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[In vitro investigations of microphages function in patients with chronic mucocutaneous candidosis (author's transl)]
Abstract:
In five patients with either familial or non-familial type of chronic mucocutaneous candidosis some properties of phagocytic function of the polymorphonuclear leucocytes (PMNL) have been studied in vitro. In each of the patients there were found: a) a decreased chemotactic activity of PMNL, b) a weakness of intake and of intracellular destruction of Candida albicans cells by PMNL, c) an impairment of phagocytosis and intracellular killing of Candida albicans as well of Staphylococcus aureus by PMNL. The rate of phagocytosis of heat-inactivated Candida albicans cells by PMNL was normal in each case. In the serum of two patients a phagocytosis inhibiting factor is supposed to exist. In PMNL of 3 patients a defective activity of NADH-dependent oxidase was found. The occurrence of hereditary CMCC in a father and his two daughters points to an autosomal dominant trait, whereas in most cases of familiar CMCC hitherto described an autosomal recessive mode of transmission was found.
Insights
This study reveals impaired phagocyte function in chronic mucocutaneous candidosis (CMCC) patients, affecting their ability to fight fungal and bacterial infections. Defective cellular processes and potential inhibiting factors contribute to this immune deficiency.
Area of Science:
- Immunology
- Cell Biology
- Genetics
Background:
- Chronic mucocutaneous candidosis (CMCC) is a group of disorders characterized by recurrent or persistent infections of the skin, nails, and mucous membranes by *Candida* species.
- Understanding the underlying immune defects is crucial for effective management and treatment strategies.
Purpose of the Study:
- To investigate the in vitro phagocytic functions of polymorphonuclear leukocytes (PMNL) in patients with familial or non-familial CMCC.
- To identify specific cellular defects contributing to the impaired immune response in CMCC.
Main Methods:
- In vitro analysis of phagocytic functions, including chemotaxis, phagocytosis, and intracellular killing of *Candida albicans* and *Staphylococcus aureus* by PMNL.
- Assessment of PMNL phagocytosis of heat-inactivated *Candida albicans*.
- Investigation of potential phagocytosis inhibiting factors in patient serum.
- Evaluation of NADH-dependent oxidase activity in patient PMNL.
Main Results:
- Patients exhibited decreased PMNL chemotactic activity.
- Impaired uptake and intracellular destruction of *Candida albicans* by PMNL were observed.
- Reduced phagocytosis and intracellular killing of both *Candida albicans* and *Staphylococcus aureus* by PMNL.
- Normal phagocytosis rates of heat-inactivated *Candida albicans* suggest specific opsonization or cell wall defects.
- A phagocytosis inhibiting factor was suspected in the serum of two patients.
- Defective NADH-dependent oxidase activity was found in PMNL of three patients.
- A familial case suggested an autosomal dominant inheritance pattern for CMCC, contrasting with previously reported autosomal recessive modes.
Conclusions:
- Patients with CMCC display significant defects in PMNL phagocytic functions, impacting their ability to combat microbial infections.
- Deficiencies in chemotaxis, phagocytosis, intracellular killing, and potentially NADH-dependent oxidase activity contribute to the pathogenesis of CMCC.
- The study highlights the heterogeneity in genetic transmission patterns of CMCC, with evidence for both autosomal dominant and recessive inheritance.