[Hyperammonemic encephalopathy in multiple myeloma]. / Hyperammonämische Enzephalopathie bei multiplem Myelom.

HISTORY AND CLINICAL FINDINGS: A 54-year old man had suffered from advanced multiple myeloma for two years. After initially good response the myeloma was refractrory to treatment with dexamethasone, cyclophosphamide, bortezomibe, zoledronate and additionally doxorubicine. The patient then complained of dyspnea without clinical signs of cardiopulmonary disease. INVESTIGATIONS: Arterial blood gas analysis showed hyperventilation with respiratory alkalosis and normal alveolo-arterial gradient as the reason for the dyspnea. With a normal MRI of the brain and lumbal puncture, a neurological disease could be excluded. Serum calcium, creatinine and serum viscosity were normal. Eventually, serum ammonia levels were found to be substantially elevated (144 µmol/l) and hyperammonemic encephalopathy was diagnosed. TREATMENT AND COURSE: Therapy with bortezomib and high dose dexamethason was repeated, and the patient also received bendamustin. Despite this treatment, he lost consciousness and died after two weeks because of aspiration pneumonia. CONCLUSION: The existence of respiratory alkalosis and multiple myeloma should prompt a search for hyperammonemia.

[Sudden onset of abdominal distension...]. / Wenn plötzlich der Bauchumfang zunimmt...

We report the case of a 39 years old patient with sudden onset of abdominal distension and icterus. Occlusion of the liver veins discovered by doppler ultrasound caused the portal hypertension and liver disease. Given the erythrocytosis the detection of JAK 2-mutation proved polycythemia vera as underlying disease for the Budd Chiari syndrome. The implantation of a TIPS wasn't successful because of an early thrombotic occlusion. Therefore a surgical mesocaval shunt was done. At the moment the patient is therapied with oral anticoagulation and hydroxycarbamide and is fully capable of work again.

[Polyps of the gallbladder]. / Gallenblasenpolypen.

Gallbladder polyps are a common incidental finding in ultrasound. They may be clinically significant, because adenomas have a malignant potential, in contrast to the benign cholesterol polyps occuring more frequently. As non-neoplastic polyps and neoplastic polyps cannot be morphologically discriminated in ultrasound, maximal diameter of a polyp has proven to be the best predictor for malignancy. Cholecystectomy is widely accepted as the treatment of choice for patients with polyps >1 cm. Patients with polyps <1 cm should have a follow-up check for a longer period of time (with the exception of very small polyps).

[Persistent fever in the elderly]. / Persistierendes Fieber im Alter.

We report the case of a 68 year old immuncompetent woman with persisting fever. Symptomatic acute CMV infection with a partial thrombosis of the left portal vein branch was diagnosed.

[Spontaneous hematomas and isolated prolongation of aPTT]. / Spontanhämatome bei isoliert erhöhter aPTT.

We report a case of a 64 year old woman with spontaneous hematomas. A prior history of bleeding disorder was not known up to now. The result of investigation of coagulation was an isolated prolonged aPTT that was caused by an inhibitor of factor VIII without underlying disease (idiopathic acquired hemophilia A). Therapy with steroids and cyclophosphamid resulted in partial remission.

[Fever and dysphagia of a young woman]. / Fieber und Dysphagie einer jungen Frau.

We report the case of a 39-year old patient with septicemia treated for pharyngitis with antibiotics since a few days. She wasn't able to swallow her antibiotics anymore because of dysphagia. Radiologic examination revealed pulmonary infiltrates and Vena iugularis interna-thrombosis. These findings and anamnesis led to the diagnosis of Lemierre syndrome inspite of lacking detection of bacteria. After changing the antibiotic therapy and start of anticoagulation further course of illness was favorable. The long duration of hospitalization was indepted to high morbidity typically seen in Lemierre syndrome.

[Right-sided lower abdominal pain and diarrhea of a young diabetic woman]. / Rechtsseitige Unterbauchschmerzen und Durchfall einer jungen Diabetikerin.

We report the case of a 24-years old diabetic women hospitalised because of right-sided lower abdominal pain and diarrhea. She fulminantly developed shock before appendectomy could be performed and was transferred to intensive care unit. Hypotension remained and laparoscopy revealed primary peritonitis and toxic shock syndrome by Group A Streptococcus which was cultivated in blood and ascites. Therapy with penicilline and clindamycine resolved symptoms. During hospitalisation Clostridium difficile colitis occurred. This complication leaded to prolonged hospitalisation.

[Lymphadenopathy and diabetes insipidus - association]. / Lymphadenopathie und Diabetes insipidus - Assoziation?

We report the case of a 62 year old women with chronic idiopathic myelofibrosis who developed acute lymphadenopathy. At the same time diabetes insipidus, osteolytic bone lesions and exanthema of the skin were diagnosed. Suspected Langerhans cell histiocytosis could be proven by repeated biopsies of affected organs. Therapy was started in accordance with the recommendations of the Histiocyte Society.

[Odynophagia of a young mother]. / Odynophagie einer jungen Mutter.

