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Biomedical subjects

L Diekmann

Publications and source records attributed to L Diekmann.

At least 37 records · Page 2Linked to original sources

Survival time in cystinosis. A collaborative study.

In a retrospective study the overall survival time of 205 cystinotic patients of six countries was determined. The median survival time was 8.5 years. The median time for 'renal death' (age at death due to uraemia or age at starting renal replacement therapy) was 9.2 years. The youngest patient dying of renal death was 5.2 years. No sex difference in survival time was noticed. Furthermore no difference in survival time was noted between the different countries. The analysis of the overall survival curve indicates no clear differences between the infantile and adolescent types of cystinosis.

Adolescent↗

[Mezlocillin concentrations in the serum of children with impaired renal function].

Mezlocillin was administered as an intravenous bolus injection to eight children, aged 4-15, requiring antibiotic therapy; the dose was either 75 mg/kg or 37.5 mg/kg. All of the patients had advanced renal insufficiency. Five of the children required chronic intermittent hemodialysis. The microbiological method of Grove and Randall was used for the quantitative determination of mezlocillin in the serum. Ten minutes after the injection, the concentration of mezlocillin in the serum was approximately 90 mg/l and 40 mg/l, respectively. The determinations 30 and 60 minutes after the injection showed that the mezlocillin concentrations had fallen rapidly to approximately 30 mg/l and 20 mg/l, respectively. The mezlocillin concentrations were still approximately 2 mg/l and 1 mg/l, respectively, 12 hours after administration. For the period between 10 and 60 minutes, the half-lives were between 33 and 57 minutes. In general, we can conclude that a single bolus injection of 75 mg/kg will result in mezlocillin concentrations in the serum of uremic children which allow us to expect good antibacterial efficacy, at least for the first few hours following the injection. On the basis of our studies, half the dosage does not appear to be sufficient.

Adolescent↗

[Treatment of the hemolytic-uremic syndrome with streptokinase and heparin (author's transl)].

In a clinical study we treated 16 children, suffering from severe hemolytic-uremic syndrome (HUS), with streptokinase or heparin. All children received the same symptomatic treatment (early dialysis, transfusion of packed red cells). A control group of 10 children was only treated symptomatically. No difference could be shown in clinical grading of severity. We analysed the results of the acute phase and of a follow up study of 1-5 years, which is not yet complete. The results show, that heparin could not improve the prognosis of HUS in comparison to streptokinase or symptomatic treatment. The results of streptokinase treatment were not better than in the control group. In 4 children we saw severe bleeding complications of streptokinase therapy. Accepting the complication rate of a drug and the incidence of chronic renal disease as criterium of the efficacy of drug treatment in severe HUS, streptokinase and heparin are not indicated. We believe, that patients should be managed symptomatically with early dialysis.

Acute Kidney Injury↗

[Pattern of serum and red cell electrolytes in acute haemolytic uraemic syndrome (author's transl)].

Sodium, potassium and magnesium were measured in serum resp. plasma and in erythrocytes of oliguricanuric children with acute haemolytic uraemic syndrome (HUS). Potassium was in serum resp. plasma and in red cells in normal range or decrease compared with other forms of acute renal failure in children. These findings were different to statements in the relevant literature, which describe hyperkaliaemia. We suppose a potassium depletion in the early state of HUS. The cause is still unknown. Furthermore we found a hyponatriaemia according to severity of edema and gastroenteritis. Sodium in red cells and magnesium in plasma and erythrocytes were slightly elevated.

Acute Disease↗

[Successful treatment of renal hypertension in a 2 1/2-year-old girl with the converting enzyme inhibitor captopril (author's transl)].

A girl aged 2 1/2 year on chronic hemodialysis, treated with converting enzyme inhibitor Captopril because of severe uncontrollable renal hypertension, returned for the first time to a satisfactory stable blood pressure. After three months of this therapy the bilateral nephrectomy was performed. Two weeks later the patient showed a stable blood pressure, normal for age. None of the known side effects was seen. Whether the oxalat deposits found with nephrectomy were due to the treatment with Captopril, cannot yet be answered.

Captopril↗

[Eye manifestations of subacute sclerosing panencephalitis].

