Sleep-disordered breathing and lung function abnormalities in adults with congenital heart disease.

PURPOSE: Advances in treatment enables most patients with congenital heart diseases (CHD) to survive into adulthood, implying the need to address comorbid conditions in this growing cohort of patients. The aim of this study was to evaluate the prevalence of sleep-disordered breathing (SDB) and lung function abnormalities in patients with adult congenital heart disease (ACHD). METHODS: Patients with ACHD underwent level 3 sleep testing (Embletta MPR polygraphy) and pulmonary function testing. Results were stratified by the underlying haemodynamic ACHD lesion group. RESULTS: Patients with ACHD (n = 100) were middle-aged (42.3 ± 14.6 years), 54% male and slightly overweight (BMI 25.9 ± 5.5 kg/m2). Polygraphy revealed a prevalence of sleep apnoea of 39% with 15% of patients presenting with predominantly obstructive apnoeic episodes, while 23% of patients presenting primarily with central sleep apnoea. The distribution of mild, moderate, and severe sleep apnoea in the total study population was 26%, 7% and 6%, respectively. Comparison of apnoea-hypopnoea index, presence of sleep apnoea, and apnoea severity did not offer significant differences between the four ACHD lesion groups (p = 0.29, p = 0.41 and p = 0.18, respectively). Pulmonary function testing revealed obstructive lung disease in 19 of 100 patients. Concomitant chronic obstructive pulmonary disease and obstructive sleep apnoea were diagnosed in 3% of patients and were associated with profound nocturnal desaturation. CONCLUSION: The findings suggest a mild propensity amongst patients with ACHD to develop SDB that seems to be unaffected by the specific underlying congenital lesion.

The Rap1 small GTPase is a critical mediator of the effects of stress on prefrontal cortical dysfunction.

The neural molecular and biochemical response to stress is a distinct physiological process, and multiple lines of evidence indicate that the prefrontal cortex (PFC) is particularly sensitive to, and afflicted by, exposure to stress. Largely through this PFC dysfunction, stress has a characterized role in facilitating cognitive impairment, which is often dissociable from its effects on non-cognitive behaviors. The Rap1 small GTPase pathway has emerged as a commonly disrupted intracellular target in neuropsychiatric conditions, whether it be via alterations in Rap1 expression or through alterations in the expression of direct and specific upstream Rap1 activators and inhibitors. Here we demonstrate that escalating, intermittent stress increases Rap1 in mouse PFC synapses, results in cognitive impairments, and reduces the preponderance of mature dendritic spines in PFC neurons. Using viral-mediated gene transfer, we reveal that the hyper-induction of Rap1 in the PFC is sufficient to drive stress-relevant cognitive and synaptic phenotypes. These findings point to Rap1 as a critical mediator of stress-driven neuronal and behavioral pathology and highlight a previously unrecognized involvement for Rap1 in novelty-driven PFC engagement.

Immunological detection of osteocalcin in meat and bone meal: a novel heat stable marker for the investigation of illegal feed adulteration.

A sandwich ELISA was developed for the detection of bovine meat and bone meal (BMBM) in feed, based on polyclonal rabbit antibodies raised against the synthetic N-terminal amino acid sequence 1-9 (YLDHWLGAP) of bovine osteocalcin. To set up a sandwich ELISA pair, a commercial mouse monoclonal capture antibody binding to a highly conserved epitope in the mid-fragment of the peptide was employed. It is shown that the bone marker osteocalcin is immunologically well detectable in BMBM extracts obtained by a simple EDTA-based procedure even in a sample heated up to 145°C. Furthermore, a genus-specific restriction of the major specificity to cattle and horse was possible. The observed bi-specificity is consistent with theoretical predictions. The assay sensitivity with bovine osteocalcin of 1 ng was sufficient to enable the detection of 0.1% BMBM in compound plant feed or fish meal, for which no cross reaction was observed. In general the quantification of osteocalcin in extracts is possible using a standard curve procedure with pure bovine osteocalcin.

Small bowel intussusceptions: issues and controversies related to pneumatic reduction and surgical approach.

