HYPERGAMMAGLOBULINqMIA, ANTIBODY DEFICIENCY, AUTOIMMUNE HqMOLYTIC ANqMIA, AND NEPHRITIS IN AN INFANT WITH A FAMILIAL LYMPHOPENIC IMMUNE DEFECT

Schaller, Jane G.; Davis, StarkeyD.; Ching, Yi-Chuan; Lagunoff, David; Williams, ChristopherP.S.; Wedgwood, RalphJ.

doi:10.1016/s0140-6736(66)92255-0

Cited by 51 publications

(17 citation statements)

References 13 publications

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“…Although patients CG (who developed chronic persistent hepatitis), AP and BB could be considered to have some degree of immune dysfunction (tables I, III), none of the present cases was suffering from any of the well-defined congenital or acquired immune deficiency syndromes which have been implicated in the devel opment of childhood AIH [19][20][21][22][23][24][25], and which were commonly seen in some of the other large series [4][5][6][7][8]. In retrospect, per haps full investigation of immune status should have been carried out in NR (who died) and in LM, since both had lifelong histories of chronic infections.…”

Section: Association With Other Disordersmentioning

confidence: 72%

Autoimmune Haemolysis in Childhood and Adolescence

et al. 1984

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The clinico-pathological features of 42 children with autoimmune haemolysis are described. Over 65 % of cases were seen before their 5th birthday. In this group males predominated by the ratio of 2.5:1, but in the older children both sexes were equally affected. The incidence decreased from 1 in 188 × 10³ in young males to 1 in 1,780 × 10³ in children over 10. Cases were classified serologically. Of particular note was the frequency of Donath-Landsteiner haemolysis which equalled that due to warm autoantibodies; together these groups made up 79% of the total cases. Most haemolytic episodes followed an acute infection. This was frequently mild and often involved the upper respiratory tract; in only 2 patients was haemolysis associated with underlying collagenosis. Typically there was a sudden onset of pallor and malaise; jaundice, splenomegaly and hepatomegaly were found in about half the subjects. Haemoglobinuria was characteristic of Donath-Landsteiner haemolysis. The illness was severe, with Hb levels falling below 6.0 g/dl in 28 patients. Prednisolone, blood transfusion and, where indicated, antibiotics were usually effective in treating the illness, with splenectomy reserved for cases where this treatment was unsatisfactory. In several individuals no treatment was required. Recovery was rapid, and complete recovery occurred in 83 % of patients, usually within 6 months. Although 2 patients died, a generally optimistic prognosis can be given, particularly in the absence of an underlying chronic disorder.

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Section: Association With Other Disordersmentioning

confidence: 72%

Autoimmune Haemolysis in Childhood and Adolescence

et al. 1984

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“…Lipton et al also predicted that T cell deficiency would be expected to limit the ability to respond to erythropoietic stress. However, Schaller et al (46) demonstrated that a patient with thymic alymphoplasia and autoimmune hemolytic anemia was able to increase erythropoiesis appropriately in response to severe anemia. If T cells actually are required for stimulation of BFU-E, the only way to account for these findings would be to propose that the small number of residual primitive precursors of T cells, which are unable to promote lymphoid differentiation (47,48), can promote normal erythropoiesis.…”

Section: Resultsmentioning

confidence: 99%

Human erythroid burst-forming units. Growth in vitro is dependent on monocytes, but not T lymphocytes.

Zuckerman¹

1981

J. Clin. Invest.

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“…SIgMID has been characterized as a rare primary immunodeficiency differentiated by a low serum IgM level, less than 2 SD or <10% of age adjusted normal controls or absolute levels of 10-20 mg/dL in infants and children [3]. The earliest recognized cases were described in children in 1966 [9,19]. More pediatric cases and the first adult cases were described in the late 1960s and were classified as dysgammaglobulinemia V [2,6,12,21].…”

Section: Discussionmentioning

confidence: 99%

“…In children, infectious agents have included Pneumocystis carinii [9], Giardia [10], Staphylococcus [10,11], Salmonella [12], Listeria monocytogenes [13], meningococcus [6,14,15], Pseudomonas [10,16], molluscum contagiosum [17], cytomegalovirus [18], and varicella [17]. These organisms account for recurrent infectious dermatitis, diarrhea, meningitis, upper and lower respiratory infections, sepsis, and in some cases, death.…”

Section: Introductionmentioning

confidence: 99%

Pediatric Selective Igm Immunodeficiency

Goldstein

Dunsky

et al. 2008

Chest

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Objective. Limited information exists on features of pediatric Selective IgM immunodeficiency (SIgMID). Previously published pediatric cases and 2 new cases are reviewed. Methods. English literature from PubMed and references from relevant articles were reviewed. Previously reported cases and 2 new cases from an allergy/immunology practice were analyzed. Results. Fortynine reported cases of SIgMID presented with respiratory infections (77.6%), gastrointestinal disease (16.3%), skin disease (12.2%), and meningitis (8.2%). Mean serum IgM level was 16.5 ± 13.8 mg/dL. Two patients were identified with SIgMID among 6300 active pediatric patients (0.03%) presenting with asthma, vasomotor rhinitis, and recurrent respiratory infections. In the 51 cases reported, none developed lymphoproliferative disease nor evolved into panhypogammaglobulinemia; four fatalities were reported. Conclusions. The prevalence of SIgMID in our pediatric population was 0.03%. In general, respiratory infections are the common comorbid conditions. Death and autoimmune disease are uncommon complications of pediatric SIgMID.

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HYPERGAMMAGLOBULINqMIA, ANTIBODY DEFICIENCY, AUTOIMMUNE HqMOLYTIC ANqMIA, AND NEPHRITIS IN AN INFANT WITH A FAMILIAL LYMPHOPENIC IMMUNE DEFECT

Cited by 51 publications

References 13 publications

Autoimmune Haemolysis in Childhood and Adolescence

Autoimmune Haemolysis in Childhood and Adolescence

Human erythroid burst-forming units. Growth in vitro is dependent on monocytes, but not T lymphocytes.

Pediatric Selective Igm Immunodeficiency

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