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Biomedical subjects

D Maas

Publications and source records attributed to D Maas.

At least 37 records · Page 2Linked to original sources

[Methyldopa-induced autoimmune hemolytic anemia. Course and long-term observations on 11 patients].

Long-term studies on 11 patients suffering from methyldopa-induced autoimmune haemolytic anaemia suggest that this self limitable episode of disturbed immune tolerance is characterized by five main parameters: 1. The autoimmune pathogenic average daily drug dose: Unfortunately, it cannot be estimated exactly due to the individually rather variable absorption of methyldopa (7-62%). The lowest oral dose in our patients was 125 mg daily. 2. The period of autoimmune induction: between the start of the methyldopa administration and the beginning of autoantibody production. It has to be estimated somewhat shorter than the time up to the clinical manifestation of the haemolytic anaemia. This varied over a wide range from 2 to 52 months. 3. The period of the active autohemolysis: between the appearance of pathogenic autoantibodies and the withdrawal of methyldopa. It depends on the recognition of the cause of the disease. In our patients it varied between 2 and 32 weeks. 4. The period of the haematologic remission: between the withdrawal of methyldopa and the normalization of the red blood cell values. It ranged between 4 weeks and 4 months. Contrary to the other parameters, the clinical remission is almost uniform in all patients. It begins immediately when the drug is stopped. This fact suggests that the induction and maintenance of the disease needs a continuous application of methyldopa and its presence in blood and tissue. The normalization of the red blood cell turnover simultaneous with the cessation of therapy, although the direct antiglobulin test remains positive, reveals a change of the autoantibodies into those apathogenic variants known from the majority of methyldopa-induced autoimmunizations. 5. The period of immunologic remission: between the withdrawal of methyldopa and the definite extinction of autoantibody production. It varied between 4 and 12 months. Transition to or later development of an autonomous warm autoantibody anaemia were not observed. The disease remits spontaneously. Its prompt reversibility resembles the same phenomenon in autoantibody anaemias induced by infectious agents. An inhibition of methyldopa-sensitive suppressor-T-cells may initiate the disturbed tolerance disease.

Adult↗

[Intestinal nodular lymphatic hyperplasia (INLH) in hypo-gammaglobulinemia (author's transl)].

Gastrointestinal symptoms frequently occur in patients with hypogammaglobulinemia of the late-onset idiopathic type, but intestinal nodular lymphatic hyperplasia (INLH) is rarely found. The characteristic radiological findings of the small bowel are reported in two patients with this well defined syndrome. The aspects of the radiological differential diagnosis of multiple small nodular filling defects of the intestine are discussed.

Adult↗

[Progressive systemic sclerosis - long-term treatment with azathioprin (author's transl)].

The study presents the results of an Azathioprin long-term therapy in 19 of 60 patients with progressive systemic sclerosis (PSS). Average treatment was 47 months (6 to 114 months). The patients received 2-2,5 Azathioprin/kg bodyweight daily. In 16 cases no further progression of PSS was noted. One patient showed minor deterioration. In particular no further deterioration in the lung and kidney manifestations were found. Two patients died. A female patient showed signs of osteomyelofibrosis after being treated for 70 months. She died 3,5 years after Azathioprin had been discontinued. The second patient died of right heart failure after recurrent pulmonary emboli. On the whole treatment with Azathioprin over a long period of time seems in most cases to hold the progression of the disease. The unfavourable prognosis can therefore be much improved.

Adolescent↗

[Wegener's granulomatosis. Roentgenographic signs and radiotherapeutic possibilities (author's transl)].

Pulmonary alterations and the clinical course of 8 patients with Wegener's granulomatosis are described. Knowledge of the variety of the clinical presentation and lung involvement is mandatory for the radiologist to achieve early diagnosis of this disease. Prognosis is better in Wegener's granulomatosis when adequate therapy is started in the first stage. Films of the nasal sinus are also important for the diagnosis. Besides immunsuppressiva radiation therapy can be helpful in local involvement of the ENT, skin, and orbital area as is demonstrated in 2 of our patients.

