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R Behrens

Publications and source records attributed to R Behrens.

At least 19 recordsLinked to original sources
PubMed1999

Late manifestation of Indian childhood cirrhosis in a 3-year-old German girl.

UNLABELLED: We report on a 3.8-year-old German girl who presented with signs of subacute liver failure based on a 4-month history. Investigations done before admission excluded infectious, metabolic and immunological diseases. Laboratory findings were increased values of aminotransferases, hyperbilirubinaemia, hyperammonaemia and deteriorated plasmatic coagulation. Caeruloplasmin and serum copper concentrations were normal; however, urinary copper excretion was elevated (80 microg/l). Liver biopsy showed a micronodular liver cirrhosis and an extremely high liver copper concentration (1400 microg/g dry weight). Epidemiological investigations revealed an elevated copper concentration (8645 microg/l, normal: <2000) and a low pH value (6.3) of the drinking water supplied by copper pipes. The girl had been exposed to copper-contaminated drinking water since the age of 2 years. CONCLUSION: Laboratory, histopathological findings and a proven chronic copper intoxication lead to the diagnosis of Indian childhood cirrhosis in a German girl. Whereas this disease is mostly described in patients with increased copper intake in infancy, our patient developed toxic liver cirrhosis with relatively late copper exposure. Indian childhood cirrhosis should be considered in the differential diagnosis of early childhood liver cirrhosis.

Child, Preschool↗
PubMed1999

Dual versus triple therapy of Helicobacter pylori infection: results of a multicentre trial.

OBJECTIVE: To compare dual therapy (omeprazole and amoxicillin) with triple therapy (omeprazole, amoxicillin, and clarithromycin) in the treatment of Helicobacter pylori infection. The efficacy of 1 mg/kg/day omeprazole was randomly compared with 2 mg/kg/day. STUDY DESIGN: 252 patients (median age, 11.0 years; range, 3-18) presenting with chronic abdominal pain underwent endoscopy and a 13C-urea breath test. Gastric biopsy specimens were taken for histological examination and for the rapid urease test. Patients were treated for two weeks: group A (n = 63) received amoxicillin (50 mg/kg; maximum, 2 g/day), group B (n = 73) received amoxicillin and clarithromycin (20 mg/kg; maximum, 1 g/day). Both groups were randomly treated with either 1 or 2 mg/kg omeprazole (maximum, 80 mg/day). Diagnostic procedures were repeated four weeks after the end of treatment. RESULTS: 11 patients were excluded; 136 patients were H pylori positive (56%), 105 of whom were re-examined after treatment. Helicobacter pylori was eradicated in 52% of group A and 83% of group B. The dose of omeprazole had no influence on the eradication rate. Specificity and sensitivity of the rapid urease test were 94% and 93%, respectively. Specificity and sensitivity of the 13C-urea breath test were 93% and 95%, respectively. CONCLUSIONS: Dual therapy can no longer be recommended. Triple therapy is more effective than dual therapy in the eradication of H pylori infection. The lower dose of 1 mg/kg omeprazole was as effective as 2 mg/kg.

Adolescent↗
PubMed1999

Another case of achalasia-microcephaly syndrome.

The first German patient with achalasia-microcephaly syndrome is described. The mother was exposed to the anti-malarial drug Mefloquine during the first 8 weeks of pregnancy.

Abnormalities, Drug-Induced↗
PubMed1998

[Eosinophil cationic protein in a 39-year-old patient with Churg-Strauss syndrome].

