Rhizomelic chondrodysplasia punctata: 16-year follow-up of a child from birth

A boy with rhizomelic chondrodysplasia punctata, diagnosed on the laboratory evidence of a high plasma concentration of phytanic acid and a low erythrocyte concentration of plasmalogens, has been followed from birth to the age of 16 years. The clinical pattern (somatic, skeletal and neurological) tallies with published findings in this disease. Unusual findings are the associated epilepsy, confirmed by EEG, and the long survival. CT brain scan and MRI showed cortical and subcortical atrophy but not gyral abnormalities or demyelination.

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Sommario Viene descritto il caso di un bambino affetto da condrodisplasia punctata rizomelica seguito dalla nascita fino ai 16 anni. La diagnosi clinica, efettuata in base alle caratteristiche somatiche, scheletriche e neurologiche del paziente è stata confermata dai reperti di laboratorio (alta concentrazione plasmatica di acido fitanico, bassa concentrazione di plasmalogeni eritrocitari). Come correlati inusuali del quadro clinico vanno segnalati l'associazione con epilessia e a la lunga sopravvivenza. La TAC e la RNM cerebrale hannovaltresì documentato atrofia corticale e sottocorticale ma non anomalie della girazione o demielinizzazione.

     

Errors in the measurements of T2 using multiple-echo MRI techniques. II. Effects of static field inhomogeneity

The accurate estimation of the spin-spin relaxation time T2 is an important goal in magnetic resonance imaging particularly because it can be used for quantitative tissue characterization. The spin-spin relaxation time T2 may be estimated using multiecho pulse sequences, but the accuracy of the estimate is dependent on the fidelity of the spin-echo amplitudes, which may be severely compromised by rf pulse and static field imperfections. In this paper, the effects of static field inhomogeneities are investigated. The propagation of the errors introduced by off-resonance effects are analyzed through computer simulations and analytical solutions of the Bloch equations. A series of experiments performed on a simple tissue phantom using a whole-body imaging system operating at 6.35 MHz corroborates the simulation and analytical results. For accurate measurements of T2 using a whole-body imaging system it is necessary to correct for these inhomogeneities. A correction scheme which would enable a more accurate estimate of T2 is currently under investigation.     

Comparative study of 3 therapeutic methods for Pouteau Colles' fracture. Apropos of 97 cases

Results of three therapeutic modalities used for the treatment of a homogeneous series of patients with a Pouteau Colles fracture are analyzed. Orthopedic treatment provided deceiving results. Pinning was, on the contrary, very effective, intrafocal pinning with immediate mobilization of the wrist appearing perfectly effective. Few complications were reported in contrast to plastered immobilizations. Good results were obtained in 80% of cases and functional recovery was rapid, indicating that this method represents undoubted progress when compared with conventional therapies.     

Caustic injuries to the esophagus in children

The ingestion of caustic chemical agents, usually accidentally by children, produces a wide range of injuries from minor mouth burns to necrosis of the esophagus and stomach. The type of agent, amount, concentration, and duration of exposure are the determining factors. The treatment for the average burn is fairly well standardized, but the serious injuries require prompt recognition of complications and appropriate therapy to prevent more serious complications.     

Diminished susceptibility to daptomycin accompanied by clinical failure in a patient with methicillin-resistant Staphylococcus aureus bacteremia.

We cared for a patient with methicillin-resistant Staphylococcus aureus bacteremia who experienced clinical failure with daptomycin. The failure was accompanied by progressive elevation of the daptomycin minimum inhibitory concentration during treatment. DNA fingerprinting confirmed that the minimum inhibitory concentration elevation occurred within the same strain of methicillin-resistant Staphylococcus aureus. This observation provides important new information to clinicians who adopt this promising drug for treatment of serious infections caused by methicillin-resistant Staphylococcus aureus.     

Stimulation der Granulopoese mit hämatopoetischen Wachstumsfaktoren

Die therapieinduzierte Neutropenie kann mit den Kolonie stimulierenden Faktoren (CSF) der Granulopoese (G-CSF und GM-CSF) vermieden,abgemildert oder in ihrer Zeitdauer verkürzt werden. Die CSF erm?glichen die Mobilisation h?matopoetischer Stammzellen in das Blut und deren Sammlung für die Transplantation nach Hochdosistherapie.Diese Faktoren sind daher essenziell in der Supportivtherapie maligner Erkranklungen. Der Arbeitskreis Supportivma?nahmen in der Onkologie (ASO) der Deutschen Krebsgesellschaft hat Richtlinien zur Therapie mit den Kolonie stimulierenden Faktoren entwickelt, die unterschiedlichen klinischen Situationen angepasst sind. Der vollst?ndige Text wurde 12/2001 in “Der Onkologe” publiziert [23], er ist auch auf der Homepage des ASO unter http://www.onkosupport.de zu finden [24]. Prof.Dr. H. Link H?matologie, Internistische Onkologie, Endokrinologie, Medizinische Klinik I, Westpfalz-Klinikum Kaiserslautern, Akademisches Lehrkrankenhaus der Johannes-Gutenberg-Universit?t Mainz, 67653 Kaiserslautern, E-Mail: hlink@westpfalz-klinikum.de