[Practical implementation of an integrated care model in mother-child treatment]. / Praktische Umsetzung eines integrierten Versorgungsmodells in der Mutter-Kind-Behandlung.

This article summarizes the specific treatment options in the combined mother-child treatment for women with postpartum psychiatric disorders. The incidence of postpartum mental illness is high, especially for postpartum depression that probably occurs in10-15 % of cases. Mental disorders not only have a negative impact on the mental health of the women affected, such as chronification and suicidal tendencies but can also adversely affect the mother-child relationship as well as the emotional and cognitive development of the child. For these reasons it is important, also under the aspects of a primary prevention with respect to the children of mentally ill mothers, to provide a needs-oriented, timely and disorder-specific interactional therapy, which also considers the long-term aspects of treatment. At the Celenus Klinik Carolabad in Chemnitz an integrated treatment model, the "Carola PLUS", could be established that caters for the particularly high demands of combined care and treatment for both mother and child.

[Bleeding origin, patient-related risk factors, and prognostic indicators in patients with acute gastrointestinal hemorrhages requiring intensive care treatment. A retrospective analysis from 1999 to 2010]. / Ursachen, patientenspezifische Risikofaktoren und prognostische Indikatoren bei akuter gastrointestinaler Blutung und intensivmedizinischer Therapieindikation. Eine retrospektive Untersuchung der Jahre 1999-2010.

BACKGROUND: Gastrointestinal bleeding (GIB) is a common problem in elderly patients involving severe comorbidities and concomitant antiplatelet or anticoagulatory therapy. The risk factors and prognostic indicators of patients with severe GIB requiring intensive care medical treatment have not been well evaluated. METHODS: A retrospective analysis of 7,376 patients from the medical intensive care unit (ICU) at the University Hospital Aachen was carried out between 1999 and 2010. RESULTS: Of 614 patients admitted to the ICU because of acute GIB, 463 (75%) presented with upper GIB (UGIB) and 151 (25%) with lower GIB (LGIB). Despite early endoscopic intervention and ICU treatment, UGIB had a mortality rate of 16%, whereas LGIB showed a significantly better prognosis (mortality <5%) in the ICU setting. Risk factors for OGIB-related mortality were hemodynamic instability, organ failure, comorbidities (especially liver cirrhosis), and rebleeding. In total, 218 patients (36%) were treated with antiplatelet or anticoagulatory drugs, which were associated with a favorable prognosis in the UGIB group. Elevated serum lactate levels upon admission were superior in predicting mortality than established indicators of prognosis such as the Rockall or the Glasgow-Blatchford score. CONCLUSIONS: Despite successful endoscopic intervention, severe acute UGIB is associated with a significant mortality rate of 16% in the ICU setting, determined by hemodynamic failure, organ dysfunction, and comorbidities. The serum lactate levels of patients with GIB on the day of admission to the ICU are prognostic.

European multidisciplinary consensus statement on the use and monitoring of metal-on-metal bearings for total hip replacement and hip resurfacing.

INTRODUCTION: There is an ongoing debate about the optimal use of metal-on-metal (MoM) bearings in total hip replacement, since there are uncertainties about local and systemic adverse effects due to wear and corrosion of these bearings. Despite various national recommendations, efforts to achieve international harmonization of specific evidence-based recommendations for best practice are still lacking. HYPOTHESIS: An international consensus study group should be able to develop recommendations on the use and monitoring of MoM bearings, preferably at the European level, through a multidisciplinary approach, by integrating the perspectives of various stakeholders. MATERIALS AND METHODS: Twenty-one experts representing three stakeholder groups and eight countries participated in this European consensus study, which consisted of a consensus meeting, subsequent structured discussion, and consensus voting. RESULTS: The current statement defines first of all benefits, local and systemic risks, as well as uncertain issues related to MoM bearings. Safety assessment after implantation of MoM comprises all patients. A closer follow-up is recommended for large head MoM (≥36mm) and resurfacing. In these implants basic follow-up should consist of x-rays and metal ion measurement of cobalt in whole blood, performed with GF-AAS or ICP-MS. Clinical and/or radiographic abnormality as well as elevated ion levels needs additional imaging (ultrasound, CT-scan and/or MARS-MRI). Cobalt values less than 2 µg/L are probably devoid of clinical concern, the threshold value for clinical concern is expected to be within the range of 2-7 µg/L. DISCUSSION: This is the first multinational, interdisciplinary, and multiprofessional approach for developing a recommendation for the use and monitoring of MoM bearings in total hip replacement. The current recommendations are in partial agreement with previous statements regarding the extent of follow-up and imaging techniques. They however differ from previous communications regarding measurement of metal ions and especially the investigated medium, technique, and eventual threshold levels. LEVEL OF EVIDENCE: Level V, expert opinion/agreement conference.

