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Biomedical subjects

M Ziprkowski

Publications and source records attributed to M Ziprkowski.

9 recordsLinked to original sources

Retroperitoneal malacoplakia.

Malacoplakia is an uncommon, pathologically distinct, granulomatous disease that occurs most frequently in the urinary tract. It can present as an abdominal mass in a patient with urinary tract infection. A case of retroperitoneal malacoplakia with CT findings and successful treatment with radiation therapy is presented.

Adolescent

The usefulness of chest radiographs in first asthma attacks.

To assess the value of routine chest radiography during acute first attacks of asthma, we studied 371 consecutive children over one year of age who presented with an initial episode of wheezing. Three hundred fifty children (94.3 per cent) had radiographic findings that were compatible with uncomplicated asthma and were considered negative. Twenty-one (5.7 per cent) had positive findings: atelectasis and pneumonia were noted in seven, segmental atelectasis in six, pneumonia in five, multiple areas of subsegmental atelectasis in two, and pneumomediastinum in one. The patients with positive films were more likely to have a respiratory rate above 60 or a pulse rate above 160 (P less than 0.001), localized rales or localized decreased breath sounds before treatment (P less than 0.01), and localized rales (P less than 0.005) and localized wheezing (P less than 0.02) after treatment; also, these patients were admitted to the hospital more often (P less than 0.001). Ninety-five per cent (20 of 21) of the children with positive films could be identified before treatment on the basis of a combination of tachypnea, tachycardia, fever, and localized rales or localized decreased breath sounds. Most first-time wheezers will not have positive radiographs; careful clinical evaluation should reveal which patients will have abnormal radiographs and will therefore benefit from the procedure.

Asthma

Fatal disseminated BCG infection. An investigation of the immunodeficiency.

A 2-year-old boy had a fatal disseminated BCG infection. Immunologic assessment showed a normal humoral response and normal numbers of E rosettes, normal thymus weight and histological features, but an abnormal response of lymphocytes in vitro and negative skin tests. Histological examination showed the presence of Gram-negative acid-fast bacilli within the macrophages. The possible mechanisms of immunodeficiency in this patient are discussed.

Animals

Congenital ichthyosis, mental retardation, dwarfism and renal impairment: a new syndrome.

A new congenital ichthyotic syndrome inherited as an autosomal recessive is described in three propositi of an Iranian family. The main clinical features are non-bullous congenital ichthyosis, mental retardation, dwarfism, and renal impairment. The nephropathy which previously has not been associated with congenital ichthyosis was manifested by raised blood urea nitrogen and creatinine levels, and a reduced creatinine clearance. The clinical and genetic features of this syndrome are discussed in relationship to the other congenital ichthyotic syndromes.

Biopsy

Corticosteroid treatment for pulmonary lymphoid hyperplasia in children with the acquired immune deficiency syndrome.

Five children with positive serology for human immunodeficiency virus (HIV) infection by enzyme-linked immunosorbent assay and Western blot were followed for chronic pulmonary disease. Lung biopsies were performed in all patients, and confirmed the diagnosis of pulmonary lymphoid hyperplasia. All children demonstrated progressive hypoxia and increasing alveolar capillary oxygen gradients over at least 1 year of follow-up. All children were on periodic intravenous gamma globulin treatment for a B-cell defect prior to the initiation of corticosteroid therapy. Prednisone was initially given at a dose of 2 mg/kg daily and was subsequently tapered to an alternate day regimen. All children showed improvement in oxygenation. No deterioration in immune function was noted, and there was no increase in bacterial infection. This study indicates that corticosteroids can successfully reverse the severe hypoxia that may result from pulmonary lymphoid hyperplasia in pediatric AIDS patients.

Acquired Immunodeficiency Syndrome