HISTORY AND CLINICAL FINDINGS: A 39-year-old woman in good health suddenly developed painful paraesthesia and incomplete paresis of all four limbs. Furthermore, arterial hypertension and tachycardia were found. The patient reported a history of chronic allergic rhinitis for 10 years and asthma one year. INVESTIGATIONS: Laboratory data showed an increased leucocyte count with a substantial increase in eosinophils in peripheral blood as well as in bone marrow. Nonspecific inflammatory markers such as erythrocyte sedimentation rate, C-reactive protein, fibrinogen as well as eosinophil cationic protein (ECP) were also increased. Histological investigations of a skin-muscle biopsy of the quadriceps revealed necrotizing vasculitis with extravascular granulomata and histiocytic giant cells. DIAGNOSIS, TREATMENT AND COURSE: Based on the diagnosis of Churg-Strauss syndrome treatment with methylprednisolone (500 mg every second day) was started, but was found to be ineffective after 10 days. Symptoms responded well to a subsequent course of 150 mg cyclophosphamide combined with 50 mg prednisolone per day. Pareses and pain were significantly reduced and all qualitative nerve functions returned to normal within two weeks of treatment. Laboratory parameters, especially the ECP, were similarly normalized. CONCLUSION: Churg-Strauss syndrome should be considered in the differential diagnosis of unexplained polyneuropathy. Determination of ECP may not only help in the diagnosis but does also facilitate monitoring of treatment and of further course of the disease.

Adult↗
PubMed1998

Safety and efficacy of interferon retreatment in children with chronic hepatitis B.

UNLABELLED: More than 50% of children with chronic hepatitis B do not respond to treatment with alpha-interferon. Since these patients continue to display high viral replication and progressive liver disease, retreatment should be considered. To date it has not been well evaluated whether a second course of treatment could increase the response rate. In two alpha-interferon retreatment trials in adult patients the response rate, defined by seroconversion from HBeAg to anti-HBe, ranged between 11% and 44%. One beta-interferon retreatment study in children reported a seroconversion rate of 32%. Regrettably, none of the studies included a control group observing the 'spontaneous' seroconversion rate after a first interferon cycle. Thus, a nonrandomized alpha-interferon retreatment study in children including control patients was performed. Alpha-interferon for retreatment was administered 3 times a week for 16-24 weeks in 15 children (5-16 years) at least 6 months after ceasing the first cycle. Four children received 5 MU/m2 of a natural alpha-interferon and 11 children 9 MU/m2 recombinant alpha-interferon 2b. Follow up was 18-47 months after initial treatment. In parallel, a control group of 19 unretreated children with comparable clinical and demographic data was followed for 12-39 months. HBeAg seroconversion was observed in 5 (33%) of the retreated children and in 5 (26%) of the control patients during follow up. The difference is not significant. In the initially nonresponding children, those with high ALT levels before the first treatment showed late HBeAg seroconversion more frequently than those with low ALT levels (P=0.017) irrespective of retreatment. The ALT level before retreatment was not a predictor for response. CONCLUSIONS: A second cycle of alpha-interferon during the 3 years following the first treatment in nonresponding children with chronic hepatitis B can be safely performed but did not increase HBeAg/anti-HBe seroconversion compared with the spontaneous seroconversion rate of patients without retreatment.

Adolescent↗
PubMed1997

Percutaneous endoscopic gastrostomy in children and adolescents.

BACKGROUND: Long-term nasogastric tube feeding is often associated with irritation of the hypopharynx or dislocation of the tube. These pitfalls may be circumvented by percutaneous endoscopic gastrostomy. Although frequently used in adults, there is limited experience with the procedure in children. METHODS: A series of 139 patients (aged 3 weeks to 36.5 years, mean age, 4.4 years; weight 3.1-60 kg, mean weight, 15 kg) underwent placement of a percutaneous endoscopic gastrostomy because of central dysphagia (n = 103); general dystrophy caused by chronic renal failure, congenital heart disease, neoplasms, or cystic fibrosis (n = 26); requirement for special diets (n = 7); malnutrition related to respiratory insufficiency (n = 2); and gastric volvulus (n = 1). RESULTS: The percutaneous endoscopic gastrostomy was placed either in the stomach (n = 122) or in the duodenum (n = 15). In two patients a direct percutaneous endoscopic jejunostomy was performed, because duodenal placement proved impossible. Percutaneous endoscopic gastrostomies were placed using intravenous sedation (midazolam, etomidate, or diazepam). None of the patients required general or inhalation anesthesia. We observed 19 complications including: dislocation of the duodenal part into the stomach (n = 5); inflammation at the insertion site (n = 3); perforation of the stomach (n = 2), which healed under conservative treatment; disconnection of the retention disk (n = 4); occlusion of the tube (n = 4), and chronic vomiting (n = 1). Mean lifetime of a percutaneous endoscopic gastrostomy was more than 1 year. CONCLUSIONS: Percutaneous endoscopic gastrostomy provides a major improvement for children requiring long-term tube feeding. High efficacy and low rates of complication suggest that percutaneous endoscopic gastrostomy should be considered more often, even in infants.

