[Controversies of partial nephrectomy for renal cell carcinoma : survey in the German-speaking countries]. / Kontroversen der Nierenteilresektion bei Nierenzellkarzinom : Umfrage im deutschsprachigen Raum.

BACKGROUND: The goal of this study was to evaluate how partial nephrectomy is technically performed in Germany, Austria, and Switzerland. METHODS: A one-page anonymous questionnaire was designed to evaluate the indication, the technical procedure, and the follow-up of R1 situation after partial nephrectomy. Furthermore, the size of the hospitals and their catchment areas were recorded. The questionnaire was sent to 341 clinics and a statistical analysis was performed. RESULTS: The response rate was 69 %. Up to 99 % of the clinics also perform partial resection in T1b tumors. Of those responding, 58 % perform this surgery laparoscopically, and 83 % of the surgeries are performed in warm ischemia. For the follow-up, 29 % suggest imaging within the first 6 weeks. According to this survey, maximum care clinics perform laparoscopic nephrectomy more frequently (p = 0.003). CONCLUSION: The survey of 236 hospitals performing partial nephrectomy shows great variability in the indication, technique, and aftercare of organ-preserving renal tumor surgery. It also shows that a large proportion of tumors >4 cm undergo organ-preserving surgery, many of them minimally invasive. The diverse handling with positive instantaneous section and R1 results suggest the need for further studies concerning long-term follow-up after minimally invasive surgery with R1 situation and renal tumors > T1a.

[Multiple intracerebral lesions. Identification of the causative agent by 16S rDNA-PCR]. / Multiple bakterielle Hirnabszesse. Erregernachweis mittels 16S-rDNA-PCR.

Molecular biological methods such as polymerase chain reaction (PCR) enable microbiologists to detect bacteria even if antibiotic treatment has already been started. Based on this case report of a 39-year-old man with multiple intracerebral lesions, we describe a PCR method called 16S rDNA-PCR which can be used to identify panbacterial DNA by focussing on the universal gene sequences for the bacterial 16S part of the ribosome.

[Aneurysm of the internal carotid artery--a differential diagnosis of paralysis of the caudal cranial nerves]. / Aneurysma der A. carotis interna. Die besondere Differenzialdiagnose einer Parese der kaudalen Hirnnerven.

Paralysis of the caudal cranial nerves, e. g. the nervus glossopharyngeus, vagus and accessorius, may cause disorders in swallowing and speaking leading to a reduction in the patient's quality of life. Glomus tumors or malignant lesions of the skull base are a frequent cause of such lesions. We report on the case of a 48 year old patient who presented an acute lesion of these cranial nerves in combination with paresis of the nervus hypoglossus as a result of an aneurysm of the internal carotid artery directly underneath the base of the skull. The aneurysm was treated by parent vessel occlusion. The results of this procedure were a shrinkage of the aneurysm and an improvement in the neurological symptoms.

[Neuritis of the optic nerve after vaccinations against hepatitis A, hepatitis B and yellow fever]. / Optikusneuritis nach Impfung gegen Hepatitis A, B und Gelbfieber mit irreversiblem Visusverlust.

BACKGROUND: Vaccinations are preventive measures against serious infections. In relation to the number of vaccinations per year, the incidence of severe complications is extremely low. PATIENT: Two weeks after vaccinations against hepatitis A, hepatitis B and yellow fever in preparation for a trip to Africa a 21-year-old woman experienced an acute and irreversible loss of vision to 0,05 and nasal visual field defect in the left eye. Whereas vision acuity did not recover the scotoma disappeared within 6 weeks. Cerebrospinal fluid showed a lymphocytic pleocytosis. Oligoglonale bands were absent. Pathological parameters were not presented in the serum. The MRI showed a hyperintense thickening of optic nerve,as well as a hyperintense focus in right temporal side using the T(2)W-Sequence. CONCLUSION: Only few cases have been reported with neurological complications, such as encephalitis, following vaccinations each of the above mentioned. As no other causes for the inflammation were found, the optic nerve involvement must have been caused by the vaccination. Which of the three vaccinations caused encephalitis can not be classified.

