Osteopetrosis: MR characteristics at 1.5 T

Four patients with proved osteopetrosis (three with the infantile malignant form and one with the benign form) were examined with magnetic resonance imaging at 1.5 T. All patients were studied in the coronal and sagittal planes using both short and long repetition time/echo time sequences. The infantile malignant form was characterized by a complete lack of signal from the marrow alternating with a signal intensity equivalent to that of the intervertebral disks, resulting in a "stepladder" appearance. In the benign form or after successful marrow transplantation in the infantile malignant form, intermediate or high signal intensity in the vertebrae was noted, suggesting the presence of some marrow elements.     

Adiponectin levels are high in children with classic congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency

Objective: It has been shown that adiponectin serves as an insulin-sensitizing adipokine. Serum concentrations of adiponectin are low in children with obesity, and increase with fat mass loss, indicating that adiponectin can serve as a biomarker. Since the prevalence of overweight and obesity is increased in children with congenital adrenal hyperplasia (CAH), our study aimed to evaluate serum levels of adiponectin in a cohort of CAH children and adolescents, and their associations with clinical parameters such as chronological age (CA), body mass index (BMI), Tanner stage (TS), medication and metabolic control.
Patients and methods: We studied 51 patients, aged between 5.6 and 19.6 years (median 11.8; 30 females, 21 males), cross-sectionally. All patients had genetically confirmed CAH and received standard steroid substitution therapy. Adiponectin was measured by an enzyme linked immunoassay. Since BMI SDS of the CAH cohort were significantly higher compared to the reference population, we built matched pairs with healthy Caucasian subjects from a normal representative cohort for sex, Tanner stage, chronologic age and BMI.
Results: Adiponectin concentrations were significantly higher in CAH patients (median 11 μg/L) compared to the matched controls (6.7 μg/L, p < 0.0001). Correlation analyses in CAH patients revealed a significant inverse relationship between adiponectin and CA, TS, BMI, serum DHEAS and serum testosterone, but no correlation with hydrocortisone and fludrocortisone dosage.
Conclusion: Currently, the importance of the elevated adiponectin concentrations in CAH children for risk assessment is not clear. However, our data imply that besides adequate metabolic control of glucocorticoid substitution, a long-term follow-up of other metabolic markers of insulin resistance should be conducted in CAH patients.     

"Strukturierter Fragebogen Simulierter Symptome"

The current article addresses the psychometric qualities of the German version of the Structured Inventory of Malingered Symptomatology (SIMS), a self-report measure of malingering. The SIMS was administered to a heterogeneous sample of forensic patients (n=62) and undergraduate students (n=204). Within the student sample, some undergraduates were instructed to feign certain pathological conditions, while others were asked to respond honestly to the SIMS items. The findings indicate that the German version of the SIMS demonstrates adequate test-retest stability and internal consistency. In the patient sample, the SIMS was found to correlate strongly with manipulative and antisocial personality features. More specifically, SIMS scores were higher in sexually delinquent patients with antisocial personality disorders. Our findings support the SIMS as a valuable screening tool for malingering of psychiatric symptoms.     

Morbidity of breast cancer patients following complete axillary dissection or sentinel node biopsy only: a comparative evaluation

Introduction The usefulness of routine axillary dissection (AD) at levels I–II in breast cancer patients has been questioned for years because of the high postoperative morbidity in the shoulder and arm region, and the increasing number of patients with negative nodes. Sentinel node biopsy (SNB) was hoped both to reduce morbidity and to improve the reliability of staging. This study was designed to provide more evidence in this matter by comparing the follow-up data of patients with AD and those with SNB only. Method One hundred forty patients who had undergone AD between 1993 and 1996 were questioned for their subjective and objective symptoms using a questionnaire and subsequently subjected to a clinical examination. Their data were compared with those of 57 patients who had undergone SNB only between 1998 and 2000. Results Local recurrences have not been seen to date. The difference between the two groups in terms of a loss of quality of life was negligible. The differences in overall complaints, number of symptoms, pain, limited range of motion of the operated upper extremity, numbness, paresthesias, and arm swelling as well as perceived disability in activities of daily living were significantly in favor of SNB. The length of hospital stay was significantly shorter for SNB patients. Conclusion SNB appears to be an accurate procedure for axillary nodal staging in breast cancer patients and is associated with reduced postoperative morbidity and length of hospital stay. But it is still investigational and should not be implemented as therapeutical standard before results of randomized trials are published.     

