ARDS as presenting symptom in an infant with CD40L deficiency (Hyper-IgM syndrome Type 1).

We report on a 4 month old male infant with respiratory syncytial virus (RSV) infection leading to acute respiratory distress syndrome (ARDS). A diagnostic algorithm including extended infectiological and immunological work-up revealed absence of CD40-ligand. ARDS was treated successfully with a complex respiratory therapy plus intravenous immunoglobulin substitution. Molecular analysis detected mutations in the CD40L gene (Hyper-IgM syndrome Type 1). The case underlines the importance of an extended diagnostic work-up in an uncommonly severe course of respiratory infection in early infancy.

Influenza A-associated rhabdomyolysis with acute renal failure.

Rhabdomyolysis induced acute renal failure as a rare complication of influenza A infection has been mainly described in adults. Consideration of this potentially life-threatening complication in pediatric patients presenting with influenza is important as clinical symptoms may be unspecific and early diagnosis leading to prompt treatment is essential to decrease associated morbidity and mortality. We report a 9 year old girl who developed severe rhabdomyolysis with myoglobinuric renal failure associated with influenza A virus infection. Receiving supportive therapy including intensive care management the patient recovered renal function completely.

[Ablative and extremity salvage tumor surgery of the lower extremity--a 10 year comparison]. / Ablative und extremitätenerhaltende Tumorchirurgie der unteren Extremität--Ein 10-Jahres-Vergleich.

AIM: The treatment of choice for local tumor control was amputation in the 1970's. Nowadays, limb salvage procedures have become the new standard, implicating that limb salvage surgery results in a better quality of life. This study attempts to prove this hypothesis. METHOD: In total, 102 patients who survived longer than ten years after tumor treatment of the lower extremities were investigated, of these, 71 patients underwent ablative procedures compared to 31 patients with limb salvage surgery. Operative revisions, education level, and occupational situation were evaluated in both groups. To analyze the outcome of every patient regarding functional results, quality of life, life contentment, and social parameters, the functional evaluation system of the Muskulo-Skeletal-Tumor Society (MSTS), the Freiburger Life-Contentment-Questionnaire (FLZ) and the Quality of Life Questionnaire (QLQ-C-30) of the European Organization of Research and treatment of Cancer (EORTC) were used. RESULTS: Patients treated with a limb salvage procedure underwent more surgical revisions (p < 0.000). Educational level and occupational situation showed no difference in both groups. Functional results reached similar levels in both groups (74.6% vs. 73.8%). Life contentment and Quality of Life measurements showed good results in both groups. The FLZ-questionnaire showed significantly better results for the ablative group in some items. CONCLUSION: The type of surgical local therapy of lower extremity tumors has no measurable effect on quality of life according to long-term follow-up in lower extremity tumors. In cases with a risk of inadequate margins when performing limb salvage surgery, an ablative procedure should be preferred.

Grading of precancerous laryngeal lesions by multiparameter image analysis at separate epithelial layers.

In order to detect early precancerous changes which do not involve the whole thickness of the epithelium, we used a novel image analysing program based on an IBAS system (Kontron, Germany) to determine nuclear DNA content (NC) as well as average nuclear area (NA) and variation of nuclear area (VA), in the entire epithelium and in three sublayers, parabasal, intermediate, and superficial. DNA aneuploidy was found in only half of the cases classified as 'high-grade' (HG) lesions, comprising carcinoma in situ (CIS) and severe epithelial dysplasias (EDIII), and was chiefly demonstrable in the parabasal third of the epithelium. The other lesions were DNA euploid. HG lesions showed highly significant increases of NA and VA at the lower levels of the epithelium when compared with 'low-grade' (LG) lesions comprising moderate and mild epithelial dysplasias (EDII and EDI). Our data show that the combination of multiparameter image analysis with conventional morphology assists in the objective grading of precancerous lesions and permits the reliable detection of high-grade lesions.

Prophylactic antibiotic treatment in therapeutic or complicated diagnostic ERCP: results of a randomized controlled clinical study.

The present study evaluated the effects of prophylactic administration of cefotaxime in patients undergoing therapeutic or complicated diagnostic ERCP. One hundred patients were randomized to two groups of 50 patients each. Patients in group 1 received an intravenous infusion of 2 g cefotaxime 15 minutes before endoscopy; patients in group 2 did not receive an intravenous antibiotic before ERCP (control group). Blood samples were drawn for bacteriologic cultures before endoscopy and 5, 15, 30, and 120 minutes after beginning the procedure. Bacteremia was detected by blood cultures (15- and 30-minute samples) in 4 patients who did not receive prophylactic antibiotics (Escherichia coli in 2 cases, Peptostreptococcus in 1, and Staphylococcus aureus in 1). Cholangitis or sepsis did not occur after the bacteremic episodes in any of these patients. In 4 other patients who did not receive cefotaxime-all of whom had biliary obstruction-clinical cholangitis or sepsis developed during the 3-day follow-up; ERCP had failed to decompress the biliary system completely in these 4 cases. Blood cultures identified bacteria in 3 of these 4 patients (Pseudomonas aeruginosa in 1, E. coli in 2). Thus, bacteremia or clinical sepsis developed in 8/50 patients (16%) in the control group without antibiotic prophylaxis, whereas no such episode was observed in patients who had received prophylactic treatment (chi 2 = 8.7; p < 0.01). The present results indicate that prophylactic administration of an antibiotic such as cefotaxime can reduce the incidence of bacteremia and sepsis in patients who undergo therapeutic or complicated diagnostic ERCP.(ABSTRACT TRUNCATED AT 250 WORDS)