[A 43-year-old patient with character changes, recurrent impaired consciousness and retrograde amnesia]. / 43-jähriger Patient mit Wesensveränderung, rezidivierenden Bewusstseinsstörungen und retrograder Amnesie.

A 43-year-old male patient with recurring impaired consciousness and retrograde amnesia was admitted to the department of neurology. During the neurological evaluation no pathological findings could initially be revealed but one day the patient was confused again and presented with inadequate behavior: at this time a blood glucose value of 40 mg/dl was measured. For further evaluation the patient was transferred to our department. As the reason for the impaired consciousness was suspected to be of neuroglucopenic origin a rapid adrenocorticotropic hormone (ACTH) stimulation test was first performed to rule out adrenal insufficiency. For further evaluation a fasting test was conducted: after 48 h an episode with neuroglucopenic symptoms occurred again which disappeared after intravenous administration of glucose. The laboratory results of glucose, insulin and c-peptide determined at this point in time led to the diagnosis of an insulinoma. By ultrasound examination a hypoechogenic lesion 1.5 cm in size could be shown in the head of the pancreas and was confirmed by magnetic resonance imaging (MRI). After duodenum-preserving partial pancreatic head resection with enucleation of the insulinoma no further neuroglucopenic symptoms occurred.

Towards OCT-Navigated Tissue Ablation with a Picosecond Infrared Laser (PIRL) and Mass-Spectrometric Analysis.

Medical lasers are commonly used in interventions to ablate tumor tissue. Recently, the picosecond infrared laser has been introduced, which greatly decreases damaging of surrounding healthy tissue. Further, its ablation plume contains intact biomolecules which can be collected and analyzed by mass spectrometry. This allows for a specific chracterization of the tissue. For a precise treatment, however, a suitable guidance is needed. Further, spatial information is required if the tissue is to be characterized at different parts in the ablated area. Therefore, we propose a system which employs optical coherence tomography as the guiding imaging modality. We describe a prototypical system which provides automatic ablation of areas defined in the image data. For this purpose, we use a calibration with a robot which drives the laser fiber and collects the arising plume. We demonstrate our system on porcine tissue samples.

Esophagogastric junction pressure topography after fundoplication.

OBJECTIVES: This study compared the pressure topography after laparoscopic Nissen fundoplication to that of normal subjects and patients with hiatal hernia and reflux disease. METHODS: Seven patients with fundoplication, 7 normal subjects and 7 patients with hiatal hernia, were studied. The squamocolumnar junction and intragastric margin of the esophagogastric junction (EGJ) were marked with metal clips. Axial and radial characteristics of EGJ pressure were mapped relative to the hernia and clipped during concurrent fluoroscopy and manometry. Responses to inspiration and abdominal compression were also analyzed. RESULTS: Fundoplication modifies the EGJ by restoration of the hiatal component of EGJ pressure and elongation of the subdiaphragmatic component. Maximal EGJ pressure after fundoplication is mainly dependent on the extrinsic effect of the hiatal canal that compresses the esophagus; the resultant length of the EGJ reflects the length of the fundic wrap. Integrity of the EGJ after fundoplication is independent of the intrinsic lower esophageal sphincter itself. CONCLUSIONS: Fundoplication alters the pressure topography of the EGJ by reducing the hiatal hernia, tightening the hiatal orifice, and constructing a subdiaphragmatic wrap of variable length. Each effect depends on different technical aspects of the surgery with the potential of substantial variability in the resultant pressure topography.

Familial congenital short small bowel with associated defects. A long-term survival.

In 1974, Royer et al. described a familial syndrome consisting of a short and sluggish small bowel, malrotation of the gut, and pyloric stenosis. These authors stressed the uniformly fatal outcome of their four cases, as well as other possibly unrecognized cases previously described in the literature. The present report deals with two more familial cases, of which one represents a long-term survivor of the syndrome. The intensive work of maintaining nutrition, controlling infection, and managing the complications of associated defects are described.

Pathogenicity of Fusarium graminearum Schwabe isolates from Poland towards seedlings of cereal species.

Ten isolates ofFusarium graminearum Schwabe originating from diseased cereal plants and kernels were tested for pathogenicity to various cultivars of wheat, rye, triticale and oats. The isolates varied greatly in their pathogenicity to the seedlings of the species, and were most pathogenic to rye and triticale, less pathogenic to barley and wheat and least pathogenic to oats.

Auxin and gibberellin-like substances synthesis by Fusarium isolates pathogenic to corn seedlings.

The four isolates studied produced considerable amounts of auxin in media with tryptophan and two of them produced also considerable amounts in tryptophan-free media. The quantity of auxin synthesized increased with decreasing pathogenicity of the fungi. All of them produced also considerable amounts of gibberellin-like substances. There was no clear reaction between the production of the above substances and the pathogenicity.