[Similarity of monozygotic twins regarding vocal performance and acoustic markers and possible clinical significance]. / Die Ahnlichkeit monozygoter Zwillinge hinsichtlich Stimmleistungen und akustischer Merkmale und ihre mögliche klinische Bedeutung.

Auditory similarities in voices of monozygotic twins have already been described in the literature. However, is there a clinical relevance? Thus, the present study was designed to identify parameters of vocal performance and acoustic features which are significantly more similar in monozygotic twins than in non-related persons. In our hypothesis, comparable prerequisites for an increased vocal load in a profession or in an artistic education of the voice could be due to these similarities. We compared intra-pair differences with data from a control group. Moreover, we examined the correlation of intra-pair differences with the age of the monozygotic twins. A greater difference in older twin pairs than in younger pairs could show the effect of an exogene influence. In addition to the few phoniatric studies in twins in the literature, we used current methods for acoustic analysis. We examined seven parameters of vocal performance and three acoustic features in 31 monozygotic twin pairs (median age 36 years, range 18-75 years) and compared them with 30 control group pairs, which consisted of non-related persons of the same age and sex, newly combined from the group of monozygotic twins ("statistical twins"). We found significant differences in seven of ten parameters (vocal range, highest and lowest vocal fundamental frequency, fundamental speaking frequency, maximum voice intensity, number of partials, vibrato of intensity; U-test by Mann-Whitney). No correlation of the differences of the identical twins with age was found in the examined parameters. The voices of identical twins are significantly more similar than those of non-related persons regarding the above mentioned features. Thus, the suitability of the voices of monozygotic twins for professions with a high demand on voice is comparable. Results of the group comparison correlate largely with the literature. The missing correlation with age could be due to the fact that the environmental effects were not measurable, and/or the development of the voice is more influenced by genetic effects.

[Prediction of the onset of voice mutation in singers of professional Boys' choirs: investigation of members of the Thomaner choir, Leipzig]. / Methoden zur Vorhersage des Eintrittszeitpunktes der Mutation bei Knabenstimmen: Untersuchungen bei Sängern des Thomanerchores Leipzig.

Die phoniatrische Betreuung der kindlichen Singstimme während des Stimmwechsels erscheint gerade bei Sängern in Knabenchören mit hoher stimmlicher Belastung wichtig. Die vorliegende Studie sucht nach Methoden, den Eintrittszeitpunkt der Mutation vorherzusagen und das Vorliegen einer Mutation differentialdiagnostisch von hyperfunktionellen Stimmstörungen oder entzündlichen Erkrankungen des Stimmorganes zu trennen. Dazu wurden 36 Knaben des Leipziger Thomanerchores im Sinne einer Longitudinalstudie alle 3 Monate über 3,5 Jahre bis zum hörbaren Einsetzen der Mutation untersucht. Es erfolgte die Bestimmung von zehn stimmlichen und acht stimmunabhängigen Parametern, die erstmals in solch umfassender Form auf ihre prädiktive Aussagekraft geprüft wurden. Die statistische Auswertung wies besonders den Serumtestosteronspiegel und die Wachstumsrate als Grössen aus, die mit dem Prämutationsverlauf korrelieren und eine konkrete Vorhersage ermöglichen. Das Vorliegen einer Mutation kann ausserdem durch den Verlauf der mittleren ungespannten Sprechstimmlage und unter Hinzuziehung des Genitalstatus bestätigt werden. Durch die exakte und prädiktive Bestimmung des Mutationsbeginnes können Erkrankungen des Stimmapparates vermieden werden, die aus dem intensiven Singen in der Mutation resultieren. Weiterhin geben die Ergebnisse dem Chorleiter wertvolle Informationen für die Planung der Besetzung seiner Knabenstimmen.

Combination of inhaled nitric oxide and intravenous prostacyclin for successful treatment of severe pulmonary hypertension in a patient with acute respiratory distress syndrome.

