Oxidation of Pt(111) under near-ambient conditions.

The oxidation of Pt(111) at near-ambient O2 pressures has been followed in situ using x-ray photoelectron spectroscopy (XPS) and ex situ using x-ray absorption spectroscopy (XAS). Polarization-dependent XAS signatures at the O K edge reveal significant temperature- and pressure-dependent changes of the Pt-O interaction. Oxide growth commences via a PtO-like surface oxide that coexists with chemisorbed oxygen, while an ultrathin α-PtO2 trilayer is identified as the precursor to bulk oxidation. These results have important implications for understanding the chemical state of Pt in catalysis.

[Evaluation of haemostasis in children treated with valproic acid]. / Untersuchungen zur Hämostase bei Kindern unter Valproatbehandlung.

UNLABELLED: Coagulation parameters were determined in children with valproic acid mono- and valproic acid-lamotrigin combination therapy. PATIENTS, METHODS: Monotherapy group (n = 22; mean age: 10.5 years) was compared to combination therapy (n = 7; 12.9 years) and a control group (n = 22; 8.7 years). The following parameters were measured: aggregation and ATP-release in whole blood (ADP: 20 µmol/l, collagen: 1 µg/ml, thrombin: 0.5 U/ml), PFA-100® closure times (CT), blood cell counts, global tests, VWF:Ag, VWF:CBA, factors VIII and XIII as well as fibrinogen. Bleeding symptoms were evaluated by using a questionnaire. RESULTS: For ADP- and collagen-induced aggregation as well as for ATP release no significant differences between the groups were detected. The combined therapy group showed significantly prolonged CT. Von Willebrand disease was not detected in any of the patients. The platelet count was significantly decreased in the monotherapy group. In six children a mild bleeding tendency was observed, mostly epistaxis. CONCLUSION: A clinically relevant influence of valproic acid on haemostasis was found only in few cases. However, before surgical procedures an extended coagulation diagnostics is recommended in patients with valproic acid therapy.

The first case of 3-methylcrotonyl-CoA carboxylase (MCC) deficiency responsive to biotin.

3-Methylcrotonylglycinuria is an inborn error of leucine catabolism with an autosomal recessive pattern of inheritance that results from a deficiency of 3-methylcrotonyl-CoA carboxylase (MCC). We report on a nine-year-old boy with severe psychomotor retardation who developed infantile spasms at the age of three weeks. Urine analysis at the age of two years revealed massive 3-methylcrotonylglycinuria and 3-hydroxyisovaleric aciduria suggesting MCC deficiency. Carnitine serum levels were decreased. Biotin therapy led to a dramatic decrease in the frequency of seizures, disappearance of hypsarrhythmia, and near normalisation of organic aciduria. Four months later a protein-restricted diet was introduced in addition and the boy remained clinically and metabolically stable. However, severe psychomotor delay persisted, and the seizures partially reoccurred. Biochemical findings showed partial MCC deficiency in cultured fibroblasts. Molecular genetic studies revealed a heterozygote missense mutation, MCCA-R385S, converting arginine to serine in a highly conserved region of the MCCA gene. This is the first patient with MCC deficiency caused by a heterozygote mutation and who demonstrated a substantial and sustained clinical and biochemical response to therapeutic doses of biotin. Sadly, this patient again also demonstrates that the main determinant of the outcome of even easily treatable metabolic diseases is timely diagnosis.

Neonatal seizures in two sisters with incontinentia pigmenti.

Familial incontinentia pigmenti (IP) (OMIM #308300) is a rare genetic disorder which segregates in an X-linked dominant way. The female-to-male ratio ranges from 20 to 37 : 1. In affected females IP causes highly variable abnormalities of the skin, hair, nails, teeth, eyes, and central nervous system (CNS). Cardiovascular anomalies, cerebral infarction, and immune dysfunction are rare complications of IP. The pathogenesis of cerebral changes in IP remains elusive. We report the case of two IP-affected sisters who presented in each case with neonatal seizures on the fifth day of life. Via cranial magnetic resonance tomographic imaging (MRI) different types of lesions in both hemispheres were demonstrable in both patients. To date the pathogenetic mechanisms for the cerebral lesions are not fully understood. However, multiple microscopic infarcts could serve as a possible explanation. The clinical course and the neurological development of the older child are favorable and so far the younger sibling appears to be developing normally, which is uncommon for patients with early onset of neurological symptoms. Symptomatic seizures in IP are an important differential diagnosis in benign non-familial and familial neonatal seizures.