[Gastrointestinal bleeding in liver cirrhosis at the ICU]. / Gastrointestinale Blutungen als Komplikation von Patienten mit Leberzirrhose auf der Intensivstation.

Due to portal hypertension and bleeding disorders, patients with liver cirrhosis are at increased risk for severe gastrointestinal bleedings (GIB), commonly requiring therapy at the intensive care unit (ICU). In order to identify epidemiological and prognostic factors for GIB in cirrhotic patients, we retrospectively analysed patients from our medical ICU from 1999 to 2010. Among 7376 critically ill patients, 650 (8.8 %) were diagnosed with liver cirrhosis. Hepatic cirrhosis was frequently found in ICU patients admitted due to severe GIB (23.2 % of 711 patients had cirrhosis). Moreover, patients with cirrhosis were at increased risk to develop severe GIB during intensive care treatment (40.9 % of 44 patients with GIB during ICU stay had cirrhosis). Besides the high rate of variceal bleedings (64.4 %) in cirrhotic patients, non-variceal haemorrhages were also common (28.5 %). We identified the MELD score and necessity of mechanical ventilation as independent risk factors for mortality in cirrhotic patients with severe GIB. Patients with liver cirrhosis and severe GIB had significantly impaired prognosis (case-related fatality rate of 26.1 % with cirrhosis vs. 6.8 % without cirrhosis), especially in cases of newly developed GIB during ICU therapy. Advanced therapeutic approaches and novel strategies are warranted to improve the critical prognosis of these high-risk patients.

The Use of Europiumstearate to Trace Polyethylene Wear Debris in Joint Fluid after Prosthetic Joint Replacement - A Feasibility Study.

Ultra-high molecular weight polyethylene (UHMWPE) is the most common counterface material against metals or ceramics in artificial hip or knee joints. Wear and the resulting particulate debris, however, limit the life span of the implant. In this study, the general feasibility of using Europium (Eu) as tracer material to quantify UHMWPE wear in joint fluid is investigated. Using Inductively Coupled Mass Spectrometry (ICP-MS), recovery experiments of Eu in artificial joint fluid were performed. In order to dope polyethylene with 50 ppm Eu, nascent UHMWPE powder was mixed with a solution of Eu-stearate. The heterogeneity of the mixture was assessed by determining the coefficient of variation (CV) of the Eu content in various weighted samples. After molding of the UHMWPE powder mixture, cylindrical pins of 10 mm diameter were machined and worn against cobalt-chromium metal disks submersed in artificial joint fluid. The Eu-content of fluid samples taken at certain time intervals was measured and compared with UHMWPE weight loss of the pins. A satisfactory homogenization of Eu in the UHMWPE powder was achieved. Tracer-based and weight-loss determined wear rates were highly correlated (Pearson correlation coefficients > 0.991). Also the detection bias was within acceptable limits. Thus both methods demonstrated good agreement.

A review on phosphate based, solid state, protonic conductors for intermediate temperature fuel cells.

The electrolytes currently used for proton exchange membrane fuel cells are mainly based on polymers such as Nafion which limits the operation regime of the cell to ∼80 °C. Solid oxide fuel cells operate at much elevated temperatures compared to proton exchange membrane fuel cells (∼1000 °C) and employ oxide electrolytes such as yttrium stabilized zirconia and gadolinium doped ceria. So far an intermediate temperature operation regime (300 °C) has not been widely explored which would open new pathways for novel fuel cell systems. In this review we summarize the potential use of phosphate compounds as electrolytes for intermediate temperature fuel cells. Various examples on ammonium polyphosphate, pyrophosphate, cesium phosphate and other phosphate based electrolytes are presented and their preparation methods, conduction mechanism and conductivity values are demonstrated.