OBJECTIVE: To investigate the combination of inhaled nitric oxide (iNO) and intravenously administered prostacyclin (i.v. PGI2) in a patient with severe pulmonary hypertension and acute respiratory distress syndrome (ARDS). DESIGN: Single case study. SETTING: Intensive care unit of a university hospital. METHODS: In an ARDS patient with severe pulmonary hypertension, gas exchange and hemodynamics were measured during combined treatment with iNO and i.v. PGI2. On two subsequent days, a protocol consisting of four 20-min periods was performed: baseline, 10 ppm iNO, 10 ppm iNO plus 4 ng kg-1 min-1, and 4 ng kg-1 min-1 PGI2 alone. At the end of each period hemodynamic and gas exchange data were obtained. RESULTS: The combination of iNO and i.v. PGI2 resulted in a marked decrease in pulmonary artery pressure and a concomitant increase in cardiac output which was more pronounced than the effect of either drug alone. During iNO, as well as during the combination of iNO and i.v. PGI2, oxygenation was improved, whereas during i.v. PGI2 alone oxygenation was worse than baseline. CONCLUSION: We conclude that the combination of iNO and i.v. PGI2 might be more useful than either drug alone when severe pulmonary hypertension leading to impaired right ventricular function is present in ARDS. A systematic study of this observation is warranted.

Long-term measurements of energy expenditure in severe burn injury.

The objective of this study was to evaluate resting energy expenditure (REE) in spontaneously breathing and artificially ventilated burn patients during the entire intensive care period. In 27 patients with 51 +/- 20% body surface area burned (BSAB) the REE was determined via indirect calorimetry. Three groups were formed according to the mortality prognosis index of Zawacki et al. In groups A, B, and C the predicted mortality rates were <20%, 20% to 80%, and >80%, respectively. The frequency of acute respiratory distress syndrome (ARDS), sepsis, renal failure, and mortality increased from group A toward group C. The REE test revealed wide individual variation and was usually overestimated by all tested formulas. The mean REE was comparable in groups A, B, and C during the first 20 days (49 +/- 16% vs. 59 +/- 21% vs. 57 +/- 18% above the REE calculated by the Harris-Benedict equation, or HBEE). The REE of patients in groups A and B declined after this period, whereas the long-term ventilated patients in the prognostically unfavorable group C showed a high REE up to the 45th day, usually accompanied by severe organ dysfunction and major metabolic disorders. During this time a nutritional regimen meeting the actual REE could not be achieved. In the clinical situation when indirect calorimetry is not available, REE can be stated to be 50% to 60% above HBEE in patients with >20% BSAB for at least 20 days. Expecting a stable clinical course in patients with a predicted mortality of <20% (group A), oral nutrition usually seems sufficient after a short period of artificial nutritional support (1 week). Patients with a predicted mortality of more than 20% have a complication-burdened clinical course and a prolonged period of ventilation (groups B and C). These patients need parenteral and enteral nutrition for at least 20 days after trauma to prevent severe malnutrition.

[MORBUS: the Sentinel Practice Network. Report on a study in South Oldenbury]. / MORBUS: Beobachtungspraxen in Niedersachsen Bericht zur Erhebung in Süd-Oldenburg.

There has been considerable public concern that emissions from intensive livestock farming may have hazardous effects on human health, particularly on the respiratory system. From October 1991 to September 1992, data on consultations of asthmatic children (up to 8 years) were obtained by a network of 25 GP and paediatric practices in South Oldenburg, a region with one of the highest livestock densities in Germany. Comparable data from a similar network of 75 practices in 3 adjacent regions (Brunswick, Hanover, Verden) with average livestock density served as a reference. In South Oldenberg, 2084 consultations of 542 asthmatic children were observed, with asthma being the reason for visit in 734 of the contacts (36%). The boy-girl ratio was 2.1:1 among index patients and 1.9:1 among consultations. Consultation rate was 25.2 contacts by asthmatic children per 1,000 total consultations of children up to 8 years in South Oldenburg, compared to 17.8 per 1,000 in Hanover, 15.7 per 1,000 in Brunswick and 13.6 per 1,000 in Verden. Consultations due to asthma scored 11.2/1,000 in South Oldenburg, 10.8/1,000 in Hanover, 7.2/1,000 in Brunswick and 6.5/1,000 in Verden. Asthmatic patients in South Oldenburg were younger (mean age 38 vs. 42 months) than those observed in the reference regions. There were no regional patterns in sex ratio, severity of asthma, respiratory allergies or atopic dermatitis. As this is an ecological study design, inferences concerning the cause of the observed regional differences can only be weak. We therefore propose a case-control study in order to obtain exposure and health data on an individual level.

