Biomedical subjects
Publications and source records attributed to W Andler.
Testicular function was examined by means of HCG-stimulation tests in 6 prepubertal or pubertal patients with leukemia or malignant tumours during cytostatic therapy and in 5 patients with nephrotic syndrome after discontinuation of treatment. The results of urinary testosterone assays suggest no damaging effect of cyclophosphamide on Leydig cell function.
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UNLABELLED: The necessity of the controversially discussed general steroid prophylaxis in Henoch-Schönlein Purpura (HSP) was analysed based on frequency, risk factors and prognosis of renal involvement. Case histories and follow up of at least 1.5 years were evaluated in all 171 patients (median age 6 years) in our institution suffering from HSP between 1.1.1987 and 30.6.1997. HSP was frequently manifest with an involvement of joints (64%) and gastrointestinal tract (58%). Renal involvement occurred in only 29% of the children. Excepted one girl (age 12 year), all children with renal disease completely recovered. Renal involvement was significantly rarer in young children (16%) and after one week therapy with prednisone (7%). In young children, renal involvement always followed abdominal pain. CONCLUSION: In our collective, a general preventing of renal disease in HSP was unnecessary. Especially in young children, the most common manifestation age, renal involvement rarely occurred and had always a good prognosis.
Autoimmune thyroiditis (AT) is the most common cause of nonendemic thyromegaly and acquired hypothyroidism in childhood and adolescence. Outgoing from an increasing number of newly diagnosed patients during the last years we performed a retrospective analysis including 34 patients treated between 1989 and 1998. Clinical data, laboratory and sonographical findings are described. Cases were ascertained by palpable thyroid enlargement or clinical signs of thyroid dysfunction and positive autoantibodies against thyroid tissue. Frequency of AT diagnosis has threefold increased since 1995 in our department. Age at diagnosis was 5.4 to 16.3 years (median 11.3). Palpable thyromegaly was present in 29 (85%) of our patients. Three patients had myxedema without goiter. Symptoms had been present for a period between 6 weeks and 7.5 years: median in all patients 6 months, median in hypothyroid patients 9 months. At time of diagnosis 15 patients suffered from hypothyroidism, 3 from hyperthyroidism and 7 patients showed normal levels of thyroid hormones. In 9 patients we found an isolated elevation of thyrotropin. 12 patients were prepubertal (Tanner I), 15 patients were pubertal (Tanner II-IV). 5 patients showed a dissociation of pubertal maturation. Ultrasound of thyroid gland showed an inhomogeneous and hypoechoic pattern in 32 patients. 30 patients were treated with thyroxine. Patients with persistent hyperthyroidism were treated with carbimazol. During the follow-up period enlargement of the thyroid gland decreased in one third of the patients. Thyromegaly caused by autoimmune thyroiditis gets an increasing importance. The high degree of obviously hypothyroid patients at the time of diagnosis reflects a delay of diagnosis. Early recognition and treatment of the disease should be achieved for a preventive care of hypothyroidism.
Valid knowledge concerning structure and contents of an ambulant training program for obese children and adolescents suggested by experts is still missing. In 1999/2000, we assessed a survey based on defined criterions in the "Arbeitsgemeinschaft für pädiatrische Diäthetik (APD)" and investigated the literature to cover and to compare based on defined criterions the spectrum of ambulant treatment models in Germany to advance guidelines for therapy in obese children. 13 ambulant training programs were detected. Training of children aged 8 to 12 years and their parents based on diet, behavioural treatment and sports therapy over couple of months without great experiences and without complete financing by health insurance were in common. Time volume of the training programs widely varies. In the seven evaluated training programs, up to one third of the patients broke off, while more than two third of the children successfully finished the training programs. The comparison of the programs failed on missing data concerning the collective, missing or different evaluation criteria, sometimes low number of patients and not standardised calculation of overweight. The survey reflects the often low experiences and the heterogeneous, insufficient evaluation of ambulant treatment models in Germany and demonstrates the great requirement of research. The schedule of criterions facilitates the comparison of programs to develop valid ambulant training programs for obese children.