We report the case of a 34-year old mother presenting in the emergency unit because of fever and odynophagia. Herpetic esophagitis with Herpes simplex virus primoinfection in a immunocompetent patient was diagnosed. The virus was transmitted by her own child which came to be hospitalised because of herpetic stomatitis. In the blood chemistry pancreas enzymes were elevated which we interpreted as herpetic pancreatitis because of coincidence with herpetic esophagitis.

[Hermaphroditos in Greek mythology--DSD in moderne medicine]. / Von Hermaphroditos der griechischen Mythologie bis zu DSD der Moderne.

We report a case of 34 year old woman how has been hospitalized at the age of 6 month with persistent vomitus. The vomitus was found to be caused by adrenal insufficiency with lack of all hormones of steroidobiosynthesis. The phenotypical femal child was diagnosed to have congenital lipoid adrenal hyperplasia with 46,XY DSD. 24 years later a homozygote mutation in the StAR-gene (L260P), which was first described in Switzerland, has been identified.

A longitudinal study of blood folate levels and depressive symptoms among young women in the Southampton Women's Survey.

BACKGROUND: Lower blood folate levels have been associated with depression in cross-sectional surveys, but no studies have examined the relationship prospectively to determine whether the relationship is causal. A follow-up study was designed to examine whether lower blood folate levels predict incident depressive symptoms. METHOD: Women aged 20-34 years registered in general practices in Southampton, UK, were asked to participate. Baseline assessment included the general health questionnaire (GHQ-12) measure of anxiety and depression, and socioeconomic factors, diet, smoking and alcohol intake. Two years later, participants' general practice (GP) records were examined for evidence of incident symptoms of depression. RESULTS: At baseline, 5051 women completed the GHQ-12 and had red cell folate levels measured, of whom 1588 (31.4%) scored above the threshold for case level symptoms of anxiety and depression on the GHQ-12. Two years later, GP records for 3996 (79.1%) were examined, but 1264 with baseline evidence of depression were excluded from follow-up analysis. Incident depressive symptoms were recorded for 307 (11.2%) of the remaining 2732. Lower red cell folate levels were associated with caseness on the GHQ-12 (adjusted prevalence ratio 0.99 per 100 nmol/l red cell folate, 95% CI 0.98 to 1.00). No relationship was found between red cell folate levels and incident depressive symptoms over 2 years (adjusted hazard ratio 1.00, 95% CI 0.97 to 1.03). CONCLUSIONS: Low folate levels were not associated with subsequent depressive symptoms. This suggests that lower blood folate levels may be a consequence rather than a cause of depressive symptoms.

[Scepticism is warranted or when the CT scan reveals to be a pitfall]. / Gesunde Skepsis tut Not oder: wenn das Spiral-CT in falscher Sicherheit wiegt....

We report the case of a 72-year old woman with known metastatic breast cancer who presented to the emergency department with progressive dyspnea on exertion and chest pain. The diagnosis of pulmonary embolism could be established by pulmonary scintigraphy after computed tomography and ultrasound of the lower extremities had been negative in spite of a moderate clinical pretest probability (Wells score). This case shows that even if we manage suspected pulmonary embolism using algorithms combining clinical probability, computed tomography and ultrasound we must remain aware of eventually missing the diagnosis and carry on investigating cases with elevated clinical probability.

["Numb chin syndrome": first presenting syndrome of multiple sclerosis?]. / "Numb chin Syndrom" als Erstsymptom einer Multiplen Sklerose?

HISTORY AND CLINICAL FINDINGS: A 34-year-old previously healthy woman presented with a five-day history of subacute onset of a numb chin. Examination on admission revealed isolated hypesthesia on the left side of the chin and lower lip. INVESTIGATIONS: Brain magnetic resonance imaging (MRI) demonstrated a lesion involving the pontine trigeminal fibers and multiple periventricular T2-hyperintense white matter lesions suggestive of inflammatory /demyelinating disease. Cerebrospinal fluid analysis revealed oligoclonal IgG bands (only in cerebrospinal fluid) and an increased IgG index. A follow-up MRI after four months demonstrated new supratentorial brain lesions, confirming a syndrome, highly suggestive of multiple sclerosis as the likely underlying diagnosis. TREATMENT AND FOLLOW-UP: The facial sensory disturbance resolved spontaneously. Prophylactic treatment with interferon-beta was started. CONCLUSION: The numb chin syndrome may be the initial presentation of a clinically isolated syndrome suggestive of multiple sclerosis. Prophylactic immunomodulatory treatment may be started after the suspicion of inflammatory/demyelinating activity is confirmed.

Effects of histamine and activators of the cyclic AMP system on protein synthesis in and release of high molecular weight glycoproteins from isolated gastric non-parietal cells.