AIM: This study aims to determine the incidence, clinical presentation, management and outcome of small bowel intussusceptions (SBI) in the paediatric population managed at our centre. METHODS: Hospital charts of SBI patients from 1999 to 2006 were reviewed retrospectively. The data collected involved the clinical presentation, diagnostic examinations, management strategy as well as outcome. RESULTS: Ileocecal intussusceptions were documented in 83 patients (74.8%) and SBI in 28 (25.2%). The median age of patients with SBI was 2.5 years (range 9 weeks to 16 years). In 18/28 patients SBI reduced spontaneously. Ileoileal SBI had an incidence of 80% in the 10/28 cases requiring intervention. In seven patients, pneumatic reductions were successful in 6/7 ileoileal intussusceptions, but unsuccessful in 1/7 where the level of intussusception (jejunojejunal intraoperative findings) could not be determined by ultrasound. Surgical intervention was required in four patients with bowel resection in three cases. CONCLUSION: Pneumatic reduction is successful in ileoileal intussusceptions with signs of bowel viability. It should be attempted with caution in patients with jejunojejunal or ileoileal SBI with pathologic lead points or bowel ischaemia. Accurate interpretation of ultrasound along with judicious implementation of pneumatic reduction or surgical options can reduce morbidity. Early diagnosis is associated with better outcomes using non-surgical reduction techniques.

Ruptured sinus of Valsalva aneurysm in two patients with subarterial ventricular septal defect.

Rupture of a sinus of Valsalva aneurysm (SVA) is a rare, but life-threatening, event and requires immediate recognition and intervention. We present two previously healthy and physically active patients who were 12 and 33 years of age when rupture of a right coronary SVA into the right ventricle occurred. A subarterial ventricular septal defect (VSD) was detectable in both patients. Cardiac surgery involved VSD closure as well as reconstruction of the aortic valve. Considering complications of subarterial VSD, such as aortic cusp prolapse, aortic insufficiency or SVA, we suggest close follow-up and surgical closure of the VSD in case of any aortic valve deformity.

Systemic iodine absorption after preoperative antisepsis using povidone-iodine in cataract surgery-- an open controlled study.

After preoperative conjunctival and periorbital antisepsis with povidone-iodine (PVP-I), the systemic absorption of iodine after cataract surgery was measured to evaluate the risk of thyroid side effects. Five different combinations of PVP-I alone or in combination with PVP-I-free antiseptics were applied to the conjunctiva and periorbital skin. An iodine-free product served as control. Iodide and creatinine in urine were analyzed before intervention and 24 and 48 h postoperatively. Depending on the concentration and application site, 0.3-4.5% of the total applied iodine or 3.6-45.4% of the free iodine were absorbed. The range of urine iodine excretion was between 11.7 and 71.0 mug iodine/g creatinine, depending on the PVP-I concentration and the site of application. The increase in iodine excretion was significant at 24 h postoperatively in trials receiving PVP-I both periorbitally and conjunctivally, depending of the concentration used. Because the iodine absorption is only slight and of doubtful clinical relevance, presurgical conjunctival antisepsis can be achieved with 1.25% PVP-I; so far clinically manifest anamnestic thyroid disorders are excluded. Presently, periorbital skin antisepsis with PVP-I cannot be recommended until data on thyroid metabolism in the population have been collected and evaluated, especially in a region currently or previously deficient in iodine.

[Asymptomatic cor triatriatum in a 2 1/4 year old patient]. / Asymptomatisches Cor triatriatum bei einer 2 1/4 Jahre alten Patientin.

We report on an asymptomatic 2 1/4 year old girl. Echocardiography revealed cor triatriatum with a membrane separating the left atrium. Transesophageal echocardiography demonstrated 3 large perforations of the intraatrial membrane. Using Doppler sonography, no pressure gradients across these orifices were identified. Obstruction of the intraatrial membrane and pulmonary hypertension were excluded by cardiac catheterization. We therefore decided against surgical resection of the membrane and to follow the patient noninvasively by echocardiography. Follow-up over 18 months revealed no development of an obstruction across the intraatrial membrane. Our case shows that immediate surgical correction is not necessary in all patients with cor triatriatum. Conservative management of these patients requires full evaluation of the hemodynamics and careful follow-up examinations by echocardiography.

Expression of AtPRP3, a proline-rich structural cell wall protein from Arabidopsis, is regulated by cell-type-specific developmental pathways involved in root hair formation.

