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Biomedical subjects

A L Moss

Publications and source records attributed to A L Moss.

At least 19 recordsLinked to original sources

Catel-Manzke syndrome: a case report of a female with severely malformed hands and feet. An extension of the phenotype or a new syndrome?

An unusual case of a female infant with Catel-Manzke syndrome is presented. Additional features not previously reported include three accessory ossicles at the bases or associated with the proximal phalanx of the index, middle, ring and little fingers bilaterally. There are also numerous bony abnormalities in both feet. Previous cases have shown no more than 2 accessory ossicles in the hand and these usually involve the index alone. The foot abnormalities are more extensive than any previously seen in this syndrome. This is only the 8th female case out of a total of 27 reported cases.

Child, Preschool↗

Ear-lobe keloids: treatment by a protocol of surgical excision and immediate postoperative adjuvant radiotherapy.

There is no universally agreed policy for treating keloid scars of the ear lobe following piercing. We treated 35 patients (34 women) for high-risk ear-lobe keloids; the average age was 24 years (range: 16-44 years). All had failed to respond to prior treatment with massage and silicone, and corticosteroid injection. The keloids were excised extralesionally and the defects were closed with interrupted prolene sutures. The operative scar was covered with topical 2% lignocaine-0.25% chlorhexidine sterile lubricant gel under a transparent adhesive dressing. Adjuvant postoperative radiotherapy of 10 Gy, applied as 100 kV photons (4 mm high-voltage therapy (HVT) Al), was given within 24 h of surgery. All keloid scars were controlled at 4 weeks' follow-up. At 1 year, three out of 34 cases followed up had relapsed (probability of control: 91.2%). At 5 years, a further four out of the remaining 31 patients had relapsed (cumulative probability of control at 5 years: 79.4%). There were no cases of serious toxicity.

Adolescent↗

Cutaneous leishmaniasis.

The incidence of leishmaniasis is increasing globally due to population and environmental changes. Ease of worldwide travel and immigrant populations means that the UK surgeon is more likely to encounter cutaneous lesions. Two cases are presented and treatment options discussed.

Antimony Sodium Gluconate↗

Aplasia cutis congenita associated with a lipoma.

Aplasia cutis congenita is a condition that can present with a wide variety of symptoms and many attempts have been made to classify it comprehensively. This report presents the first case of a child with aplasia cutis congenita confirmed on histology with an associated lipoma or lipomatosis directly underlying it. It exemplifies the difficulty in diagnosing and treating this rare condition.

Ectodermal Dysplasia↗

Multiple pilomatrixomata and myotonic dystrophy: a familial association.

The association of pilomatrixoma and myotonic dystrophy has been described in the past in 13 publications in the English literature. The association seems to involve the development of pilomatrixomata before signs of myotonic dystrophy. Myotonic dystrophy is the commonest adult dystrophy and is an autosomaldominant disease with a variable phenotypic penetrance. The disease is determined by a genetic locus on chromosome 19q and can be diagnosed using methods of DNA testing. We describe the 25th case of a patient with both conditions together with a review of the literature. To our knowledge, no other patient has had such a large number of histologically proven pilomatrixomata.

Adult↗

Pyoderma gangrenosum in a child with congenital partial deficiency of leucocyte adherence glycoproteins.

Congenital deficiency of beta 2 integrin leucocyte adhesion molecules is a rare immunodeficiency and is often fatal. Neutrophils are unable to bind to ligands on the endothelium, and so cannot leave the circulation during inflammation or infection. When leucocyte adhesion deficiency (LAD) is caused by abnormally low expression of beta 2 integrins, it is termed LAD type 1. We describe a 5-year-old girl with a history of recurrent bacterial infections since early childhood who developed necrotic skin ulcers resembling pyoderma gangrenosum and a persistent circulating neutrophilia. Histologically, the lesions showed deep ulceration with a diffuse lymphohistiocytic infiltrate, but with a relative sparsity of neutrophils. Subsequent investigation revealed a complete absence of CD11a/CD18 beta 2 integrins on the surface of the patient's neutrophils, confirming the diagnosis of LAD type 1. The ulcers responded to treatment with oral prednisolone and colchicine.

CD11 Antigens↗

Leech-borne Serratia marcescens infection following complex hand injury.

Leeches are commonly used in the postoperative course of plastic surgical operations where there is venous congestion in a pedicled or free flap. They provide a temporary relief to venous engorgement whilst venous drainage is re-established. It is known that leeches can carry Aeromonas hydrophila infection, and a second or third generation cephalosporin antibiotic has traditionally been given as prophylaxis against infection. We report a new observation that leeches can carry Serratia marcescens and give rise to clinically significant infection. The implication for prophylaxis and treatment of leech-associated cellulitis is discussed.

Adult↗