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Biomedical subjects

L Flom

Publications and source records attributed to L Flom.

6 recordsLinked to original sources

Beemer-Langer syndrome with manifestations of an orofaciodigital syndrome.

We report on an infant girl with hydrops, macrocephaly, high forehead, flat face, hypertelorism, broad nasal bridge, median cleft lip and alveolar ridge, grooved palate, accessory frenula, small tongue, milia, severe rib and limb shortness, brachydactyly, talipes equinovarus, Dandy-Walker malformation, accessory spleen, unfixed mesentery, ectopic pancreas, and renal cysts. This patient resembles seven previously reported patients with the Beemer-Langer syndrome, a distinct lethal short rib syndrome characterized by hydrops, markedly short ribs and limbs, median cleft lip with or without cleft palate, flat face, and macrocephaly. Polydactyly is usually absent. Our patient's oral anomalies suggest an orofaciodigital syndrome, but the severe rib and limb shortness distinguish it from those disorders.

Adult↗

Pediatric musculoskeletal magnetic resonance imaging.

MRI has demonstrated clinical applicability and improved diagnostic capability in the musculoskeletal system to a degree rivaling its success in the neurologic system. For certain regions such as the joints, the superficial nature of these structures is ideal for the application of localized radiofrequency coils, enabling considerably improved signal, spatial resolution, and detectable contrast. The multiplanar capability is often invaluable in assessing such regions as the weight-bearing surfaces of the hips. It has been shown to be the most sensitive single test for diagnosing avascular necrosis, and has yielded very high resolution images of the internal architecture of the joints and soft tissues in an entirely noninvasive manner and without the need for the administration of exogenous contrast agents. Short TE images are quite useful in evaluating fatty background regions of interest, such as the bone marrow and subcutaneous tissue, whereas long TR and TE studies are frequently more suitable for soft tissue pathology. The lack of ionizing radiation and the continued failure of intensive research to disclose any deleterious effects at the levels used for clinical diagnostic imaging make this tool especially appealing for application to the pediatric population. With the continual development of new pulsing sequences, contrast agents, and further advances in imaging/spectroscopic correlation, magnetic resonance imaging of the musculoskeletal system is ensured of a solid role in the clinical diagnostic evaluation of the pediatric population.

Bone Diseases↗