The bottom line: Outcomes after conservation treatment in anal cancer

At the Department of Radiation Oncology, Westmead Hospital, between 1980 and 2000, 60 patients with squamous cell carcinoma of anal canal or margin (including 15 with Stage IIIA or IIIB) were treated radically; 55 received chemoradiation (89% were prescribed mitomycin C and 5‐fluorouracil). Five‐year overall survival was 64% (95% confidence interval (CI): 48–79%), with a median survival of 9.75 years (median follow up 5.6 years, range 5 months to 22.5 years). Ten patients have died of disease. At 2 years the local control rate was 86%, and colostomy‐free survival was 83%. Relapse after 2 years was uncommon. Tumour size was the main factor driving outcomes, especially survival. Patients with larger tumours (T > 4 cm) had a hazard ratio for survival of 5.7 (95% CI: 1.8–17). Fourteen (24%) patients experienced treatment interruptions as a result of acute toxicity, including one death from neutropoenic sepsis. Seven (12%) patients, in total, experienced one or more late toxicities, grade 3 or above, including four women (all postmenopausal) who developed a radiation‐induced bone injury. Most patients with anal cancer can expect to retain a functional sphincter after chemoradiation/radiation. Further studies are in progress to determine the optimal chemoradiation protocol.     

Alveolar capillary dysplasia. Report of a case of prolonged life without extracorporeal membrane oxygenation (ECMO) and review of the literature

We describe an unusual infant with the diagnosis of alveolar capillary dysplasia who had a relatively prolonged life without extracorporeal membrane oxygenation (ECMO). We have used this case as a springboard for a thorough review of the literature. This was a full-term female infant who presented with a picture of persistent pulmonary hypertension of the newborn. She was treated as such, with various ventilatory modes, alkalinizing agents, surfactant therapy, tolazoline, prostacyclin and nitric oxide. Because of the prolonged clinical course the possibility of alveolar capillary dysplasia was raised. The parents refused ECMO. Despite all efforts she progressively deteriorated and died at 22 days of age. Macro- and microscopic examination of the lung at autopsy were diagnostic of alveolar capillary dysplasia. A detailed review of 39 cases published in the literature with comments regarding incidence, etiology, pathophysiology, clinical picture, diagnosis and treatment is presented.     

Efficacy of antimicrobial treatment in non-dysenteric persistent diarrhoea in a community setting

Objectives : To determine the efficacy of antimicrobial treatment in non-dysenteric persistent diarrhoea in a community setting. Methods : In this double-blind field trial, 156 children aged 4 36 months with persistent diarrhoea not associated with Giardia lamblia infestation seeking treatment in a community outpatient clinic, were randomized to receive a combination of nalidixic acid and metronidazole, metronidazole alone, or placebo for 7 days. Results : In comparison with placebo, metronidazole treatment did not result in a significant reduction in the mean post-enrolment diarrhoeal duration and stool frequency, increase in the proportion of patients recovered by days 3, 5 and 7 of treatment, and increase in weight gain at days 7 and 14. Comparing the combination of nalidixic acid and metronidazole with metronidazole alone, 17.5% more children treated with the combination recovered by day 3 of treatment ( p = 0.08) and the mean stool frequency ascertained on day 7 for the previous 24 h was 26.8% less in them ( p = 0.05). The weight gains at days 7 and 14 were similar in the two groups. Conclusions : These findings indicate that metronidazole offers no therapeutic benefit in persistent diarrhoea not associated with Giardia lamblia and nalidixic acid has only a modest clinical benefit, which is not substantial enough to warrant its routine use.     

Long-term variability of zooplankton populations in aquatic mesocosms

The natural variability on a spatial and temporal scale was examined in the zooplankton community of mesocosms from Syngenta Crop Protection AG (Stein, Switzerland), with the focus on improving the experimental design and evaluation of mesocosm studies. Analysis was performed using zooplankton data collected during a three-year period in 3 (1996 and 1998) to 12 (1997) ponds. Interreplicate variability was measured as the variance among the 3 to 12 replicates at each sampling date. Temporal variation was examined as seasonal variability by comparing different sampling dates within a year and as year-to-year variation by comparing pooled data year by year. Univariate and multivariate methods were used for the evaluation of population and community data, respectively. Results from the present study indicate that because of the low interreplicate variability, only data from high-abundance species could be evaluated with a precision able to detect effects less than 20%. For the majority of the zooplankton populations, abundances were lower than 10 organisms/L, with frequent zero counts resulting in a weak evaluation of the data with a precision able to detect effects of greater than 20 and 110%. Ordination analysis of the community data from the three years revealed that approximately 29% of the total variance could be explained by year-to-year differences, whereas 11% could be attributed to seasonal variability within a year. The residual variance can be attributed to interreplicate variability and sampling error. These results were in line with findings for individual populations. The present analysis demonstrated that the inherent variability of a system should be investigated for a proper design and evaluation of mesocosm studies and promotes the use of multivariate tools for a more comprehensive interpretation of mesocosm data.