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Biomedical subjects

M Brandis

Publications and source records attributed to M Brandis.

At least 91 records · Page 5Linked to original sources
PubMed1995

Zinc deficiency in an exclusively breast-fed preterm infant.

UNLABELLED: A formerly premature, exclusively breast-fed infant with severe zinc deficiency syndrome is presented. He showed the characteristic erosive skin changes, including alopecia, as seen in acrodermatitis enteropathica. In addition, he manifested a failure to thrive and irritability. The diagnosis was confirmed by reduced serum levels of zinc (2.3 mumol/l) and alkaline phosphatase (45 U/l). We consider the reduced zinc supply in the breast milk (5.7 mumol/l) as the most likely cause of the disease. Therapy consisted of oral zinc supplements (50 mumol/kg/day) for a period of 30 weeks. Symptoms and laboratory values normalized completely and did not recur on a normal diet. CONCLUSION: A diet of breast milk can, in rare circumstances, cause insufficient zinc intake resulting in severe zinc deficiency syndrome with characteristic dermatological features. Therapy consists of temporary oral zinc supplementation at a daily dose of 50 mumol/kg.

Alkaline Phosphatase↗
PubMed1995

Haemangiomatosis in children: value of MRI during therapy.

MRI findings of 18 examinations of six children with haemangiomatosis and one with extended arteriovenous malformation are reported. Structures involved were the liver, liver and lung, periorbital area and the thigh. Response to interferon therapy, in particular, was assessed. With MRI the disease can be characterized and the extent of the lesions and size of the haemangiomas measured. Coronal views provide excellent demonstration of the involved structures in liver and lung haemangiomatosis. Two children showed response to interferon therapy with a reduction in lesion size and subsequently in number. Signal intensity decreased slightly on T2-weighted images. During treatment, however, no definitive fibrotic zones were seen. Following complete regression, signal intensity of the liver parenchyma was homogeneous in both weightings, that is, no fibrotic areas were visible 18 months after the beginning of treatment. Two children showed no response and one child died from congestive cardiac failure. The periorbital haemangioma was reduced in size and the lesion in the thigh might be classified as an arteriovenous malformation. In children MRI can replace CT as it is a reliable imaging modality for diagnosing haemangiomatosis and monitoring therapy.

Child↗
PubMed1995

Value of RARE-MRI sequences in the diagnosis of lymphangiomatosis in children.

Three patients suffering from extensive cavernous lymphangiomatosis are presented here. They were examined by MRI using RARE-MR hydrography (rapid acquisition with relaxation enhancement) as well as conventional spin-echo sequences. RARE sequences, which depict each fluid-filled lymphatic space, can be used for screening. RARE-sequences help to shorten investigation time, particularly in cases involving the skeleton. The imaging strategy can be changed according to the results of this sequence. It may be performed prior to spin-echo sequences and facilitates follow-up investigations. RARE sequences distinguish between lymphangiomatosis and hemangiomatosis, or a combination of the two.

Adult↗
PubMed1995

Hemophagocytosis: a complication of acute Q fever in a child.

We describe an 11-year-old boy with acute Q fever complicated by hemophagocytosis. In addition to pancytopenia, the boy presented with hyponatremia, hypofibrinogenemia, and changes in lipid metabolism. Examination of a bone marrow aspirate revealed striking hemophagocytosis. All of the pathological changes were caused by an activated macrophage system. We also discuss the possible role that pathogenetic mechanisms play in these changes.

Acute Disease↗
PubMed1995

EBV-associated lymphoproliferative syndrome with a distinct 69 base-pair deletion in the LMP-1 oncogene.

We describe an immunocompetent 12-year-old boy with chronic EBV infection and lymphoid interstitial pneumonitis. Lymph node biopsies showed effacement of the architecture with polymorphic cellular infiltrates, consisting predominantly of T cells and natural killer cells. No clonal rearrangement of TCR or immunoglobulin genes was seen. DNA was extracted from hilar lymph nodes; sequencing of the carboxy terminal region of the latent membrane protein 1 (LMP-1) oncogene revealed a 69 base-pair deletion and four point mutations. Immunosuppressive treatment with prednisone and cyclosporine reversed the lymphadenopathy.

