A Chinese adolescent girl with Fechtner-like syndrome

We describe a 15-y-old girl with Fechtner-like syndrome, who is the first Chinese reported to have this rare syndrome. She presented with left homonymous hemianopia and neuroimaging revealed haemorrhage in both parietal and occipital lobes. Peripheral blood smear showed macrothrombocytopenia and intracytoplasmic inclusion bodies inside leucocytes. Thrombocytopenia and proteinuria responded to intravenous immunoglobulin and pulsed methylprednisolone. This case illustrates that life-threatening haemorrhage can occur in patients with Fechtner syndrome. Although there was no effective treatment reported in the literature, high dose steroid and immunoglobulin seemed to be useful in our patient. Our patient also had nephritic-nephrotic syndrome with renal insufficiency, which is unusual in adolescent female patients.     

SUICIDE ATTEMPT DUE TO METOCLOPRAMIDE-INDUCED AKATHISIA

Akathisia as a side-effect of metoclopramide has received increasing attention in consultation-liaison psychiatry in recent years. A case of metoclopramide-induced akathisia resulting in a suicide attempt is reported in order to highlight the suffering of such patients and the factors that lead to misdiagnosis.     

Carcinoid tumor of the ampulla of Vater. Morphology, diagnosis and therapy of a rare papilla tumor

We report on a patient with a carcinoid tumour of the ampulla of Vater and review the 33 known cases of the literature with regard to clinical presentation, morphology and therapy. As it is apparent from the location of the tumour, obstructive jaundice, pancreatitis and nonspecific upper abdominal complaints are the most frequent symptoms. The ampullary carcinoid belongs histogenetically to the APUD cell system with the ability of multiple endocrine activity. The expression of somatostatin peptide and neuron-specific enolase are the most common histopathological findings. A systemic function of these hormones, however, has not yet been shown. For diagnostic purposes in surgical pathology we found synaptophysin and chromogranin A to be important markers. For surgical treatment, partial duodenopancreatectomy remains the current therapy of choise, if the tumour exceeds two centimeters in diameter.     

Current practice patterns in pancreatic surgery: Results of a multi-institutional analysis of seven large surgical departments in germany with 1454 pancreatic head resections, 1999 to 2004 (german advanced surgical treatment study group)

Despite decreasing mortality rates, morbidity is still high after pancreatic head resection. Comparative data in the United States and Europe show a relationship between hospital volume and mortality. Treatment strategies vary frequently, partially because of the lack of evidence-based data. We performed a multi-institutional analysis in Germany evaluating current numbers, indications, techniques, and complication rates of pancreatic head resection. Questionnaires were completed by seven high-volume surgical departments regarding quantitative and qualitative aspects of pancreatic head resections in the period from 1999 to 2004 (five prospective and two retrospective institutional databases). A total of 1454 pancreatic head resections (944 for malignancy) were reported. Mean annual hospital volume ranged from 14 to 52 (10 to 43 in malignancy). Mortality was between 1.1% and 4.8%, morbidity was between 24% and 46%, and pancreatic leakage was between 9% and 20%. In malignant disease, all centers perform standard lymphadenectomy and regard arterial infiltration as a contraindication for resection. However, the rate of portal vein resection varied from 0% to 28%. No consensus is seen on the type of surgery for malignancy and chronic pancreatitis. After resection for pancreatic cancer less than one fourth of the patients receive adjuvant therapy. The results of our analysis in Germany confirm that pancreatic head resection can be performed with low mortality in specialized units. Variations in indications, operative technique, and perioperative care may demonstrate the lack of evidence-based data and/or personal and institutional experience. The low number of patients receiving adjuvant therapy after resection of pancreatic cancer suggests that more efforts must be made to establish novel adjuvant therapies under randomized study conditions. Presented at the Forty-Sixth Annual Meeting of The Society for Surgery of the Alimentary Tract, Chicago, Illinois, May 14–18, 2005 (oral presentation).     

Predictive value of normal sperm morphology: a structured literature review

The aim of the study was to conduct a structured review of theliterature published on the use of normal sperm morphology,as an indicator of male fertility potential in the in-vitrofertilization (IVF) situation, and to establish the universalpredictive value of this semen parameter. Published literaturein which normal sperm morphology was used to predict fertilizationand pregnancy, during the period 1978-1996, was reviewed. Atotal of 216 articles were identified by the sourcing methodology,but only 49 provided data that could be tabulated and analysed.Of these, only 18 provided sufficient data for statistical analysis.Fifteen studies used the strict criteria to evaluate sperm morphology,two used World Health Organization (WHO) guidelines and oneused both the strict criteria and the WHO guidelines. All thestudies (n=10) using the 5 and 14% normal sperm morphology thresholds(strict criteria) produced positive predictive values for IVFsuccess. In the prediction of pregnancy, 82% (9/11) and 75%(6/8) of the studies produced positive predictive values whenusing the 5% and 14% thresholds respectively. Aggregating thedata produced around the 5% normal sperm morphology threshold(strict criteria), the overall fertilization rates were 59.3%(1979/3337; per oocyte) for the 4% group and 77.6% (10345/13327;per oocyte) for the >4% group, and the overall pregnancyrates were 15.2% (60/395; per cycle) and 26.0% (355/1368; percycle) respectively. The no-transfer rates across the 5% thresholdwere 24.0% (86/359; per cycle) in the 4% group compared to 7.4%(80/1088; per cycle) in the >4% group. The inclusion of anaccurately evaluated normal sperm morphology count as an integralpart of the standard semen analysis makes this analysis stillthe most cost-effective means of evaluating the male factor.     

Comparing sulindac with indomethacin for closure of ductus arteriosus in preterm infants

Objectives: A prospective study comparing the efficiacy and side-effects of oral sulindac with intravenous indomethacin in clinically stable preterm infants (<1750 g) requiring non-invasive closure of haemodynamically significant patent ductus arteriosus.
Methodology: As maturity and birthweight are the two major determinants of ductal closure, infants were matched as closely as possible for these parameters. An eligible patient was first assigned to the sulindac group and a subsequent patient with similar gestational age (± 1 week) and birthweight (±100 g) to the previously recruited infant would automatically receive indomethacin. A total of eight infants were enrolled in each group.
Results: The ductus arteriosus was successfully closed in all eight infants receiving indomethacin, and in seven of eight infants receiving sulindac. No significant differences were found with regards to the ductal size between the two groups at diagnosis or on each of the consecutive days of treatment ( P >0.25). More renal adverse effects were encountered in the indomethacin group. Significant differences in changes from baseline value for urine output, plasma sodium, urea and creatinine concentrations were noted at 24, 48 and 72 h after commencement of treatment between the two groups ( P <0.05). All the parameters returned to normal or pre-treatment levels 48 h after stopping therapy. Unexpectedly, severe gastrointestinal complications were encountered in the sulindac group.
Conclusions: Sulindac is capable of promoting ductal constriction in clinically stable preterm infants without compromising the renal function. The spectrum of gastrointestinal complications observed in sulindac treated infants were similar to those described for indomethacin. The use of sulindac for ductal closure in the preterm infant should remain experimental.