Glanzmann's thrombasthenia, is one of the rarest congenital, genetically inherited platelet disorder. It has an incidence of about 1:1,000,000, but is more common in populations with increased consanguinity. Glanzmann's thrombasthenia is characterized by deficiency or dysfunction of glycoprotein (GP) lib and Ilia, which are the receptors of fibrinogen. Both sexes are equally affected. Typical m...

Cornelia de Lange is a genetic syndrome which affects between 1/10.000 and 1/60.000 neonates, but its genetic bases are still not clear. Its principal clinical characteristics are the delay in growth and development, hirsute, structural anomalies in the limbs and distinctive facial characteristic. Dental problems are frequent and include: ogival palate, micrognathia, dental malalignment, delaye...

BACKGROUND This study aimes to evaluate the failure to thrive (FTT) recurrent event over time. METHODS This longitudinal study was conducted during February 2007 to July 2009. The primary outcome was growth failure. The analysis was done using 1283 children who had experienced FTT several times, based on recurrent events analysis. RESULTS Fifty-nine percent of the children had experienced t...

disorders and infectious diseases (including immunisations), both of which must surely be of relatively frequent occurrence in general practice, receive no mention. Warning signs of possible non-accidental injury could with benefit have been included. The mythology of tongue tie is deservedly stressed but there is no mention of teething in a similar context. Management advice is often impractic...

The patient was a Hindu male child of good family, aged thirteen months and the last child of a family of ten children, of whom one child had died in the fourth month of infancy and another of Spanish influenza in its eighth month in 1918, the rest of the family enjoying quite good health. In April 1923 the patient, who seemed in quite good health up to that time, got fever and diarrhoea which ...

WARNING: Life-threatening and fatal events in infants and young children Postmarketing cases of seizures, cardiopulmonary arrest, and death in patients under the age of 3 years have been reported with use of Xylocaine 2% Viscous Solution when it was not administered in strict adherence to the dosing and administration recommendations. In the setting of teething pain, Xylocaine 2% Viscous Soluti...

Familial severe hypodontia of the permanent dentition is a rare condition. The genetics of this entity remains unclear and several modes of inheritance have been suggested. We report here an increase in the number of congenitally missing teeth after the mating of affected subjects from two unrelated Norwegian families. This condition may be the result of allelic mutations at a single gene locus...

background: this study aimes to evaluate the failure to thrive (ftt) recurrent event over time. methods: this longitudinal study was conducted during february 2007 to july 2009. the primary outcome was growth failure. the analysis was done using 1283 children who had experienced ftt several times, based on recurrent events analysis. results: fifty-nine percent of the children had experienced th...