Vitamin K prophylaxis in the newborn.
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Biomedical subjects
Publications and source records attributed to W Künzer.
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Tick-borne encephalitis (central European encephalitis) and Lyme disease (erythema chronicum migrans) are diseases mainly transmitted by ticks of the Ixodes ricinus species. The tick-borne encephalitis, a virus infection, can produce severe encephalitic symptoms. There is no specific treatment but the infection can be prevented by active or passive immunization. Lyme disease is caused by a spirochete (Borrelia burgdorferi). Due to the variety of often uncharacteristic symptoms this infection is not easily recognized. However, early diagnosis and antibiotic treatment are necessary to prevent chronic damage to skin, joints, heart or nervous system.
Tick-borne encephalitis (central european encephalitis) and Lyme disease (erythema chronicum migrans) are diseases mainly transmitted by ticks of the ixodus ricinus species. The tick-borne encephalitis, a virus infection, can produce severe encephalitic symptoms. There is no specific treatment but the infection can be prevented by active or passive immunization. Lyme disease is caused by a spirochete (borrelia burgdorferi). Due to the variety of often uncharacteristic symptoms this infection is not easily recognized. However, early diagnosis and antibiotic treatment are necessary to prevent chronic damage to skin, joints, heart or nervous system.
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The terms "consumption coagulopathy" and "disseminated intravascular coagulation" are used synonymously, though the former expression refers to the process of consuming the haemostatic potential, whereas the latter is based upon the generalized formation of microthrombi. Both terms apply to an acquired disturbance of blood clotting leading to an increased turnover of coagulation factors and platelets by which the production sites are being exhausted. Such a process is triggered off by generalized activation of the haemostatic system: after a period of hypercoagulability, haemostasis changes into hypocoagulability with subsequent haemorrhagic diathesis. Additionally, the generalized activation of the haemostatic system leads to a formation of microthrombi in the microcirculation. Since consumption coagulopathies are bound to be secondary disorders, any underlying disease prone to lead to disseminated intravascular coagulation, should be treated as early and as intensively as possible. Solely by this and by restoring circulatory functions impaired by the underlying disease, it is possible in the majority of cases to stop the consumptive coagulopathy and to repair its sequelae. The shock frequently going along with a consumption coagulopathy requires immediate therapy: correction of hypothermia, treatment of acid-base and electrolyte disorders as well as fighting against hypovalaemia, anuria, and uraemia. Dextran does not serve only as plasma expander, but also corrects hypercoagulability and improves the rheological qualities of circulating blood. If these measures fail to stop the consumptive reaction of blood coagulation and/or fail to restore microcirculation in vital organs, indication for the use of anticoagulants or fibrinolytic drugs is given.(ABSTRACT TRUNCATED AT 250 WORDS)
A male premature triplet with healthy siblings was born with characteristic symptoms of a Rossi syndrome: pterygiums at neck, axillae, elbows and in a moderate way at hips and knees with inhibition of extension of the affected joints, dislocation of the right hip, club-feet, cutis laxa et hyperelastica, facial dysmorphism, double pelvis renalis with ureter duplex on the right side, kryptorchidism, hypoplastic genital. The boy died after 38 days because of a haemorrhage of the lungs. An intrauterine immobilisation is discussed as cause of the syndrome.
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A 12.5 year old girl was admitted to hospital with the typical signs of hemolytic uremic syndrome, and systemic lupus erythematodes as well. On the basis of clinical, blood chemistry, and histological findings we assumed an hemolysis-induced form of hemolytic-uremic syndrome as the most likely pathogenic mechanism. The child also suffered from congenital IgA-deficiency and produced an inhibitor against coagulation factor VIII. Congenital IgA-deficiency, systemic lupus erythematodes, inhibitor-induced hemophilia and hemolytic uremic syndrome are suggested to form a pathogenic sequence.
Xipho-omphalopagus twins with a pericardial bridge, extended liver tissue union and considerable intestinal herniation from one abdominal cavity to the other were separated successfully at the age of three months. Special diagnostic procedures including cardiac and abdominal sonography, catheterism of the umbilical vein with portal angiography, radionucleotide liver and bile duct imaging and separate oral glucose tolerance tests provided important information for perioperative and surgical patient management. Relevant items for determination of the favourable data and method of surgery are discussed.