Base Composition↗
PubMed1995

Endothelin (ET-1) is involved in the contrast media induced nephrotoxicity in children with congenital heart disease.

In 77 children with congenital heart disease urinary endothelin-1 (ET-1), an indicator of intrarenal endothelin release, was compared to urinary excretion of total protein, albumin, immunoglobuline G (IgG), alpha 1-microglobuline (alpha 1-MG), N-acetyl-beta-D-glucosaminidase (NAG) and villin. Urine samples were collected the day before and immediately after cardiac angiography with high (Conray 70; n = 56; CON) or low osmolality contrast media (Solutrast 300; n = 21; SOL) to assess the relationship between urinary endothelin and glomerular and tubular nephrotoxicity of contrast media. The children were further subdivided according to age: less than 1 year-CON 1 (n = 20); SOL 1 (n = 12) and 1-18 years CON 2 (n = 36); SOL 2 (n = 9). Results (median): 1. There are no significant changes in total protein-, albumin- and IgG-excretion as parameters of glomerular toxicity. 2. Tubular toxicity of contrast media is shown by significant increase of alpha 1-MG-(10.0 to 23.2 mg/g Crea; p < 0.001), NAG-(5.9 to 9.6 mg/g Crea; p < 0.001) and Villin-excretion (1.0 to 2.0 STS, p < 0.001) in all children. 3. Endothelin excretion (101.0 to 163.0 ng/g Crea, p < 0.001) and concentration (42.5 to 56.0 pg/ml; p < 0.001) were elevated after angiography in all children. 4. The changes in endothelin excretion are correlated to the changes in alpha 1-MG (r = 0.65; p < 0.001) and NAG (r = 0.43, p < 0.001) in all children.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1994

Renal involvement in tuberous sclerosis complex: a retrospective survey.

In a retrospective survey performed in Germany and Switzerland, 207 patients (ranging in age from newborn to 70 years) were evaluated in order to establish the frequency, prognosis and diagnostic awareness of kidney involvement in the tuberous sclerosis complex. Renal manifestations were observed in 48% of patients: renal cysts (33 patients), renal angiomyolipoma (AML) (30 patients), a combination of both (8 patients), renal cell carcinoma (3 patients), life-threatening events such as haemorrhage (4 patients), hypertensive crisis (2 patients) and chronic renal failure (10 patients) were also documented. The diagnostic imaging techniques of ultrasonography, intravenous urography, computed tomography and magnetic resonance imaging (MRI) are important but do not always yield definitive information. Differentiation between AML and cysts can be achieved using special MRI techniques (RARE). The potential for renal involvement should be monitored in all patients with the tuberous sclerosis complex.

Adolescent↗
PubMed1994

Dithio-bis-mercaptoethanesulphonate (DIMESNA) does not prevent cellular damage by metabolites of ifosfamide and cyclophosphamide in LLC-PK1 cells.

Ifosfamide (IF) is an alkylating cytostatic with urotoxic (haemorrhagic cystitis) and nephrotoxic (Fanconi syndrome) side effects. Cyclophosphamide (CP), a structural isomer of IF, shows urotoxic but no nephrotoxic side effects. The development of haemorrhagic cystitis during therapy with IF or CP can be prevented by the uroprotective drug sodium-2-mercaptoethanesulphonate (MESNA). However, even in the presence of MESNA, Fanconi syndrome may still develop after therapy with IF. Using the renal tubular cell line LLC-PK1, we investigated whether there is a protective effect of either MESNA or of its major metabolite DIMESNA, in combination with metabolites of IF or CP, on thymidine incorporation, uridine incorporation or total protein. DIMESNA, the dimer of MESNA, is the dominant form of the molecule in the circulation; the proximal tubular cell must convert this back to MESNA at the expense of glutathione, before it can exert its uroprotective action. We did not find a protective effect of DIMESNA under any of the experimental conditions tested. LLC-PK1 cells exposed to 3 mmol/l DIMESNA did not convert DIMESNA to MESNA. The toxic effect of the CP metabolite 4-OOH-CP was more pronounced in the presence of DIMESNA than in its absence. MESNA completely prevented the toxic effects of acrolein and of 4-OOH-CP. The toxic effects of 4-OOH-IF and of chloracetaldehyde, two major metabolites of IF, were significantly reduced in the presence of MESNA. However, even at 30-fold molar excess of MESNA over a 4-OOH_IF, thymidine incorporation remained reduced by 40% compared with controls, indicating incomplete protection of tubular cells against metabolites of IF. Similarly, the effect of chloracetaldehyde was not completely reversed by MESNA.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗
PubMed1994

