ; Glomerulonephritis, IGA ; diagnosis ; pathology ; Humans ; Kidney ; pathology ; Male ; Medicine, Chinese Traditional ; Middle Aged ; Prospective Studies ; Yang Deficiency ; pathology ; Yin Deficiency ; pathology

A case of a clinical syndrome of selective hereditary complete IgA deficiency with partial T-lymphocyte inactivation is reported. A 14 year old boy was admitted with chjef complaint of persistent wide shallow ulcerated non-vesicular patches on the skin folds of whole body, chronic upper respiatory infections and gastointestinal upsets. General appearance showed us as if the undemourished or malabsorption status had been accompanied. The duration of illness was about 10 years under the incorrect or mistaken diagnosis and skin lesions have incompletely responded to the treatment. Peripheral T-lymphocyte determination was carried out by the method of Wybran to be tumed out active T-cell 14%, total T-cell 48%. Tuberculin test showed negative with 5 TU. Immunoglobulin values were respectively as follows: IgG 325 mg%, IgM 60 mg%, and IgA trace. And we diagnosed this case as selective IgA defiency with partial T-lymphocyte inactivation. We started the immunochemotherapy with tuberculo-protein complex, Tubercin-3 and antibictics for 2 months. After 2 months with above regimen. the skin lesions were markedly improved. T-cell count also retumed to the normal limit(active 35%, total 59%) and levels of TgG and IgM raised(IgG 617.5 mg%, IgM 75mg%), but IgA still showed trace amount, Tuberculin test tumed out positive.
Adolescent ; Dermatitis* ; Diagnosis ; Humans ; IgA Deficiency* ; Immunoglobulin A* ; Immunoglobulin G ; Immunoglobulin M ; Immunoglobulins ; Lymphocytes* ; Male ; Skin ; T-Lymphocytes ; Tuberculin Test ; Ulcer

PURPOSE: People who suffer from chronic or recurrent respiratory disease have immunoglobulin deficiency due to hypofunction of B cell. Among these immunoglobulin deficiency, it has been reported that only one or two subclass of IgG are deficient with normal quantity of IgG and IgA. Therefore it is necessary to measure IgG subclasses in chronic or recurrent respiratory infection for diagnosis. The purpose of this study is to measure IgG subclass in children with chronic respiratory disease and administration of RU 41.740 which was obtained from Klebsiella pneumonia, K2O strain(it has proven role in control of immunity) comparing the changes of immunoglobulin levels and IgG subclasses with the control group. So that better therapeutic approaches can be made for these diseases. Subjectsand METHODS: Thirty-six patients who visited to Konkuk university hospital due to chronic and recurrent coughing from January 1993 to June 1994, were selected for this research. Their age ranged from 2~13 year old and patients with evident history of allergy or tuberculosis were excluded. RU 41.740 with antibiotics were given to 31 subjects and 5 subjects were given placebo with antibiotics. For all subjects IgG, A, M, E, IgG1, G2, G3, G4 were measured. Immunoglobulins were remeasured from 15 subjects who received RU 41.740 and from 5 subjects who received placebo. Results : 1) Five subjects out of 36 were low or low-normal IgG group and all combined with IgG subclass deficiency, and all of low or low-normal IgG group had IgG3 deficiency. Eighteen subjects out of 31 normal IgG group accompanied IgG subclass deficiency and 12 subjects had IgG3 deficiency, which is most common IgG subclass deficiency. 2) Four subjects out of 36 were IgA deficiency group. Three subjects from 4 accompanied IgG subclass deficiency and had IgG4 subclass eficiency. 3) After the RU 41.740 administration, the levels of IgG and IgG subclasses increased significantly in study group. There were no significant change in IgG and IgG subclasses levels in placebo group CONCLUSIONS: In patients with chronic or recurrent respiratory disease, it is necessary to measure IgG subclass. These diseases tend to be chronic and recurrent and can be severely complicated causing bronchiectasis and incurable otitis media. Generally these diseases are not responsive to antibiotics. Therefore therapies should be directed to increasing immunological function for better results.
Anti-Bacterial Agents ; Bronchiectasis ; Child ; Cough* ; Diagnosis ; Humans ; Hypersensitivity ; IgA Deficiency ; Immunoglobulin A ; Immunoglobulin G* ; Immunoglobulins* ; Klebsiella ; Otitis Media ; Pneumonia ; Tuberculosis

BACKGROUND: Sinusitis is a common and frequently recurrent illness in children. Respiratory allergy has been recognized as a major factor that predisposes children to recurrent and chronic sinusitis. Another important causative factors of recurrent sinusitis in children is immunodeficiency diseases. Among them, humoral immunodeficient disease especially is associated with recurrent sinusitis. Most common immune defect in recurrent sinusitis is immunoglobulin deficiency. OBJECTIVES: The aim of this study is to obtain a quantitative data of serum immunoglobulins in children with recurrent sinusitis, to investigate a relationship between recurrent sinusitis and immunoglobulin deficiency. MATERIALS AND METHODS: 30 childrens were selected who had been diagnosed as recurrent sinusitis at Nowon Eulji hospital in 1996. The serum immunoglobulins were evaluated by Latex agglutination immunoassay and ELISA. RESULTS: The serum IgG was within normal limits and IgA deficiency appeared in 1 patient but serum IgM appeared higher than normal value over 3 years patients. The serum IgG subclass deficiency appeared in 3 patients for IgG(1), 7 patients for IgG(2), 14 patients for IgG(3), 10 patients for IgG(4). The combined serum IgG subclass deficiency appeared in 4 patients for IgG(2) and IgG(3), 1 patient for IgG(2) and IgG(4), 4 patient for IgG(3) and IgG(4), 1 patient for IgG(1) and IgG(2) and IgG(3). CONCLUSION: Immunoglobulin deficiency is approved to be closely associated with recurrent sinusitis in children.
Agglutination ; Child* ; Enzyme-Linked Immunosorbent Assay ; Humans ; Hypersensitivity ; IgA Deficiency ; Immunoassay ; Immunoglobulin G ; Immunoglobulin M ; Immunoglobulins* ; Latex ; Reference Values ; Sinusitis*