Production of trichothecenes and zearalenone by Fusarium species isolated from wheat.

Fusaria isolates from wheat were tested for ability to produce trichothecenes and zearalenone. Four isolates of F. culmorum out of 13 produced vomitoxin (DON) and 3 Ac-DON, one produced diacetoxysirpenol and 12 zearalenone. Particularly high yield of zearalenone was observed in cultures of sever pathogenic isolates. Higher temperature (20 degrees C) during first week of incubation favoured yield of zearalenone. About 50% of zearalenone was produced by surface mycelium.

The effect of hiatus hernia on gastro-oesophageal junction pressure.

BACKGROUND: Hiatus hernia and lower oesophageal sphincter hypotension are often viewed as opposing hypotheses for gastro-oesophageal junction incompetence. AIMS: To examine the interaction between hiatus hernia and lower oesophageal sphincter hypotension. METHODS: In seven normal subjects and seven patients with hiatus hernia, the squamocolumnar junction and intragastric margin of the gastro-oesophageal junction were marked with endoscopically placed clips. Axial and radial characteristics of the gastro-oesophageal junction high pressure zone were mapped relative to the hiatus and clips during concurrent fluoroscopy and manometry. Responses to inspiration and abdominal compression were also analysed. RESULTS: In normal individuals the squamocolumnar junction was 0.5 cm below the hiatus and the gastro-oesophageal junction high pressure zone extended 1.1 cm distal to that. In those with hiatus hernia, the gastro-oesophageal junction high pressure zone had two discrete segments, one proximal to the squamocolumnar junction and one distal, attributable to the extrinsic compression within the hiatal canal. Inspiration and abdominal compression mainly augmented the distal one. Simulation of hernia reduction by algebraically summing the proximal segment pressures with the hiatal canal pressures restored normal maximal pressure, radial asymmetry, and dynamic responses of the gastro-oesophageal junction. CONCLUSIONS: Hiatus hernia reduces lower oesophageal sphincter pressure and alters its dynamic responsiveness by spatially separating pressure components derived from the intrinsic lower oesophageal sphincter and the extrinsic compression of the oesophagus within the hiatal canal.

Manometric characteristics of the upper esophageal sphincter recorded with a microsleeve.

OBJECTIVES: We compared manometric recordings of the upper esophageal sphincter (UES) recorded with a miniature sleeve to those obtained using standard manometry. METHODS: The UES pressure of eight volunteer subjects was measured by station pull-through (SPT), by rapid pull-through (RPT), and with a microsleeve sensor for 30 min, followed by 15 min of esophageal acid infusion. Deglutitive UES relaxation recorded with a microsleeve and solid state sensor were compared. RESULTS: The UES pressure recorded with the microsleeve (25+/-9 mm Hg) was significantly less than that by SPT (114+/-18 mm Hg) or RPT (152+/-19 mm Hg), and was unaffected by acid infusion. Periods of low UES pressure were observed during long interswallow intervals (11+/-4, range 6-18 mm Hg). Deglutitive relaxation duration and intrabolus pressure measured with the microsleeve were less than those recorded by the solid state transducer. CONCLUSIONS: "Normal" UES pressure is heavily dependent on measurement technique; pressures obtained with a miniature sleeve are a fraction of those obtained by SPT or RPT. During periods of relative comfort with minimal swallowing, UES tone is approximately 10 mm Hg, similar to that during sleep. Volume modulation of deglutitive UES relaxation is demonstrable with a microsleeve, albeit with less precision than with a solid-state transducer.

Zearalenone formation byFusarium crookwellense /Burgess, Nelson and Toussoun/ isolates from Poland and their pathogenicity towards cereals.

Fusarium crookwellense /B.N. and T./ isolated from affected cereals in Poland formed zearalenone on wheat grain up to 602 mg/kg. Tested isolates have been found strong to severe pathogens of wheat, rye, triticale and barley seedlings and corn ears with pathogenicity similar to that ofF. culmorum andF. graminearum.

Formation of Avenacein Y by Fusarium avenaceum Fries Sacc. isolates from Germany and pathogenicity of the isolates to cereal seedlings.

Twelve isolates ofFusarium avenaceum Fries Sacc. originating from diseased corn plants from Germany produced Avenacein Y in amounts ranging from 0.001 to 1.6 g/kg of wheat grain. The isolates proved most pathogenic to triticale seedlings, less pathogenic to rye seedlings and least to wheat. Pathogenicity of the isolates was not correlated with their ability to produce Avenacein Y.

The connection between age, job control and sickness absences among Finnish food workers.