The tightly regulated expression patterns of structural cell wall proteins in several plant species indicate that they play a crucial role in determining the extracellular matrix structure for specific cell types. We demonstrate that AtPRP3, a proline-rich cell wall protein in Arabidopsis, is expressed in root-hair-bearing epidermal cells at the root/shoot junction and within the root differentiation zone of light-grown seedlings. Several lines of evidence support a direct relationship between AtPRP3 expression and root hair development. AtPRP3/beta-glucuronidase (GUS) expression increased in roots of transgenic seedlings treated with either 1-aminocyclopropane-1-carboxylic acid (ACC) or alpha-naphthaleneacetic acid (alpha-NAA), compounds known to promote root hair formation. In the presence of 1-alpha-(2-aminoethoxyvinyl)glycine (AVG), an inhibitor of ethylene biosynthesis, AtPRP3/GUS expression was strongly reduced, but could be rescued by co-addition of ACC or alpha-NAA to the growth medium. In addition, AtPRP3/GUS activity was enhanced in ttg and gl2 mutant backgrounds that exhibit ectopic root hairs, but was reduced in rhd6 and 35S-R root-hair-less mutant seedlings. These results indicate that AtPRP3 is regulated by developmental pathways involved in root hair formation, and are consistent with AtPRP3's contributing to cell wall structure in Arabidopsis root hairs.

Characterization and expression of four proline-rich cell wall protein genes in Arabidopsis encoding two distinct subsets of multiple domain proteins.

We have characterized the molecular organization and expression of four proline-rich protein genes from Arabidopsis (AtPRPs). These genes predict two classes of cell wall proteins based on DNA sequence identity, repetitive motifs, and domain organization. AtPRP1 and AtPRP3 encode proteins containing an N-terminal PRP-like domain followed by a C-terminal domain that is biased toward P, T, Y, and K. AtPRP2 and AtPRP4 represent a second, novel group of PRP genes that encode two-domain proteins containing a non-repetitive N-terminal domain followed by a PRP-like region rich in P, V, K, and C. Northern hybridization analysis indicated that AtPRP1 and AtPRP3 are exclusively expressed in roots, while transcripts encoding AtPRP2 and AtPRP4 were most abundant in aerial organs of the plant. Histochemical analyses of promoter/beta-glucuronidase fusions localized AtPRP3 expression to regions of the root containing root hairs. AtPRP2 and AtPRP4 expression was detected in expanding leaves, stems, flowers, and siliques. In addition, AtPRP4 expression was detected in stipules and during the early stages of lateral root formation. These studies support a model for involvement of PRPs in specifying cell-type-specific wall structures, and provide the basis for a genetic approach to dissect the function of PRPs during growth and development.

[Atraumatic Clostridium septicum infection in granulocytopenia]. / Atraumatische Clostridium-septicum-Infektion bei Granulozytopenie.

A fatal Clostridium septicum infection occurred in three patients. Case 1. A 55-year-old man died of septicaemia resulting from granulocytopenia of uncertain aetiology; it was associated with perforation of ileal mucosal ulcers. Autopsy revealed neutropenic enterocolitis and diffuse gas formation, especially in the brain, caused by Clostridium septicum. Case 2. A 18-year-old boy developed a caecal invagination during imipenem-induced granulocytopenia. A fulminant postoperative Clostridium septicum infection ended fatally. At autopsy many ulcers were found at the site of invagination with gas formation involving all organs. Case 3. Myonecrosis of the left arm, caused by Clostridium septicum, developed without external cause in a 12-year-old girl with congenital neutropenia. Despite aggressive surgical intervention she died of toxic shock. Autopsy revealed caecal mucosal ulcers as the portal of entry of Clostridium septicum.

Combination of interferons and cytostatic drugs for treatment of advanced colorectal cancer.

Three phase I/II trials were performed in patients with metastatic colorectal cancer using immunochemotherapy--a combination of recombinant interferon beta and gamma with low doses of cytostatic drugs. The third regimen, consisting of a cytostatic component containing 5-fluorouracil plus carboplatin plus mitomycin C besides the interferons, produced a high remission rate of 47%: 14/30 patients responded. The tolerability of this protocol was good and it could be administered on an out-patient basis.

[Catalases of mycobacteria as antigens. I. Isolation, purification and characterization of catalases from different mycobacteria (author's transl)]. / Katalasen von Mykobakterien als Antigene. I. Isolierung, Reinigung und Charakterisierung von Katalasen verschiedener Mykobakterien.

Preparations of catalase isolated from the strains M. tuberculosis, M. kansasii and M. bovis BCG are produced for testing their antigenic activity. After desintegration of the bacteria the highest activity remained in the precipitation with 50% saturated ammonium sulphate solution. The further purification of the catalase-fractions occurred with the aid of column chromatography on Sephadex G 200 and DEAE-Sephadex-A 50 after ultrafiltration. In this way the relative activity increased in M. tuberculosis 3- to 4-fold, in M. kansasii 12-fold and in M. bovis BCG 16-fold. The catalase preparations are uniform and nearly free from other protein compounds as indicated by the results of immunoelectrophoresis and Ouchterlony test.