Photo-induced effects on self-organized TiO2 nanotube arrays: the influence of surface morphology.

Self-organized TiO(2) nanotubes with packed, vertically aligned morphology and different lateral characteristics were grown on Ti metal substrates by controlled electrochemical anodization in phosphate/HF and ethylene glycol/HF electrolytes. The wetting, photo-induced superhydrophilicity, and photocatalytic activity of the nanotubular materials were investigated under ultraviolet irradiation. The photoactivity of the TiO(2) nanotube arrays was analysed in terms of their morphological characteristics that were determined by means of scanning electron microscopy and atomic force microscopy in conjunction with geometrical modelling. The wetting and the UV-induced superhydrophilicity could be accordingly modelled by the Cassie-Baxter mode arising from the large scale roughness of the nanotubular arrays in combination with the Wenzel mode due to the small scale roughness induced by ridges at the outer tube surface. The photocatalytic activity of the TiO(2) nanotube arrays was further found to correlate quantitatively with the variation of the geometric roughness factor, verifying the strong impact of morphology on the photo-induced properties of the vertically oriented TiO(2) tubular architecture.

[Cutaneous malignant peripheral nerve sheath tumor in neurofibromatosis type 1]. / Kutaner maligner peripherer Nervenscheidentumor bei Neurofibromatose Typ 1.

Patients with neurofibromatosis have an increased risk of developing malignant tumors in comparison to the general population. We describe a woman who developed a malignant peripheral nerve sheath tumor in a pre-existing neurofibroma.

[Juvenile hyaline fibromatosis]. / Juvenile hyaline Fibromatose.

Juvenile hyaline fibromatosis is a rare autosomal recessive disease of the connective tissue. We present the case of a 6-year-old normal mental developed boy with confluent pearly papules behind the ears and in the paranasal folds, firm nodules of the scalp, the back and metaphalangs, and severe gingival hypertrophy.

Dye-sensitized solar cells based on thick highly ordered TiO(2) nanotubes produced by controlled anodic oxidation in non-aqueous electrolytic media.

Dye-sensitized solar cells (DSSCs) were prepared using TiO(2) nanotubes, grown by controlled Ti anodic oxidation in non-aqueous media. Smooth, vertically oriented TiO(2) nanotube arrays, presenting a high degree of self-organization and a length of 20 µm, have been grown using ethylene glycol electrolyte containing HF. As-grown nanotubes exhibit an amorphous structure, which transforms to the anatase TiO(2) crystalline phase upon post-annealing in air at 450 °C. Atomic force microscopy (AFM) revealed the porous morphology together with high roughness and fractality of the surface. The annealed tubes were sensitized by the standard N719 ruthenium dye and the adsorption was characterized using resonance micro-Raman spectroscopy and adsorption-desorption measurements. The sensitized tubes were further used as active photoelectrodes after incorporation in sandwich-type DSSCs using both liquid and solidified electrolytes. The efficiencies obtained under air mass (AM) 1.5 conditions, using a back-side illumination geometry, were very promising: 0.85% using a composite polymer redox electrolyte, while the efficiency was further increased up to 1.65% using a liquid electrolyte.

The efficiency of nanotube formation on titanium anodized under voltage and current control in fluoride/glycerol electrolyte.

The formation of nanotubes on titanium is compared for anodizing under controlled voltage and controlled current in a fluoride/glycerol electrolyte. Rutherford backscattering spectroscopy and nuclear reaction analysis are employed to determine the film compositions. Film morphologies are examined by electron microscopy. The findings reveal films of approximate composition TiO(2).0.15TiF(4) that probably also contain derivatives of glycerol. Controlled voltage conditions resulted in more uniform final nanotube dimensions, for a particular charge density, and the highest efficiency of film growth, with the charge of the titanium in the film representing ∼48% of the charge passed during anodizing. Under current control, the efficiency decreased from ∼40% to ∼23% with increase of the current density from 0.1 to 0.5 mA cm(-2). Further, the thickness of the barrier layer was sometimes enhanced under current control, possibly due to a non-uniform current distribution and consequently elevated local temperature.

