Air way foreign body aspiration with normal chest X-ray and CT scan

Air way foreign body Aspiration [AFA] is not an unusual event in young children. Unfortunately a chocking episode is not always present or remembered by parents and normal imaging doesn't rule out foreign body aspiration. We report a 9 month old male with laryngeal foreign body aspiration, normal CXR and chest CT, misdiagnosed as respiratory infection and bronchial asthma

Autosomal recessive chronic granulomatous disease, IgA deficiency and refractory autoimmune thrombocytopenia responding to anti-CD20 monoclonal antibody

Immunodeficiency and autoimmune disease may occur concomitantly in the same individual. Some of the immunodeficiency syndromes, especially humoral defects are associated with autoimmune disorders. Hematological manifestations such as thrombocytopenia and hemolytic anemia are the most common presentations. Persistent antigen stimulation due to an inherent defect in the ability of the immune system to eradicate pathogens is the primary cause leading to autoimmunity in patients with primary immunodeficiency states. We describe a 10 year old Iranian girl with chronic granulomatous disease -the autosomal recessive type with mutation of NCF1 gene P47- associated with selective IgA deficiency, refractory immune thrombocytopenia that showed an excellent response to Rituximab [Anti-CD20 monoclonal antibody]. Patients with primary immunodeficiencies may have variable autoimmune manifestations. So for early detection and appropriate treatment, autoimmune diseases should always be suspected in such patients

Mucocutaneous candidiasis or psoriasis? A case report

Pustular psoriasis is a rare form of psoriasis in childhood. The prevalence of psoriasis in various parts of the world varies from 0.1% to 3% and the most frequently observed variant is the plaque type, followed by the guttate psoriasis. A 4-year-old boy with a history of repeated self-limited arthritis, onycholysis, recurrent erythematous skin, diaper rash, fever and pustular lesions, had several hospital admissions with no benefits. After a 2-year delay in the diagnosis, he was treated as a case of pustular psoriasis which was shown by skin biopsy

Systemic and local reactions of bee venom immunotherapy in Iran

Severe allergic reactions during specific immunotherapy may occur in the treatment of hymenoptera sting allergy. The objective of the present study was to examine the characteristics of allergic reactions during specific immunotherapy in patients with allergy towards hymenoptera venom in the Iranian population. A prospective study was performed using the clinical reports of 27 patients with anaphylaxis to bee venom [Apis melifera, Geupes vespula and Geupes Polites]. Ten patients treated with Cluster protocol during 2002 and 2006 After diagnosis of hymenoptera sting allergy according to history and intradermal tests, the patient were treated with Cluster protocol immunotherapy. The protocol lasted 6 weeks with an increase in the concentration of venom from 0.01? g/ml to 100 ? g/ml. None of the patient received premedication. All patients with hymenoptera venom allergy received 120 injections. Anaphylactic reactions were classified according to the Mueller-classification. The frequencies of systemic reactions during Cluster protocol were 8.33% and 5% for yellow jacket and honey bee venom respectively. No patient experienced severe systemic reaction. Cluster protocol for hymenoptera immunotherapy is a reliable method for the treatment of anaphylactic reactions to bee venom. It is safe with low cost and do not need hospitalization

Autoimmune lymphoproliferative syndrome; a case report

Autoimmune lymphoproliferative syndrome is a disorder of lymphoid system regulation characterized by chronic splenomegaly, lymphadenopathy and autoimmune phenomena especially immune-mediated cytopenias. The hallmark of the disease is the presence in peripheral blood and lymphoid tissue of increased numbers of a normally rare T lymphocyte subset, usually referred to as [double-negative] T cells. Here the authors report a 16-year-old boy when he was first hospitalized for diffuse petechiae, purpura and epistaxis at 9 years of age.One year later,he was readmitted for high fever and recurring cytopenia. On examination several enlarged, nontender lymph nodes involving cervical and submandibular areas and a huge spleen were detected.Lymph node biopsy was performed two times. According to flowcytometry of peripheral blood and immunophenotyping of lymph node tissues which revealed increased numbers of CD3[+]CD4[-]CD8[-] T-lymphocytes autoimmune lymphoproliferative syndrome was suggested for him. Autoimmune lymphoproliferative syndrome should be considered in differential diagnosis of any patient with unexplained Coomb's positive cytopenias, hypergammaglobulinemia, generalized lymphadenopathy and splenomegaly. The confirmation of the diagnosis should be based upon genetic analysis and detection of the affected genes involved in fas pathway

