PubMed Health⌕ Search

Biomedical subjects

D Anders

Publications and source records attributed to D Anders.

At least 19 recordsLinked to original sources

Complement analysis in children with idiopathic membranoproliferative glomerulonephritis: a long-term follow-up.

Fifty children with idiopathic membranoproliferative glomerulonephritis (MPGN), aged 2-14 years at apparent onset, were monitored for the presence of C3 nephritic factor (C3 NeF) and signs of complement activation in serum. In addition, C3 allotyping was performed in 32 patients. Observation time ranged from 2 to 20 (median 11) years. C3 NeF activity was detected at least once in 60% of the patients (in 11 of 26 with type I, in 15 of 17 with type II, and in four of seven with type III). C3 NeF-positive patients had significantly reduced levels of CH50 and C3 and elevated levels of C3dg/C3d. During follow-up, C3 levels were persistently normal in 62% of the patients with MPGN type I and in 43% with type III but in only 18% with type II. C3 allotype frequencies differed from those found in healthy controls with a significant shift to the C3F/C3FS variants in C3 NeF-positive patients. C3b(Bb)P as a marker for alternative pathway activation was not increased in C3 NeF-positive patients. Despite the presence of C3 NeF activity, C3 levels remained normal in six patients throughout the observation period. C3 NeF became undetectable in six patients, whereas seven developed C3 NeF activity during follow-up. There was no significant difference in renal survival probability in patients with or without C3 NeF activity. Neither C3 variants nor continuous low C3 or low CH50 levels had any prognostic value for the clinical outcome. No factor H deficiency was detected.

Adolescent↗

Orofacial findings in the Klippel-Trénaunay syndrome.

The Klippel-Trénaunay syndrome is a triad of congenital anomalies characterised by haemangiomas, varicosities, and unilateral bony and soft tissue hypertrophy. Hypertrophy usually affects one distal limb, but trunk or face may be affected. Cutaneous haemangiomas (nevus flammeus) of varying extent and irregular contour are often present in the hypertrophic regions. Varicosities may also be part of the vascular lesions of the syndrome. Orofacial manifestations include facial asymmetry, jaw enlargement, and malocclusions as well as premature tooth eruption. Two cases of the Klippel-Trénaunay syndrome are presented here. Both of these show the typical hemifacial hypertrophy and premature eruption of teeth on the affected side. In the first case only the left mandibular region was affected. In contrast, in the second there was hypertrophy of the whole left side of the body including upper and lower jaws. This boy also suffers from congenital ideokinetic retardation, while the first was otherwise normal. Both cases differ from previously reported cases of the Klippel-Trénaunay syndrome in lacking any prominent facial nevus flammeus. In the first case there was also malformation of the crown of the first permanent molar on the affected side that has not been described previously.

Child↗

The time course of nerve growth factor content in different neuropsychiatric diseases--a unifying hypothesis.

Nerve growth factor (NGF) is the prototype of related neurotrophic proteins, the so-called neurotrophins. NGF is essential for proper development of sympathetic and neural crest-derived sensory neurons of the peripheral nervous system (PNS) as well as of the neurons in the cholinergic basal forebrain (CBF). In the mature peripheral and central nervous system (CNS) NGF is also biologically active; NGF facilitates neuronal plasticity and regulates synaptic transmission and connectivity. Besides this well established neurotrophic function, recent findings suggest a role of NGF in neuroimmune and stress-associated processes, which NGF imparts not only as the classical "target-derived messenger", that is retrogradely transported within NGF-sensitive neurons, but also as para- and autocrine cytokine modulating the function of non-neuronal cells. Since neurotrophins are produced in very small amounts in vivo, NGF-sensitive cells have to compete for the limited NGF even under physiological conditions, so that normally only less than 10% of NGF receptors (NGFR) are saturated with their endogenous ligand. Consequently, it is feasable that minute changes in NGF concentrations can influence neuronal function in an extensive way. Hence, one plausible pathomechanism of disease(s) may be that a deficiency in NGF leads to malfunction of NGF-sensitive neurons. The change in NGF concentrations in the course of several diseases, namely during alcoholic and diabetic neuropathy as well as in Alzheimer's disease (AD) and several lesion-models of the CBF, indicates that fluctuations of endogenous NGF concentrations in PNS and CNS follow a distinctive pattern. An initial deficit of NGF at the onset of pathological processes is typically followed by its temporary elevation, during which some neuronal deficits may be partially ameliorated. However, if the disease progresses a decrease of NGF is typically observed, which appears to be a "normalization" of formerly elevated NGF concentrations. In our hypothesis we postulate that after acute or chronic injuries NGF is up-regulated as an intrinsic attempt to regenerate NGF-sensitive neurons. After long-term exposure to noxious processes, however, this compensatory increase of NGF cannot be maintained and eventually breaks down. The extent of such a compensatory up-regulation may depend on age and condition of NGF-sensitive neurons as well as on the type of lesion (acute or chronic). Furthermore, we also postulate that an exceeding level of NGF or its chronic elevation could even be detrimental to neuronal functioning under certain conditions. Thus, endogenous NGF has the capacity to modulate and even to compensate different kinds of harmful processes and in this way it may reinstate the homeostatic equilibrium. In our view, it seems to be a more appropriate approach to regard NGF changes as independent of classical constructs of neuropsychiatric diseases. Perhaps our understanding of NGF may even model for a new approach to the aetiology of multifactorial neuropsychiatric disorders.

