Extra-lobar pulmonary sequestration with prenatal diagnosis. A report of 5 cases and review of the literature.

UNLABELLED: THE PURPOSE of this work was to study the pre- and postnatal features of extra-lobar pulmonary sequestration (ELPS) and consider their diagnosis and treatment. METHODS: Five ELPS diagnosed prenatally (1986-1992) were reviewed retrospectively. RESULTS: In 2 cases, prenatal diagnosis was based on the presence of a left suprarenal mass for which tumor markers proved negative postnatally. These 2 infants underwent surgery at 3 weeks of age for supposed neuroblastoma or teratoma. In the other 3 cases, diagnosis was based on the presence of a solid mass at the left base of the thorax. The systemic vessel was visualized in 2 of these cases; mediastinal displacement was noted in one case and hydrothorax (which recurred after puncture) in the other. The latter infant was born at 34 weeks of amenorrhea, and hydrothorax disappeared postnatally after excision of the ELPS. The other two infants were asymptomatic at birth and underwent surgery respectively on the 8th day and during the 6th month of life. CONCLUSION: ELPS can take the form of a mass in the abdomen or at the base of the thorax. For subdiaphragmatic ELPS, surgical excision (possibly preceded by percutaneous puncture) is required if the diagnosis is uncertain. Supradiaphragmatic ELPS can be complicated prenatally by hydrothorax or even hydrops, requiring drainage in utero. If the infant is asymptomatic postnatally, systematic surgical excision should be considered. Extra-lobar pulmonary sequestrations (ELPS) are masses of nonfunctional lung tissue vascularized by an abnormal systemic artery and covered with a pleural layer isolating them from the rest of the parenchyma. The diagnosis of these malformations is based increasingly on obstetrical ultrasonography. The purpose of this study was to specify the prenatal features of these malformations and define the diagnostic approach and therapeutic strategy.

Measurement of insulin-like growth factor I (IGF-I) in normal adults, patients with liver cirrhosis and acromegaly: experience with a new competitive enzyme immunoassay.

A competitive enzyme immunoassay for the determination of human insulin-like growth factor I in microtiter plates was established. Using a polyclonal antiserum raised in rabbits against hIGF-I ovalbumin conjugate the assay system was able to detect IGF-I at a range of 12-800 pg/well with a sensitivity of 10 pg/well. It showed a low (< 0.5%) cross reactivity with hIGF-II. The serum concentrations of IGF-I found by EIA agreed well with those found in a conventional RIA (r = 0.965, p < 0.001). Effects of age and sex on IGF-I levels were studied in 260 normal adults. There was no evidence for sex differences but a steep decline of values from the third to the fourth and from the eight to the ninth decade, respectively. To asses the diagnostic capability of the IGF-I determination in liver cirrhosis, 71 sera of patients classified according to Child classes (A-C) were measured. Although significantly diminished concentrations were found in class B vs A and in class C vs B, the diagnostic sensitivity in cross-sectional examinations proved to be low (class A: 0.33, class B: 0.67). Only in the case of extensively destroyed liver parenchyma (Child C: 0.94) IGF-I was a good indicator of impaired hepatocellular capacity. In 29 patients with acromegaly serum IGF-I levels were investigated. All patients with active acromegaly showed increased IGF-I levels. In contrast, in inactive or weakly active acromegaly values were considerably lower.(ABSTRACT TRUNCATED AT 250 WORDS)

Lymphocyte transfusion in recent onset type I diabetes mellitus--a one-year follow-up of cell-mediated anti-islet cytotoxicity and C-peptide secretion.