Bradycardia and hypoxaemia due to position-dependent hypoperfusion of the brain stem in a preterm infant.

UNLABELLED: The case is reported of a male baby with a decreased time average velocity of the basilar artery to 32%, measured by Doppler sonography in dextro-rotated head position. The decrease was due to a hypoplastic right vertebral artery with compression of the contralateral vertebral artery at the craniocervical junction during dextro-rotation of the head. This finding was more prominent in prone than in supine position. A decrease in oxygen saturation and heart rate to < 70% and 60 bpm, respectively, was monitored during dextro-rotation. The polysomnography also revealed postural-dependent bradycardia, decrease of the oxygen saturation, and rising carbon dioxide partial tension in prone position with dextro-rotation of the head. CONCLUSION: Hypoperfusion of the brain stem caused by postural changes leads to further clinically relevant changes. Therefore an association with an acute life-threatening event and sudden infant death syndrome is speculated.

The Kleine-Levin syndrome - effects of treatment with lithium -.

Kleine-Levin syndrome (KLS) is a rare disorder which affects mainly adolescents. Periods of extreme somnolence alternate with megaphagia, psychomental changes and behavioural symptoms. The cause and pathogenesis of KLS remains unknown. Several treatments have been tried and recently lithium has been proposed for a prophylactic use in single cases. In view of the rarity of KLS, long-term results of lithium therapy have not been described yet. We report the clinical course of five adolescents with KLS who were treated with lithium. All patients showed significant EEG and polysomnographic changes during the episodes and had normal results in the interval. All patients had relapses while being treated with lithium. But episodes of hypersomnia under lithium therapy were shorter and monosymptomatic with lack of behavioural symptoms. Statistical modelling showed that the risk for a relapsing episode under maintenance of lithium drops per months of therapy from 100 % to 93 %, and furthermore that the maintenance of lithium shortens the mean duration of episodes to 19 %. No severe side effects were observed. In conclusion, in KLS with a high frequency of episodes and severe behavioural changes lithium may become a treatment option.

[BCG osteitis. A case report]. / Die BCG-Ostitis. Ein Fallbericht.

Report of a 8 month old girl with a BCG-Ostitis in the head of the left humerus. The diagnosis was ascertained by typical radiological, histological and immun-histochemical changes. Tuberculostatic therapy resulted in healing. The value of the BCG-vaccination remains beyond all question.

[Generalized herpesvirus infection with severe consumption coagulopathy in a newborn infant]. / Generalisierte Herpesvirusinfektion mit schwerer Verbrauchskoagulopathie bei einem Neugeborenen.

In a mature newborn the symptoms of a disseminated HSV infection were evident at the 6th day of life. Later on bleeding occurred as a result of severe consumption coagulopathy. During treatment with Acyclovir the bleeding situation was controlled by fibrinogen replacement. The infant survived and is under normal psychologic and motorical development now. The treatment result is taken for the good virostatic efficacy of Acyclovir. It inhibits the DNA polymerases and therefore the DNA replication within the herpes viruses selectively. This high degree of selectivity is caused by its selective penetration into the infected cells, its faster transformation by the viral thymidine kinase as well as by its stronger affinity for HSV coded polymerase in detail. The diagnosis had been confirmed by detection of herpes viruses within the blister fluid and cerebrospinal fluid as well as by serological findings.

University wellness program: the effectiveness of students as nutrition counselors.

The Whole Body Health Program at San Jose State University was designed for employees who were interested in improving their general nutritional intake and fitness. This university wellness program furnishes students in nutrition and other health-related fields with valuable training and experience while also providing university employees with a work-site wellness program. As part of the wellness program and under faculty supervision, nutrition students conduct the Dietary Assessment Program by interviewing, assessing, and counseling clients. To determine whether the program was effective in improving participants' dietary habits, 16 previous participants were contacted by telephone. Fourteen agreed to be reinterviewed, and new dietary data were collected. Both the original (pre-program) and current (post-program) 24-hour recalls were evaluated according to the Recommended Dietary Allowances (RDAs), polyunsaturated to saturated (P:S) fat ratio, and percent of energy from fat and from carbohydrate. Reported mean consumption of cholesterol decreased from pre- to post-program for all subjects (pre = 383 mg, post = 242 mg; p less than or equal to .05). Those originally consuming more than 300 mg achieved a greater decrease in reported cholesterol consumption (pre = 487 mg, post = 234 mg; p less than or equal to .01).(ABSTRACT TRUNCATED AT 250 WORDS)