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C K Naspitz

Publications and source records attributed to C K Naspitz.

At least 109 records · Page 6Linked to original sources
PubMedDate not supplied

Hereditary angioedema type II--a study of two families.

Hereditary angioedema (HA) is caused by a quantitative or qualitative deficiency of C1 esterase inhibitor (C1 INH). We present a study of nine patients with HA belonging to two different families. The symptoms started before 10 years of age in most cases (78%). Facial edema (lips, eyes) and of the extremities (feet, hands) were the most frequent complaints. Three patients presented edema of the glottis and one of them underwent a tracheostomy twice. Laboratory tests, outside the acute crisis, revealed low levels of C4 in all patients. The serum levels of C1 INH were normal in seven patients; however, functional activity was not observed in any of them. After the use of a modified androgen (danazol), control of symptoms was observed in all patients, although functional activity was re-established in only five patients.

Adolescent↗
PubMedDate not supplied

Reduction in the oral doses of theophylline in asthmatic children during concomitant treatment with ketotifen.

Sixteen children with moderate or severe bronchial asthma underwent a double-blind study receiving either ketotifen (K) or placebo (P) in order to verify the sparing effect of K on theophylline. The study lasted 18 weeks. Slow release theophylline was administered to all patients at a dose of 300 mg every 12 hours for the first six weeks of the study ("washout"). From the seventh week on, eight patients received K (1 mg, 2x/day) and the others P. The theophylline dose was maintained and reduced every 4 weeks to 200 mg and 100 mg. The initial clinical characteristics were identical in both groups. The serum levels of theophylline decreased below the ideal range (less than 10 mcg/ml) in groups K and P with a 100 mg dose of theophylline. However, in group K patients, fewer reported worsening of symptoms (number of days with cough and/or wheeze, altered daytime or nighttime peak expiratory flow rate and consumption of beta agonist) when compared to group P. Increased PC20 of methacholine only occurred in group K patients.

Administration, Oral↗
PubMedDate not supplied

Ataxia-telangiectasia: a clinical and laboratory review study of 14 cases.

Fourteen patients with ataxia-telangiectasia and 10 relatives were studied. Besides neurological examination, laboratorial investigations as to their immunological condition were carried out. In this study, our attention was attracted to the most frequent and relevant laboratorial data being decreased serum and salivary levels of IgA, decreased rate of blastic transformation of lymphocytes and increased serum levels of alpha-fetoprotein (AFP). On the other hand, no patient showed increased levels of carcinoembryonic antigen (CEA). Our population reported a consanguinity rate of 28.5%.

Ataxia Telangiectasia↗
PubMedDate not supplied

[Juvenile rheumatoid arthritis: review and update].

Major current clinical, laboratory, and etiopathogenic aspects of juvenile rheumatoid arthritis (JRA) are focused by this article. The authors make a comparison of data of their patients to those described in the literature. A retrospective clinicolaboratory study was performed of 130 patients with JRA followed in the Pediatric Rheumatology Department of the Division of Allergy, Rheumatology, and Clinical Immunology of the Service of Pediatrics of Escola Paulista de Medicina from 1982 to 1989. These patients (73 female and 57 male) were analyzed for the type and age of onset, clinical manifestations (articular and extraarticular), laboratory data, and prognosis. The findings of the authors are similar to those reported, with the exception of eye involvement (chronic iridocyclitis) and of the presence of the IgM rheumatoid factor (RF) and the antinuclear antibody (ANA), which were lower than those described by other authors. With respect to prognosis, the authors verified the greatest number of remissions (40%) with the pauciarticular type, whereas the polyarticular type presented the lowest number of remissions (27%). The two deaths occurred in the series were of children with the systemic type of the disease. Although etiology and the mechanism responsible for its release are not yet known, therefore, not allowing a definite type of treatment, experience gathered throughout the years has made it possible for physicians who attend to these patients not only to make an early diagnosis but also to resort to more efficient means of control.