Brain Diseases↗

Outcome of idiopathic membranoproliferative glomerulonephritis in children. Arbeitsgemeinschaft Pädiatrische Nephrologie.

The aim of this multicentre study was to analyse the long-term outcome of idiopathic membranoproliferative glomerulonephritis (MPGN) according to histological type and to the presence of C3 nephritic factor. Fifty patients aged 2-14 years at the onset of the study were followed over 2-20 years; 26 patients had MPGN type I, 17 had type II and 7 had type III. Treatment was variable. At the last observation, 30 patients had reached terminal and four pre-terminal renal failure. The median survival probability until renal death was 15.3, 8.7 and 15.9 years for disease types I, II and III respectively (difference between MPGN types I + III versus type II: p = 0.013). The presence of an initial nephrotic syndrome was associated with a more rapid progression (p = 0.018). C3 nephritic factor was of no prognostic value. We conclude that the outcome of MPGN mainly depends on the histological type observed.

Adolescent↗

[Sexually aggressive acts of an adolescent with Klinefelter syndrome].

Using the case of an average gifted 15 years old youth with Klinefelter-syndrome (ks), who acted in a sexually aggressive manner, first the characteristics of this syndrome are described. In the following personality development and family background are compared to three hitherto published case examples of youths with ks or similar symptomatics on the one hand, and forty other sexually aggressive acting youths without ks on the other hand. In closing the possible significance of a treatment with a testosterone substitute is discussed.

Adolescent↗

[Effect of cocoa on excretion of oxalate, citrate, magnesium and calcium in the urine of children].

Cocoa is a strong carrier of oxalic acid (average: 400 mg per 100 g). In three calcium oxalate stone formers clinical observation had been suggestive of excessive intake of cocoa products contributing to calculus formation. We studied the effect on renal oxalate excretion of an oral cocoa load (30 g per m2 body surface given on 2 consecutive days) in 12 former stone formers (group 1), 14 children with isolated microscopic haematuria (group 2), 13 healthy boys (group 3), and 12 healthy girls (group 4). A new enzymatic method was used to measure oxalic acid in cocoa products as well as in urine samples by a two step reaction: 1. Oxalate decarboxylase, 2. formiate dehydrogenase with photometry of NADH. In addition, the daily excretion of the following substances was measured: Citrate, magnesium, and calcium. There was a significant increase of urinary oxalate excretion from an average of 14.5 mg/24 hours before to an average of 22.2 mg/24 hours after the load in healthy children, and a similar increase in stone formers, but not in children with microscopic haematuria. The excretion of citrate and magnesium did not change following cocoa intake. The calcium excretion was higher in stone formers than in the other groups, but the difference was significant only compared to group 2. It is concluded that the risk of calculus formation may increase following continuous and excessive intake of cocoa products in children with a tendency toward hypercalciuria. Counselling of the stone formers resulted in a marked drop of the daily oxalate excretion, and there was no recurrence of calculus formation over a period of 6 years.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

[Clinical, sonographic and radiologic findings in spinal dysraphism of the lumbosacral region].

Demonstrating the clinical, sonographic and radiologic findings in three pediatric patients suffering from caudal spinal dysraphism the various diagnostic and therapeutic problems of neural tube defects are discussed. Important conclusions are as follows: (1) Early diagnosed and operated malformations of the caudal spine may cause neurologic, neurosurgical, urogenital, gastrointestinal and orthopedic complications even after uneventful years. Mostly the reason is a prevented ascensus of the conus medullaris. Therefore a longstanding follow-up of these patients is mandatory. (2) The patient's examiners have to look for cutaneous manifestations of spinal dysraphism even in the sacral region to avoid secondary damage. (3) Sonography today is a valuable diagnostic method not only applied for screening examinations but also to establish definite diagnoses in many cases. The application of other methods is therefore guided by sonographic findings.

Child↗

[Metabolism of parenterally administered amino acids in chronic kidney failure in childhood].

