[Recurrent hypoglycemia and a large intraabdominal tumor in a 61-year-old woman]. / Rezidivierende Hypoglykämien und ein großer intraabdominaler Tumor bei einer 61-jährigen Patientin.

HISTORY AND ADMISSION FINDINGS: A 61-year-old woman was found unconscious by her husband. The emergency doctor detected hypoglycemia (blood glucose 1.7 mmol/l). This was the first such event, the patient had not been known to have diabetes mellitus. At admission the physical examination and the laboratory findings revealed no abnormalities. INVESTIGATIONS: A fasting test was aborted shortly after the start because of the onset of neurological symptoms. An insulinoma was excluded by detecting suppressed levels of insulin and C-peptide. Computed tomography of the abdomen revealed a mesenteric tumour of 9 cm in diameter, which was identified immunhistologically as a grade 1 follicular lymphoma (FL).  After exclusion of endocrinological causes the recurrent hypoglycaemia was diagnozed as part of a paraneoplastic syndrome associated with a non-islet cell tumour hypoglycaemia (NICTH) with a newly diagnosed FL. TREATMENT AND COURSE: Specific medication with the CD20 antibody rituximab (375 mg/m2, once per week for a total of four cycles) was initiated. There were no further episodes of hypoglycaemia. After one year the patient remains free of any symptoms. CONCLUSIONS: After exclusion of any endocrinological reasons for hypoglycemia, differential diagnosis should include NICTH as paraneoplastic syndrome. In rare cases a hematological malignancy may be the underlying disease. The specific treatment of this disease likewise represents the causal treatment of NICTH.

[Glycogenosis type IV (Andersen disease). Clinical data, pathology, and genetics in a fatal perinatal case]. / Glykogenose Typ IV (Andersen). Klinik, Pathologie und Genetik eines letalen perinatalen Falles.

Here we report the case of a newborn with glycogenosis type IV (Andersen disease), who died shortly after birth. The diagnosis was established in the first instance by light microscopy and histochemistry, and subsequently ultrastructurally. DNA could be extracted from a fibroblast cell culture by sequencing the causative GBE1 gene (glycogen branching enzyme 1). Two compound heterozygous mutations in the gene were identified. The differential diagnosis should include Lafora disease as well as polyglucosan body disease. Since there is no effective therapy for glycogenosis type IV to date, prenatal diagnosis is mandatory.

[Carney complex. Clinical, pathological and genetic features in two generations of a family]. / Carney-Komplex. Klinik, Pathologie und Genetik bei 2 Generationen einer Familie.

Clinical symptoms of Carney complex occurred in two female members of one family (mother and daughter). In addition to the clinical symptoms, we describe the pathological findings in the adrenals (pigmented nodular hyperplasia of the adrenal cortex), heart (myxoma) and skin/soft tissues (superficial angiomyxomas). Genetic investigation revealed a mutation on the long arm of chromosome 17 in both patients; this mutation had previously been described only in Carney complex type 1. Unilateral adrenalectomy was performed in both these cases, 13 years ago and 7 months ago, respectively. Lifelong cardiac surveillance is mandatory to prevent death from embolism or arrhythmia.

Four worksite weight loss programs with high-stress occupations using a meal replacement product.

The objective was to evaluate the efficacy of worksite weight reduction programs at high-stress worksites. We employed a longitudinal study based on two meal replacements daily with subjects choosing a third 'sensible' meal. The subjects were 492 healthy, overweight men and women working in high-stress occupations (police, hospital health professionals, flight crew members, firefighters). The mean group ages ranged from 32.17 +/- 5.70 to 44.50 +/- 16.40 years; the mean group body mass indexes (BMIs) ranged from 27.40 +/- 2.54 to 32.90 +/- 3.39 kg/m(2). The completion rate for the 12 weeks was 79.8%. Reductions in mean weight and mean BMI were greater than in medically supervised clinical trials with non-worksite adults. Firefighters lost the most weight and medical personnel the least. Follow-up found considerable retention of weight loss. Men lost significantly more weight than women (p < 0.006). We conclude that employees in some high-stress settings may participate productively in worksite weight reduction and maintenance programs that use meal replacements.

