Hepatosplenic abscesses due to Brucella melitensis: report of a case involving a child and review of the literature.

Suppurative disease of the liver and/or spleen is a rare and serious complication of human brucellosis. In the English-language literature, only nine cases have been reported, all involving adults with chronic infection. We report the case of a young child in whom abscesses of the liver and spleen developed during acute brucellosis. Brucella melitensis was cultured from an aspirate of the liver and from the bone marrow. After percutaneous drainage of the liver abscess, the patient responded to a 56-day course of antimicrobial therapy. To our knowledge, this is the first reported case of hepatosplenic abscess due to a Brucella species in a child.

A practical radiologic approach to skeletal dysplasias in infancy.

Although skeletal dysplasias occur infrequently, the recognition of these disorders is important both for the patient and for genetic counseling. Our present understanding of these diseases has come about largely as a result of continued study by a small group of individuals who have become authoritative experts. This accumulated knowledge, as well as newer methods of antenatal detection, has significantly heightened the general interest in these disorders.

Use of low osmolar contrast for gastrointestinal studies in low-birth-weight infants.

Metrizamide is a safe, water-soluble contrast medium suitable for bedside gastrointestinal studies in low-birth-weight infants. We describe our experience with 26 patients and 34 examinations. We present illustrative cases and indicate the clinical applications. Similar results are anticipated when less expensive nonionic contrast agents are approved by the Food and Drug Administration for pediatric use.

Wolman's disease. Ultrasound and CT diagnosis.

An infant with the typical clinical and radiographic findings of Wolman's disease was also examined with ultrasound and CT. Although the radiographs are distinctive, the newer modalities add another dimension of examination.

Progressive ventriculomegaly following minor intracranial hemorrhage in premature infants.

Progressive hydrocephalus following subependymal-intraventricular hemorrhages (SEH-IVH) in premature infants has been noted after moderate to severe degrees of hemorrhage. The ventricular dilatation often has been noted to resolve spontaneously, or not continue to progress after four weeks of age. 11 premature infants with moderate to marked degrees of ventricular enlargement following minor degrees of SEH-IVH have been identified over an 18-month period, and in six of these infants the dilatation continued to progress after one month of age. This sequence of events has not been well documented, and it emphasizes the need to closely follow all infants who have sustained any degree of intracranial hemorrhage. The authors recommend routine real-time ultrasound brain-scans at one month of age for all infants with SEH-IVH, and continued close observation with serial head-measurements of those with enlarged ventricular systems at that time.

Postmortem observations on beta-glucuronidase deficiency presenting as hydrops fetalis.

This study reports a case of type VII mucopolysaccharidosis (beta-glucuronidase deficiency) presenting as lethal hydrops fetalis. Skin fibroblast cultures established postmortem revealed deficient beta-glucuronidase activity. Mucopolysaccharides were stored in various cells of the brain, heart, kidney, liver, and spleen. The stages of maturation of the bones, kidneys, and brain were discrepant, the brain being the least mature organ. A delay in central nervous system maturation may account for psychomotor retardation in some patients with this enzyme deficiency.

Leprechaunism (Donohue's syndrome) in a low birth weight infant.

A 910 gm infant with leprechaunism, the smallest reported infant with this syndrome, was described. Marked discrepancies were noted between gestational age as assessed by physical examination, bone and dental roentgenographic studies, and renal histology. Radiographic and pathologic features of this syndrome were discussed. Failure to thrive characterized the course of this infant despite adequate caloric intake. The precarious nature of these infants and difficulties encountered in obtaining adequate studies to delineate the cause of this syndrome were emphasized.

Tuberous sclerosis: a case report with aortic aneurysm and unusual rib changes.

An unusual case of tuberous sclerosis with thick sclerotic ribs, thoracic and abdominal aortic aneurysms and sacrococcygeal chordoma is described. The patient has been followed for 17 years and the radiographic features of the ribs and especially the aortic aneurysms are unusual features of this condition.