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Biomedical subjects

R J Koopman

Publications and source records attributed to R J Koopman.

32 records · Page 2Linked to original sources

Sarcoidosis.

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Adult↗

Calciphylaxis.

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Calciphylaxis↗

[Subfascial lipoma of the forehead].

Three patients with subfascial lipoma of the forehead are presented. These deeply placed lipomas are seen in up to fifty percent of the lipomas of the scalp. A characteristic feature is the intact skin line pattern over the tumour. Surgical treatment involves incision of the aponeurosis of the M. occipitofrontalis.

Adult↗

Erysipelas-like skin lesions associated with Campylobacter jejuni septicemia in patients with hypogammaglobulinemia.

Three cases are reported of hypogammaglobulinemic males with recurrent Campylobacter jejuni septicemia and erysipelas-like cellulitis without diarrhoea. In one patient Campylobacter jejuni grew from skin biopsy specimens. The findings in another patient were strongly suggestive of osteomyelitis caused by Campylobacter jejuni. Since the susceptibility of hypogammaglobulinemic patients to infection with Campylobacter jejuni is probably related to a lack of serum bactericidal activity against Campylobacter jejuni due to lack of IgM, two patients in whom previous antimicrobial treatment failed were treated with plasma infusions. This regimen supplemented with imipenem resulted in cure of these relapsing infections. Campylobacter jejuni septicemia must be considered in hypogammaglobulinemic patients who present with periodic fever and cellulitis.

Adult↗

Autosomal dominant transmission of the NAME syndrome (nevi, atrial myxoma, mucinosis of the skin and endocrine overactivity).

A family affected in two generations with the NAME syndrome is described, and the literature on this phenotype reviewed. The term "NAME" was originally proposed as an acronym for "nevi, atrial myxoma, myxoid neurofibromata and ephelides". However, in order to give a more comprehensive description of this syndrome, we recommend the following alternative interpretation of "NAME": nevi, atrial myxoma, mucinosis of the skin, endocrine overactivity. Previous family observations suggested a dominant, and probably autosomal, inheritance but an X-linked dominant transmission could not be excluded. We describe male-to-male transmission, strongly supporting the concept of the autosomal inheritance of this trait.

Adult↗

[An infant with short stature and red cheeks (Rothmund-Thomson syndrome)].

An infant with short stature and progressive skin lesions of cheeks and dorsum of the hands is described. Further problems such as recurrent diarrhoea and respiratory infections suggested zinc-deficiency, malabsorption-syndrome, Bloom syndrome and early Lupus Erythematosus respectively. Finally Rothmund-Thomson syndrome was diagnosed. This rare genetic disorder is characterized by variable expression of typical cutaneous changes, cataracts, skeletal anomalies, short stature, abnormal hair growth and defective nails and teeth, mental retardation, hypogonadism and a typical facial appearance.

Diagnosis, Differential↗

[Acral nevi following chemotherapy].

Multiple melanocytic nevi showing an unusual accumulation on the soles of the feet were observed in an 8-year-old boy after he had received chemotherapy for acute lymphatic leukemia. This observation confirms the occurrence of chemically induced melanocytic nevi as well as their affinity for the acral sites.

Antineoplastic Combined Chemotherapy Protocols↗

Tuberculosis cutis miliaris disseminata as a manifestation of miliary tuberculosis: literature review and report of a case of recurrent skin lesions.

The development of severe adverse reactions to antituberculous drugs in a patient with miliary tuberculosis led to unorthodox, suboptimal antituberculous therapy. The patient's failure to respond to therapy was discovered when acid-fast bacilli were detected in new skin lesions. Such lesions have been described in the literature as tuberculosis cutis miliaris disseminata; 24 cases (in addition to that described herein) have been reported thus far. The patient eventually recovered completely after detection and drainage of a large retrofascial tuberculous abscess. This case illustrates the importance of careful examination of the skin in clinical medicine, as tuberculosis cutis miliaris disseminata is an easily overlooked sign of miliary tuberculosis.

Abscess↗

[Proteus syndrome. Expansion of the phenotype. Apropos of 3 pediatric cases].

In 1979 Cohen et Hayden and in 1983 Wiedemann et al. delineated a syndrome consisting of partial gigantism of the hands and/or feet, nevi, hemihypertrophy, subcutaneous tumors, macrocephaly or other skull anomalies and possible accelerated growth and visceral affections. Hitherto the literature pertaining to this syndrome consists of somewhat more than 100 cases of which some, that have been described previously or subsequently under other headings, were rediagnosed as being Proteus syndromes. Of these, more than half show vascular anomalies closely resembling those observed in the Klippel-Trenaunay syndrome, but in the Proteus syndrome appear to be more haphazardly distributed over the integument. We report 3 pediatric patients with the Proteus syndrome, all showing cutaneous angiodysplasias. These patients were initially diagnosed as suffering from "severe or atypical Klippel-Trenaunay syndrome". In one of these, cardiac tumors were observed soon after birth which subsequently showed spontaneous involution and were therefore considered to be rhabdomyomas. In the Proteus syndrome cardiac pathology is rare, and cardiac tumors have not been described previously. Moreover, we observed umbilical hernia in two of our patients, a feature which has hitherto not been reported in patients with the Proteus syndrome. In all our patients a broad thoracic cage resembling a "body-builders chest", asymmetrical and disproportional macrodactyly and broad, flat feet were conspicuous. These broad, flat feet with macrodactyly and large spaces between the first and second digits were designed by the parents of one of our patient as "chimpanzee's feet". Macrodactyly, "chimp's" feet and a broad thoracic cage are considered by us to be clinical hallmarks of the Proteus syndrome.(ABSTRACT TRUNCATED AT 250 WORDS)

Angiodysplasia↗