Beverage viscosity is inversely related to postprandial hunger in humans.

Accumulating evidence indicates that energy-yielding beverages evoke weaker appetitive responses than more solid food items, but the properties responsible have not been characterized. The present study attempted to isolate an influence of viscosity. At weekly intervals, 84 adults ingested 325-ml (220 kcal) shakes that were matched on weight, volume, temperature, energy, macronutrient content, energy density, rate of consumption, cognitive expectations, palatability, appearance, and requirements for mechanical processing, but varied in viscosity. Twice appetitive ratings were obtained over the subsequent 4 h, while all intake was proscribed, and twice ratings were kept until the first spontaneous eating occasion comprised of > or =100 kcal. Dietary intake was recorded over the 24 h after shake ingestion. Significantly greater and more prolonged reductions of hunger were observed with the thicker shake. No significant differences were noted in the size or time to first meal or 24 h energy intake. These data indicate viscosity exerts an independent inverse effect on hunger in humans.

Obesity prevalence by age group and 5-year changes in adults residing in rural Wisconsin.

OBJECTIVE: To estimate the prevalence of overweight and obesity and weight changes over a 5-year period in a rural adult population by age group. SUBJECTS: Residents of Marinette County, Wis: 613 women and 352 men. METHODS: Age, body weight, and height data were collected during October and December 1997 in a volunteer convenience sample of white men and women (aged 20 to 74 years). Age groups in 10-year increments were defined by age in 1992. Weights obtained from medical and employment records from 1992 by medical personnel, as well as self-reported weights, were recorded for 1992 to assess weight changes from 1992 to 1997. RESULTS: In 1992 women and men had mean body weights of 72.1 and 88.4 kg, respectively. By 1997, the average weight for women in the same data set was 79.3 and that for men was 96.4 kg, a gain of 7.3 and 7.9 kg, respectively. For both women and men, those in the youngest age group (age 20 to 30 years) experienced the greatest 5-year increase in body weight: 11.0 and 12.1 kg, respectively; the next largest increase occurred in the second youngest group (age 30 to 40 years). The majority of the normal-weight subjects (58%, 229 of 393) moved into an overweight or obese category, the overweight and obese generally gained weight, and only 20 of 572 overweight or obese individuals moved from an unhealthful to a healthful body weight during the 5 years. APPLICATIONS/CONCLUSIONS: The data indicate a need for community-wide weight-gain prevention programs for young adult men and women in rural areas. Weight-reduction treatment programs should be geared toward older adults, as most have already attained an unhealthful weight, which is likely to increase.

Five-year self-management of weight using meal replacements: comparison with matched controls in rural Wisconsin.

We compared 5-y weight changes of subjects after a self-managed meal-replacement weight-control program (MR) with that of matched control subjects in rural Wisconsin. Healthy but overweight females (n = 108) and males (n = 50) received free products and weighed in twice a year from 1992 through 1997. Weights from three area clinics were obtained from 1992 records for 528 healthy males and females, whose weights were again obtained in late 1997. In addition, 1997 weights were obtained on 378 area males and females who reported their 1992 weight by questionnaire. Three control subjects were then selected for each MR participant, matched for age (within 5 y), body mass index (within 2 kg/m(2)), sex, and race. After 5 y, 141 MR participants weighed in, 2 died, and 19 were excluded primarily because of pregnancy. The 50 males were -5.8+/-5.4 kg and the 84 females were -4.2+/-6.9 kg from their 1992 weights. Conversely, 142 matched male controls gained 6.7+/-10.2 kg and 247 female controls gained 6.5+/- 10.7 kg. In conclusion, success determinations of MR and other long-term weight-control programs should be made by comparing changes with matched controls as the background rate of weight gain becomes increasingly more significant the longer the study is run.

Effectiveness of a single topical application of 10|x% trolamine salicylate cream in the symptomatic treatment of osteoarthritis.

