Treatment of a Large Skull Defect and Brain Herniation in a Newborn With Adams-Oliver Syndrome.

Adams-Oliver syndrome (AOS) is a rare congenital disorder characterised by a wide variety of clinical expression ranging from the occurrence of aplasia cutis congenita (ACC), transverse limb defects, and cutis marmorata telangiectica to extensive lethal anomalies. In this article, we present the conservative and surgical management of a male newborn infant diagnosed with AOS. Surgical treatment included wound management, the removal of protruding brain, and treatment of cerebrospinal fluid (CSF) leakage.

Targeting Cyclooxygenase-2 in Pheochromocytoma and Paraganglioma: Focus on Genetic Background.

Cyclooxygenase 2 (COX-2) is a key enzyme of the tumorigenesis-inflammation interface and can be induced by hypoxia. A pseudohypoxic transcriptional signature characterizes pheochromocytomas and paragangliomas (PPGLs) of the cluster I, mainly represented by tumors with mutations in von Hippel-Lindau (), endothelial PAS domain-containing protein 1 (), or succinate dehydrogenase () subunit genes. The aim of this study was to investigate a possible association between underlying tumor driver mutations and COX-2 in PPGLs.

Increased hypothalamic serotonin turnover in inflammation-induced anorexia.

Background: Anorexia can occur as a serious complication of disease. Increasing evidence suggests that inflammation plays a major role, along with a hypothalamic dysregulation characterized by locally elevated serotonin levels. The present study was undertaken to further explore the connections between peripheral inflammation, anorexia and hypothalamic serotonin metabolism and signaling pathways.

Genotype by environment interaction for production, somatic cell score, workability, and conformation traits in Dutch Holstein-Friesian cows between farms with or without grazing.

In this study, genotype by environment interaction was investigated for production traits, somatic cell score (SCS), workability traits, and conformation traits for Holstein-Friesian cows producing on farms with or without grazing in the Netherlands. Additionally, heritabilities and repeatabilities were estimated in both farm systems. Data were available for 1,019 Dutch farms, and farm type was known for those farms, 142 farms without grazing and 877 farms with grazing.

Pharmacokinetics of nifedipine slow-release during sustained tocolysis.

Objective: The pharmacokinetics of nifedipine as a tocolytic agent has not been studied in great detail in pregnant women and has instead focused on immediate release tablets and gastrointestinal therapeutic system (GITS) tablets. The aim of this study was to determine nifedipine slow-release half-life and distribution volume in pregnant women and to compare these with pharmacokinetic parameters of nifedipine in non-pregnant subjects described in the literature.

Materials: This is a study parallel to a trial studying women with threatened preterm labor between 26 + 0 and 32 + 2 weeks after initial tocolysis and a completed course of corticosteroids, who were randomly allocated to maintenance nifedipine (slow-release tablets 20 mg 4 times daily) or placebo.

Patient-specific implants compared with stored bone grafts for patients with interval cranioplasty.

Interval cranioplasty after craniectomy carries the risk of infection and failure. There is no consensus regarding the choice of reconstruction technique. In addition to the replacement of the stored autogenous bone graft, the use of patient-specific implants (PSIs) has become popular.

A treatment algorithm for patients with large skull bone defects and first results.

Large skull bone defects resulting from craniotomies due to cerebral insults, trauma or tumours create functional and aesthetic disturbances to the patient. The reconstruction of large osseous defects is still challenging. A treatment algorithm is presented based on the close interaction of radiologists, computer engineers and cranio-maxillofacial surgeons.

Recognition of impaired atomoxetine metabolism because of low CYP2D6 activity.

Ten out of 100 children treated for attention deficit hyperactivity disorder with standard doses of atomoxetine were selected by a neurologist for cytochrome P450 2D6 and cytochrome P450 2C19 genotyping, based on late response (>9 weeks) and adverse effects (gastrointestinal problems, sleeping disorders, malaise, inactivity, and mood instabilities). After genotyping, eight children were confirmed to have compromised cytochrome P450 2D6 activity because of at least one nonfunctional or less functional allele. Cytochrome P450 C19 is a minor pathway in atomoxetine metabolism and therefore of less importance.