Short-term immunotherapy: a prospective, randomized, double-blind, placebo-controlled multicenter study of molecular standardized grass and rye allergens in patients with grass pollen-induced allergic rhinitis.

BACKGROUND: Short-term immunotherapy (STI) can be beneficial for patients who are noncompliant with long-term specific immunotherapy. OBJECTIVE: The efficacy and tolerance of STI with seven preseasonal injections of molecular standardized allergens from grass and rye pollen has been investigated in a double-blind, placebo-controlled multicenter study with 87 patients at 12 German University hospitals. METHODS: Symptoms of the eyes, nose, and bronchi and use of symptomatic drugs were documented daily in diaries by patients with allergic rhinitis to grass and/or rye pollen and without bronchial asthma. Patients were monitored by skin prick test titration and measurement of levels of specific IgE and IgG4. RESULTS: The median nasal score for the 10 weeks with the strongest symptoms during the grass pollen season was significantly lower (p = 0.014) with 35.0 for STI (n = 41) versus 69.0 for placebo (n = 40); the overall symptom score was 54.0 for STI versus 97.5 for placebo (p = 0.020). Only STI-treated patients exposed to less than 40 pollen grains per cubic meter per week showed a significantly lower nasal symptom score of 39.0 versus 75.0 for placebo (p = 0.006); these patients also had fewer nasal symptoms and less use of topical nasal drugs (p < 0.001). The threshold dose in skin prick tests was significantly higher, being 9.06 histamine equivalent for skin prick test (HEP) for STI-treated patients who received the maximum dose (n = 22) versus 4.33 HEP for placebo (p = 0.005). Specific IgE levels were significantly higher, being 55.9 SU/ml for STI versus 39.2 SU/ml for placebo after seven injections (p = 0.006) and level of specific IgG4 was 5.36% for STI versus 1.28% for placebo (p < 0.001). No severe systemic reactions were observed. CONCLUSION: STI with seven preseasonal injections with molecular standardized allergens is effective and well tolerated.

Performance of a prototype for a large-aperture multipass Nd:glass laser for inertial confinement fusion.

The Beamlet is a single-beam prototype of future multibeam megajoule-class Nd:glass laser drivers for inertial confinement fusion. It uses a multipass main amplifier, adaptive optics, and efficient, high-fluence frequency conversion to the third harmonic. The Beamlet amplifier contains Brewster-angle glass slabs with a clear aperture of 39 cm x 39 cm and a full-aperture plasma-electrode Pockels cell switch. It has been successfully tested over a range of pulse lengths from 1-10 ns up to energies at 1.053 mum of 5.8 kJ at 1 ns and 17.3 kJ at 10 ns. A 39-actuator deformable mirror corrects the beam quality to a Strehl ratio of as much as 0.4. The 1.053-mum output has been converted to the third harmonic at efficiencies as high as 80% and fluences as high as 8.7 J/cm(2) for 3-ns pulses.

[Metastasis of adenocarcinoma of the ovary to the subglottis. A laryngologic rarity]. / Metastase eines Adenokarzinoms des Ovars in der Subglottis. Eine laryngologische Rarität.

Malignant tumors of the larynx are an important disease entity in otorhinolaryngology and mainly involve squamous cell carcinomas. Different epithelial or mesenchymal malignant tumors are reported only sporadically and metastases localized in the larynx are even rarer. In this report we present a patient who was found to have a laryngeal metastasis from an adenocarcinoma. An ovarian carcinoma treated 7 years previously was the primary tumor and first metastasized into the left lung and then into the subglottig area of the larynx. Because the surgical procedures and chemotherapy used for each lesion were successful and no local recurrences were found, the same therapeutic rules for the treatment of metastases to the larynx should be applied as in cases of primary malignancies.