UNLABELLED: The evaluation of treatment programs in obese children and adolescents is uncommon but necessary to prove effectiveness and to improve treatment modalities. We studied the effectiveness of the a one-year structured outpatient training programme "OBELDICKS" consisting of physical exercise, nutrition education and behaviour therapy in 132 participants based on the criterions developed by the Institute of Medicine and German Obesity Group (degree of weight reduction, improvement of comorbidity and health behaviour, minimising of side effects). Furthermore, we analysed degree of overweight (SDS-BMI) two years after the end of the outpatient training (n = 60). - 74 % of participants reduced their overweight (intention to treat). The mean reduction of SDS-BMI was 0.43. 34 % of the participants was not obese any more at end of the training. The degree of overweight was significantly (p < 0.001) lower two years after intervention compared to baseline. The comorbidity was improved (significant reduction of the frequencies of hypertension, dyslipidaemia and hyperuricaemia). The nutrition, exercise and eating habits (cognitive control and disinhibition of control) were significantly improved. Side effects were not found. CONCLUSION: The effectiveness based on the criterions of the Institute of Medicine and the German Obesity Group was proven for the outpatient training "OBELDICKS". Long-term weight reduction can be achieved in most of the obese participating children due to this long-term, specialised treatment.
48 patients between one and seventeen years of age were stimulated with GRF (1-29) NH2. Seventeen of them showed a constitutional delay in growth and puberty, twenty-three suffered from growth-hormone deficiency (GHD) and eight had a suspected GHD as a result of pharmacological tests. Although the results demonstrate a high specificity for GH release in children with constitutional delay of growth and patients with functioning hypophyseal gland, the sensitivity for diagnosis of GHD was poor. The majority of GHD patients suffer from hypothalamic disorders and therefore respond to GRF. In conclusion, the GRF-stimulation test may be sufficient for differentiation of hypophyseal or hypothalamic disorders. However, GRF-stimulation test shows too many false positive results in hypothalamic growth disorders and therefore is not recommended for diagnosis of GHD.
Newborn infants with inborn errors of lipid metabolism are relatively rare. There are only a few reports of hyperlipoproteinemia in infancy. In a six-day-old "healthy" newborn infant with a weight of 2960 gm a milky blood serum was found. Diagnosis of primary hyperlipoproteinemia type I was established by exclusion of other diseases and by analysis of blood lipids. Lack of lipoprotein lipase activity in postheparin plasma confirmed the diagnosis. The parents of the patient were normolipemic but showed reduced lipoprotein lipase activity. Thus, they were recognized as heterozygotes. A low fat (6-19 gm fat/d, 2-3 gm fat/kg bodyweight/d) diet with a high proportion of medium-chain triglycerides (78% MCT) lowered the initially extremely high serum triglyceride level (3674 mg/dl) significantly (214-711 mg/dl). Until the age of 7 months body weight and length followed the 10. percentile, head circumference followed the 50. percentile. Unexpectedly the patient died of pulmonary infection at the age of 8 months.
Bleomycin is now firmly established in the treatment of germinative scrotal tumors. Because of its low bone-marrow toxicity it is used for a number of malignant diseases in combination with other cytostatic drugs. However, the pulmonary toxicity of Bleomycin therapy was pointed out at an early stage: it can lead to a fatal lung fibrosis (Blum et al. 1973). This paper considers the pulmonary side-effects on the basis of a case observed by the author.
This report outlines the endocrine abnormalities associated with intracranial germinoma in childhood. The presence of special tumoral markers as alpha 1-fetoprotein and beta-HCG could be of diagnostic value and is important in respect to therapy control. However the reported case shows that despite known radiosensitivity and appropriate radiotherapy localized tumor relapse may arise soon after treatment.
The pathogenesis of neonatal transient hyperthyroidism has not been fully established, but the placental transfer of thyroid stimulating immunoglobulins such as thyroid stimulating antibodies is considered the main cause. Clinical signs usually subside with the disappearance of thyroid-stimulating IgG in the serum of the neonate. We report the clinical course of a neonatal transient hyperthyroidism.
