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Biomedical subjects

B Wolach

Publications and source records attributed to B Wolach.

At least 109 records · Page 6Linked to original sources
PubMed1987

Tx polyagglutination in three members of one family.

A case of acute haemolytic anaemia is described in a child. Tx polyagglutination of his red cells was observed, but no direct association with the anaemia could be proved. Polyagglutination was suspected because of irregularities in the AB0 blood grouping. Confirmation of the cryptantigen Tx was made when the patient's red cells were tested with lectins including Arachis hypogaea, Glycine soja, and Vicia cretica. Examination of family members showed Tx polyagglutination on the red cells of 2 siblings. The Tx polyagglutination was a transient phenomenon lasting 4-5.5 months, and could have been caused as the result of some unidentified bacterial or viral infection. Guidelines for transfusion therapy are suggested in patients in whom polyagglutination is recognised.

Adolescent↗
PubMed1985

Severe congenital neutropenia with unique features of dysgranulopoiesis.

Congenital dysgranulopietic neutropenia (CDN) is a recently proposed entity that describes a small subgroup of children with clinically severe neutropenia. We followed and studied a 3-year-old girl with neutropenia (less than 500/mm3) and recurrent severe infections in whom repeated marrow evaluations revealed large (30-50 microns) multinucleated promyelocytes to polymorphonuclear cells with as many as 4 to 16 nuclei or nuclear lobes, respectively. In addition to the nuclear endoreduplication, ultrastructural and cytochemical evaluation of these cells demonstrated abnormalities in granule genesis and centriole structure. Concomitantly, immunoperoxidase staining indicated that many of the granules were devoid of lactoferrin but not lysozyme. In vitro proliferation studies revealed normal to increased thymidine labeling, normal numbers of colony-forming cells, and normal colony-stimulating activity from blood and marrow mononuclear cells, findings consistent with ineffective myelopoiesis. However, serum folate, B12, and lysozyme levels were normal. The nuclear and cytoplasmic abnormalities in this patient result in an extreme example of CDN, distinct from previously described cases.

Agranulocytosis↗
PubMed1985

Neonatal pneumothorax, a rare complication of pulmonary cystic lymphangiectasis.

An unusual case of neonatal pneumothorax in a full-term female infant is reported. The collapse was caused by congenital pulmonary lymphangiectasis and rapidly led to death; the lesion was discovered at autopsy. Multiple cardiac malformations were also associated. The literature and pathogenesis are reviewed and discussed.

Female↗
PubMed1984

Plasma lactoferrin reflects granulocyte activation via complement in burn patients.

Complement activation and neutropenia have been observed in thermally injured animals. In burn patients, granulocyte chemotaxis and morphological loss of specific granules occur. We conjectured that complement is activated in humans and, in turn, induces granulocytes to secrete lactoferrin (LF), a marker of granulocyte activation. Twenty burn patients were evaluated for absolute granulocyte count (AGC), plasma levels of anaphylatoxins (C3a, C4a, C5a), and lactoferrin. The AGC directly correlated with the extent of the burn on day 1. Similarly, plasma LF on day 1 correlated with the percent burn. Those with greater than 30% burn had plasma LF between 10 and 40 micrograms/ml (normal LF = 1.5 +/- 1.8 micrograms/ml). In five patients without further complications followed serially, plasma LF did not return to normal until 2 to 5 weeks. In all patients, there was evidence of complement activation; C4a ranged between 283 and 13,064 ng/ml and C3a between 19 and 852 ng/ml. In some patients, C5a was detectable, but the values correlated inversely with the extent of burn. On the other hand C3a and C4a levels did not correlate with the extent of burn but threefold to fivefold rises of C3a levels on days 7 and 9 predicted gram-negative sepsis. Although plasma LF did not predict sepsis, levels greater than 12 micrograms/ml on day 1 heralded the onset of neutropenia on day 3 in 60% of patients with 30% burn. Six of 20 patients developed pulmonary radiographic changes and, in five of the six, the changes occurred by day 3. Plasma LF in all six patients on day 1 was greater than 17 micrograms/ml. In two patients with greater than 50% burn, depletion of granulocyte LF was demonstrated histochemically. These studies indicate that complement is activated in burn patients, which is associated with granulocyte secretion. Measurement of plasma anaphylatoxins and LF may serve as useful aides in clinical management of these patients.

Adult↗
PubMed1983

The mechanism of action of the antiinflammatory agents dexamethasone and Auranofin in human polymorphonuclear leukocytes.

Human polymorphonuclear neutrophils (PMN) were treated with the antiinflammatory agents dexamethasone or Auranofin. PMN treated with dexamethasone in a dose range of 0.25-1 microM or Auranofin, 5-15 mM, were stimulated with 10(-7)M N-formyl-methionyl-leucyl-phenylalanine (FMLP). These agents were shown to inhibit the functional responses of degranulation and superoxide production in a dose-dependent manner. Similarly, the change in electrophoretic mobility, reflecting cell surface charge, was blocked. While both agents inhibited change in the fluorescence of the calcium chelate probe chlorotetracycline (CTC), the pattern of inhibition was significantly different. Dexamethasone appeared to inhibit the CTC response during its latter phases, while Auranofin inhibited all aspects of the CTC response. Auranofin was additionally shown to significantly decrease specific binding of FMLP, as well as the number of FMLP receptors. The two agents thus appear to act by different mechanisms. Dexamethasone is shown to have an effect on membrane-bound calcium release as measured by CTC, while Auranofin interferes with receptor binding.