Twenty children (8 months to 17 years old) with chronic renal insufficiency were infused with a 10% L-amino acid solution (1 ml/kg B.W. over 3-5 min). Amino acid blood concentrations were measured in the basal state and repeatedly after the end of infusion. The elimination constant, half-life, clearance, corrected transfer and pool size were calculated for the individual amino acids. The results were analyzed for gender specific and age-related differences. The control group consisted of 19 healthy children. (1) Almost all basal free amino acid concentrations in the chronic renal patients showed abnormalities typical for this disease. (2) Calculation of the pharmacokinetic data demonstrated differences between chronic renal patients with predominantly glomerular and those with predominantly tubular disease. (3) The half-lives and elimination constants did not differ from normal. Evaluation of age-related changes demonstrated no abnormalities for these 2 parameters in renal patients. (4) On the average, the clearance value for the group with primarily glomerular disease was 57% of normal; that of the group with predominantly tubular disease was 115% of normal. (5) Analogously, the average corrected transfer was 56% and 113% of normal; the pool size was 67% and 139% of normal. (6) No gender specific differences were found. (7) The clearance values for lysine, threonine, glycine and proline in infants and toddlers exceeded those found in older children. (8) The corrected transfer values for threonine, arginine, histidine, glycine, alanine and serine in children 8 to 31 months of age were significantly higher than those found in the rest of the children. (9) No significant age-related differences in pool size were found.

Adolescent↗

[Membranoproliferative glomerulonephritis. Morphological observations and considerations on pathogenesis].

Membranoproliferative glomerulonephritis (MPGN) is a morphologically defined disease which can be subdivided into three types on the basis of different fine structural basement membrane changes. Type I (43%) is characterized by subendothelial deposits, essentially intact basement membranes, and normal C3 serum values on long term follow-up. Type II (32%), also known as dense deposit disease, is characterized by slight thickening of the basement membranes due to an electron dense, argyrophilic alteration of the basement membrane material the turnover of which seems to be disturbed. The carriers of this lesion can be recognized by persistently depressed serum C3 values. Type III (25%) is accepted by some working groups on the basis of mixed subendothelial and subepithelial deposits with discontinuity of the basement membrane, absence of classical intramembranous dense deposits but presence of a constant or inconstant serum C3 depression. The three types are demonstrated by light and electron micrographs of paediatric renal biopsies and discussed in detail with regard to different pathogenic mechanisms likely to be operative in different fine structural lesions (formation, removal and incorporation of deposits).

Adult↗

Basement membrane-changes in membranoproliferative glomerulonephritis: a light and electron microscopic study.

This study is based on 31 renal biopsies from 28 patients with idiopathic membranoproliferative glomerulonephritis (MPGN). 18 cases were classified as "pure" MPGN, 10 as lobular GN. For light microscopy two staining procedures were found to be of particular value: Pearse trichrome (PAS+Orange G) and Jones-Chromotrope (methenamine silver + Chromotrope 2R). These techniques reveal conspicuous basement membrane (b.m.) lesions which are not observed in other types of GN and are characterized by thickening of the b.m. and a bright orange or red coloration, respectively, which can well be separated from normal (PAS-positive or argyrophilic) basement membranes. Electron microscopy was performed in 13 cases in order 1. to analyze the fine structure of this lesion, 2. to match the results with the current subclassification concept which is essentially based on the discrimination of intramembranous dense (IMDD) and subendothelial deposits. The typical finding of a continuous intramembranous electron-dense material which proves to be argyrophilic in ultrathin sections was present in 3 (23%) of the cases.

Adolescent↗

Interstital nephritis in autoimmune hemolytic anemia.

A case is reported of a 15-year-old boy with chronic autoimmune hemolytic anemia who developed renal insufficiency 3 years after splenectomy. An interstitial nephritis with striking lymphocytic infiltrates and sclerosed glomeruli could be demonstrated by percutaneous renal biopsy. Renal symptoms disappeared promptly after corticosteroid therapy. The renal lesions are thought to have arisen as part of the autoimmune disease.

Adolescent↗

[Sialadenosis as secondary organ manifestation of anorexia nervosa (author's transl)].

In a 13-year-old boy the occurence of a sialadenosis of the submaxillary glands was observed in association with anorexia nervosa (a.n.). The non-inflammatory salivary gland enlargement became apparent after the boy had suffered a progressive weight loss of 5 kilograms during a period of three years prior to admission. The psychogenic origin of the disorder could be confirmed by 1. the history of the patient being almost identical compared to the few observations of a.n. in males, 2. the absence of any other organ involvement, and 3. follow up over a period of two years during which time the boy regained normal weight, and the salivary gland enlargement disappeared spontaneously. The diagnosis of sialadenosis was based on histological examination.

Acute Disease↗