Effects of the Ca2+ antagonist nimodipine on functional deficits in the peripheral and central nervous system of streptozotocin-diabetic rats.

Diabetes mellitus can lead to functional and structural deficits in both the peripheral and central nervous system. The pathogenesis of these deficits is multifactorial, probably involving, among others, microvascular dysfunction and alterations in intracellular calcium homeostasis. The present study examined the effects of treatment with the Ca2+ antagonist nimodipine (20 mg/kg, intraperitoneal injection, every 48 h) on functional deficits in the peripheral and central nervous system in streptozotocin-diabetic rats.

A 2-step strategy to reduce the need for methionine-loading tests to diagnose hyperhomocysteinemia.

An increased plasma homocysteine level may increase the risk of cardiovascular disease. The methionine-loading test is commonly used to detect additional subjects with hyperhomocysteinemia who have normal fasting levels of homocysteine but increased post-methionine-load levels. We developed a 2-step strategy to restrict the methionine-loading test to those subjects with intermediate fasting homocysteine levels to confirm the presence of hyperhomocysteinemia.

The potent melanocortin receptor agonist melanotan-II promotes peripheral nerve regeneration and has neuroprotective properties in the rat.

The neurotrophic and neuroprotective potential of the alpha-melanocyte-stimulating hormone (alpha-MSH) analog cyclo-[Ac-Nle(4),Asp(5),D-Phe(7),Lys(10)]alpha-MSH-(4-10) amide (melanotan-II), a potent melanocortin receptor agonist, was investigated. The sciatic nerve crush model was used as a paradigm to investigate the neurotrophic properties of melanotan-II. Melanotan-II significantly enhanced the recovery of sensory function following a crush lesion of the sciatic nerve in the rat at a dose of 20 microg kg(-1) per 48 h, s.

Neuronal Ca2+ disregulation in diabetes mellitus.

The Ca(2+) hypothesis of brain ageing and dementia may account for part of the available data on the pathogenesis of dementia and certain neurodegenerative disorders. The hypothesis proposes that disturbances in the homeostasis of neuronal cytosolic free Ca(2+) are part of a final common pathway, ultimately leading to neuronal dysfunction and cell death. The hypothesis also proposes that a small change in cytosolic free Ca(2+) sustained over a long period of time will result in similar damage as a large change over a short period.

Effects of enriched housing on functional recovery after spinal cord contusive injury in the adult rat.

To date, most research performed in the area of spinal cord injury focuses on treatments designed to either prevent spreading lesion (secondary injury) or to enhance outgrowth of long descending and ascending fiber tracts around or through the lesion. In the last decade, however, several authors have shown that it is possible to enhance locomotor function after spinal cord injury in both animals and patients using specific training paradigms. As a first step towards combining such training paradigms with pharmacotherapy, we evaluated recovery of function in adult rats sustaining a spinal cord contusion injury (MASCIS device, 12.

Scanning electron microscopic evaluation of nonocclusive excimer laser-assisted anastomosis in rabbits.

Background: The nonocclusive Excimer laser-assisted bypass technique has been described in previous studies and proved to be a promising bypass operation in vascular brain surgery. Little is known about the morphological regeneration process of the laser-assisted anastomosis in time. By way of a scanning electron microscopic study we examined the way in which the anastomosis site created by the nonocclusive Excimer laser-assisted anastomosis technique becomes endothelialized.

Clinical characteristics of type 1 diabetic patients with and without severe hypoglycemia.

Objective: To investigate the frequency of severe hypoglycemia (SH) and hypoglycemic coma and to identify clinical and behavioral risk indicators in a nonselected population of type 1 diabetic patients.

Research Design And Methods: This study involved a retrospective clinical survey of 195 consecutive patients using a questionnaire addressing the frequency of SH (i.e.