METHODS: The relationship between sickness absence and job control among industrial food workers of different ages (n = 114) was studied. RESULTS: The number of absence spells, particularly short absence spells, was higher among younger workers, as expected. Using multivariate analysis, low job control was associated with an increased number of all absence spells (P < 0.02). CONCLUSIONS: Job control may be an important factor in determining sickness absence among industrial workers.

Mycotoxins in cereal grain. Part IX. Zearalenone and Fusaria in wheat, barley, rye and corn kernels.

Toxigenic Fusaria, producing zearalenone, appeared in 31% of cereal grain samples. However zearalenone was present only in 0.5% of 584 cereal samples assayed during 1979-1981 at level 0.2-1.2 mg/kg. Fungi able to form zearalenone were present mainly in grain collected from breeding stations. Fusarium culmorum was dominant among toxigenic isolates from domestic cereals and yield of zearalenone was up to 700 mg/kg.

Living on the edge: a phenomenological study of medically uninsured working Americans.

An estimated 35 to 50 million Americans have no medical insurance; the vast majority are employed persons and their dependents. This phenomenological study was developed to make visible the experience of working Americans living on the edge-forced to walk a fine line between health and illness without the safety net of medical insurance. A purposive sample of 12 individuals was asked, "What is it like to be working and without medical insurance? Based on textual analysis, using an adaptation of Colazzi's method, themes were grouped into four theme clusters: A Marginalized Life, Up Against Rocks and Hard Places, Making Choices-Chancing It, and Getting By-More or Less. These are illustrated through commentary and direct quotation to depict an overall sense of the experience. Implications for nurses charged with addressing the needs of the medically uninsured and for nursing as a whole are discussed.

Increased frequency of transient lower esophageal sphincter relaxation induced by gastric distention in reflux patients with hiatal hernia.

BACKGROUND & AIMS: This study aimed to determine if hiatal hernia influences vulnerability to reflux and transient lower esophageal sphincter relaxation (tLESR) during gastric distention in patients with gastroesophageal reflux disease (GERD). METHODS: Eight normal subjects and 15 patients with GERD were studied. A metal clip attached to the squamocolumnar junction (SCJ) was beneath the hiatus in all control subjects. Eight GERD patients with >/=1-cm SCJ-hiatus separation were considered hernia patients, and 7 with <1-cm separation were considered nonhernia patients. Manometry and esophageal pH were recorded for 30 minutes, after which the stomach was loaded with acid dextrose and the recording continued for 2 hours with intragastric air infusion of 15 mL/min. RESULTS: Baseline reflux was comparable among groups. Gastric distention increased the frequency of reflux by the tLESR mechanism in all groups. Controls and nonhernia patients had median increases of 4.0 and 4.5 in tLESR frequency, respectively, and hernia patients had a median increase of 9.5/h. tLESR frequency was highly correlated with the SCJ-hiatus separation (r = 0.76; P < 0.001). CONCLUSIONS: Gastric air infusion was a potent stimulus for tLESR and reflux. The resultant tLESR frequency was directly proportional to the separation between the SCJ and hiatus, suggesting that the perturbed anatomy associated with hiatal hernia predisposed to eliciting tLESRs in patients with GERD.

Operative manometry and endoscopy during laparoscopic Heller myotomy. An initial experience.

BACKGROUND: We report our initial experience using operative esophageal manometry as an adjunct to endoscopy to determine the completeness of esophagogastric high-pressure zone (HPZ) obliteration during laparoscopic Heller myotomy. METHODS: Between July 1997 and October 1998, we performed laparoscopic Heller myotomies in 20 patients (eight male, 12 female; median age, 41 years). Mean duration of symptoms was 3.2 +/- 2.6 years (r = 0.5-11), and 45% of the patients had received prior dilation or toxin injection. A 16-channel esophageal manometry catheter was placed prior to anesthesia, with sites crossing the lower esophageal sphincter (LES). An endoscope was passed intraoperatively to localize the squamocolumnar junction, and the myotomy was performed. While the translucency was imaged in the area of the incision, we determined the adequacy of myotomy by visual assessment of LES and gastric cardia opening in response to endoscopic air insufflation. Manometry was then performed to detect any potential residual high pressure at the myotomized esophagogastric junction (EGJ). If it was found, the locus of persistent pressure was identified by probing along the myotomy, and residual muscle fibers were cut to yield a minimum pressure at the EGJ. RESULTS: A persistent HPZ was identified after the initial myotomy in 10 of 20 patients (50%). A Dor fundoplasty completed the operation. The mean operating time was 2.6 +/- 0.5 h (median, 2.5; r = 2-3.5 h), and the mean hospital stay was 1.6 +/- 1 days (median, 1, r = 1-5 days). The mean LES pressure was 2 +/- 3 mmHg immediately postmyotomy (p < 0.001 compared with preoperative value). Of 20 patients, only two have reported recurrence of dysphagia (10%). One had a recurrent HPZ on manometry, and one developed esophagitis, which resolved with omeprazole. CONCLUSIONS: Our initial experience suggests that operative esophageal manometry is a useful adjunct to upper endoscopy during laparoscopic Heller myotomy, quantitatively assuring obliteration of the nonrelaxing LES and HPZ.