[Systemic spread of wear debris--an in-vivo study]. / In-vivo-Analyse zur systemischen Verteilung von metallischen Abriebpartikeln.

AIM: The aim of this study was to elucidate whether there is a systemic spread of wear debris from peripherally applied stainless steel and titanium particles into the blood and subsequently to parenchymatous organs. Furthermore, we report on histological findings at the implantation site. METHOD: In Syrian Gold hamsters we implanted 2 mm3 wear debris of stainless steel and titanium into the dorsal skin fold chamber. Over a period of 2 weeks we took blood samples and afterwards explanted the implant area, the heart, lung, liver and spleen. One half of the organs and the implant area were used for histological analysis. The other half of the organs and the blood samples were analysed by optical emission spectrometer with inductively coupled plasma and graphite furnace atomic absorption spectrophotometry for their contents of chromium, nickel and titanium. RESULTS: In the group with titanium particles, histological analysis of the implant site showed moderate phagocyted wear in granulocytes but no other pathological findings. Animals treated with stainless steel wear debris had a massive inflammatory reaction, in some cases leading to necrosis. The analysis of the blood and one half of the organs showed increased levels of chromium and, already 24 hours after implantation, raised values for nickel. The result of the hamsters treated with titanium showed significantly elevated levels of titanium ions in the organs, but not in the blood samples. Histology of the organs did not reveal pathological findings. CONCLUSION: In this study we could show a massive inflammatory reaction for stainless steel wear debris in contrast to titanium wear debris at the implantation site. The elevated blood levels of chromium and increased values of other metals in the organs suggest the haematogenic distribution of ions from the peripherally implanted wear debris.

New options for prenatal diagnosis in autosomal recessive polycystic kidney disease by mutation analysis of the PKHD1 gene.

Due to the poor prognosis of severe autosomal recessive polycystic kidney disease (ARPKD), there is a strong demand for prenatal diagnosis (PD). Reliable PD testing is possible by molecular genetic analysis only. Although haplotype-based analysis is feasible in most cases, it is associated with a risk of misdiagnosis in families without pathoanatomically proven diagnosis. Linkage analysis is impossible in families where DNA of the index patient is not available. Direct mutation analysis of the recently identified polycystic kidney and hepatic disease 1 gene opens new options in families to whom a reliable PD cannot be offered on the basis of linkage analysis. We for the first time report two cases with PD based on mutation detection, illustrating the new options for PD in ARPKD.

Spondyloepiphyseal dysplasia Omani type: a new recessive type of SED with progressive spinal involvement.

We report a large inbred kindred from Oman with a distinct type of spondyloepiphyseal dysplasia (SED). We evaluated eight individuals from two consanguineous sibships, one male and seven females between the ages of 2 and 22. The pedigrees strongly suggest autosomal recessive inheritance and both families are likely to be related through distant consanguineous loops. The clinical features include near to normal length at birth, short stature with final height of 110-130 cm, shortening of the upper segment due to severe progressive kyphoscoliosis, severe arthritic changes with joint dislocations, rhizomelic limbs, genu valgum, cubitus valgus, mild brachydactyly, camptodactyly, microdontia, and normal intelligence. Minor radiographic metaphyseal changes were found, but major manifestations were in the spine and the epiphyses. During the first year of life the vertebral bodies are of normal height but the endplates are irregular and intervertebral space is narrow. With age, the vertebral endplates become increasingly irregular, the intervertebral space diminishes further and individual vertebrae start to fuse resulting in a severe short trunk dwarfism with kyphoscoliosis. The epiphysis are small and precocious osteoarthropathy was observed involving small and large joints. The elbow, wrist, and hip joints were affected starting in infancy and showed restricted movement. Osteoarthropathy and spinal involvement resulted in physical handicap in early adulthood. Comparison of these patients with other skeletal dysplasias suggests that they represent a previously undescribed variant of SED.

