Haemorrhages were observed in four wholly breastfed infants beyond the neonatal period. These infants were observed within a period of 8 weeks and showed the following characteristics: 1. Onset of bleedings was unexpected and without prior indication. 2. They were of a serious nature and involved the CNS in two children. 3. In all cases infants between 4 and 6 weeks of life were affected. 4. All infants had been wholly breastfed. 5. All were male. 6. There was a prompt improvement after administration of vitamin K or after blood or blood derivatives. Although preliminary own investigations do not indicate general lowering of vitamin-K-dependent coagulation factors in wholly breastfed infants in the postneonatal period, these 4 cases observed within a short time confirm the necessity to consider vitamin K deficiency in haemorrhages in infants in the postneonatal period. Diagnostic steps have to be initiated immediately.

In adults with vitamin K deficiency, correction of abnormal coagulation tests with vitamin K therapy cannot be expected for several hours; therefore, bleeding patients are given plasma concentrates to bridge the time before vitamin K becomes effective [1,2]. In newborns a time gap of several hours, in some instances up to 12 h, is reported [3,4]. The aim of the present study is to investigate the time interval between administration of vitamin K and, first, a significant increase of the PT value (in %) and, second, a demonstrable hemostatic effect to reverse the vitamin K deficiency bleeding with administration of vitamin K.

6 der 23 (entsprechend 26%) in Freiburg betreuten Patienten mit Hämophilie sind Anti-HIV-positiv.

Die Annahme, daß bei Neugeborenen generell ein Vitamin-K-Mangel vorliege, beruht auf einem Irrtum der Pädiatrie in der ersten Hälfte dieses Jahrhunderts.

Im Jahre 1981 wurden in den USA die ersten Krankheitsfälle des acquired immunodeficiency syndrome — AIDS — bekannt (CDC 1981). Während zunächst vorwiegend Erwachsene erkrankten, die einer der bekannten Hochrisikogruppen angehörten (Tabelle 1), wurden bereits 1983 die ersten AIDS-Fälle bei Säuglingen und Kindern beschrieben (Rubinstein 1983). Ende 1985 waren neben 15719 Jugendlichen und Erwachsenen mit AIDS auch 229 Kinder unter 13 Jahren dem CDC bekannt geworden. Der Kinderanteil unter den ca. 715 AIDS-Kranken in der Bundesrepublik wird mit 2% angegeben (Eichenlaub 1986).

Eine der Spätkomplikationen bei Hämophilen nach langjährigem Krankheitsverlauf ist die chronische Arthropathie, meist der großen Gelenke. Sowohl durch konservative wie auch aggressive Methoden wurde bislang mit unterschiedlichem Erfolg versucht, die Blutungsfrequenz zu senken und so der fortschreitenden Gelenkdestruktion Einhalt zu gebieten [5].

Vollbluttests haben sich gerade in der Pädiatrie als vorteilhaft erwiesen, da sie nur ein geringes Problemvolumen erfordern und außerdem alle gerinnungsaktiven Substanzen und deren Interaktionen erfassen. Sie sind darüber hinaus aufgrund der einfachen Probenbereitung rasch durchführbar.

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)

Die Spätform der Vitamin K-Mangelblutung wird mit zunehmender Häufigkeit diagnostiziert. Berichteten wir 1983 (SUTOR u. Mitarb., 1983) über 4 Patienten, so übersehen wir jetzt 57 Säuglinge mit Vitamin K-Mangelblutung jenseits der 1. Lebenswoche (Tabelle 1), wodurch uns die folgende Charakterisierung des Krankheitsbildes ermöglicht wird.

Seit etwa drei Jahren ist in der Behandlung der schweren Blutungsübel ein weiterer entscheidender Fortschritt erzielt worden. Maßgeblich dafür war die Verfügbarkeit von neuen, durch Hitze oder andere technische Verfahren behandelten Plasmafakto-renkonzentraten, die ein erhebliches Maß an Sicherheit für die Inaktivierung von Viren verschiedener Herkunft garantieren. Man kann wohl annehmen, daß auch das HTLV III/LAV-Virus, für die AIDS-Erkrankung ursächlich angeschuldigt, mit diesen neuen Verfahren weitgehend inaktiviert wird [14, 15].

A 14 year old boy was admitted for vomiting, anorexia, flank pain and leukocyturia/hematuria. Shortly after admission, he developed anuria and acute renal failure so that hemodialysis had to be started. Pre- and post-renal causes were excluded. There were no signs of acute glomerulonephritis; liver enzymes were normal. The 123Iodine-Hippuran scan showed a shock kidney pattern lacking tubular clearance. Renal biopsy revealed an interstitial nephritis with edema and a mixed cellular infiltration. History was empty for nephrotoxic agents except for mushroom ingestion: Five days before admission the boy ate Cortinarius speciocissimus mushrooms, the toxine of which is known to be nephrotoxic, causing irreversible renal failure in severe cases (Orellanus Syndrome). Renal function did not improve much and renal transplantation was performed after 14 months on hemodialysis. In interstitial nephritis of unknown etiology the possibility of mushroom poisoning should be considered.

Discussion about vitamin K prophylaxis has again become a matter of major concern since cases of life threatening bleeding due to late onset vitamin K deficiency have been observed in Germany. This review tries to summarize present knowledge about the clinical presentation, pathogenesis and the potential prevention of vitamin K deficiency caused bleeding in the neonatal period and infancy.

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.