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Biomedical subjects

D Lachmann

Publications and source records attributed to D Lachmann.

At least 37 records · Page 2Linked to original sources
PubMed1977

[Abnormal lipoprotein (LP-X) in the first months of life with particular reference to obstructive jaundice (author's transl)].

Abnormal lipoprotein (LP-X) represents a specific parameter for the presence of obstructive jaundice in the adult. Since LP-X has also been detected in the serum of newborn infants, both full-term and premature, and in early infancy, in the absence of clinical evidence of obstructive jaundice, extensive investigations were undertaken in infants during the neonatal period to clarify this phenomenon. The present study reports the data obtained in over 2000 sera from over 370 infants (mature newborn and premature newborn and young infants), tested more or less continuously by means of the Rapidophor method, initially on a qualitative, and subsequently, on a semi-quantitative basis. LP-X appears within the first fortnight in newborn infants, irrespective of the mode of feeding. The LP-X concentration was correlated to the birth weight. Premature infants displaying signs of immaturity possessed markedly higher LP-X levels than mature newborn infants. LP-X was not correlated to the alkaline phosphatase level, nor to the gammaglutamyl transferase activity; the bilirubin level, likewise, had no connection with the LP-X concentration. Patients with proven obstructive jaundice showed distinctly higher LP-X concentrations (greater than 56 mg/100 ml), whereby the rise in LP-X level in some cases preceded the appearance of the clinical manifestations of obstructive jaundice. The following hypotheses are advanced in order to explain the presence of LP-X during the neonatal period and are discussed on the basis of clinical observations in adults, the physiological conditions in the newborn infant and the results of the present study: The liver, which occupies the central position amongst metabolic organs, also in the case of the lipoproteins, is at a physiological stage of organic and functional maturation during this early period of life. Under these circumstances, a pseudo-obstructive mechanism on the basis of insufficient excretion of biliary lipoproteins, in conjunction with a simultaneous "physiological" deficiency of lecithin: cholesterol acyl transferase could lead to the appearance of LP-X in the serum. Catabolism of the resultant LP-X cannot take place owing to an inadequate activity of lipoprotein lipase. Functional immaturity can be presumed in the case of both enzyme systems during the neonatal period. On attainment of a degree of maturity compatible with the appropriate neonatal stage, the LP-X values become negative between the 7th and the 16th week of life. It is conceivable that the appearance of LP-X in the newborn infant can be ascribed to LP-X1, since the "physiological" LP-X concentrations in the neonatal period (values of up to 20 mg/100 ml) are distinctly lower than the values found in obstructive jaundice. LP-X determination can be rated as a useful supplementary investigation in the differential diagnosis of extrahepatic biliary atresia during the first weeks or months of life...

Alanine Transaminase↗
PubMed1977

[To the Treatment of myelomeningocele (author's transl)].

Social importance of myelomeningocele specially in Great Britain is stressed and surgical treatment in 24 hours after birth is necessary. Severe complications as infections of the CNS and others in consequence of the neurogenic bladder are demonstrated. The manifold problems of these infants and their families need an individual plan for treatment and care. Interdisciplinary cooperation is the condition for the success of rehabilitation.

Child Development↗
PubMed1977

[LP-X in the first month of life (author's transl)].

An abnormal lipoprotein can be detected in the sera of patients suffering from intra- and extrahepatic cholestasis. The specificity of LP-X detection for the diagnosis of cholestasis in adult persons was proved by extensive investigations. The aim of this study was to examine the specificity of LP-X with regard to cholestasis in the postnatal phase of premature and newborn infants. 1056 sera of 270 newborns were tested and in 75% of these cases LP-X was found to be positive. Three hypothetical explanations are given concerning the appearance of LP-X in the sera of premature and newborn infants. The semi-quantitative determination of LP-X also represents a possibility for differential diagnosis of obstructive jaundice in the neonatal period.

Adult↗
PubMed1976

[Glutathione reductase deficiency with membrane defect in hereditary hemolytic anemia].

Glutathione reductase activity and phospholipid metabolism in red cell membranes were determined in a family with hereditary hemolytic anemia. A marked decrease of glutathione reductase activity and stability of reduced glutathione was found in combination with enhanced phospholipid-phosphate metabolism and decreased activity of the membrane-stabilizing enzyme lysolecithin-acyltransferase. In all cases splenectomy beneficially influenced the hemolytic process. Family studies revealed a dominant-autosomal genetic transmission.

Adolescent↗
PubMed1976

[BCG-septicemia and mucocutaneous candidiasis due to familial combined immunodeficiency (authors transl)].

The history of a patient with mucocutaneous candidiasis and BCG-septicemia is reported. Combined immunodeficiency has been diagnosed as the underlying cause. A sister of the patient died at seven months of age under similar circumstances. The importance of the immunological diagnosis in severe bacterial and mycotic infection during early infancy has been emphasized. In such cases vaccinations with life vaccine are strongly contraindicated.

Autopsy↗
PubMed1976

[Clinical aspect and therapy of BCG-lymphadenitis (author's transl)].

