an investigation was carried out on a young unrelated couple, both 29 years old, with two boys and three girls, 3 months to 8 years old. one boy and the mother were healthy, the other four children and the father were affected. the family, originally from the district of nour, in northern iran, near the caspian sea, was first counseled at the genetic clinic in tehran in 1984. several trips were...

thymoma is the most common neoplasm of anterior mediastinum with incidence peaks during the fourth, fifth and sixth decades of life. two-thirds of patients are asymptomatic and identified accidentally by chest x-rays . of the total, 40-70 % of patients have one or more parathymic syndromes of which myasthenia gravis (mg) is the most common and reported in 10-50% . gullain-barre syndrome (gbs) h...

conclusions there was a significant association between pms scores and the prevalence of metabolic syndrome. further studies are needed to confirm and validate the relationships between lipid profile abnormalities and metabolic disorders with pms. objectives this study was designed to investigate the association of hormonal and metabolic factors with pms among iranian women of reproductive age....

Male mice emit ultrasonic vocalizations (USVs) towards females during male-female interaction. It has been reported that USVs of adult male mice have the capability of attracting females. Although the waveform pattern of USVs is affected by genetic background, differences among strains with respect to USV and the effects of these differences on courtship behavior have not been analyzed fully. W...

We report a six year old male with mental retardation, postaxial polydactyly and syndactyly, atrichia congenita totalis, severe seborrhoeic dermatitis, recurrent staphylococcal skin sepsis, and Perthes' disease of the hip. His birth may have resulted from an incestuous mating.

Pachyonychia congenita is a rare genodermatosis that can affect the larynx. Laryngeal obstruction is very unusual with only a few cases reported. A 2-year-old girl presented with typical clinical features of pachyonychia congenita shortly after birth. At age 9 months, following an upper respiratory infection, she developed stridor and hoarseness and was found to have severe laryngeal obstructio...

Pachyonychia congenita type II is an autosomal dominant inherited rare genodermatosis characterized by dystrophic wedge shaped thickened nails with subungual hyperkeratosis, symmetric palmoplantar keratoderma, steatocystoma multiplex. Here we report a 23-year-old male with characteristic features of dystrophic nails, palmoplantar keratoderma, steatocystoma multiplex, follicular hyperkeratotic p...

Pachyonychia congenita (PC) is a rare genodermatosis which may be associated with painful, focal hyperkeratosis on the soles. Plantar sweating at high ambient temperatures increases the blistering of the callosities. We report three patients with PC who had great problems in walking, especially during summer time. They were treated with intracutaneous plantar injections of botulinum toxin type ...

severe overgrowth and tallness is very rare in human beings. the most common cause is gigantism due to the excessive secretion of the growth hormone, especially, before the closure of long bones’ epiphyseal growth plates. there are other rare disorders that are categorized on overgrowth syndromes. herein, we report an extremely rare, or even perhaps a unique, patient from iran. the clinical and...