A report is given on 10 children with subacute sclerosing panencephalitis (SSPE) whose clinical illness began with visual or mental impairment. All patients showed typical clinical and neurologic features and various ophthalmological manifestations. The latter correspond to lesions generally dealt with in a number of reports and demonstrate for the individual case that ophthalmological manifestation can precede clinical manifestation. The ophthalmologist can give an important contribution to the diagnosis. In some cases the focal retinal lesions can be similar to retinal lesions in the case of toxoplasmosis. A positive antibody titer for toxoplasmosis, which can occur parallel with the typical measles or SSPE virus antibody titer, occasionally causes the misdiagnosis of toxoplasmosis acquisita. In the 10 cases reported on, the diagnosis SSPE was confirmed on the basis of the typical clinical features and elevated measles virus antibody titer. In no case the permission for autopsy was gained. The genesis of SSPE is discussed with reference to a genetic defect, to an autoimmune reaction, or as a result of a measles virus-like infection. In the diagnosis, primarily unclear 'focal retinal lesions' should be at any rate investigated with respect to measles or SSPE virus antibody titer.

Antibodies, Viral↗

[Sodium, potassium and magnesium in plasma and erythrocytes during haemodialysis of children (author's transl)].

In 19 children with chronic renal insufficiency sodium, potassium and magnesium in plasma and erythrocytes were measured before and after haemodialysis. Creatinine and the acid-base status were also determined. The mean predialysis values of sodium in plasma were within normal limits, in erythrocytes in the lower normal range. Potassium was elevated in plasma, and in erythrocytes within the normal linits. Magnesium was elevated in both plasma and erythrocytes. The changes during dialysis are described. Statistically significant relations of investigated variables are also described. The results are discussed.

Adolescent↗

[Familial nephropathy with retinitis pigmentosa and peripheral dysostosis].

The paper reports two siblings, 6 and 3 3/4 years old, with a congenital nephropathy (nephronophthisis), retinitis pigmentosa, heart failure and peripheral dysostosis. The severe histological changes of the kidneys with tubular atrophy and interstitial fibrosis caused the death of the older sister at the age of 7 years. The symptoms of our patients are discussed in comparison to the syndrome described by MAINZER et al.

Bone Diseases, Developmental↗

[Acute renal failure in childhood (author's transl)].

From 1960 until 1975 we treated 88 children with acute renal failure. The majority of these patients were infants and children up to three years. In this report the etiology, symptomatology and prognosis of acute renal failure in childhood are discussed. During the last years the cases observed were more severe, especially when associated with the haemolyticuraemic syndrome. The overall mortality was 27%, and did not change since the introduction of dialysis. Important prognostic factors are discussed.

Acute Kidney Injury↗

[Septicaemia in the neonatal and infant period (author's transl)].

The paper reported about septicaemia in the neonatal and infant period. Continuing former investigations by Kienitz in the same pediatric clinic we have got a survey from 1933 to 1974. In the last years we saw increasingly more infections with gram-negative organisms, especially by Pyocyaneus with a severe prognosis. The prognosis did not improve during the last 10 years. The over all mortality was 34-35%. Frequency, symptoms, problems of species and resistance were described. Besides that we reported about an outbreak of enterobacter in our neonatal unit.

Drug Resistance, Microbial↗

[Familial occurrence of hydronephrosis and of the mega-ureter -- megacystis syndrome (author's transl)].

The authors describe the familial occurrence of hydronephrosis, the father and two daughters being affected. In another family, mother and son suffered from the mega-ureter-megacystis syndrome. Cases described in the literature are reviewed. It is stressed that relatives of patients with congenital anomalies of the urinary tract should be carefull screened for possible renal abnormalities.

Abnormalities, Multiple↗

[Report of pheochromocytoma in childhood (author's transl)].

This paper reports the results and specific variations in diagnostic and therapy of pheochromocytoma in childhood. Two patients aged 10 and 12 years are described. Previous reports about 155 cases are summarized in respect to localization and frequency of occurrence. In our experience the catheterization of the vena cava is a small-risk method to localize the tumor and to obtain informations about the amount and type of cathecholamines. The value and limits of the diagnostic procedure - until now seldom used in children - are mainly discussed. The improvement of symptoms and the marked circulatory changes by the treatment with the alpha-adrenergic blocking agent Dibencyline are described. The histological differentiation between benign and malign growth proves to be uncertain.

Adrenal Gland Neoplasms↗