BCG-Lymphadenitis is a local complication of BCG-vaccination. The rate of complication depends on the kind of vaccine used, the dose and the used technique. In the last 9,5 years 32 infants with BCG-Lymphadenitis complicated with suppuration or perforation have been treated surgically. For 6 patients the treatment included a pre- and postoperative tuberculostatic therapy, for 11 patients included antibiotics. In 15 cases only surgical treatment was performed. In all 3 groups satisfactory results have been achieved, therefore tuberculostatic therapy seems not to be necessary and an antibiotic therapy ought to be reserved for cases with secondary miscellaneous infection. There is no doubt that it is possible to shorten the duration of this disease through early extirpation and to achieve a favourable cosmetic result too.

Anti-Bacterial Agents↗
PubMed1976

[Hemangioma simplex of the larynx in an infant (author's transl)].

Symptoms of inspiratory stridor, dyspnoa and cyanosis appeared in a four month old boy. After the direct laryngoscopy and biopsy a hemangioma simples of the larynx was diagnosed. The tumor itself was successfully treated by cryosurgery. The advantages of this therapy were pointed out and the cryosurgery can be recommended in cases of larynxangioma.

Cryosurgery↗
PubMed1975

[Preliminary investigations of the serum alpha-1-fetoprotein level in mature and premature newborn infants (author's transl)].

Semiquantitative determination of the serum AFP concentration was carried out in 170 newborn infants (mature and premature) by means of crossover electrophoresis on admission to hospital and at 1 to 2 weekly intervals thereafter. The infants were divided into 3 groups according to their birth weight and also grouped according to gestational age into 3 further groups. The serum AFP level was correlated with the age in weeks of the infants for each group separately. Mature newborn infants with a birth weight of over 2500 g show a rapid fall in serum AFP titre; AFP is no longer detectable after the 9th week of life. In premature infants with a birth weight of 1500 to 2500 g, the fall in AFP titre is delayed and reaches zero between the 7th and 13th week of age. There is a markedly slower decrease in serum AFP concentration in the group of immature infants with a birth weight of under 1500 g; the limit of detectability lies between the 13th and the 17th week of age. An almost identical pattern is revealed when the infants are grouped according to gestational age. The possibility of diagnosing the small for date babies and severe liver disease by means of the serum AFP titre is discussed.

Age Factors↗
PubMed1975

[Clinical picture and therapy of gastroschisis (author's transl)].

The results of operative treatment of gastroschisis have been improving in recent years. The survival rate is now between 50 and 60 p.c. It is very important to perform primary repair as soon as possible after birth. According to our own experiences resection of the bowel should be avoided as it gives a poor prognosis. Combined malformations of gastroschisis are very rare and their treatment should be tired since there is no other alternative. An own successfully treated case of combined malformations of gastroschisis with atresia of the small bowel is presented. Stenosis of the duodenum and tubular duplication are described

Abdominal Muscles↗
PubMed1975

[Incomplete, combined hereditary immunodeficiency with generalized tuberculosis after BCG-vaccination from bacille Calmette Guérin (author's transl)].

A case of hereditary incomplete, combined immunodeficiency is reported. The patient, a 20-week-old boy, suffered from severe, generalized tuberculosis after BCG-vaccination. A conspicuous discrepancy was found between the normal number of lymphocytes in the peripheral blood and the severe morphological changes in the organs of the lymphoreticular system (deficient development of thymic structures; absence of lymphocytes in the thymus and the thymus-dependent areas of the periphery as well as in the bursa-dependent structures). A partial primary defect of the stem cells and a secondary insufficiency of the functions with incomplete differentiation due to an insufficiency of the primary lymphatic organs are discussed as possible causative factors. The inability to develop epitheloid cells in connection with the tuberculous infection is interpreted in part as a sequence of a T-cell insufficiency.

Autopsy↗
PubMed1975

[Oculocerebrovertebral syndrome].

After description of the oculovertebral syndrome Weyers-Thier a personal observation is reported. It was characterized by severe cerebral malformations (agenesis of the olfactory lobes, incomplete agenesis of corpus callosum with large sagittal arachnoidal cyst) associated with polycystic brain damage. The relationship of this "oculovertebral syndrome" to the "oculoauriculovertebral syndrome" is discussed. The combination of various malformations in our observation indicates an embryonic lesion between the 4th and 11th week. Additional polycystic brain damage was due to perinatal anoxy. The causal factor of the malformative syndrome remains unknown, but similar lesions were experimentally induced by fetal hypoxia.

Brain↗
PubMed1975

[On the differential diagnosis of hemolytic anemia (author's transl)].

Differential diagnosis of hemolytic anemia is discussed, with regard to the classification into corpuscular and extracorpuscular types of hemolysis. Presence of antibodies in a patient's serum, indicate extracorpuscular-acquired hemolytic anemia. Coombs test may be positive or negative according to the antibody present. A primary disease has to be excluded in each case. Abnormal hemoglobin, defect of the corpuscular-hereditary type. Direct laboratory enzyme estimation indicate enzyme deficiency (Glucose-6-P-dehydrogenase, Pyruvate-kinase, Glutathione-reductase). Hemoglobinelectrophoresis and special tests for unstable hemoglobins indicate this type of disturbance. For defect of the membrane measuring of osmotic fragility might be helpful. Activity of membrane enzymes and introduction of 32P in the fractionated membrane lipids, point out special types of a deficient membrane. 6 of our own cases are discussed.

Anemia, Hemolytic↗