Adolescent↗
PubMed1997

Congenital microvillous atrophy: report of two cases.

2 infants with chronic severe diarrhoea from birth and with lethal outcome have been studied. Small intestinal biopsies were examined by light and electron microscopy. Severe villous atrophy, complete loss of microvilli or rudimentary forms, intracytoplasmic vesicles, and microvillous inclusions are the characteristic features of the disease. The etiology is unknown. A disturbance in the transport of brush-border proteins to the cell surface is assumed to be the reason for congenital microvillous atrophy.

Atrophy↗
PubMed1996

Different expression of Helicobacter pylori gastritis in children: evidence for a specific pediatric disease?

BACKGROUND: Infection with Helicobacter pylori causes active chronic gastritis. Once the infection is acquired, gastritis will persist for almost the rest of one's life. To date, very few data are available on H. pylori gastritis in relation to age. Therefore, we attempted to investigate whether H. pylori gastritis in children exhibits features different from H. pylori gastritis in adults of two different age groups. MATERIALS AND METHODS: Fifty consecutive children with a median age of 11 years (range, 3-18 years) were compared with two groups of 50 adult patients, one group with a median age of 43 (range, 19-56 years) and another group with a median age of 70 years (range, 59-86 years). All patients had H. pylori gastritis unrelated to active peptic ulcer disease. Two biopsy specimens were taken from the antrum and two from the corpus, and the following gastritis parameters were evaluated: degree and activity of gastritis, H, pylori colonization, replacement of foveolar epithelium by regenerative epithelium, mucous depletion, presence of atrophic gastritis with intestinal metaplasia, and presence of lymphoid follicles. RESULTS: Degree and activity of gastritis, extent of H. pylori colonization, degree of replacement by regenerative epithelium, extent of mucous depletion, degree of atrophic gastritis with intestinal metaplasia, and the presence of lymphoid follicles in the antrum, as well as the presence of lymphoid follicles in the corpus differed significantly (chi-square test: p < .05). All these differences--except the once frequent occurrence of atrophic gastritis with intestinal metaplasia in adults--were attributable to a higher expression of these gastritis parameters in children. CONCLUSIONS: We conclude that H. pylori gastritis, particularly in the antrum, is more severely expressed in childhood. One reason for this might be a child-specific immune response to an infection with H. pylori. Alternatively, infection may represent a pediatric disease characterized by a nonatrophic, highly expressed form of gastritis, which changes its appearance once the host becomes adapted over time.

Adolescent↗
PubMed1995

Effect of growth hormone on IGF-I levels in a patient with growth hormone deficiency and Wilson disease.

This is a case report of a boy with a combination of two rare disorders:growth hormone deficiency (GHD) and Wilson disease. To our knowledge, no comparable case has yet been published in the literature. GHD was diagnosed at the age of 4.5 years (height standard deviation score (SDS) -4.85). However, because of a difficult family background, growth hormone (GH) therapy could not be started. The boy was not seen again until the age of 7.7 years (height SDS -4.77), when GHD was reconfirmed and GH therapy could be initiated (dose 0.6 IU/kg/week). At that time, elevated liver enzymes (GPT 128 U/l, GOT 67 U/l, gamma-GT 28 U/l) confused diagnostic procedures. On GH, growth velocity SDS increased from -1.86 to +4.50 in the first year and +3.87 in the second year, and height SDS increased to -4.26 and -3.59. However, serum IGF-I levels did not normalize (max. 67 ng/ml), and liver enzymes were still elevated. At the age of 10 years, Wilson disease was diagnosed in view of low concentration of serum ceruloplasmin, elevated urinary copper excretion and high copper content in a liver biopsy sample. Under a combined therapy with D-penicillamine and GH, serum liver enzymes decreased, and IGF-I levels increased to normal. Height SDS for chronological age has improved constantly.

Ceruloplasmin↗