In most cases, acute diarrhea in childhood heals spontaneously, but it may become the form of chronic diarrhea in immunodeficient children and then cause weight loss, dehydration, malabsorption and malnutrition. The immunodeficient diseases associated with chronic diarrhea include severe combined immunodeficiency syndrome, common variable immunodeficiency, acquired immunodeficiency syndrome, agammaglobulinemia or selective IgA deficiency. IgA deficiency is the most common primary immunodeficiency. Because many IgA deficient individuals seem to have compensated for their deficiency with increased IgM production and various nonimmunologic factors, the incidence of gastrointestinal involvement is not prominent. Some of those with IgA deficiency and recurrent infections have been found to also have IgG subclass deficiency. IgA deficiency with IgG2 and IgG4 subclass deficiency have high susceptability to infection and chronic diarrhea. IgG subclass deficiency, when present, is more likely to be found in association with a partial IgA deficiency rather than complete IgA deficiency. We report a 3-month-old male with intractable diarrhea accompanied by IgA, IgG2, and IgG4 deficiency.
Acquired Immunodeficiency Syndrome ; Agammaglobulinemia ; Child ; Common Variable Immunodeficiency ; Dehydration ; Diarrhea* ; Humans ; IgA Deficiency ; Immunoglobulin A* ; Immunoglobulin G* ; Immunoglobulin M ; Incidence ; Infant ; Male ; Malnutrition ; Severe Combined Immunodeficiency ; Weight Loss

Disibling pansclerotic morphea, a severe form of generalized morphea, has a relentless clisabling course, producing marked contract,ion and joint deformity due to sclerosis of the skin and the deeper structures. We report herein an unusual case of morphea in a 25 year old male who had suffered from chronic recurrent ulceration with scar like sclerotic changes overlying limb folds, ankles, nape and abdomen since 4 years of age. The indurated lesions on the chest and abdomen were ivory colored, sclerotic guttae and plaques having a, violac ous border. At l4 years of age, the skin lesions were diagnostically ambiguous, wherea.s the immunologic results revealed a selective IgA deficiency with partial T lymphacyte inactivation. About 10 years later, the follow up visit showed this severe form of morphea over generalized area and disabling contractures of knees, elbows and alkles, but IgA value returned nearly to the nomal level.
Abdomen ; Adult ; Ankle ; Cicatrix ; Congenital Abnormalities ; Contracture ; Elbow ; Extremities ; Follow-Up Studies ; Humans ; IgA Deficiency ; Immunoglobulin A ; Joints ; Knee ; Male ; Scleroderma, Localized* ; Sclerosis ; Skin ; Thorax ; Ulcer

Selective IgA deficiency is one of the most common primary immunodeficiency. Some patients with IgA deficiency also have deficits in one or more immunoglobulin G subclasses. It has been estimated that up to 25% of patients with certain primary immunodeficiencies will develop tumors, primarily B-cell lymphomas during their lifetime. We hereby present 2 cases of malignant lymphomas, one diffuse large cell lymphoma and another mixed cellularity Hodgkin's disease, respectively, which developed in patients with selective IgA and IgG subclass deficiency.
Child* ; Hodgkin Disease ; Humans ; IgA Deficiency ; Immunoglobulin A* ; Immunoglobulin G* ; Lymphoma* ; Lymphoma, B-Cell ; Lymphoma, Large B-Cell, Diffuse ; Lymphoma, Non-Hodgkin

BACKGROUND: The association between type 1 diabetes and immunoglobulin A deficiency (IgA-D) has long been recognized in many populations. The aim of this study was to assess the prevalence of IgA-D in patients with type 1 diabetes mellitus all coming from a defined geographical area and to investigate the clinical features of these subjects. METHODS: The records of 150 consecutive patients with type 1 diabetes mellitus referred in a period of one year were analyzed. A detailed history was obtained for each patient. Information was collected concerning age, gender, time of onset of diabetes, and presence of other autoimmune diseases. RESULTS: Out of 150 patients with type 1 diabetes, eight (5.3%) had a diagnosis of IgA-D. There were one female and seven male; all these patients were diagnosed by screening: none of them had history of recurrent infections. Autoimmune thyroiditis was coexisting in five patients (62%). Although other associated autoimmune disorders were found in a number of patients, there was no different prevalence rate in IgA deficient patients. CONCLUSION: This study shows the prevalence of IgA-D in Sicilian patients with type 1 diabetes as 5.3% which is much higher than reported in other Italian studies. Moreover, our data show a high prevalence of IgA-D in male gender and describe thyroiditis as the most frequent autoimmune disease present in these patients. Finally, in our case report, IgA-D diagnosis always followed routine IgA measurement when case finding for celiac disease with no history of recurrent infections in each patient.
Autoimmune Diseases ; Celiac Disease ; Cross-Sectional Studies* ; Diabetes Mellitus, Type 1* ; Diagnosis ; Female ; Humans ; IgA Deficiency ; Immunoglobulin A* ; Male ; Mass Screening ; Prevalence ; Thyroid Gland ; Thyroiditis ; Thyroiditis, Autoimmune