[Recent information on tick-borne diseases (II)]. / Neues über Zeckenkrankheiten (II).

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.

[Recent information on tick-borne diseases (I)]. / Neues über Zeckenkrankheiten (I).

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.

[Therapy of consumption coagulopathies]. / Therapie der Verbrauchskoagulopathien.

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)

[Arthrogryposis multiplex congenita with associated abnormalities. Case report of a fatal course in a premature triplet]. / Arthrogryposis multiplex congenita mit assoziierten Missbildungen. Kasuistischer Beitrag über den letalen Verlauf bei einem Drillingsfrühgeborenen.

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.

[Terminal renal failure caused by interstitial nephritis following mushroom poisoning by Cortinarius speciocissimus]. / Terminale Niereninsuffizienz durch interstitielle Nephritis nach Pilzvergiftung durch Cortinarius speciocissimus.

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.

[Beta 2 microglobulin in the serum as a parameter of glomerular kidney function in the first days of life]. / beta 2-Mikroglobulin im Serum als Parameter der glomerulären Nierenfunktion in den ersten Lebenstagen.

Though serum creatinine is a very reliable parameter for predicting glomerular filtration rate in infancy, this does not apply to the first hours and days of life. As there is no placental barrier for creatinine, serum creatinine at birth reflects maternal renal function at the moment of delivery and, during the first days of life, establishment of the steady state condition between creatinine serum level and actual infantile glomerular filtration rate. Serum creatinine levels of cord blood and maternal blood in term and preterm infants of 25-42 weeks gestational age are almost identical (maternal blood 0.82 +/- 0.34 mg-%, cord blood 0.87 +/- 0.34 mg-%, n = 77, r = 0.94), whereas there is no correlation between maternal and infantile beta 2-microglobulin concentrations (maternal blood 2.1 + 1 mg/1, cord blood 3.3 +/- 0.6 mg/l, n = 78, r = 0.05). There is no free diaplacental exchange for this low molecular weight protein. The determination of cord blood beta 2-microglobulin levels therefore predicts the newborn's renal function independently of the mother's. It is possible to differentiate between prenatal and perinatal genesis of renal damage in case of renal failure in the newborn, and to study the elimination of creatinine preloading in maternal renal insufficiency. Although we are not yet able to give an exact quantitative prediction of glomerular filtration rate by determining beta 2-microglobulin we believe it to be the best parameter of glomerular renal function in this age-group.

[Hemolytic uremic syndrome following juvenile lupus erythematodes disseminatus]. / Hämolytisch-urämisches Syndrom im Gefolge eines kindlichen Lupus erythematodes disseminatus.

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.

Diagnostic procedures leading to successful separation of xipho-omphalopagus twins.

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.

Cerebral infarction in term neonates: diagnosis by cerebral ultrasound.

Cranial ultrasound (US) through the newborn's open fontanelle can diagnose not only intracerebral hemorrhages but also diffuse and localized hypoxic-ischemic encephalopathies. Sonographically, it was possible to distinguish between different courses of cerebral ischemia in seven neonates: ischemic infarction, usually in the area of the middle cerebral artery: borderline infarction; transient ischemia. The patients showed lateralized seizures during the first days of life with a corresponding focus in the electroencephalogram (EEG). Computed tomography showed areas of partially reduced density corresponding to the regions of increased echogenicity in ultrasound. The course was various; prognosis was good except in one patient. Etiologically, embolism, thromboses or hypoxemia were responsible for cerebral infarction. In some cases secondary bleeding ensued. The prognostic value of cerebral lesions was dependent on the involved area, gestational age, and any concurrent hypoxic cerebral damage.

[Need for the determination of chloramphenicol levels in the treatment of bacterial-purulent meningitis with chloramphenicol succinate in infants and small children]. / Die Notwendigkeit von Chloramphenicol-Spiegelbestimmungen während der Behandlung bakteriell-eitriger Meningitiden bei Säuglingen und Kleinkindern mit Chloramphenicol-Succinat.

17 cases of purulent meningitis in 15 children, aged 1 day to 5 years (median 8 months) were treated with continuous i.v. infusion of chloramphenicol succinate. Free chloramphenicol in serum and cerebrospinal fluid (C.F.) was assayed by high performance liquid chromatography (HPLC). CF chloramphenicol levels averaged 45 +/- 14% of the serum level. Out of 16 patients only five received the usually recommended dosage. In three others because of initially or progressively high serum levels the dose had to be diminished. In eight others because of subtherapeutic levels the dose had to be raised. The highest dose (390 mg/kg body weight/d) was required in a 2 month old boy. He was shown to have a clearance rate for free chloramphenicol considerably higher than has been reported so far. Maturation of the metabolism could be observed in a small-for-date newborn who acquired a grey baby syndrome during the treatment of his first meningitis. Several weeks later he required exactly the recommended dose to reach therapeutic chloramphenicol levels. As a consequence of these observations we strongly recommend meticulous drug monitoring of chloramphenicol in order to meet the large biological variations seen particularly in neonates and young infants in their capacity to reach and maintain therapeutic serum levels.

