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Biomedical subjects

R Lurie

Publications and source records attributed to R Lurie.

At least 19 recordsLinked to original sources
PubMed2004

Laron syndrome (primary growth hormone insensitivity): a unique model to explore the effect of insulin-like growth factor 1 deficiency on human hair.

BACKGROUND: Classical Laron syndrome is a recessive disease of primary insulin-like growth factor 1 (IGF-1) deficiency and primary growth hormone insensitivity. Affected children have, among other defects, sparse hair growth and frontal recessions. The hair is thin and easy to pluck. Young adults have various degrees of alopecia, more pronounced in males. OBJECTIVE: The aim of the present study was to investigate the effect of primary IGF-1 deficiency on hair structure. The study sample included 11 patients with Laron syndrome--5 children (2 untreated) and 6 adults (5 untreated). Hairs were examined by light and electron microscopy. RESULTS: The most significant structured defect, pili torti et canaliculi, was found in 2 young, untreated patients. Grooving, tapered hair and trichorrhexis nodosa were found in the remainder. IGF-1-treated patients had either none or significantly fewer pathological changes compared to the untreated patients. CONCLUSION: This is the first documentation of the role of primary IGF-1 deficiency on hair structure in human beings.

Adult↗
PubMed2001

Hypotrichosis with juvenile macular dystrophy is caused by a mutation in CDH3, encoding P-cadherin.

Congenital hypotrichosis associated with juvenile macular dystrophy (HJMD; MIM601553) is an autosomal recessive disorder of unknown etiology, characterized by hair loss heralding progressive macular degeneration and early blindness. We used homozygosity mapping in four consanguineous families to localize the gene defective in HJMD to 16q22.1. This region contains CDH3, encoding P-cadherin, which is expressed in the retinal pigment epithelium and hair follicles. Mutation analysis shows in all families a common homozygous deletion in exon 8 of CDH3. These results establish the molecular etiology of HJMD and implicate for the first time a cadherin molecule in the pathogenesis of a human hair and retinal disorder.

Adolescent↗
PubMed1996

Trichorrhexis nodosa: a manifestation of hypothyroidism.

A 23-year-old woman presented with diffuse white hair nodules, hair fragility, and the inability to grow long hair. Examination of specimens under the light microscope showed the morphologic characteristics of trichorrhexis nodosa. Results of tests revealed hypothyroidism. Treatment with a daily dose of 0.1 mg L-thyroxine sodium for six months restored euthyroidism and concurrently normalization of the hair defect was observed. The corrected hair defect observed after replacement therapy may point toward a causal relationship between hypothyroidism and trichorrhexis nodosa. To the best of our knowledge, this is the first report of trichorrhexis nodosa associated with hypothyroidism.

Adult↗
PubMed1996

Acquired pili torti--a structural hair shaft defect in anorexia nervosa.

The hair of seventeen girls aged 13 to 19 years with anorexia nervosa (AN) was studied and compared with the hair of fifteen healthy girls aged 13 to 18 years with no complaints of hair loss but with similar habits of hairstyling and care. Light microscopic examination of the hair revealed no defect in the control group. In the AN group, fourteen patients (82.3 percent) had pili torti, an acquired hair shaft defect that has not yet been described in association with this disease. We estimate that the hair defect was due to malnutrition combined with ingestion of exaggerated amounts of yellow vegetables and vitamin supplements, causing a significant increase in levels of serum carotene, retinyl esters, retinol, and retinoic acid. We suggest a possible link between excess vitamin A and pili torti in patients with AN.

Adolescent↗
PubMed1995

Helical hairs: a new hair anomaly in a patient with Netherton's syndrome.

A new hair shaft defect, helical hair, is presented. This hair abnormality was found to accompany trichorrhexis invaginata and pili torti in an infant with Netherton's syndrome. The patient's main clinical features included erythroderma since birth, failure to thrive, recurrent infections, hepatosplenomegaly, lymphadenopathy, eosinophilia, hypergammaglobulinemia, and high serum IgE levels. A possible mechanism for the formation of helical hair is reviewed.

Child, Preschool↗
PubMed1992

Treatment with bifonazole shampoo for seborrhea and seborrheic dermatitis: a randomized, double-blind study.