Auranofin↗
PubMed1982

Correlation of in vitro and in vivo effects of gold compounds on leukocyte function: possible mechanisms of action.

The anti-inflammatory effects of gold compounds include suppression of PMN lysosomal enzyme release. Since lysosomal products can provoke PMN aggregation, we assessed the effect of two gold compounds, auranofin and GST, on suppressing aggregation, degranulation, and metabolic functions of the cells. Aggregation of 1 x 10(7) cytochalasin B-treated PMNs in response to 2 x 10(-7)M FMLP, as assessed by light scattering, was inhibited in a dose-dependent fashion by both drugs. Concentrations of auranofin ranging from 5 to 20 microM caused 30.8% to 89% inhibition, whereas 200 microM GST reduced aggregation by only 32%. FCS or BSA added to suspensions of normal PMNs considerably reduced the gold compound inhibitory effect on PMN aggregation. Cell viability assessed by dye exclusion and lactate dehydrogenase release was unaffected by the drugs. The suppressive activities of the drugs could not be removed by washing the PMNs. Correspondingly, the drugs suppressed lysosomal enzyme release induced by FMLP of PMNs rendered secretory with cytochalasin B. Concentrations of 20 microM auranofin and 200 microM GST resulted, respectively, in a 61.5% and 19.3% reduction of release of lysozyme, 61.7% and 27.1% reduction of beta-glucuronidase, 84.8% and 33.7%s reduction of myeloperoxidase, and 50.0% and 25.0% reduction of lactoferrin. Furthermore, auranofin inhibited 14C-1-glucose oxidation through the hexose monophosphate shunt in response to stimulation by either PMA or methylene blue. The in vivo studies suggested that auranofin could prevent neither neutropenia induced by zymosan-activated serum nor a corresponding rise in plasma lactoferrin levels. These findings suggest that the beneficial effect of gold compounds in rheumatoid arthritis are unlikely to be related to their ability to dampen PMN activation in vivo.

Animals↗
PubMed1981

Transient Donath-Landsteiner haemolytic anaemia.

Five children with 'paroxysmal cold haemoglobinuria' are described. In all of them an upper respiratory tract infection preceded the episode of acute intravascular haemolysis and potentially pathogenic organisms were recovered from four. The haematological features are presented and the importance of technique in the detection of the pathognomonic antibody is stressed and discussed in detail. Although the clinical manifestations are usually self-limiting and of short duration, it may be necessary, because of the rapidity and severity of anaemia, to provide interim support with appropriate blood transfusion. Since the disease is not usually recurrent and not clearly related to exposure to cold, it is suggested that it be re-named Donath-Landsteiner haemolytic anaemia.

Antibodies↗
PubMedDate not supplied

Acinetobacter septicemia: a threat to neonates? Special aspects in a neonatal intensive care unit.

Acinetobacter is one of the organisms responsible for nosocomial infections in intensive care, neurosurgery, burn and hemodialysis units. There are only a few reports on Acinetobacter infections in neonatal intensive care units. Over a 31 month period, nine cases of Acinetobacter sepsis occurred in our unit, with four deaths. There was a cluster of four cases within 3 days. In this study the English literature on this pathogen is reviewed and it is suggested that Acinetobacter should be added to the list of organisms causing severe nosocomial infection in neonatal intensive care units.

Acinetobacter↗
PubMedDate not supplied

Poliomyelitis-like syndrome following asthmatic attack (Hopkins' syndrome)--recovery associated with i.v. gamma globulin treatment.

A report on a 15-year-old male with a diagnosis of poliomyelitis-like syndrome (Hopkins' syndrome) following an asthmatic attack is presented. The prognosis of Hopkins' syndrome is usually poor and the patients remain with permanent paralysis of the affected limb. The outcome correlates with severity of the initial injury to the anterior horn cell as reflected by abnormal electrophysiologic studies. This is the first case report of treatment with i.v. gamma globulin in Hopkins' syndrome which resulted in a nearly complete recovery.

Adolescent↗
PubMedDate not supplied

Some aspects of the humoral and neutrophil functions in post-comatose nawareness patients.

Post-comatose unawareness (PCU) is one of the possible outcomes of severe brain injury. Patients with severe brain injury have an increased susceptibility to severe nosocomial infections for multifactorial reasons, including immune suppression at different levels. We studied different immunological aspects in 11 PCU patients. Impaired humoral immunity was found in 27% of them. Two patients had decreased haemolytic activity of the classical complement pathway, associated with decreased levels of the components C1q, C1r and C4. Another patient had very low levels of IgG2 and IgG4. The neutrophil killing activity was impaired in these three patients, but was completely restored with the addition of a heterologous serum, suggesting a humoral defect. Neutrophils showed normal chemotaxis and random migration, and the superoxide anion release by neutrophils was also found to be normal. Understanding the immunological events in PCU patients contributes to a better and more intensive therapeutic approach, which might accelerate the rehabilitation of these patients.

Adolescent↗
PubMedDate not supplied

Giant hemangioma with thrombocytopenia and osteolysis successfully treated with prednisone.

A 6-week-old girl with a hemangioma with thrombocytopenia, consumption coagulopathy, extensive bleeding, disappearance of the right pubic bone, and destruction of part of the right iliac bone was successfully treated with prednisone. Treatment lasted 30 weeks. Bleeding ceased within a few days; bone rebuilding was first observed at 30 days and was extensive at the end of treatment. When the child was 3 years old, pelvic films were normal. The occurrence of Kasabach-Merritt syndrome associated with complete osteolysis as in our patient is rare and bone reconstruction is unusual.

Female↗