Pulmonary embolism and portal vein thrombosis in an immunocompetent adolescent with acute cytomegalovirus hepatitis.

Cytomegalovirus infection is usually asymptomatic or resembles infectious mononucleosis with fever, pharyngitis, arthralgias, lymphadenopathy, and atypical lymphocytosis. Even though primary CMV infection is usually self-limited in healthy individuals, significant complications can develop in immunocompromised patients. Venous or arterial thromboembolic phenomena are uncommon, yet very serious complications of CMV infection.

Rectal strictures in Crohn's disease and coexisting perirectal complications.

Background: The significance of the presence of rectal strictures in Crohn's disease has not been well studied. The aim of this study was to examine patients diagnosed with Crohn's disease associated with rectal strictures and to describe co-existing manifestations of perianal disease (abscesses, fistulae, or skin tags) and strictures located elsewhere in the colon or small intestine.

Methods: A cohort of 70 Crohn's disease patients with rectal strictures were compared with controls without rectal strictures matched for age, gender, and duration of disease.

Molar tooth sign in Joubert syndrome: clinical, radiologic, and pathologic significance.

Joubert syndrome is a rare autosomal-recessive condition characterized by early hyperpnea and apnea, developmental delay, and truncal ataxia. We previously described key ocular motor signs in Joubert syndrome and the molar tooth sign resulting from dysplasia of the isthmic segment of the brain stem, superior cerebellar peduncles, and vermis. In this study, we obtained clinical and developmental data in 61 cases, and radiologic data in 46 of these, to determine the prevalence of the molar tooth sign in a large sample, and to ensure that magnetic resonance images obtained for study were representative of the Joubert syndrome population at large.

Central nervous system structure and function in Sturge-Weber syndrome: evidence of neurologic and radiologic progression.

Sturge-Weber syndrome is characterized by the presence of a port-wine nevus, epilepsy, stroke-like episodes, headache, and developmental delay. We studied 20 cases to test the hypothesis that decreased cerebral blood flow alters neurologic function by affecting cellular glucose metabolism. Group A consisted of 10 patients with a mean age of 1.

High prevalence of bihemispheric structural and functional defects in Sturge-Weber syndrome.

Abnormal cerebral venous drainage is associated with hypoxia and glucose deprivation, which can account for progressive neurologic deterioration in Sturge-Weber syndrome. Although developmental delay is common in Sturge-Weber syndrome, bihemispheric calcification is uncommon. Computed tomography (CT) and magnetic resonance imaging (MRI) were used to study the neuroanatomy, while single photon emission computed tomography (SPECT) was used concurrently to evaluate perfusion and glucose metabolism using 99mTc hexamethylpropyleneamine oxime (HMPAO) and [18F] fluorodeoxyglucose (FDG), respectively.