This study was designed to evaluate the effectiveness in relief of pain and rigidity of a 10|X% trolamine salicylate cream compared with a placebo cream identical in smell and appearance, for subjects with osteoarthritis in their hands. This was a one-application, randomized, double-blind, placebo-controlled, parallel study conducted in 81 patients. Pain and stiffness were assessed in the morning upon subjects' awakening (baseline) and at 30, 45, and 120 min after a 4-min rubbing application. Analgesic response was determined using the sum of pain intensity differences (SPID) and the sum of stiffness intensity differences (SSID); the sum across the observation points derived from a pain/stiffness rating scale. Trolamine salicylate was significantly superior to the placebo in improving SPID (p = 0.0492) and in improving SSID scores for both hands (p = 0.0283). Treatment differences in absolute pain and stiffness scores were significant (p |Ml 0.05) at 45 min after application. A 10|X% trolamine salicylate cream was shown to be safe and effective for the temporary relief of minor pain and stiffness associated with osteoarthritis in the hands. This formulation has no smell or counter-irritating properties; patient acceptability was good.

Electrocardiographic QTC interval: short-term weight loss effects.

OBJECTIVE: Prolongation of the QTc interval is a risk-factor for ventricular arrhythmias and sudden death. There is a correlation between body mass index (BMI) and QTc, with longer intervals observed in obese subjects. Dieting and weight loss on selected diets are also associated with prolongation of the QTc interval, although the mechanisms and risk factors of this syndrome are not clearly established. The aim of the current investigation was to determine if short-term weight loss over seven days while living on a metabolic ward influences cardiac repolarization as indicated by QT interval duration. DESIGN AND SUBJECTS: The electrocardiogram QT, RR, and QTc (QT/RR(0.5)) intervals were assessed in 30 healthy obese (BMI > 27 kg/m2) men and women ingesting a balanced 1120 kcal/d formula diet (23% kcal as fat and 52 g protein/d). RESULTS: Weight loss (3.9 +/- 1.7 kg) over seven days had no measurable effect on the RR interval and there was a significant (P = 0.02) shortening of the QT interval (mean +/- s.d., 0.395 +/- 0.028-0.386 +/- 0.027 s) and borderline significant (P = 0.11) shortening of the QTc (0.411 +/- 0.028-0.404 +/- 0.025 s) interval. The QTc interval normalized in three subjects with prolonged baseline QTc (> 0.44 s). A statistically significant (P = 0.007) reduction in systolic blood pressure early in the course of weight loss (day 2) accompanied the electrocardiographic changes. CONCLUSION: These results fail to support the development of cardiac repolarization abnormalities in obese patients who ingest a nutritionally balanced low calorie diet over seven days. A significant reduction in QT interval duration and amelioration of abnormally prolonged baseline QTc intervals suggest improved cardiac repolarization with early weight loss.

Varieties of serous surface papillary carcinoma of the peritoneum in northern Germany: a thirty-year autopsy study.

From a total of 62,858 autopsy files kept over a 30-year period (1961-1990), all cases that met the following criteria were extracted: (a) main tumor masses in the peritoneum with no evidence of origin from any adjacent organ; (b) histologic features suggestive of serous ovarian carcinoma; (c) ovaries definitely recognizable as having either no tumorous involvement or tumor confined to the surface and cortex of the ovaries; and (d) in patients with a history of abdominal operations, availability of slides and reports. From 670 stages III and IV serous carcinomas of the ovary, we retrieved 57 cases (8%) of serous surface papillary carcinoma (SSPC) of the peritoneum. All SSPCs occurred in women with an age range from 47 to 84 years (median 66 years). Eight cases (14%) were grade I, 36 (63%) were grade II, and 13 (23%) were grade III. Histologically, four cases (7%) resembled malignant epithelial mesothelioma in major parts of the tumor, and in one case endometrioid differentiation of the tumor was conspicuous. Additional histochemical and immunohistochemical examinations were performed in 30 cases of SSPC. In 14 cases (47%) neutral mucosubstances were identified by periodic acid-Schiff positivity after diastase predigestion, and in 16 cases (53%) acid mucosubstances were identified by alcian blue staining. In one of these cases the alcian blue-positive substances were abolished under predigestion by testicular hyaluronidase. Tumor cells stained positive for cytokeratin (100% of the cases), B72.3 (90%), Ber-EP4 (83%), CD 15 (57%), placental alkaline phosphatase (53%), CA 125 (43%), vimentin (23%), and carcinoembryonic antigen (10%). The survival rates at 1 year for SSPC (0%) were significantly shorter (p = 0.03) in comparison with stages III and IV ovarian carcinomas (34%). Problems of definition and differential diagnosis of SSPC are discussed.