Lower esophageal sphincter relaxation characteristics using a sleeve sensor in clinical manometry.

OBJECTIVE: We undertook this study to determine the characteristics of swallow-induced lower esophageal sphincter (LES) relaxation in the setting of clinical manometry using a standardized methodology. METHODS: We reviewed 170 manometric recordings performed using a perfused manometric assembly with a sleeve sensor and a computer polygraph. Patients were categorized as patient controls, gastroesophageal reflux disease (GERD), diffuse esophageal spasm (DES), or achalasia. Tracing were semiautomatically analyzed for basal LES pressure, LES pressure during deglutitive relaxation (relaxation LES pressure), duration of LES relaxation, timing of LES relaxation, and the success rate of primary peristalsis. RESULTS: Forty-six patient controls, 93 with GERD, five with DES, and 26 with achalasia were identified. GERD and achalasia patients had lower or higher basal LES pressures than patient controls, respectively. Compared with patient controls, achalasia patients had higher relaxation LES pressures, lower percent LES relaxation, and shorter durations of LES relaxation. The best single measure for distinguishing achalasia was the relaxation LES pressure; using the 95th percentile value of patient controls (12 mm Hg) as the upper limit of normal, its sensitivity and positive predictive value for the diagnosis of achalasia were 92% and 88%, respectively. Coupled with the finding of aperistalsis, a relaxation LES pressure > or = 10 mm Hg achieved 100% sensitivity and positive predictive value among these patients. CONCLUSIONS: Sleeve sensor recording is a practical method for clinical manometry that reliably records LES relaxation characteristics and is amenable to both a standardized manometry protocol and a semiautomated analysis routine. Relaxation LES pressure has a high diagnostic value for achalasia.

The nutritional therapy of histidinemia.

Control of the plasma histidine level in histidinemia is possible with the use of an amino acid mixture free of histidine and a carefully monitored intake of histidine. This regimen is compatible with good physical growth and normal mental development. If further clinical experience demonstrates that widespread nutritional intervention in this disease is warranted, it should be possible to obtain good biochemical control.

Multilevel lumbar disc herniation in 12-year-old twins.

Herniation of a lumbar disc in the pediatric age group is rare. A 12-year-old female twin developed backache and left sciatica after a mild lifting injury. Magnetic resonance imaging of the spine showed multilevel lumbar disc herniation. The patient was managed conservatively and her symptoms subsided within 6 weeks. Magnetic resonance imaging of her asymptomatic twin sister revealed a similar pattern of disc degeneration and multilevel herniation. This report emphasizes the hereditary nature of juvenile lumbar disc degeneration.

Bolus transit assessed by an esophageal stress test in postfundoplication dysphagia.

INTRODUCTION: Dysphagia is common after Nissen fundoplication but the relationship between dysphagia and bolus transit is poorly defined. This study compared bolus transit of fundoplication patients to normal individuals. METHODS: Twelve fundoplication patients and 20 healthy volunteers rated their ability to swallow eight bolus consistencies from no difficulty (0) to extreme difficulty (3) to compute a dysphagia score (range = 0-24). A 16-lumen manometric assembly was positioned across the esophagogastric junction (EGJ) and subjects were imaged fluoroscopically in a supine posture while swallowing 5 cc liquid barium and a 5-cc marshmallow-like viscoelastic barium bolus. Videofluoroscopic images were analyzed for total esophageal transit time and the fraction of time required to cross the EGJ. Manometric tracings were analyzed for the intrabolus pressure proximal to the EGJ, intragastric pressure, and distal peristaltic amplitude for each bolus. RESULTS: Dysphagia scores for fundoplication patients were significantly higher (7.3 +/- 5.1, range = 1-17) than for normals (0.5 +/- 0.6, range = 0-2). This correlated with longer total transit times for liquids and solids (r = 0.60, P < 0.01) and a greater percentage of transit time attributable to the EGJ transit. Retrograde flow at the EGJ (escape of bolus proximally up the esophagus) and peristaltic dysfunction were more frequent in fundoplication patients. However, no differences existed in manometric parameters between groups. CONCLUSIONS: Fundoplication impairs both liquid and solid esophageal bolus transit. Dysphagia perceived by fundoplication patients correlated with increased transit time, particularly across the EGJ. Combined quantitative evaluation with manometry and fluoroscopy reveals functional defects in fundoplication subjects, which are not evident by either modality alone.