Successful isolated liver transplantation in a child with atypical hemolytic uremic syndrome and a mutation in complement factor H.

A male infant was diagnosed with atypical hemolytic uremic syndrome (aHUS) at the age of 5.5 months. Sequencing of the gene (CFH) encoding complement factor H revealed a heterozygous mutation (c.3644G>A, p.Arg1215Gln). Despite maintenance plasmapheresis he developed recurrent episodes of aHUS and vascular access complications while maintaining stable renal function. At the age of 5 years he received an isolated split liver graft following a previously established protocol using pretransplant plasma exchange (PE) and intratransplant plasma infusion. Graft function, renal function and disease remission are preserved 2 years after transplantation. Preemptive liver transplantation prior to the development of end stage renal disease is a valuable option in the management of aHUS associated with CFH mutations.

[Therapeutic local anesthesia in backache]. / Therapeutische Lokalanästhesie beim Kreuzschmerz.

Low back pain for which operative therapy is not indicated, can prove resistant to treatment even with the aggressive application of conservative measures and the use of suitable drugs. Here, local anesthesia can often have a decisive influence on pain. On account of possible complications, particular consideration must be given to pharmacological aspects. Moreover, certain minimum demands must be met by the equipment and personnel staffing of a pain clinic, and also the possibilities for monitoring the patient. Within the framework of an interdisciplinary pain clinic, the various blockade techniques employed for therapeutic local anesthesia provide suitable possibilities for combatting this type of pain.

[Capsular ligament injuries of the knee joint. Significance of anamnesis and findings]. / Kapselbandverletzungen des Kniegelenks. Deutung der Anamnese und Befunderhebung.

Despite the enormous developments in imaging procedures, the patient's history and clinical examination remain the basis for the diagnosis of fresh injuries to the knee. The description of the mechanisms of the accident as revealed by the case history already provides important clues. The clinical examination must cover all the anatomical detection of lesions of individual parts of the capsular ligaments. With an appropriate knowledge of anatomy, and adequate experience in performing the examination, the general physician (with no specialized orthopedic knowledge) can obtain a good idea of the nature and severity of the injury.

[MRI in osteochondrosis dissecans]. / MRI bei der Osteochondrosis dissecans.

The osseous manifestations of osteochondrosis dissecans are well demonstrated by conventional and computerised tomography. Beyond that, magnetic resonance imaging (MRI) is effective in evaluating the vitality and loosening of an osseous dissecate. Subchondral cavities and cartilaginous defects are detected with high accuracy. Further, MRI seems to be a useful method in childhood to differentiate a variant irregularity of the osseous articular surface from definite osteochondrosis dissecans.

[Imaging of the internal structures of the knee by MR tomography]. / Die Darstellung der Kniegelenksbinnenstrukturen durch die MR-Tomographie.

With magnetic resonance imaging it is possible to demonstrate the cruciate ligaments of the knee reproducibly without the need for invasive measures. Prerequisites for this are high-resolution coil systems and a stratum thickness of 5 mm or less. Examination of 29 patients and volunteers showed that normal cruciate ligaments can be delineated as homogeneous zones of low signal intensity and high contrast from the surrounding fatty tissue, while in cases of injury to the cruciate ligaments an interruption of the ligament structure, inability to detect them in anatomic position, or an intermediate signal is observed. In 13 cases operated on, with 12 injuries of the cruciate ligaments, the MR findings corresponded to findings at surgery. In 6 patients who had previously had cruciate ligament plasties there correlation with clinical findings was only partial.

Total urinary estrogens in complicated pregnancies.

One hundred fifty-seven pregnancies complicated by different degrees of diabetes, toxemia, and hypertension were studied with serial urinary placental estrogen determinations. A simple and fast method for total placental estrogen determination was used. The level of total estrogen excretion was related to Apgar score in cases of class B diabetes, severe toxemia, and also in moderate toxemia when estrogen excretion was falling. Mean estrogen levels did not differ as a function of severity of diabetes. Levels did differ with severity of toxemia; however, only the difference in mean estrogen excretion between mild and severe toxemia was significant. Estrogen excretion was very low in hypertension but was not related to Apgar score. This study concludes that total urinary estrogens constitute only a single parameter necessary in the management of high-risk pregnancies.