[Infections following spleen exstirpation].
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to W Künzer.
Explore the source record for details and available documents.
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.
Explore the source record for details and available documents.
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.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Five galactosemic and 5 normal children received an oral load of galactose under standardized conditions. The maximal blood galactose level after 1.5 hours was 12.6 +/- 2.0 (S.D.) mmol/l in individuals with a deficiency of uridylyl transferase (EC2.7.7.12) as compared to 5.8 +/- 1.2 (S.D.) mmol/l in the controls. The concentration of serum urate in galactosemics increased to 155% of the fasting level (P less than 0.005); no rise was detectable in the controls. The elimination of urate with the urine was augmented by the same amount in both groups. Our studies provide evidence for an increased catabolism of hepatic nucleotides. This may lead to a deficiency of nucleotides which is proposed as a cause of galactosemic liver injury.
The authors report on an infant with myeloproliferative syndrome of the myelomonocytic type. The findings fulfilled the criteria for juvenile chronic myeloid leukemia except that there was no increase of fetal hemoglobulin and no depression of erythrocyte carbonic anhydrase I. In one half of the bone marrow cells a Ph1-chromosome was found. During the course of the disease the Ph1-positive clone was successively replaced by cell lines with different chromosomal aberrations. The relationship between the cytogenetic mosaicism and the clinical and laboratory findings is discussed.
Recently we gained increasing evidence of the fact that cholostatic syndromes in young infants are frequently associated with preceeding shock and disseminated intravascular coagulation. This is illustrated by the case of a 4 weeks-old male infant with urosepticemia due to E. coli, which was successfully treated with additional streptokinase. Therefore, in the age-group of early infancy we have to pay attention to the liver as a potential shock-organ similar to the well known shock organs such as kidneys, lungs, skin and brain.
The effect of orally administered agar on the serum concentration of bilirubin was tested in 366 premature and newborn infants. For this purpose two different concentrations of agar were added to the milk for 8-10 days. On none of the groups tested did the serum level of bilirubin show a significant decrease after administration of agar. Therefore the attempt to lower the level of serum bilirubin in premature and newborn infants by inhibiting enteric reabsorption by means of adsorbents must be considered a failure.