Adult↗

[Complex formation between monoclonal IgM and albumin in a patient with macroglobulinaemia (author's transl)].

A remarkable interaction between a monoclonal IgM(x) protein and autologous albumin was found in the serum of a 76 year old male patient (Ke.E) without morphological characteristics of Waldenströms disease. The components were bound noncovalently. Immunoelectrophoretic analysis of 50 additional monoclonal IgM and 10 IgA sera showed a complex formation with albumin in 62% (IgM) and in 90% (IgA). The degree of interaction was, however, less pronounced in comparison with that observed in the serum of Ke.E.

Aged↗

[On the question of drug-induced pseudo-LE syndrome: preliminary results in 58 cases (author's transl)].

Drug intake had been checked in 58 patients with pseudo-LE-syndrome. In view of the findings of the Zürich group a connection between the intake of Venopyronum¿ coated tablets and a pseudo-LE syndrome was strongly suspected in 45. In a further seven patients who also had taken the drug no definite connection could be shown because there was no temporal relationship between drug and disease. Three patients had taken Venopyronum-triplex capsules. Only three patients categorically denied ever having taken the drug in any form.

Acute Disease↗

[Heterogenicity of mitochondrial antibodies (author's transl)].

Sera from 137 patients with mitochondrial antibodies were tested against two different mitochondrial antigens. The mitochondrial antibodies from patients with pseudo-lupus erythematosus (PLE antigen) reacted exclusively with antigen which sedimented on moving-zone centrifugation at a density of 1.10, and contained no antigenic activity when tested against sera from patients with primary biliary cirrhosis (PBC). Purified PBC antigen had no PLE antigen activity at a density of 1.19, and all sera from patients with autoimmune liver disease fixed complement with this fraction. Sera from 54 of 55 patients with PLE reacted with the antigen of the PLE-gradient fraction. But 71 patients with liver disease had no such uniform reaction: sera from 39 patients fixed complement only with the PBC fraction, whereas 32 reacted stimultaneously with both the 1.10 and 1.19 density gradient fraction. The latter pattern was especially found in patients with chronic active hepatitis in whom antibodies to smooth muscle and nuclei were frequently detected.

Autoantibodies↗

[Polycystic disease of the kidneys and pregnancy (author's transl)].

A summary of pregnancy-developments of patients with polycystic disease of the kidneys is presented. The polycystic disease of the kidneys is a chronic progessive affection which takes both the kidneys in almost every case. The development of this disease can be divided in four stages. During the first, asymptomatic stage there is no disturbance of the pregnancy. In the second stage at the beginning of the decrease of the kidney-function therapy in pregnancy consists in confinement to bed and usual kidney-disease-diet. When complications as chronic recurrent pyelonephritis and hypertension rise and the final stage -- renal failure -- is achieved the pregnancy has to be finished as quick as possible in the early pregnancy by interruption and in the late pregnancy by sectio cesarian. Both methods should be followed by sterilisation of the patient.

Abortion, Therapeutic↗

[Mitochondrial antibodies induced by drug administration in patients with and without pseudo LE syndrome (author's transl)].

The pseudo LE syndrome was first described in 1972. It is a severe, sometimes fatal condition, in which high titres of mitochondrial antibodies are a constant feature. In the vast majority of cases detected, Venopyronum in dragée form (containing phenopyrazone, horse-chestnut extract, and cardiac glycosides from various plants), had been taken prior to onset of the clinical symptoms. It is probably commoner, that following intake of the drug mitochondrial antibodies appear without clinical manifestations. In both situations, disposition, dose and duration of treatment are important factors. There is reasonable ground to believe that not just a single component, but rather the combination of various substances in the preparation is responsible for the induction of an autoimmune process. At the present time it appears that an idiopathic form, without drug contact, also exists.

Adult↗