An Arab Moslem kindred is reported in which six abnormal male fetuses were born in three closely related sibships. They had amelia, malformed head and other severe skeletal and visceral malformations. The similarities and differences between the developmental and inherent characteristics found in our patients and in families with Roberts syndrome are discussed.
Here we report on 2 sibs with immotile cilia syndrome (ICS), born to consanguineous Arab parents. Both had the Kartagener triad. In addition, one sib had polysplenia and extrahepatic biliary atresia. This observation, together with other literature reports, suggests that the occurrence of ICS with polysplenia and extrahepatic biliary atresia represent the result of a single dysmorphogenetic process. It is concluded that polysplenia and extrahepatic biliary atresia are rare and unusual manifestations of ICS.
We report the results of ultrasound studies on 11 children with Schoenlein-Henoch purpura. In 8 children the ultrasound examination was normal. In two of three patients with macroscopic haematuria, haemorrhagic cystitis, a previously undescribed finding in Schoenlein-Henoch purpura, was found. In one case the gall bladder wall and the wall of several adjacent loops of bowel were markedly thickened. Follow up examinations after clinical recovery demonstrated complete resolution of these abnormalities. Our observations demonstrate that diagnostic ultrasound may have an important role before more expensive and invasive procedures are employed in patients with Schoenlein-Henoch purpura.
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