Novel mutations in the Atlastin gene (SPG3A) in families with autosomal dominant hereditary spastic paraplegia and evidence for late onset forms of HSP linked to the SPG3A locus.

Hereditary spastic paraplegias (HSP) comprise a genetically and clinically heterogeneous group of neurodegenerative disorders characterised by progressive spasticity and hyperreflexia of the lower limbs. Autosomal dominant hereditary spastic paraplegia linked to the SPG3A locus on chromosome 14q11-21 accounts for approximately 10% of autosomal dominant hereditary spastic paraplegia (ADHSP). It is caused by mutations in the SPG3A gene encoding the protein atlastin. To date, only five disease-causing mutations in the SPG3A gene have been described. We analysed 13 SPG4-negative families for mutations in the SPG3A gene and identified a mutation in 38% (5/13). Two of the mutations are novel, c.481G>C (p.A161P) and c.740A>C (p.H247P). One of the novel mutations was found both in a family with early onset of symptoms and in a late onset family. Furthermore, we report on numerous polymorphisms detected in the SPG3A gene.

Congenital generalized lipodystrophy, mental retardation, deafness, short stature, and slender bones: a newly recognized syndrome?

We present three patients with congenital generalized lipodystrophy, sensorineural deafness, low birth weight, short stature, delayed cognitive development, and progressive bone changes characterized by overtubulation and rarefaction of long bones with dense metaphyseal striations occurring in adolescence. Abnormalities of lipid and carbohydrate metabolism, hepatosplenomegaly, acanthosis nigricans, and hirsutism were not found. Comparison of the condition of these patients with known syndromic lipodystrophies suggests that they represent a previously unrecognized genetic disorder. The occurrence in sibs born to consanguineous parents and a third patient from the same tribal unit suggest autosomal recessive inheritance.

Autosomal recessive type of Adams-Oliver syndrome: prenatal diagnosis.

We report on three pregnancies complicated by Adams-Oliver syndrome in a consanguineous Turkish couple. Two cases were correctly diagnosed prenatally at 22+3 and 13+0 weeks gestation following the first case of Adams-Oliver syndrome in which severe anomalies of the extremities were observed at 26+5 weeks' gestation. In this first case, the diagnosis of Adams-Oliver syndrome was made following termination of pregnancy at 27+2 weeks' gestation. In all three cases, autopsy was performed. All fetuses showed anomalies of the extremities, aplasia cutis and symmetric defects of the skull, with bone being replaced by collagenous tissue. Although there have been numerous cases of the postnatal diagnosis of Adams-Oliver syndrome following termination of pregnancy, this is the first description of the prenatal diagnosis of this disorder.

Prenatal diagnosis in Coffin-Lowry syndrome demonstrates germinal mosaicism confirmed by mutation analysis.

Coffin-Lowry syndrome is a rare X-linked, semi-dominant mental retardation syndrome resulting from mutations of the ribosomal S6 kinase 2 (RSK2) gene. In the present report, a male patient affected with Coffin-Lowry syndrome is shown to have a nonsense mutation of the RSK2 gene. His unaffected mother does not have this mutation in her lymphocytes. In her third pregnancy prenatal diagnosis by mutation analysis has detected gonadal mosaicism. As this is the second report of germinal mosaicism in Coffin-Lowry syndrome, the finding has important implication for genetic counselling.

Mesomelic campomelia, polydactyly and Dandy-Walker cyst in siblings.

The present report describes two fetuses, one female and one male, with thus far undescribed skeletal malformations. The mother was a gravida 2, para 0. Both pregnancies were terminated in the second trimester because of multiple congenital anomalies diagnosed ultrasonographically resembling a short rib-polydactyly syndrome. Both fetuses were found to have postaxial hexadactyly of the hands and feet, marked bilateral campomelia of the forearm and shank bones, and a Dandy-Walker cyst. In addition, the fourth ventricle was dilated in the first sibling and the second sibling had an inverse intestinal malrotation. A literature search failed to reveal similar observations.