1. Glycoprotein and protein synthesis in and release from pig isolated, enriched gastric mucous cells were measured by the incorporation of N-acetyl-[14C]-D-glucosamine and [3H]-L-leucine, respectively, into cellular and released acid precipitable material. 2. Histamine and activators of the adenosine 3':5'-cyclic monophosphate (cyclic AMP) system maximally stimulated total protein and glycoprotein synthesis in and release from the cells at concentrations of histamine (10 microM), forskolin (10-100 microM), 3-isobutyl-1-methylxanthine (100 microM), and dibutyryl cyclic AMP (1-3 mM), respectively. In the presence of 3-isobutyl-1-methylxanthine (30 microM) histamine stimulation was enhanced. 3. As shown by gel chromatography, stimulation by histamine (100 microM), forskolin (10 microM), 3-isobutyl-1-methylxanthine (100 microM) and dibutyryl cyclic AMP (1 mM) resulted in a release of high molecular weight (approximately 2 x 10(6) daltons) glycoproteins from the cells. The histamine H2-receptor antagonist, ranitidine (100 microM), blocked the effect of histamine. 4. We conclude that cyclic AMP-dependent processes are involved in the regulation of protein and glycoprotein synthesis in and the release of high molecular weight (mucous) glycoproteins from pig gastric non-parietal cells and that histamine may be a physiological activator of this system.

Effects of PGE2, carbenoxolone, cholecystokinin, and the thromboxane-mimetic U46619 on protein and glycoprotein production by isolated pig gastric mucosal cells.

We studied the effects of prostaglandin (PG) E2, carbenoxolone, cholecystokinin-octapeptide (CCK-OP), and the thromboxane-mimetic U46619, all known to stimulate gastric mucus secretion in vivo, on protein and glycoprotein synthesis in and release from isolated and enriched pig gastric mucous cells, as measured by the incorporation of [3H]L-leucine and N-acetyl-[14C]D-glucosamine respectively into cellular and released acid insoluble material. PGE2 stimulated glycoprotein and protein synthesis (EC50 7 and 30 nmol/L, respectively) and release (EC50 50 and 140 nmol/L, respectively) in a concentration-dependent manner, whereas carbenoxolone, CCK-OP and U46619 failed to enhance the incorporation of the tracers. We conclude that stimulation of mucus secretion by PGE2 is related to direct effects on protein and glycoprotein production of gastric non-parietal cells, whereas indirect effects may be involved in the stimulation by carbenoxolone, CCK-OP, and U46619.

Stimulation of glycoprotein and protein synthesis in isolated pig gastric mucosal cells by prostaglandins.

The purpose of this study is to evaluate the effects of different prostaglandin derivatives on protein and glycoprotein synthesis and secretion in isolated and enriched pig gastric mucous cells, as measured by the incorporation of [3H]L-leucine and N-acetyl-[14C]D-glucosamine respectively into acid insoluble macromolecules (AIM). PGE2 and 16,16-dimethyl-PGE2 enhanced the incorporation of the amino sugar into cellular (EC50 8 and 75 nmol/l) and secreted (EC50 30 and 270 nmol/l) AIM in a concentration dependent manner during a 20 hours incubation. After incubation for eight hours or more they also stimulated the incorporation of [3H]L-leucine into cellular AIM. PGF2 alpha was considerably less potent (EC50 greater than 1 mumol/l) than the E-type prostaglandins. Iloprost, a stable prostacyclin analogue, was ineffective.

Effects of histamine on protein and glycoprotein production of isolated pig gastric mucosal cells.

The production of glycoprotein and protein by isolated pig gastric non-parietal cells was measured by incorporation of N-acetyl-[14C]-D-glucosamine [( 14C]GlcNAc) and [3H]-L-leucine [( 3H]Leu), respectively, into acid insoluble material (AIM). Histamine enhanced incorporation of the tracers into cellular and released AIM in a concentration-dependent manner. The H2 receptor antagonist ranitidine completely blocked the effects of histamine (100 mumol/l) on [3H]Leu incorporation into cellular and released AIM (IC50 37 and 32 mumol/l, respectively) but had no inhibitory effect on the 16,16-dimethylprostaglandin-E2 - and forskolin-stimulated tracer incorporation. The H1 receptor antagonist mepyramine did not inhibit the histamine effect. We conclude that histamine is a stimulant of protein, via H2 receptors, and glycoprotein production of isolated pig gastric non-parietal cells.

Incorporation of N-acetyl-[14C]D-glucosamine and [3H]L-leucine by isolated pig gastric mucosal cells.

Glycoprotein and protein production of isolated pig gastric mucosal cells were determined by the incorporation of N-acetyl-[14C]D-glucosamine ([14C]GlcNAc) and [3H]L-leucine ([3H]Leu) into acid-insoluble macromolecules (AIM). In four cell fractions (F1-F4), obtained by counterflow centrifugation, specific [14C]GlcNAc incorporation was greatest in the mucous cell-enriched F2. Tracer incorporation by F2 cells, proceeded linearly up to 20 h, was inhibited by cycloheximide or incubation at 0 degree C, and enhanced by PGE2 1 mumol/l. Gel chromatography of released AIM revealed that PGE2-stimulated [14C]GlcNAc incorporation was predominantly directed into high molecular weight (2 X 10(6) daltons) glycoproteins, whereas [3H]Leu incorporation was mainly related to proteins of albumin-like molecular weight. We conclude that incorporation of [14C]GlcNAc by enriched pig gastric mucous cells (F2), further analyzed by gel chromatography, is a suitable probe to study the production of high molecular weight gastric mucous glycoproteins in vitro.