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Article
Laparoscopic omental fixation technique versus open surgical placement of peritoneal dialysis catheters
Background: Continuous ambulatory peritoneal dialysis (CAPD) is an effective form of treatment for patients with end-stage renal disease. Open insertion of peritoneal dialysis (PD) cathet...
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Article
Isolated cutaneous response to granulocyte-monocyte colony stimulating factor in fatal idiopathic disseminated Bacillus-Calmette-Guerin infection
Severe disseminated Bacillus-Calmette-Guerin (BCG) infection is very rare and has been regarded as idiopathic when no immunodeficiency is present. This entity seems to be due to several new types of inherited...
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Article
Successful bone marrow transplantation in a case of Griscelli disease which presented in accelerated phase with neurological involvement
Griscelli disease (GD) is a rare disorder characterized by pigment dilution, immunodeficiency and occurrence of accelerated phase consisting of hemophagocytosis, pancytopenia and neurological manifestations. A...
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Article
Impaired IgG Antibody Production to Pneumococcal Polysaccharides in Patients with Ataxia–Telangiectasia
Various factors seem to be etiologic in the susceptibility to sinopulmonary infections in ataxia–telangiectasia (A-T) patients, i.e., low serum and salivary IgA, low serum IgG2, and even aspiration of saliva. S. ...
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Article
Development of systemic lupus erythematosus in a patient with selective complete C1q deficiency
A 7-year-old male with recurrent erythematous and desquamated skin lesions and respiratory infections was diagnosed as selective complete C1q deficiency following detailed studies of the complement system. Hi...
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Article
Hyperimmunoglobulin M syndrome associated with systemic amyloidosis type AA
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Chapter and Conference Paper
Clinical Variants of Ataxia-Telangiectasia
Although ataxia-telangiectasia (A-T) can be diagnosed on purely clinical grounds and often on inspection, a number of case reports have shown clinical variability that makes diagnosis difficult without laborat...
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Article
Complement component deficiencies and infection: C5, C8 and C3 deficiencies in three families
Three families are described with complement component deficiencies. In one family, five children had C5 deficiency; in a second family, two children had C8 deficiency and one child in a third family had C3 de...
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Chapter
Immune Dysfunctions in Ataxia-Telangiectasia
Ataxia-telangiectasia (AT) is an autosomal recessive immunodeficiency, characterized by progressive ataxia, oculocutaneous telangiectasia, recurrent sinopulmonary infections, absent or reduced serum IgA, low s...
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Article
44 THYMIC HUMORAL FACTOR (THF) THERAPY IN A PATIENT WITH DI GEORGE SYNDROME
A total of 55 doses of THF (1.5 mg/kg/day) was given to a 5.5 month old male with Di George Syndrome.Skin tests (for PHA and Candida) were negative,E rosettes were low (24%),blastogenic transformation response...
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Article
Epstein Barr Virus (EBV) Associated Antibody (Ab) Patterns in Ataxia-Telangiectasia (A-T)
Serum anti-EBV ab titers determined by immunofluorescent techniques in 27 patients (pts) (ages 3.5-20 yr) with A-T showed elevated anti-viral capsid antigen (ag) (VCA⩾1:320) and anti-early ag (EA⩾1:10) titers ...
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Article
Possible involvement of endogenous histamine in the myotropic effect of clonidine on the isolated rabbit aorta
Clonidine has a contractile effect in the isolated rabbit aorta which can be blocked by alpha-adrenergic antagonist, phentolamine. Histamine H1-receptor blocker, mepyramine, partly antagonizes its myotropic effec...
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Article
REDUCED CELLULAR IMMUNITY WITH NORMAL IMMUNOGLBULINS AND A DEFECTIVE ANTIBODY PRODUCTION IN TWINS
The primary immunodeficiency diseases represent themselves with a broad spectrum but sometimes reaching a diagnosis is difficult because of mild symptoms and normal tests. An example of this was a set of twins...
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Article
MEMREANE MARKERS IN ALL: A STUDY OFF 21 CASES
Twenty one cases of ALL (13 males, 8 females) were analysed for the presence of T and B membrane markers in blood or bone marrow prior to treatment. Their ages were between 2 and 16 years. E rosettes with SREC...