Follow-up studies of immunological disorders in patients with ataxia-telangiectasia

Abstract

We studied a 10 years old boy with ataxia-telangiectasia suffered from T cell ALL.We characterized his tumor cell with immunological techniques.His affected female sib, who had very low serum IgG, IgA and elevated IgM concentration, died from pulmonary infection at 10 years old. In his family members, there were high frequency of cancers.The patient showed moderate cellular immune deficiency such as deficient delayed type skin hypersensitivity and low lymphocyte counts during early childhood, but did not show susceptibility to sinopulmonary infection. At age 10, he presented fever, malaise and lymphadenopathy. Peripheral blood smear and bone marrow aspirate showed abnormal cells.Biopsied cervical lymphnode was also diffusely infiltrated with abnormal cells. The mor phology of the cells was considered to be typical acute lymphoblastic leukemia.Surface marker studies of blast cells were done. The tumor cells had E-receptor, and did not show surface immunoglobulin. Studies using a series of monoclonal antibodies revealed 9.6, la-like antigen positive, and common ALL antigen, OKT3, OKT4, OKT6, OKT8negative. Tumor cells were shown to be T lymphcyte origin and immature thymocyte origin provably.Chest X-ray did not show mediastinal enlargement. After partial remission with Vincristine, Prednisone, Cyclophosphamide and cytosine arabinoside combination therapy (COAP),

Associations between Antiepileptic Use and Hypogammaglobulinaemia: Findings from a Population-Based Case-Control Study Using Data Linkage

“…Although the mechanisms by which AEDs cause HGG are unclear, low immunoglobulin subclass concentrations in children treated with carbamazepine or sodium valproate have been attributed to the effect of AEDs on B cell maturation or regulatory T lymphocytes, which directly affect immunoglobulin isotype production [34, 40]. Dosch et al [14] observed that AEDs impacting immunoglobulin concentrations block sodium channels; therefore, it may be possible that this mode of action causes the observed side effect.…”

Effectiveness of Topical Steroid in Phimosis: A longitudinal Observational Study

“…This study highlights that four weeks of conservative treatment could be tried before circumcision reducing the risk of anesthesia 16 as well as complications associated with surgery. 16,17 A tight foreskin may manifest with symptoms such as itching, smegma deposits, straining, ballooning, balanoposthitis, dysuria, or urinary infection termed as symptomatic or pathological phimosis.…”

“…[10][11][12] Gentle retraction of the prepuce, known as physiotherapy, was suggested as an important factor in the spontaneous resolution of physiologic phimosis. 17 Although forcible retraction of the prepuce should be avoided because of pain, bleeding, adhesion, and cicatrix formation, which might lead to secondary phimosis, careful and gentle retraction has been encouraged for the more rapid resolution of severe physiologic phimosis. 6,17 A previous study found that gentle retraction of the foreskin with topical application, reported a 50% success rate even with placebo cream and physiotherapy and suggested that physiotherapy per se would be effective to resolve physiologic phimosis.…”

“…17 Although forcible retraction of the prepuce should be avoided because of pain, bleeding, adhesion, and cicatrix formation, which might lead to secondary phimosis, careful and gentle retraction has been encouraged for the more rapid resolution of severe physiologic phimosis. 6,17 A previous study found that gentle retraction of the foreskin with topical application, reported a 50% success rate even with placebo cream and physiotherapy and suggested that physiotherapy per se would be effective to resolve physiologic phimosis. 6 In concurrence to these researches, we also found that steroid therapy would be an effective therapy for physiological phimosis.…”