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Biomedical subjects

J S Prendiville

Publications and source records attributed to J S Prendiville.

33 records · Page 2Linked to original sources
PubMed1989

Hereditary benign telangiectasia.

We describe a father and two daughters with hereditary benign telangiectasia. Both girls had numerous telangiectases distributed over the upper limbs, trunk, face, and vermilion border of the lips. The father displayed less extensive lesions and had been unaware of their presence. All three patients were in good health; the younger daughter had a past history of mild recurrent epistaxis. Differentiation of this benign entity from other primary telangiectatic disorders is discussed.

Adolescent↗
PubMed1989

Kindler syndrome: report of two cases and review of the literature.

We evaluated two patients with hereditary bullous poikiloderma. Both had acral bullae, generalized poikiloderma with prominent atrophy, and acral keratoses. One patient, with sporadic disease, had, in addition, urethral and subglottic stenoses, webbing of digits, and poor dentition. The other patient, whose disease was dominantly inherited, had koilonychia. The results of cutaneous histopathology, electron microscopy, and immunofluorescence mapping studies are presented. It is possible that Kindler syndrome and Weary's hereditary acrokeratotic poikiloderma are variants of the same disease.

Age Factors↗
PubMed1988

Focal facial dermal dysplasia: bitemporal lesions resembling aplasia cutis congenita.

Focal facial dermal dysplasia is the preferred designation for an inherited disorder characterized by congenital scarlike lesions on the temples. Review of the literature reveals a spectrum of associated facial anomalies that cannot be clearly divided into discrete clinical syndromes. To reconcile the apparent genetic diversity of previously reported cases, we postulate autosomal dominant inheritance with incomplete penetrance. An isolated case of a 4 1/2-month-old infant with typical bitemporal markings and otherwise normal facies is described.

Apgar Score↗
PubMed1986

Cryofibrinogenaemia and acute gangrene in systemic sclerosis.

A 49 year old man with systemic sclerosis developed ischaemia of his extremities with massive peripheral gangrene. High levels of circulating cryofibrinogen were detected in the plasma and the significance of these findings is discussed.

Acute Disease↗
PubMedDate not supplied

Pain management of junctional epidermolysis bullosa in an 11-year-Old boy.

Epidermolysis bullosa is a group of hereditary blistering disorders for which there is no definitive therapy. Wound care is an important component of management. Regular dressing changes are required to protect blistered and eroded skin, and to prevent secondary infection and sepsis. These dressing changes can be very painful for patients with extensive erosions. We report our experience of pain management in an 11-year-old boy with severe junctional epidermolysis bullosa. Amitryptiline and cognitive behavioral techniques were effective in relieving chronic pain and discomfort. Oral midazolam 0.33 mg/kg administered 20 minutes prior to baths and dressing changes substantially improved his tolerance of wound care.

Amitriptyline↗
PubMedDate not supplied

Treatment of molluscum contagiosum with oral cimetidine: clinical experience in 13 patients.

Our purpose was to determine if oral cimetidine, a histamine-receptor antagonist, might be of benefit in the treatment of extensive molluscum contagiosum in children. We present 13 pediatric patients in whom conventional treatment modalities for molluscum contagiosum were unsuccessful or difficult to apply. They were treated with a two-month course of oral cimetidine 40 mg/kg/day. All but three children who completed treatment experienced clearance of all lesions. These children had no new lesions but had persistence of several lesions. One child did not take the drug and did not clear. No adverse effects were observed. We conclude that oral cimetidine may be of benefit in the management of widespread or facial molluscum contagiosum in immunocompetent children.

Administration, Oral↗
PubMedDate not supplied

Ocular findings of incontinentia pigmenti in a male infant with Klinefelter syndrome.

A male infant with Klinefelter karyotype (47, XXY) manifested both the typical dermatologic findings of the X-linked dominant disorder incontinentia pigmenti (Bloch-Sulzberger syndrome) and ocular findings including retinal pigmentary changes, peripheral retinal avascularity, and preretinal fibrovascular proliferation. To our knowledge, this is the first reported case of incontinentia pigmenti with this specific abnormal genotype manifesting ocular findings.

Fluorescein Angiography↗