[Prevalence and treatment of hypercholesterolemia in patients with manifest vascular disease according to practice guidelines of the current cholesterol consensus].

Objective: To determine the prevalence of hypercholesterolaemia and the use of lipid-lowering medication in patients with a manifest vascular disease.

Design: Prospective, cross-sectional.

Methods: In patients who visited the University Medical Centre Utrecht, the Netherlands, for the first time with a manifestation of atherosclerosis in the period 1 September 1996-15 November 1998, we determined by a single measurement of the cholesterol if they were eligible for lipid-lowering medication according to the cholesterol cut-off value mentioned in the new Dutch cholesterol guidelines (1998) of the Dutch Institute for Health Care Improvement.

rhGGF2 protects against cisplatin-induced neuropathy in the rat.

In many patients treated with cisplatin a peripheral sensory neuropathy develops. This side-effect is considered dose-limiting, and therefore restricts the total dose of cisplatin that can be administered. Recent in vitro and in vivo studies suggest that recombinant human Glial Growth Factor 2 (rhGGF2) has neuroprotective effects.

Combined treatment with alphaMSH and methylprednisolone fails to improve functional recovery after spinal injury in the rat.

To date, relatively little progress has been made in the treatment of spinal cord injury (SCI)-related neurological impairments. Until now, methylprednisolone (MP) is the only agent with clinically proven beneficial effect on functional outcome after SCI. Although the mechanism of action is not completely clear, experimental data point to protection against membrane peroxidation and edema reduction.

[Diabetic ketoacidosis presenting as acute abdomen].

Three patients, two women aged 21 and 67 and a man aged 43 years, presented at the emergency department with diabetic ketoacidosis and abdominal symptoms mimicking an acute abdominal condition. In two of them laparotomy was performed which proved to be negative. Abdominal symptoms resolved after correction of metabolic, fluid and electrolyte disturbances.

Second manifestations of ARTerial disease (SMART) study: rationale and design.

The Second Manifestations of ARTerial disease (SMART) study is a single-centre prospective cohort study among patients, newly referred to the hospital with (1) clinically manifest atherosclerotic vessel disease, or (2) marked risk factors for atherosclerosis. The first objectives of the SMART study are to determine the prevalence of concomitant arterial disease at other sites, and risk factors in patients presenting with a manifestation of arterial disease or vascular risk factor and to study the incidence of future cardiovascular events and its predictors in these high-risk patients. At least 1000 patients, aged 18 to 80 years, will undergo baseline examinations, including a questionnaire on cardiovascular disease, height, weight and blood pressure measurements, blood tests for glucose, lipids, creatinine and homocysteine, urinary tests for microproteinuria, resting twelve-lead electrocardiogram, ultrasound scanning of the abdominal aorta, kidneys and the carotid arteries, measurements of common carotid intima-media thickness and arterial stiffness, and a treadmill test to assess atherosclerosis of the leg arteries.

Functional recovery after central infusion of alpha-melanocyte-stimulating hormone in rats with spinal cord contusion injury.

Melanocortins, peptides related to alpha-melanocortin-stimulating hormone (alpha MSH) and adrenocorticotropic hormone (ACTH), are known to improve axonal regeneration following peripheral nerve injury and stimulate neurite outgrowth from central nervous system (CNS) neurons both in vitro and in vivo. The neurite outgrowth promoting capacity of alpha MSH has prompted us to investigate the effects of intrathecal application of alpha MSH on functional and electrophysiological recovery in a well-characterized model of spinal cord contusion injury. Different doses of alpha MSH were applied via osmotic minipumps into the cisterna magna for 10 days, thereby delivering the peptide directly into the CNS.

A descriptive study of visits by animal health specialists in pig farming: type, frequency, and herd-health management factors.

This research was carried out to analyse the visits specialists of the Dutch Animal Health Service made to growing and fattening pig farms. The type and frequency of the visits and identified herd-health management factors that did not meet accepted standards were investigated. In total 373 visit reports were studied.