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Biomedical subjects

S J Woerner

Publications and source records attributed to S J Woerner.

4 recordsLinked to original sources

Olfactory neuroblastoma (esthesioneuroblastoma) in a 2-year-old boy.

A 2-year-old boy presented with sudden blindness and epistaxis resulting from olfactory neuroblastoma with intracranial extension. This disease is uncommon in children, with only 27 cases reported in patients less than 17 years of age. This article reviews the clinical and histological features of this tumor and discusses treatment. Therapy has generally consisted of surgery and radiation. Our child and 2 others treated with radiation and combination chemotherapy (including cyclophosphamide and anthracyclines) have done well with follow-ups of up to 9 1/2 years. Chemotherapy is an important form of adjuvant therapy in this disease.

Antineoplastic Combined Chemotherapy Protocols↗

Intracranial hemorrhage in children with idiopathic thrombocytopenic purpura.

Intracranial hemorrhage is a rare but life-threatening complication of childhood idiopathic thrombocytopenic purpura. We present three cases of this complication encountered at our institution, in addition to a tabulation of 15 previously reported cases. Prevention, diagnosis, and management of intracranial hemorrhage in idiopathic thrombocytopenic purpura are discussed. The importance of avoidance of antiplatelet drugs as well as the significance of the location of the intracranial hemorrhage are emphasized. Posterior fossa hemorrhages are especially dangerous because of the possibility of rapid cerebellar herniation and brainstem compression. Management of intracranial hemorrhage should be prompt and aggressive, and splenectomy should always be performed prior to any neurosurgical procedure. It is encouraging that 11 of 18 patients had a favorable outcome.

Adolescent↗

The Bowen-Conradi syndrome -- a highly lethal autosomal recessive syndrome of microcephaly, micrognathia, low birth weight, and joint deformities.

This paper describes six Hutterite children from five families who appear to have been affected by the same syndrome that was described in two brothers by Bowen and Conradi [1]. Our additional cases confirm that the major features of the syndrome include porportionate intrauterine growth retardation, microcephaly, micrognathia, a prominent nose, rocker-bottom feet, joint limitation, and failure to thrive, with death within the first year of life. Bowen-Conradi syndrome is an autosomal recessive trait and pedigree records show that all six families now known are related to each other through two couples born in the late 1700s but that there are additional earlier possible sources of the responsible gene. The differential diagnosis of this syndrome is discussed.

Abnormalities, Multiple↗

Infantile botulism.

Botulism has not traditionally been considered as occurring in infants under one year of age because they generally do not ingest foods potentially containing preformed Clostridium botulinum toxin. We report a case of infantile botulism in a 3 1/2 month old infant who presented as a "floppy baby," and discuss the probable pathobiology involved.

Botulism↗