[Brush cytology and HPV detection in chronic laryngitis and papillomatosis]. / Abstrichzytologie und HPV-Nachweis bei chronischer Laryngitis und Papillomatose.

BACKGROUND: We investigated the utility of cytologic studies (including follow-up studies) in our specialty in determining the prevalence of human papillomavirus, specifically in the larynx. We utilized Papanicolaou's method of exfoliative cytology and in situ hybridization of the culture (biotin-marked DNA probes). PATIENTS: 486 patients with chronic hyperplastic laryngitis and 74 patients with papilloma were enrolled in the study. RESULTS: In 198 typings in 132 patients (59 patients with papilloma, 41 with chronic hyperplastic laryngitis, 17 with cancer, and 15 healthy), we found distributions of the individual types that were independent of the diagnosis. Particularly often, we encountered human papillomavirus types 18 and 31 in the presence of dysplasia, human papillomavirus type 16 in the presence of cancer, and type 11 in general. CONCLUSIONS: Cytologic examination and human papillomavirus typing of the culture permit us to define a third risk category in addition to the known risk groups smokers and dysplasia patients. Besides this, cytologic follow-up studies can provide further insight into the behavior of preneoplastic epithelial changes.

[Lethal, non-menstrual toxic shock syndrome associated with Staphylococcus aureus sepsis]. / Letal verlaufendes nicht-menstruelles Toxic-Shock-Syndrom bei Staphylococcus aureus-Sepsis.

We report a rare case of non-menstrual toxic shock syndrome (TSS) in the course of Staphylococcus aureus sepsis in a 31-year-old primigravida who developed high fever and severe pulmonary and cardiovascular failure within a few hours at the end of the 29th week of a twin pregnancy. Mechanical ventilation was necessary due to signs of adult respiratory distress syndrome (ARDS) and catecholamines were needed to maintain a somewhat adequate blood pressure. A forceps delivery was performed immediately. Postoperatively, the patient was brought to the intensive care unit (ICU) due to the suspicion of severe septic shock. In addition to the extreme cardiovascular instability and massive disturbance of pulmonary gas exchange, the clinical picture was characterised by a disseminated intravascular coagulopathy (DIC) with marked petechial bleeding and ecchymoses on all extremities. Moreover, a confluent, spotty exanthem of the trunk and extremities could be seen. Despite all therapeutic efforts, the patient died within a few hours after admission to the ICU with signs of multiorgan failure. Post-mortem, multiple staphylococcal abscesses were found in the kidneys, liver, and uterus. Moreover, acute ulcerous endocarditis of the mitral valve and septic myocardial foci with myocarditis were seen. The Staph. aureus strain isolated from the blood cultures was shown to produce TSS toxin 1 (TSST-1) and enterotoxin B. In summary, the clinical picture can be interpreted as severe staphylococcal sepsis complicated by TSS. TSS is a specific type of infectious disease, occurring mainly in young women during the menstrual period (80%-90%), but it has also been reported in non-menstrual cases (10%-20%). It is characterised by sudden-onset high fever, hypotension, rash, mucosal hyperaemia, and various additional symptoms such as myalgia, vomiting, and diarrhoea. The clinical course depends on the extent of the organ failure due to decreased tissue perfusion during hypotension. Severe cases are accompanied by multiple organ-system failure including impaired renal function, which is reversible in nearly all cases. Respiratory failure ranges from interstitial and alveolar aedema to ARDS in 10% of cases; severe DIC is seen in 10%-15%. Another severe clinical complication is cardiac insufficiency. The etiology of TSS is based on a localized or, rarely, systemic infection with certain Staph. aureus strains that are capable of producing toxins, the most important one being TSST-1. Staph. aureus strains can also produce various other enterotoxins that may be involved in the pathogenesis of TSS. The pathogenetic importance of the toxins is supported by the antibody titers in TSS patients: more than 80% of healthy adults show high levels of antibody titers, whereas 90% of TSS patients exhibit low levels in the acute phase followed by a significant increase during convalescence. It is not clear whether the toxins cause TSS by a direct effect or by release of mediators due to their function as superantigens. The clinical characteristics of non-menstrual TSS are identical to those of menstrual TSS, but it can occur in many clinical settings in both sexes at any age. Severe clinical courses are more frequent in non-menstrual TSS: the mortality is about 8%-11% in non-menstrual TSS compared to 2%-5% in menstrual TSS. The diagnosis is based mainly on clinical signs and the isolation of toxin-producing Staph. aureus strains. Besides antibiotic therapy, treatment is primarily directed to the correction of hypotension and additional organ-system failure. Other therapeutic measures such as the elimination of toxins by plasma separation or the administration of antibodies or gamma-globulins are subjects of investigation with no general recommendations at this time.