[Physiology of hemostasis in newborn infants. I]. / Physiologie der Hämostase der Neugeborenen. Teil I.

Ever since neonatal hemostasis has been studied systematically, a great number of single data und laboratory parameters have been collected which all differ more or less distinctly from results gained in adults. This has been leading repeatedly to the conclusion that the hemostasis in newborns is still immature or somehow insufficient. At least in healthy and term infants this does not apply; each pre- and postnatal stage of development has its own optimally functioning hemostasis changing with age due to respective physiological peculiarities which nevertheless may gain considerable pathogenetic significance for preterm or sick newborns. A good example are for instance the vascular structures of the periventricular germinal matrix in prematures born during the critical period around the 24th and 34th week of gestation. This assumption being in agreement with an important principle of developmental physiology is proven in this paper particularly for platelets and plasmatic coagulation.

[Pathology of hemostasis in newborn infants. II]. / Pathologie der Hämostase der Neugeborenen. Teil II.

Already in newborns almost all congenital and acquired disorders of hemostasis can be encountered. Especially gestational age and developmental peculiarities of hemostasis influence the incidence of the different causes for hemorrhages. Due to laboratory progress the well-known question of vitamin K deficiency and related bleeding has again become a point of interest and can be answered more clearly now than some years ago. Other significant disturbances of neonatal hemostasis are disseminated intravascular coagulation, intracranial hemorrhages, and the thrombocytopenias of the newborn. Disseminated intravascular coagulation is a pathogenetically important and frequent complication of numerous diseases in term as well as particularly in preterm infants. Ultrasonography gave new information about frequency and prognosis of intracranial hemorrhages in affected newborns. Finally, qualitative and quantitative disorders of the platelets present always a true challenge for the neonatologist in terms of differential diagnosis and differential therapy.

[Spinous process osteomyelitis of the thoracic vertebrae 10 and 11 in a newborn infant]. / Dornfortsatzosteomyelitis der Brustwirbel 10 und 11 bei einem Neugeborenen.

A case of a newborn with osteomyelitis of the spinous processes 10 and 11 is presented. The first clinical sign was a dorsothoracal abscess. Radiologically a destruction of the 10th, later also the 11th spinous process could be demonstrated. Staphylococcus aureus was isolated. The clinical course was mild. The pathogenesis of the disease is discussed. Such a case of newborn osteomyelitis has apparently not been described in the literature.

[Transient erythroblastopenia]. / Passagere Erythroblastopenie.

5 patients 10 to 20 months old with severe normochromic, normocytic anemia and reticulocytopenia are reported. All patients recovered within 10 days to 5 weeks. No steroid therapy was given. Failure to recognize the clinical entity leads to unnecessary diagnostic and therapeutic procedures including the transfusion of blood.

Combined autoimmune neutro- and thrombocytopenia.

Two children aged 3 and 11 years are described in whom a combined autoimmune neutro-and thrombocytopenia developed in association with pronounced cell-membrane bound IgG. These latter antibodies were detected by the unlabelled immunoperoxidase (PAP) method. Prednisone therapy proved to be beneficial.

Plasma elimination of antithrombin III (heparin cofactor activity) is accelerated in term newborn infants.

Antithrombin III (AT III) levels are markedly increased in newborn infants following exchange transfusion with adult blood, and subsequently return to pre-exchange values. This transient rise in AT III (heparin cofactor activity), was used to estimate its plasma elimination half-life. AT III activities were measured serially, before and after double-volume exchange transfusions with heparinised blood in newborn infants requiring therapy for severe hyperbilirubinaemia. The plasma elimination half-life of AT III activity was calculated to be 3.9 +/- 1.4 h (mean +/- SEM). Compared with published data on the kinetics of AT III infusions in adults, the neonate has a considerably accelerated turnover. This finding has important implications for the design of future therapeutic trials of AT III concentrates and provides further evidence that plasma proteins, including components of the coagulation system, appear to have different kinetics in the neonatal period.

Serum C-reactive protein in early diagnosis of bacterial infections in premature infants.

Serum C-reactive protein (CRP) is known to be produced by full-term infants and children in many diseases causing severe inflammation. We examined the usefulness of CRP as an early indicator of bacterial infection in premature newborn infants. CRP was obtained from 100 patients enrolled in a prospective study. All babies were suspected of having bacterial infection (meningitis-septicaemia) because of complications during pregnancy and/or symptoms suggestive of infection during the perinatal period. CRP was measured with the radial immunodiffusion technique. Examinations were done daily as long as elevated serum CRP levels were found. 100% (6/6) of our patients with culture-proven bacterial infections showed elevated CRP values within 24 h after the first clinical or laboratory signs suggesting sepsis. In 52.3% (11/21) of cases most probably suffering from infection, CRP rose within 72 h after the appearance of other symptoms. Even extremely immature infants were able to react with elevated CRP concentrations. Peak values of CRP were independent of birth weight. On the other hand, only 2.7% (2/73) of babies without findings of infection had slightly elevated amounts of CRP for a short time. Thus, serum CRP levels are a helpful parameter for the early diagnosis of severe bacterial infection in premature infants.