Antibodies, Antinuclear↗
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Chemotaxis of leukocytes from peripheral blood of patients with juvenile rheumatoid arthritis (JRA).

Spontaneous and stimulated migration of leukocytes from peripheral blood was studied in 15 patients with JRA and 15 controls. Cells from both groups showed equal migratory ability. However, in the presence of the activated serum of the JRA patients, there was a significant decrease in migration of leukocytes from JRA patients and from controls, suggesting the existence of a serum factor responsible for this chemotactic impairment. The effect of washing on the migration of these cells was also studied. A significant decrease could be observed in the spontaneous and stimulated migration of washed cells from both JRA patients and controls.

Antigen-Antibody Complex↗
PubMedDate not supplied

[Familial Bloom's syndrome associated with neuroblastoma].

Bloom's syndrome (BS) is an autosomal recessive disease characterized by short stature, sensitivity to sunlight, and telangiectasic malar erythema. It is associated to chromosomal breakage, to primary combined immunodeficiency, and to a high incidence of neoplasias. The authors report the case of two siblings with BS and associated immunodeficiency. Both patients were male and 5 (A) and 4 (B) years old at the time of diagnosis. Chronic diarrhea, recurrent otitis media, purulent rhinitis, conjunctivitis and pyodermatitis were reported by patient A. Patient B was admitted with diagnosis of bilateral neuroblastoma and had the tumor resected. Later on, he presented with oral moniliasis, herpetic stomatitis, and skin abscesses. This patient did not have recurrent infections. Immunological evaluation showed normal serum levels of CH50, C3, and C4 for both patients. Serum IgG, IgA, IgM, and salivary IgA levels were: 455 mg/dl, 15mg/dl, 20mg/dl, 0.6mg/dl for A, and 400mg/dl, 15mg/dl, 20mg/dl, and 0.2mg/dl for B, respectively. Serum antipolio antibodies (1, 2, and 3) were normal, and low levels of isohemagglutinins were observed in both patients. T cells subset determination showed: patient A--OKT3 = 66%, OKT4 = 33%, OKT8 = 32%, and 4/8 ratio = 1.0; patient B--OKT3 = 70%, OKT4 = 32%, OKT8 = 34%, and 4/8 ratio = 1.0. In vitro cellular immune response to PHA was depressed only in patient B. Patients karyotype showed chromosomal breaks with sister chromatid exchanges. Neither patient had abnormal alphafetoprotein and carcinoembryonic antigen serum levels. The rarity of such associations justifies the presentation of the cases.

Bloom Syndrome↗
PubMedDate not supplied

[Anaphylactic shock].

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Anaphylaxis↗
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[The wheezy baby].

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Asthma↗
PubMedDate not supplied

Secretory IgA levels in normal and atopic individuals. Influence of breast and/or bottle feeding.

The SIgA and IgA quantification by radial immunodiffusion was carried out in the saliva of 449 normal individuals, whose ages vary from 1 day to 32 years old. SIgA was determined in an agar plate containing purified serum against SIgA of human colostrum and the IgA in commercial plates containing serum anti IgA. The SIgA values grouped in different age groups were much variable, allowing only to establish minimum critical values for each group. In the saliva of 71 patients IgA was dosed by both methods. The set of obtained data showed significant correlation (p less than 0.05). The dosage of standardized quantities of SIgA and IgA in conventional plates containing serum anti IgA, allowed to establish corresponding values similar to precipitation ring diameter. In this way, it was possible to establish a "critical value" for salivary IgA by the use of conventional plates. Thus, the IgA quantitative determination by radial immunodiffusion with antibody against serum IgA was carried out in saliva samples of normal and atopic individuals (asthma and/or rhinitis). The patients' ages varied from 1 month to 32 years old. The obtained values were grouped according to defined age group. We observed non-measurable values in 70% of the children aged less than 1 month; from then on, the values were increasing, reaching adult levels from the 6th year on. We did not observe differences between the salivary IgA levels of normal and atopic children. In children aged less than 6 months breast and/or bottle feeding (natural, mixed or artificial) there were no differences between the IgA levels observed in saliva.