Organochlorine residues in human breast milk: analysis through a sentinel practice network.

STUDY OBJECTIVE: The study aimed to assess through a sentinel practice network the validity of data on levels of organochlorine residues in human milk along with personal, lifestyle, and exposure variables of breastfeeding women; to compare the results of this new approach with those of the Lower Saxony breast milk surveillance programme; and to test hypotheses on potential determinants of contamination levels. DESIGN: Eligible women were enrolled into this cross sectional study by a network of 51 paediatric practices when bringing their babies for a U3 infant screening examination (4th to 6th week after delivery). Lifestyle and exposure factors were obtained by questionnaire. All milk samples were analysed for hexachlorocyclohexane, hexachlorbenzole, DDT, dieldrin, polychlorinated biphenyls (PCB) and heptachlor; half the samples were also analysed for dioxin. Analytic statistics were computed using polychotomous logistic regression (PLR). SETTING: The study was conducted in Lower Saxony, Germany, from summer 1992 to summer 1993. PARTICIPANTS: Altogether 156 primiparous, breast feeding German women, aged 25-35 years, who had been born and had grown up in West Germany, were studied. MAIN RESULTS: Compared with the regular programme, participants in this study had their milk analysed sooner after delivery and were more likely to have grown up in rural areas, less likely to have been exposed to hazardous substances, less likely to have a diet of health food, and slightly less likely to be a smoker at the time of the study. Breast milk contamination levels were comparable in both studies, and in all but two cases well below the tolerable concentrations established by the Deutsche Forschungsgemeinschaft (German Research Fellowship). After adjustment for potential confounders using polychotomous logistic regression, there were statistically significant positive associations between breast milk contamination and age (PCB, test for trend: p = 0.006), average dietary fat intake per week (dioxin, p = 0.01), and proximity of residence to hazardous sites (dioxin, p < 0.05), and negative associations between residue levels and relative body weight at the time of the study (PCB; p < 0.0001) and difference in body weight (weight minus weight before the pregnancy; PCB, p = 0.0002), respectively. CONCLUSIONS: Sentinel practice networks are a feasible and low-biased approach to population based breast milk studies. The contamination levels and associations found are biologically plausible and comparable with the results of other studies. To reduce organochlorine residue levels in human milk in the short term, breast-feeding women should be advised not to try to reduce their weight until after lactation. Public promotion of a lower dietary fat intake may reduce the lifetime accumulation of organochlorine compounds in the human body fat tissue in the long term, resulting in lower concentrations in breast milk as well.

[Clinical importance of hypocaloric parenteral feeding]. / Klinische Bedeutung der hypokalorischen parenteralen Ernährung.