The aim of neonatal screening programs for congenital hypothyroidism is to ensure early treatment in order to prevent brain dysfunction. There are several reasons why infants are missed in the screening program. We report on three patients with congenital hypothyroidism, who had a pathological screening result and initiation of therapy was delayed. The first patient had an increased TSH level, but she was missed because of mistakes in the confirmatory serum test. During the follow-up the patient showed typical symptoms of hypothyroidism and got a thyroxine supplementation not before the age of three years. The second patient did not get a therapy before the age of six months because of the noncompliance of the parents and physicians. The third patient had a central hypothyroidism. The neonatal screening-program revealed no measurable TSH activity. Although the child had clinical signs of a severe hypothyroidism diagnosis was not made before the age of 5.5 months. Although different reasons are known for screening errors, all these 3 patients were missed because of failures in the follow-up of a pathological screening result, indicating a poor quality in the follow-up procedure.
Short stature is a common reason for presenting a child to the pediatrician. Emotional deprivation may cause short stature and may simulate growth hormone deficiency. Diagnosis of emotional deprivation as the cause of growth retardation is difficult and misdiagnosed frequently despite of suspicious clinical signs. We report on 2 patients with growth retardation because of emotional deprivation. At the age of 5 years both children had a severe growth hormone deficiency. They received therapy with growth hormone and showed an increase of growth velocity to > or = 8 cm in the first year of treatment. But in the third year of treatment both patients showed a diminished response to the growth hormone therapy. During the period of observation the features of emotional deprivation became obvious through the extreme behavioural abnormalities. Both children showed disturbances in their social behaviour, and striking disorders concerning eating and digestion. The families of these children had severe social problems; alcoholism, low income and rejection of the child were risk factors. Removal from the current environment led to a characteristic increase of the growth velocity. Growth hormone deficiency was spontaneously reversible, so that treatment with growth hormone was terminated. The social environment and the psychical prosperity are essential growth factors in childhood and adolescence. The common features and risk factors of emotional deprivation are described. Spontaneous catch-up growth after removal from the current environment distinguishes this form of short stature from the other organic growth disorders.
Spondylodiscitis is a frequently unrecognized disease in childhood because of unspecific symptoms and late arising or radiological signs. The heterogeneous symptoms, the value of diagnostic procedures and the outcome of 8 patients suffering from spondylodiscitis in the time period of 1989 to 1995 are demonstrated. Guiding symptoms were back pain and refusal to walk or sit. Furthermore, abdominal pain and psoas abscess were the only symptoms in two cases. The pathogenomonical narrowing of the disc space arose in X-ray films earliest 3 weeks after onset of symptoms. The MRI was the best method for early diagnosis and detection of complications. In contrast to persistent radiological changes the clinical outcome was good in most of the patients.
A twelve year old boy presented with a sudden onset of recurrent nausea and vomiting. During the past six weeks he had a weight loss of 13 kg. While he was in the hospital, persistent tachycardia and a slightly elevated blood pressure were noted. The gastroenterologic, cardiologic and neuropediatric examinations were normal. To exclude the differential diagnosis of hyperthyroidism, thyroid hormones were checked. They showed clearly elevated levels of tri-iodothyronine and thyroxine, while thyrotropin was suppressed. The boy did not have a goiter. Under thyrostatic therapy his clinical condition improved quickly. Among our 20 patients with hyperthyroidism he was the only one whose main symptom was severe vomiting.
Between 1991 and 1996 three cases of MCAD-deficiency (medium-chain-acyl-Co-A dehydrogenase deficiency) were diagnosed in the Vestische Kinderklinik. All patients showed hypoketotic hypoglycaemia with hyperuricaemia. In the group of hypoketotic hypoglycaemia without lactat acidosis MCAD-deficiency is the only metabolic disease presenting regularly with hyperuricaemia. Thus, hyperuricaemia in a patient with hypoketotic hypoglycaemia is a strong indicator for MCAD-deficiency. Measurement of uric acid is easily available before sophisticated metabolic analysis are completed.