Primary retroperitoneal mucinous cystadenomas with gastric epithelial differentiation.

We present two rare cases of retroperitoneal mucinous cystadenomas (RMC) by clinical data, light microscopy, electron microscopy, histochemistry, and immunohistochemistry. Both tumors occurred in women. We excluded attachment to any organ. Additional immunohistochemical examinations were performed for comparison in 10 cases of ovarian mucinous cystadenomas (OMC). Both RMC showed no significant immunohistochemical differences from the OMC. Ultrastructurally and histologically the RMC resemble OMC reported in the literature. Immunohistologically, we proved gastric epithelial differentiation by using one monoclonal antibody (2B5) recognizing gastric mucin and one polyclonal antibody against pepsinogen II (PG II). In spite of this, the histogenesis of RMC in the presented cases in the end remains uncertain.

Quantitation of the dynein pool in unfertilized sea urchin eggs.

A dynein-like ATPase activity has been isolated previously from soluble extracts of unfertilized sea urchin eggs. However, the use of non-quantitative isolation techniques, in particular affinity for microtubules or Ca2+/calmodulin, has precluded accurate estimates of dynein pool size. We have taken the unique approach of using dynein-like ATPase activity to quantitate the egg dynein pool. This approach is based on the isolation by anion-exchange chromatography on DEAE-Sephacel of a peak of dynein-like ATPase activity comprising 65% of soluble ATPase activity in the cytosolic extract. Identification of cytoplasmic dynein was based on dose-dependent inhibition by erythro-9-[3-(2-hydroxynonyl)]adenine and orthovanadate, low GTPase activity and a sedimentation coefficient of 12 S. Two high molecular weight polypeptides corresponding to the A- and D-bands of axonemal dynein were shown to copurify with dynein-like ATPase activity and to undergo specific photocrosslinking with [alpha-32P]ATP, suggesting that they were egg dynein catalytic polypeptides. The specific ATPase activity of these putative catalytic polypeptides was determined to be 1.2 mumol.min-1.mg-1. The specific dynein-like ATPase activity of the crude soluble extract of unfertilized sea urchin eggs was determined to be 0.004 mumol.min-1.mg-1. The concentration of putative dynein catalytic polypeptides was therefore determined from the ratio of the specific activities of crude to pure cytoplasmic dynein catalytic polypeptide to be 0.33% of soluble protein, or 99 pg per egg. This is approximately 3-fold greater than the mass of dynein catalytic polypeptides estimated to be present in cilia at the blastula stage of sea urchin embryonic development. The large amount of cytoplasmic dynein in unfertilized eggs suggests that it could act as a precursor of embryonic ciliary dynein. Three minor peaks of ATPase activity were also resolved from cytosolic extracts and shown to be dynein-like. However, their GTPase activities were 2-4-fold higher than that of cytoplasmic dynein, raising the possibility that egg cytoplasm may contain several isoforms of dynein.

Effects of variation of necropsy time and fasting on liver weights and liver components in rats.

In rats, percent liver weight loss is greater than percent body weight loss within the 8 A.M.-4 P.M. period of the working day. The liver weight loss is principally the result of decreased water content, either carbohydrate (glycogen) bound water in rats with access to feed, or protein bound water in rats fasted overnight. During this period, percent kidney weight loss is approximately equal to percent body weight loss. To optimize the sensitivity of kidney and liver weight evaluation, it is recommended that rats be fasted overnight and that relative liver and kidney weights be expressed based on body weights taken immediately prior to necropsy. Since these procedures will not entirely eliminate necropsy time-related organ weight differences, the animal necropsy sequence must be randomized to distribute the remaining differences across all treatment groups.