In 19 patients with newly diagnosed Type I diabetes mellitus a single transfusion of 1.9 x 10(9) to 1.5 x 10(10) lymphocytes was performed. Fifteen Type I diabetic patients who did not receive a transfusion were used as controls. Anti-beta-cell cell-mediated cytotoxicity was measured using an insulin release assay. Stimulated C-peptide secretion (100 g glucose orally, 1 mg glucagon i.v.) was used to estimate residual beta-cell function. Both parameters were measured prior to transfusion and after 12 months. The transfusions were followed by a fall of cytotoxicity below the 95% confidence limit of the controls in 11 of the 19 patients ('responders') (15.7 +/- 1.7 ng insulin/islet/20 h vs 6.7 +/- 1.3 P less than 0.001), while the other eight transfused patients ('non-responders') (13.5 +/- 1.9 vs 17.1 +/- 2.9, ns) and the non-transfused control patients (11.6 +/- 1.1 vs 14.2 +/- 2.4, ns) displayed persistently high cytotoxicity levels. In the responder group a slight improvement in stimulated C-peptide secretion was observed (136 +/- 43 pmol/dl vs 148 +/- 38, ns) whereas in the non-responder (127 +/- 28 vs 106 +/- 25, ns) and in the control group (130 +/- 17 vs 97 +/- 19, P less than 0.05) the stimulated C-peptide responses declined during the 12-month follow-up. Thus, lymphocyte transfusion may have beneficial effects by suppressing anti-beta-cell cytotoxicity and preserving C-peptide secretion.

Allogeneic bone marrow transplantation for leukaemia and aplastic anaemia. Results of the Leipzig BMT Group.

In acute leukaemias there was a stable plateau in the survival curve at 45% after two years if grafted in first complete remission (n = 20) but only 13% of the patients are disease-free alive if grafted in a more advanced stage of the disease (n = 8). In 16 patients transplanted for chronic myeloid leukaemia the overall survival is 40%, in cases with graft-versus-host disease (GVHD) prevention by cyclosporine survival rate could be improved. Only 8 patients with severe aplastic anaemia, partially in low performance status were able to be transplanted; three died of infections, another by acute GVHD. The fatal complications in our study characterize the international well-known major problems in BMT: GVHD, interstitial pneumonitis, infections, graft failure in aplastic anaemia and recurrence of leukaemia, especially in more advanced leukaemia stage.

Plasma exchange for removal of anti-A and anti-B group antibodies in AB0 major-incompatible bone marrow transplantation.

Between 1980-1986 seven patients underwent AB0-major incompatible bone marrow transplantation. Incompatible anti-A and anti-B antibodies could be decreased by the plasma exchange from titers 8-256 down to 0-8. Our practice combined with additional depletion of erythrocytes from marrow allowed to transplant all patients successfully, however, one patient demonstrated an acute haemolytic reaction during infusion of marrow. Plasma exchange was fairly well tolerated, however, 4 patients developed slight urticarial reactions. Both thrombocytopenic patients overcompensated the platelet loss caused by the exchange.

[Discontinuous plasma exchange with the conventional hemodialysis technic and blood cell centrifugation]. / Diskontinuierlicher Plasmaaustausch mit konventioneller Hämodialysetechnik und Blutzellenzentrifugation.

A conventional haemodialysis system, to which the patient is connected as for acute dialysis, forms the basis of the plasma substitution method. At each session around 2 litres of plasma were exchanges within 3 hours. Freshly frozen donor plasma was mainly used as the substitution solution. A total of 20 substitutions were carried out in 5 patients in a period of 7 months. The indications were: 3 x major mismatch in the ABO system before bone marrow transplantation, myasthenic crisis, violent attacks connected with lupus erythematosus with rapid development of renal insufficiency. The therapeutic goal of quickly eliminating immunopathogenetic proteins was achieved in all cases. It was possible to reduce the level of isoagglutinins, haemolysins, autoantibodies (myasthenia gravis) and immunity complexes (LE) effectively. The clinical course confirmed the excellent effectivity. There were no serious complications despite the serious primary diseases. Volume imbalances, changes of body temperature, precipitations of cryoglobulins, electrolyte disturbances or citrate intoxications were always well controllable thanks to the combination of plasma substitution and dialysis.