Toxicity of ifosfamide, cyclophosphamide and their metabolites in renal tubular cells in culture.

Ifosfamide (IF) and cyclophosphamide (CP) are highly effective alkylating cytostatic drugs. IF and CP have to be activated through a metabolic step in vivo; numerous metabolites are known. While both IF and its structural isomer CP have severe urotoxic side effects, only IF is also a nephrotoxic drug, causing tubular damage resulting in Fanconi syndrome in some cases. Little information is available regarding the pathogenic mechanism of tubular damage by IF. We used the renal epithelial cell line LLC-PK1, which has many properties of the proximal tubule, in order to investigate the toxicity of IF and CP and of their reactive metabolites 4-hydroxy-IF (4-OH-IF), 4-hydroxy-CP (4-OH-CP), acrolein and chloroacetaldehyde (CAA). Protein content of monolayers, DNA and RNA synthesis were determined by standard techniques (thymidine and uridine incorporation). IF and CP had the lowest toxicities of all compounds tested. Both drugs inhibited thymidine incorporation by about 30% at a concentration of 300 mumol/l after 1 h incubation. 4-OH-IF and 4-OH-CP were significantly more toxic than the parent drugs. Thymidine incorporation, the most sensitive parameter, was reduced by about 70% by 300 mumol/l of either compound. In addition, 4-OH-CP reduced the total protein content of monolayers. 4-OH-IF did not effect protein content and RNA synthesis. Acrolein, the most toxic metabolite tested, reduced all three parameters significantly at concentrations of 50-75 mumol/l after 1 h.(ABSTRACT TRUNCATED AT 250 WORDS)

Acetaldehyde↗
PubMed1994

Endothelin excretion during ketoacidosis does not correlate with tubular dysfunction.

Urinary excretion of endothelin was measured by radioimmunoassay in children with diabetes mellitus during severe ketoacidosis and 12 days later when blood pH and blood glucose concentrations were normal. Metabolically stable diabetic children served as controls. Results from apparently healthy children without diabetes mellitus were used as normal values. Renal tubular injury was evaluated by the urinary excretion of the proximal tubular marker alpha 1-microglobulin and the distal tubular marker Tamm-Horsfall protein (THP). During ketoacidosis we detected a decreased glomerular filtration rate associated with highly significant changes in the excretion of alpha 1-microglobulin, indicating proximal tubular damage, and THP, suggesting disturbance of cells of the ascending loop of Henle. The daily excretion of endothelin was unaltered but the ratio of endothelin/creatinine or endothelin excretion/creatinine clearance were significantly enhanced. In conclusion, we could demonstrate that, despite a proximal and distal tubular cell dysfunction, endothelin excretion is not elevated during ketoacidosis. The increased endothelin excretion when related to creatinine clearance may be a consequence of disturbed proximal tubular function or dysfunction of the renal medulla.

Adolescent↗
PubMed1994

Renal excretion of endothelin in children is influenced by age and diuresis.