Adolescent↗
PubMedDate not supplied

Evaluation of ketotifen in the prophylactic treatment of bronchial asthma in children.

The usefulness of ketotifen for reducing bronchial hypperreactivity, acting as a prophylactic drug, was studied in children with extrinsic asthma. In an open-label phase (phase A), 2.267 asthmatic children received ketotifen orally at a dosage of 1 mg twice daily for three months. Another study, double-blind, placebo-controlled (phase B), enrolled 75 children (38 ketotifen; 37 placebo); they received the same dosage of ketotifen or placebo for five months, after a one-month placebo baseline control period. A progressive improvement in the severity of asthmatic attacks was seen throughout the three-month period (phase A). At the end of the trial, the percentage of patients with moderate or intense attacks was reduced from 77% at baseline to 18%. Significant reductions were noted in the duration and frequency of asthma attacks at each monthly evaluation. The need for the use of symptomatic medication declined significantly during the 3-month trial. In the five-month, double-blind, placebo--controlled study (phase B), significant reduction in the frequency of dyspnea was noted at the end of the trial; in the placebo group, this frequency increased. This clinical observation was confirmed by the mean consumption of sympathomatic drugs during the five-month treatment period: it declined in the ketotifen--treated patients but increased in those who received placebo. The low incidence side effects attests to the safety of ketotifen in children. Our results demonstrate that ketotifen is both safe and effective for prophylactic use in asthmatic children.

Adrenal Cortex Hormones↗
PubMedDate not supplied

Maximum bronchodilator effect of pirbuterol and procaterol administered as sprays with and without an aerochamber.

OBJECTIVE OF THE STUDY: To determine the maximal bronchodilator dose of procaterol and pirbuterol administered by inhalation with an without an aerochamber (Aerocâmera) to children with acute bronchial asthma attacks. TYPE OF STUDY: Prospective. 18 children (6-15 years of age) with acute bronchial asthma attacks (FEV1 < 80% of the predicted value) received pirbuterol (N = 10) or procaterol (N = 8) by metered-dose inhaler, one puff every 5 minutes, for a total of five doses. During another acute attack, the same patient received the same medication with the aid of a spacer (Aerocâmera). Clinical evaluation and pulmonary function tests were performed 5 minutes after each inhaled dose. RESULTS: For both drugs, maximal bronchodilation was obtained after the second dose. No significant side effects were observed even after procaterol doses of 50 micrograms or pirbuterol doses of 1000 micrograms. The results were unaffected by the use of the spacer. CONCLUSIONS: The doses that induced maximal bronchodilation were 400 micrograms pirbuterol and 20 micrograms procaterol. Although the spacer did not change the results, it is a valuable aid for patients who have difficulty in using the metered-dose inhaler (M.D.I.).

Administration, Inhalation↗
PubMedDate not supplied

Cartilage-hair hypoplasia syndrome: immunological evaluation of two cases.

PURPOSE: Immunological evaluation of patients with cartilage-hair hypoplasia. TYPE: Prospective and retrospective studies. PLACE: Division of Allergy, Clinical Immunology and Rheumatology-Dept. of Pediatrics-"Escola Paulista de Medicina". PATIENTS: Two children with cartilage-hair hypoplasia syndrome. METHODS: Clinical and immunological evaluation. Humoral immunity (immunoglobulin levels, poliovirus antibodies, etc.) and T cell immunity (in vitro cultured lymphocytes stimulated with PHA, Con A and PWN, total T cell and subset determination) were studied. RESULTS: Cellular immunodeficiency and hypogammaglobulinemia were observed in one patient and normal values in the other. CONCLUSIONS: Immunological evaluation (cellular and humoral) should be performed in all patients with cartilage-hair hypoplasia.

Antibody Formation↗