Forty-four patients with seborrhea and seborrheic dermatitis of the scalp were treated with either bifonazole shampoo (22 patients) or the vehicle shampoo (22 patients) in a randomized, double-blind vehicle-controlled study. The patients were instructed to wash their scalps 3 times weekly for 6 weeks and were examined every 3 weeks. Responses were evaluated by clinicians using a clinical grading of scaling, erythema and overall improvement, and also by the patients, who assessed pruritus and overall improvement, using a scale of 0 to 3. The improvement following the bifonazole shampoo was found to be significantly greater than that achieved with the vehicle shampoo in regard to the clinical evaluation of scaling (p = 0.01) as well as patient evaluation of pruritus (p = 0.008) and overall improvement (p = 0.03). No major adverse side effects were recorded.

Adolescent↗
PubMed1991

Esophageal involvement in pemphigus vulgaris: a clinical, histologic, and immunopathologic study.

Eleven newly diagnosed patients and one patient with pemphigus vulgaris who relapsed underwent endoscopy of the upper gastrointestinal tract. Three patients had blisters or erosions and two had longitudinal lines of erythema in the esophageal mucosa. In four patients histopathologic examination showed findings of pemphigus vulgaris but direct immunofluorescence was positive in all patients. This study demonstrates that the immunopathologic disturbance in pemphigus vulgaris involves the entire length of the esophagus, although only some patients have clinical or histologic involvement. To the best of our knowledge this is the first histopathologic and direct immunofluorescence study of esophageal involvement in patients with untreated pemphigus vulgaris.

Adult↗
PubMed1990

Esophageal involvement in pemphigus vulgaris: report of two cases and review of the literature.

Two patients with esophageal involvement of pemphigus vulgaris are presented, who were diagnosed histopathologically and by direct immunofluorescence. In most dermatological textbooks the possibility of esophageal involvement is not mentioned. In the English medical literature 11 cases have been reported. It seems that if upper gastrointestinal endoscopy had been done on a routine basis it might have shown that esophageal involvement is more widespread than present findings indicate.

Aged↗
PubMed1990

A possible case of drug-induced familial pemphigus.

Two sisters developed pemphigus vulgaris and pemphigus erythematosus within 3 years. The diagnosis was confirmed by clinical, histologic and immunofluorescent antibody studies. One of the sisters experienced a common cold before the pemphigus developed and displayed a positive macrophage migration inhibition (MIF) test to a combination drug compounded of paracetamol, caffeine, chlorpheniramine maleate and phenylephrine HCl, which she had received 2 weeks prior to the appearance of the cutaneous lesions. It is suggested that her pemphigus was triggered by the drug. Although the patient had a strong genetic and familial predisposition to pemphigus, her clinical symptoms did not become evident until they were activated through an exogenous factor, namely, the causative drug. This case offers an example of a possible interaction between endogenous, genetic factors, and exogenous, triggering factors in the development of full-blown disease.

Acetaminophen↗
PubMed1988

Temporomandibular joint ankylosis in rheumatoid arthritis. A case report.

Rheumatoid arthritis can result in ankylosis of the temporomandibular joint. The objectives of treatment are to maintain ascending mandibular ramus height, improve joint function and prevent occlusal-facial deformity. These are achieved by the use of a glenoid fossa-condylar total joint replacement prosthesis.

Ankylosis↗
PubMed1987

Haemorrhage from the maxillary artery. A case report.

Haemorrhage from the maxillary artery can be life-threatening. The literature related to ligation of the external carotid artery at various levels to control such haemorrhage is reviewed and a case presented where a severe haemorrhage from the maxillary artery was controlled by ligation of the external carotid artery distal to the posterior auricular/occipital trunk.

Adult↗
PubMed1984

Effects of water and saline irrigation during bone cutting on bone healing.

The healing of standardized bony defects prepared in the mandibles of 24 vervet monkeys with a rotating bur uncooled, or cooled with saline or water, was studied. The experiment showed no difference in healing after 56 days irrespective of the coolant used or whether the bone was cut with or without a cooling agent.

Animals↗