Recurrent infections and bilateral uveitis in a patient with CD8 deficiency

CD8 deficiency is a rare primary immunodeficiency with low or absent peripheral CD8 cells which results from TAP deficiency, Zap 70 deficiency and CD8 alpha gene mutation. We report a 14 year old female who presented with a history of recurrent pneumonia, bronchiectasis, otitis, severe varicella, herpetic lesions of mouth, bilateral uveitis, and cataract formation since the age of 8 years. She had growth failure, a huge spleen and moderate clubbing. In immunologic workup, humoral and phagocytic systems were normal. DTH response to candida, PPD and DT were negative but LTT response to PHA mitogen was normal. HLA typing showed normal class I expression. Flowcytometry of peripheral blood showed CD8: 0 to 2% [absolute count, 0-60 cells/mm3] with increased CD4/CD8 ratio on several occasions. Diagnosis of this patient cannot be HLA class I deficiency [TAP1 or TAP2], because class I expression had been normal. It is possible to be Zap -70 deficiency or CD8 alpha gene mutation. Bilateral uveitis in our patient was a unique presentation which might have resulted because of immune dysregulation in CID

Descriptive study of 226 patients with allergic rhinitis and asthma

The prevalence of allergic diseases such as asthma and allergic rhinitis is high in general population and aeroallergens are the most common allergens that cause air way inflammation. The aim of this study was an evaluation of clinical and laboratory findings in allergic patients and identification of the most common aeroallergen in these patients. A cross–sectional retrospective study was conducted on 226 allergic patients who were referred to allergic clinic of Karaj city, and skin prick test response to aeroallergens were studied. The most common risk factors in these patients were total IgE more than 100 IU/ml and a positive family history of atopy. Skin prick testing results showed that the most common aeroallergens were: herbacee II [62%], sycamore [57%], chenopodium [53%], tree mix [50%], herbacee III [47%], grass [43%], ash [40%], herbacee I [37%], cedar [27%], cockroach [25%], and mite D.P [19%], D.F [18%]. High prevalence of skin reactivity to weeds [chenopodium and herbacee] and sycamore indicates variation in the prevalence of aeroallergen reactivity in different regions with different climates

Neutropenia in patients with primary antibody deficiency disorders

Neutropenia is characterized by decrease in the absolute number of circulating neutrophils and an increase susceptibility to infections. The current study was performed in order to explain the clinical and laboratory findings of patients with antibody deficiency disorders associated neutropenia. The patients' records of 19 neutropenic cases out of 207 patients with antibody deficiencies, who had been referred to Children's Medical Center and enrolled in Iranian primary immunodeficiency registry, were reviewed. Nineteen cases [14 male and 5 female], with a mean age of 10.7 +/- 5.7 years, were associated with neutropenia [9.2%]. The disorders with associated neutropenia were Hyper IgM syndromes [3 of 8], Common variable immunodeficiency [13 of 109], and X-linked agammaglobulinemia [3 of 45]. The median age for the onset of disease and diagnosis age were 15 months [1-134] and 3.8 years [6 months-13 years], respectively. The most common infections during the course of illness were pneumonia [13 cases], diarrhea [12 cases], oral candidiasis [9 cases], otitis media [6 cases], sinusitis [6 cases], cutaneous infections [5 cases], and abscess [5 cases]. Other less frequent infections were: conjunctivitis, oral ulcers, meningitis, and osteomyelitis. Three neutropenic patients died because of recurrent infections. Neutropenia may occur in any of the primary immunodeficiency disorders. Persistent or severe infections always pose a supposition, which deserves further evaluation for detecting an underlying immune deficiency syndrome and neutropenia, since a delay in diagnosis may result in a serious organ damage or even death of the patient

Report of six cases of Chediak-Higashi syndrome with regard to clinical and laboratory findings

Chediak Higashi Syndrome [CHS] is a rare, primary immunodeficiency disorder with an autosomal recessive [AR] inheritance and characterized by recurrent infection, partial occulocutaneous albinism and an accelerated phase. In this report we describe clinical and laboratory findings from 6 CHS patients. Clinical and laboratory information of six patients who were referred to our center during the last 20 years [from 1983 - 2003] were reviewed. Onset age of disease was between 3 months to 10 years. All patients had history of consanguineous parents and two patients were siblings. All patients had oculocutaneous albinism, nystagmus, recurrent infections which included upper and lower respiratory tract [U and LRT] infections, stomatitis, thrush, and skin abscesses and hepatitis. In laboratory findings, all patients had neutropenia and normal immunoglobulins and normal CD3, CD4, and CD8, CD19 Lymphocyte by flowcytometry and three of the four patients had chemotatic defect. Five patients certainly had giant granule in bone marrow neutrophil and in one patient it was equiovocal. Three patients had an accelerated phase, and for one patient bone marrow transplantation was done that was tolerated well and had been well after 7 years. We emphasize the need for early diagnosis on basis of characteristic facies and diagnostic laboratory examinations and early bone marrow transplantation [BMT] in patients