[Indication for prophylactic and therapeutic thrombocyte substitution]. / Indikation zur prophylaktischen und therapeutischen Thrombozytensubstitution.

The substitution of thrombocytes is on principle indicated only when a disturbance of the formation is present. The prophylactic application, i.e. before the occurrence of a haemorrhage, which is sometimes life-limiting, is to be preferred, however, on account of the danger of sensitization it is bound to particular conditions: temporarily limited need of substitution, decreased immune reagibility of the recipient (basic disease, therapy), medico-therapeutically induced thrombocytopenia (cytostatic drugs); example: haemoblastoses. Prerequisites are subtile control of clinical effectiveness and formation of antibodies. In planned bone marrow transplantation only preparation of individual donors (not related!) with HLA-A/B match are to be used. The same is applied to already alloimmunized patients, in which case a negative lymphocytotox cross test must still be present. Patients without option to transplantation or without HLA-antibodies may receive also random preparations of individual donors or mixed preparations. AB0-minor-incompatibilities are without significance, AB0-major-incompatibilities are not permissible. The introduction of thrombocyte-specific testings is urgently to be aspired to.--Exclusively therapeutic substitution of thrombocytes, i.e. in manifest haemorrhage, is performed in a primarily not limited period of substitution, in a completely immune competent recipient with regularly low values of thrombocytes without cytoreductive therapy (example: aplastic anaemia). In these cases on account of high danger of sensitization only preparations of individual donors with HLA-A/B-match should be used. In cases of exception in thrombocytopenic, life-threatening haemorrhages on account of metabolic disturbances (immune thrombocytopenia, massive transfusions) the substitution with thrombocytic mixed concentrates is satisfied.--The substitution of thrombocytes is a common task of clinic and blood donation service, which opens new possibilities of therapy (e.g. transplantation of bone marrow).

[Thrombocyte substitution in acute leukemia. Effect of histocompatibility on the clinical efficacy]. / Thrombozytensubstitution bei akuter Leukämie. Einfluss der Histokompatibilität auf die klinische Wirksamkeit.

Thrombocyte substitution is an essential prerequisite for intensive cytoreductive therapy in acute leukemia. Evaluating 228 thrombocyte transfusions in 17 patients shows that the clinical effectiveness of thrombocyte concentrates can be increased by making the coordination of HLA antigens of donor and receiver as good as possible. When measured in the corrected increment (CI) 24 hours after transfusion, the effectiveness of A3/B1 match preparations (CI = 7.0 +/- 1.6) is significantly higher than that of random preparations (CI = 3.0 +/- 0.5). With the presence of HLA antibodies an effective substitution (CI24 greater than or equal to 4.5) can only be achieved by A3/B1 match thrombocytes. This can only be realized by applying the fourfold thrombapheresis of single donors.

[Supportive hemotherapy in anemias]. / Supportive Hämotherapie der Anämien.

The substitution of blood and erythrocytes, respectively, has to be performed as an aimed supportive haemotherapy according to measure. Therefore full blood is indicated only in loss, otherwise only concentrates of erythrocytes may be transfused, in order to exclude side-effects as a potential danger. The latter should above all produced buffy-coat-free. Washed preparations of erythrocytes should be used only in such a case, when there are present clear clinical demands, such as transfusion reactions which can otherwise not be removed, and which for their part limit the success of the transfusion. The practically plasma-free concentrates of erythrocytes which are extremely poor in alloantigens and preserved at deep temperature should at present be used for reasons of capacity only in persons who are potentially to be transfused many times, above all in recipients of grafts.

[The responsibility of the transfusing physician]. / Zur Verantwortlichkeit des transfundierenden Arztes.

In a chronological representation a survey of important duties of the transfusing physician in blood transfusions is given. When fulfilling conscientiously the special, legal and organisational demands, the transfusing physician may authoritatively influence the decrease of disturbances of transfusion.

[Isolation and characterization of mistletoe extracts (Viscum album L.). I. Affinity chromatography of mistletoe extracts on immobilized plasma proteins].

