Summary
The case of a 9-month-old baby girl who failed to develop normally due to nutritional neglect and secondary immunodeficiency characterized by marked thymic involution is reported. The child died of systemicPseudomonas aeruginosa (P. aeruginosa) infection manifested in pnenmonia, lung abscesses, bacterial endocarditis and ecthyma gangrenosum. At autopsy the child was 64 cm in height (normal for a 4- to 5-month-old child) and 5.1 kg in weight (normal for a 2- to 3-month-old child). Multiple gangrenous ecthymas, consisting of deep ulcers, induration and inflammation, were observed in the skin over the entire body. The lungs showed hemorrhagic pneumonia, multiple lung abscesses, and necrotizing arteritis in the abscesses and surrounding areas. The thymus weighed 2.3 g and showed marked involution. Histological examination showed so-called nutritional thymectomy characterized by servere cortical atrophy and clustering, cystic dilation and amorphous changes of the Hassall's corpuscles. In the heart, dark brown verrucae were present at the attachment sites of the tendinous cords of the papillary muscle in the anterior and posterior cusps of the mitral valve, suggesting infections endocarditis. Bacteriological examination demonstratedP. aeruginosa in the ecthymas, lung abscesses and blood. As primary immunodeficiency was considered unlikely, immunodeficiency secondary to thymic involution following malnutrition seemed to have led to a fatal systemic infection withP. aeruginosa, whose virulence is generally weak. This suggests a close association of the development of such infection and immunodeficiency with child neglect.
Zusammenfassung
Beschreibung eines 9-Monate alten weiblichen Säuglings, welcher sich wegen Mangelernährung verzögert entwickelte. Die Entwicklungsstörung führte zu einer Immundefizienz, welche durch eine ausgeprägte Thymus-Involution charakterisiert war. Das Kind starb an einer systemischen Infektion durch Pseudomonas aeruginosa mit Manifestation einer Pneumonia, Lungenabszessen, bakterieller Endokarditis und Ecthyma gangraenosum. Bei der Obduktion war das Kind 64 cm lang — entsprechend einem Alter von 4–5 Monaten — und wog 5,1 kg — entsprechend einem Lebensalter von 2–3 Monaten. Die Haut des gesamten Körpers wies multiple gangränöse Ecthyme auf, welche aus tiefen Geschwüren, Verhärtung und Entzündung bestanden. Die Lungen zeigten eine hämorrhagische Pneumonie, zahlreiche Abszesse und eine nekrotisierende Arteriitis in den Abszessen und in den umgebenden Geweben. Der Thymus wog 2,3 g und zeigte eine ausgeprägte Involution. Die Histologie zeigte eine sog. „ernährungsbedingte Thymektomie”, welche durch schwere kortikale Atrophie charakterisiert war und durch Anhäufung, zystische Erweiterung und amorphe Veränderungen der Hassall'schen Körperchen. Im Herzen waren tiefbraune Warzen vorhanden. An den Anheftungsseiten der Sehnenfäden der Papillarmuskeln im vorderen und hinteren Segel der Mitralklappe, wie bei infektiöser Endokarditis. Die bakterielle Untersuchung wies Pseudomonas aeruginosa in den Ecthymen, den Lungenabszessen und im Blut nach. Da eine primäre Immundefizienz als unwahrscheinlich erachtet wurde, wurde eine sekundäre Immundefizienz aufgrund einer Thymusinvolution, bedingt durch Nahrungsmangel, für ursächlich für die Pseudomonas aeruginosa-Infektion gehalten, ein Keim, welcher generell wenig virulent ist. Dieser Umstand legt eine enge Assoziation zwischen der Entstehung dieser Infektion und der Immundefizienz durch Kindesvernachlässigung nahe.
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Ohshima, T., Nakaya, T., Saito, K. et al. Child neglect followed by marked thymic involution and fatal systemic pseudomonas infection. Int J Leg Med 104, 167–171 (1991). https://doi.org/10.1007/BF01369723
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DOI: https://doi.org/10.1007/BF01369723
Key words
- Child neglect
- Malnutrition
- Thymic involution
- Secondary immunodeficiency
- Pseudomonas aeruginosa infection