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A new senitive and specific micromethod for determination of UDP-glucuronic acid is described Extracts from 2.5 mg of liver are incubated with twice washed guinea pig microsomes (as a source of UDP-glucuronyl transferase) and [14C]p-nitrophenol. The content of UDP-glucuronic acid is calculated from the [14C]p-nitrophenyl glucuronide/[14C]p-nitrophenol radioactivity ratio and the known amount of introduced [14C]p-nitrophenol. These values are corrected for losses of UDP-glucuronic acid by a calibration experiment, containing in addition to the aforementioned constituents, a known amount of UDP-glucuronic acid. The mean concentration of UDP-glucuronic acid in mice liver was found to be 171 mumol/kg wet weight.
Using the paper-radioimmunosorbens test (PRIST) normal values were obtained from 200 non-allergic children. The IgE level was aged-dependent, in part with significant differences between various age groups. These normal values were compared with those in children with seborrhoeic dermitis, constitutional neurodermatitis, parasitoses, urticaria, Quincke oedema, Schönlein-Henoch purpura, pityriasis rosea, multiform exudative erythema, erythema nodosum and infantile papular acrodermatitis. Half-life of IgE in neonates was 16.2 hours.
Among twelve patients with homozygous alpha1-antitrypsin deficiency (Pi-type Z), five cases of infantile liver disease were diagnosed. The course of the disease was extremely variable; only one patient died of liver cirrhosis at the age of fourteen. In four cases the clinical, biochemical and histological (2 cases) findings became normal over a follow-up period of one to fifteen years. The results of these observations demonstrate that in alpha1-antitrypsin deficiency even when associated with proven liver disease the prognosis need not be unfavorable.
Hypercoagulation and intravascular coagulation developed a nearly 12-year-old girl with a one-year history of typical anorexia. Because of extreme cachexia she had been treated with numerous drugs elsewhere, among them ACTH an infusions of amino-acids. At the end of the second week of hospitalisation acute venous thrombosis of the right leg developed which was treated with heparin. Severe disseminated intravascular coagulopathy and thrombosis of the right leg were diagnosed on admission, the previously administered heparin was neutralised and streptokinase given for 60 hours, heparin was then given for several days, and the cachexia then treated. Both the local and general condition of the patient having been cured, the emotional state has also very much improved during the last year.
Seven patients with classic hemophilia A had alteration of primary hemostasis after treatment with lyophilized antihemophilic globulin (LAHG). The following test results, which were normal before treatment, became abnormal after treatment: bleeding time, bleeding intensity, and platelet adhesiveness. In two patients, the fibrin-fibrinogen-degradation products increased to more than 40 microgram/ml. In three patients, the bleeding symptoms became worse with LAHG therapy although no inhibitor against Factor VIII was demonstrated. In one of these patients, the bleedings symptoms disappeared when the use of LAHG was discontinued and prednisone was given; at the same time, the altered primary hemostasis returned to normal. In the remaining two patients, prednisone did not have any effect. In these two patients, however, the bleeding stopped, and the bleeding time became normal immediately after freshly prepared blood-group compatible cryoprecipitate was given.
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Alpha-1-antitrypsin (alpha-1-AT) is a potent protease inhibitor. Its deficiency predisposes to serious diseases such as "neonatal hepatitis" and "obstructive pulmonary emphysema". Due to the existence of multiple codominant alleles at one single locus, there are several genetic variants from alpha-1-AT. In homozygous persons the protease inhibitor type (Pi type) MM is prevailing, in heterozygous persons the Pi types MZ and MS. So far one knows at least 24 different alleles. Their phenotypes differ as well in their electrophoretic position as in the protein concentration of the serum. Pi type MM guarantees a normal concentration of alpha-1-AT in the serum, whereas Pi type ZZ causes of serious alpha-1-AT deficiency which bears a particularly high risk of disease.
Case report on a 14 months old infant who swallowed a glas splinter while being fed from a jar with commercially prepared strained baby food (fruits). A perforating laceration in the upper posterior wall of the esophagus gave rise to a purulent inflammation of the paraesophageal tissue within 24 hours requiring incision and drainage. The suspected splinter was not found, neither by two esophagoscopies nor by the incision. Therefore the stools were collected for six days and carefully screened with a special technique. Thus a tiny curved glas splinter was detected fitting exactly into a corresponding defect of the upper inner rim of the used jar.