Biomedical subjects
Publications and source records attributed to H Kemperdick.
A case of Pierre Robin-Syndrome with associated rib gap defects is reported. Rib defects are described still now only in childs with micrognathia. Respiratory embrassement may be caused.
Mesomelic dwarfism of the Langer type is described. This is characterised by shortening and bending of the middle portions of the extremities. This is compared with three cases of "mesomelic" changes in the forearm. The classification of this condition is discussed.
Two own observations of pycnodysostosis initiated our description of this disease. Inherited as an autosomal recessive trait the syndrome is characterized by a medium degree generalized osteosclerosis, open craniial sutures and fontanels until adult lige, hypoplastic mandibles with deficiency of the angle, prominence of the forehead and occiput, hypoplasia of the paranasal sinuses, commonly observed dysproportionate dwarfism, acro-osteolysis and increased bone fragility. Contrary to osteopetrosis the prognosis is good.
The differential diagnosis of Meckel's syndrome and Ellis-van-Creveld's syndrome with encephalocele is summarized utilizing two own observations. Radiographic criteria are of special importance. In EvCS typical signs of chondrodysplasia are present whereas in MS we found pelvic changes similar to Down's syndrome and striking rhomboid deformities of the tibiae. Clinically relevant is ectodermal dyplasia in EvCS, especially, when cystic kidneys in MS are not palpable.
Two boys aged up to 2 weeks suffered from enterobacter-sepsis. In both cases osteomyelitis developed in spite of treatment with Gentamycin or Gentamycin combined with Chepazolin. Both children were, taking accont of the risks, then treated with Chloramphenicol (100 mg/kg body weight/24 hours) and the first patient also, for a short time, with tetracyclin. In the second patient we saw a marrow depression dependent on Chloramphenicol and its dosage which disappeared rapidly, when the drug was withheld.
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A 12 year-old female patient suffering from multifocal Ewing's Sarcoma underwent bone marrow transplantation in March 1992. The donor was the patient's HLA-identical brother. On day 38 following BMT, an occluding catheter thrombosis of the superior vena cava was diagnosed. Lysis therapy using rt-PA was initiated. During therapy, serious bleeding occurred and administration was temporarily discontinued. Normalisation of previously high fibrinogen levels during an acute phase reaction was seen concomitantly with systemic fibrin and probably also fibrinogen fragments as demonstrated using the Western blot technique. Lysis therapy resulted in regained catheter patency, while thrombosis of the superior vena cava persisted. The reduction in the need for the transfusion of packed thrombocytes following lysis was seen as being a positive result. The use of rt-PA following BMT should be carefully weighed against the risks and requires careful patient observation. Due to the systemic fibrinolytic and fibrinogenolytic effects combined with mucositis and thrombocytopenia as a result of transplantation therapy, a high risk of bleeding complications seems likely.
A 9.6-year-old patient was treated exclusively with an oligopeptide diet at initial diagnosis and at first relapse of Crohn's ileocolitis. The patient achieved complete remission in both episodes. Control radiologic examinations 14 months after diagnosis revealed complete disappearance of radiologic manifestations of Crohn's disease.