Hypocaloric parenteral nutrition (HPN) is mainly and frequently used in surgical medicine since it allows a reliable and standardized supply of nutrients. Ready-mix solutions which are suitable for peripheral venous nutrition generally contain about 50 g carbohydrates (glucose and/or xylitol), 25 g amino acids and electrolytes per liter. The osmolarity of the solutions averages between 600 and 800 mosmol/l. HPN has two distinct advantages: firstly the minimal effects on carbohydrate metabolism and secondly the good improvement in nitrogen balance. If 2 g/kg and day glucose are administered, even postoperatively, the mean blood sugar levels are only just above the normal range and an amino acid dosage of 1 g/kg and day, compared with liquid substitution alone or the administration of small amounts of carbohydrates, leads to an approximately 60% improvement in postoperative N-balance. Experience gained with HPN in surgical medicine to date permits the following recommendation: 1. HPN should not be used after small and moderate interventions with short nutritional abstinence; it is not necessary to administer nutrients in such cases. 2. HPN may be used after moderate to serious surgical interventions; e.g. after gastrointestinal resections in the case of elective surgery on patients with a normal nutritional status. However, studies have yet to prove the clinical efficacy of HPN, e.g. as evidenced by shorter hospitalization or a reduced perioperative morbidity or mortality, although this reservation equally applies to the routinely administered complete parenteral or enteral nutrition.(ABSTRACT TRUNCATED AT 250 WORDS)

[Data quality in the MORBUS Sentinel Project--expiratory wheezing in infants]. / Zur Datenqualität im Sentinelprojekt MORBUS--Exspiratorisches Giemen bei Kleinkindern.

Since 1991 the MORBUS project is being conducted to establish and run a sentinel network of 100 general and paediatric practices in three regions of Germany. A number of health conditions have been and will be monitored consecutively with special emphasis on environmentally determined health problems. From March to June, 1991, 1054 contacts of 1- and 2-year-old children with expiratory wheezing were reported. Quality of these event data was assessed by means of internal completeness. Important clinical information was missing in about 10% of all cases without evidence for regional differentiations. Data quality by this criterion was better in first contact than in re-contact cases (9.7% vs 18.1% missing). Questions concerning the parents (allergies, smoking, education) were less frequently answered (up to 24% missing) than questions of obvious medical relevance to the child. Completeness of parental information varied considerably between regions. There was no association between the medical specialty of the doctors and the quality of their data. In a longitudinal view, there was a slightly positive trend over time in the proportion of clinically incomplete case reports at borderline statistical significance (p = 0.057). Apart from these minor findings then, there was an overall good consistency of completeness in the MORBUS data on expiratory wheezing. By optimizing questionnaires and data transmission, it should be possible to increase the data quality even further.

[Spontaneous liver rupture as a rare complication of the HELLP syndrome]. / Die spontane Leberruptur als seltene Komplikation des HELLP-Syndroms.

The HELLP syndrome (haemolysis, elevated liver enzymes, low platelet count) was first referred to by Weinstein in 1982 as an extremely progressive form of gestosis. In addition to the more common gestotic symptoms, such as oedema, proteinuria and hypertension, the clinical picture is characterized by microangiopathic haemolysis, thrombocytopenia and, especially, impaired hepatic function. Within this clinical picture severe complications can occur, such as eclamptic attacks, renal dysfunction, intracranial haemorrhage, intrahepatic haemorrhage and coagulopathy. An imbalance in prostanoid metabolism has been implicated in the pathogenesis. A decrease in synthesis of the vasodilator and thrombocyte aggregation inhibitor prostacyclin leads to a preponderance of the vasoconstrictor thromboxane A2, which promotes thrombocyte aggregation. This results in local vascular spasms and endothelial lesions, which in the case of hypercoagulopathy are accompanied by the formation of fibrin deposits with resultant vascular constriction. Intravascular fibrin deposits indicate that the coagulation system has been compromised and can lead to consumption coagulopathy in approximately 10% of cases. In the majority of cases, however, one finds low-grade disseminated intravascular coagulation (DIC), i.e. mild hypercoagulopathy with thrombocytopenia, a tendency to thrombocyte aggregation and fibrinogen deficiency in the presence of usually normal plasmatic coagulation. These vascular changes occur particularly in organs that have high blood flow, such as liver, kidneys and placenta. In the liver, sinusoidal obstruction causes vascular congestion, leading to an increase in intrahepatic pressure, dilatation of Glisson's capsule, development of subcapsular hepatic haematomas and hepatic rupture. Hepatic haematoma virtually always requires surgical treatment, and otherwise the patient has hardly any chance of survival. Nevertheless, mortality is around 35%.(ABSTRACT TRUNCATED AT 250 WORDS)