Endothelin is a peptide with vasoactive and diuretic potential. Its release has been demonstrated from endothelial and renal epithelial cells. Urinary excretion of endothelin, as shown by others, is thought to reflect intrarenal production. We measured endothelin by RIA in a population of healthy children from Germany and Hungary (group 1), neonates (group 3) and children before and during forced diuresis (groups 2a and 2b). Group 1 consisted of 24 children living in Germany and 13 children resident in Hungary. The age range in this group was 2.9-17 years. Daily excretion correlated significantly with age (r = 0.48, p < 0.001), but endothelin excretion corrected for body surface area remained constant with regard to the age group studied. This indicates that body or kidney size may influence endothelin excretion, respectively. There was no difference in endothelin excretion between the two countries. In premature infants and neonates (group 3), daily excretion of endothelin was highest in infants with very low gestational ages and decreased in full-term neonates to values not significantly different to the group of older children. The high values in premature infants may have been influenced by mechanical ventilation of physical stress, which cannot be differentiated in this study, however. In contrast to reported results in adults, renal excretion of endothelin was correlated positively to urine flow in all groups. Furthermore, the influence of forced diuresis was evaluated in 10 children with oncological disease before (group 2a) and during (group 2b) forced diuresis with fluid load (3 l/m2; n = 4) and fluid load with furosemide injection (0.3-1.0 mg/kg body weight; n = 6).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1994

Salmonella enteritidis orchitis in a 10-week-old boy.

Extraintestinal manifestations of salmonellosis in paediatric patients are found predominantly in infants less than three months of age. Genital involvement is a rare complication. We present a short review of the literature and describe the case of a 10-week-old boy suffering from severe diarrhoea, who presented with a swelling of the right testicle after six days of illness. He underwent surgery on suspicion of testicular torsion, whereby orchitis was diagnosed. Salmonella enteritidis was cultured from the intraoperative swab. All cultures from blood, CSF and urine remained sterile. We conclude that orchitis must be taken into consideration as an extraintestinal complication of enteric salmonellosis and as a differential diagnosis of testicular torsion. In addition, we wish to emphasize that any infant less than three months of age with suspected or proven salmonellosis, should receive early antibiotic treatment.

Anti-Bacterial Agents↗
PubMed1993

Incidence and etiology of ifosfamide nephrotoxicity: report of a meeting held in Rhodes, Greece, October 3, 1991, sponsored by Asta Medica, Frankfurt, Germany.

The nephrotoxic potential of ifosfamide is now clearly recognized but the true incidence is unknown and risk factors are uncertain. There are, as yet, few studies which systemically explore these issues although many centers have collected data from patients receiving ifosfamide. These support the need for collaborative studies to look at the influence of probable risk factors such as age, cumulative/dose, schedule, and exposure to other nephrotoxic drugs. The ability to detect acute subclinical changes in renal function may provide the opportunity to predict subsequent clinical toxicity. The consensus of opinion recorded so far provides the basis of recommendations for future studies.

Child↗
PubMed1993

Renal proximal and distal tubular function is attenuated in diabetes mellitus type 1 as determined by the renal excretion of alpha 1-microglobulin and Tamm-Horsfall protein.

Diabetic nephropathy is usually characterized by glomerular dysfunction; the view that tubular damage occurs as a consequence, however, has been disputed. To verify this hypothesis we compared glomerular with proximal and distal tubular parameters in 62 patients with diabetes mellitus type I. The duration of disease ranged between 0 and 39 years and the glomerular, proximal tubular, and distal tubular parameters were investigated in 24-h urine samples. Excretion of albumin as a marker of the glomerulum, alpha 1-microglobulin and N-acetyl-beta-D-glucosaminidase as parameters of proximal tubule, and Tamm-Horsfall protein as parameter of distal tubule were determined by sensitive enzyme-linked immunosorbent assays. Patients were divided into five groups (D1-D5) according to the duration of diabetes as follows: D1, less than 1 year; D2, 1-4 years; D3, 5-9 years; D4, 10-14 years; D5, longer than 14 years. Healthy individuals (n = 61) aged 3-42 years served as controls. Significantly increased excretion of proximal tubular parameters were found in early course while albumin excretion was still in the normal range. In addition, proximal tubular alpha 1-microglobulin showed an increase during the course of diabetes duration, probably indicating an early proximal tubular impairment. Distal tubular Tamm-Horsfall protein showed increasing excretion in D1-D4, which may reflect disturbance of the thick ascending loop of Henle. Our results therefore stress the importance of tubular parameters such as alpha 1-microglobulin during early diabetes mellitus type I since they may serve as early markers of renal dysfunction and may precede albumin excretion.(ABSTRACT TRUNCATED AT 250 WORDS)

Acetylglucosaminidase↗