The agglutinating effect of lectin from mistletoe (Viscum album L.) relative to erythrocytes and tumor cells is destroyed or reduced by plasma proteins. There is a competition between lectin receptors of plasma proteins, especially immunoglobulins and erythrocytes, as well as tumor cells. The preparation of insolubilized immunoglobin fractions allows one to separate lectin from the mistletoe extract. There exist chemical connections between the lectin fixed to the adsorbent and part of the toxic components.

[Isolation and characterization of mistletoe extracts (Viscum album L.). II. Effect of agglutinating and cytotoxic fractions on mouse ascites tumor cells]. / Isolierung und Charakterisierung von Inhaltsstoffen der Mistel (Viscum album L.). II. Wirkung von agglutinierenden und zytotoxischen Fraktionen auf Mäuse-Aszites-Tumorzellen

Lectin from mistletoe (Viscum album L.) was studied for its relations with the toxins from Viscum album, ascites tumor cells of mouse, and human immunoglobulins. Using affinity chromatography on glutaraldehyde-crosslinked IgG (human) from viscum crude extract, a fraction was isolated which exhibited full agglutination capacity and high toxicity. The supernatant showed no agglutination capacity but a strong toxic effect on mouse ascites tumor cells. This toxic effect could not be influenced by further additions of insolubilized IgG. Chromatography on DEAE cellulose also gave agglutinating fractions with toxic effects and a non-agglutinating toxic portion. Column chromatography on Sephadex G 75 allowed separation of toxic from agglutinating components. The molecular weight of the toxin remaining after lectin removal was above 10,000. Lectin was found to bind more readily to mouse ascites tumor cells than to erythrocytes.

[Qualitative demands on a anti-N blood group test reagent from Vicia graminea (anti N vg)]. / Qualitative Anforderungen an ein Blutgruppentestreagens Anti-N aus Vicia graminea (Anti Nvg)

Qualitative demands made on anti-NVg are enunciated. Apart from a short description of the production and directions for the use indications for testing the specific effect, the purity and the titre are given. Furthermore, serological peculiarities of the anti-NVg for special questions are reported. Anti-NVg shall enlarge the series of the out superseding the usual anti-N-serum of the blood-group-serological diagnostic remedies with-rabbit.

[Effect of protamine sulphate on erythrocyte agglutination by anti-rh antisera. Cause of time-dependence (author's transl)]. / Die Wirkungsweise von Protaminsulfat auf die Erythrozytenagglutination durch Rh-Antiseren Ursache der Zeitabhängigkeit

The effect of protamine sulphate on Rh antisera causes agglutination also in saline milieu and is time-dependent. Synchronous to the serologically demonstrable decrease of the protamine concentration a normalization of the immunoelectrophoretic picture (especially of the albumin arc) and a decline of the precipitate formation by heparin occur. These effects are based on enzymatic degradation of protamine by a protaminolytic enzyme contained in human serum. This enzyme could not be inhibited by sodium fluoride, sodium azide, ammonium oxalate, EDTA or alpha, alpha'-dipyridyl to a serologically desired degree. The change of the behaviour with regard to agglutination of human erythrocytes in the system Rh antibody - protamine sulphate results primarily from blocking acid groups on the erythrocyte surface and not from chemical modification of IgG antibodies. In this system protamine sulphate leads to the formation of agglutinates but it does not represent an obligate constituent of the formed agglutinate.

Immunologic reactivity to incompatible blood group substances of the ABO system in scleroderma.

The immunization with incompatible blood groups substances of the ABO system yielded distinct increases in the isohemagglutinin titers and their scores in 16 patients suffering from systemic sclerosis (scleroderma) and in 16 controls. The difference between these two groups was not statistically significant, which suggests an intact humoral immunological reactivity of these antigens. However, investigations with further antigens would be necessary to judge the immunological factors supposed to be involved in the pathogenesis of systemic sclerosis. The method may be recommended for testing the humoral immunological response in similar problems.