Effect of BH4 on blood phenylalanine and tyrosine variations in patients with phenylketonuria

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Pediatric Differentiated Thyroid Carcinoma in The Netherlands: A Nationwide Follow-Up Study

Introduction: Treatment for differentiated thyroid carcinoma (DTC) in pediatric patients is based mainly on evidence from adult series due to lack of data from pediatric cohorts. Our objective was to evaluate presentation, treatment-related complications, and long-term outcome in patients with pediatric DTC in the Netherlands. Patients and methods: In this nationwide study, presentation, complications and outcome of patients with pediatric DTC (age at diagnosis ≤18 years) treated in the Netherlands between 1970 and 2013 were assessed using medical records. Results: We identified 170 patients. Overall survival was 99.4% after median follow-up of 13.5 (range 0.3–44.7) years. Extensive follow-up data were available for 105 patients (83.8% women), treated in 39 hospitals. Median age at diagnosis was 15.6 (range 5.8–18.9) years. At initial diagnosis, 43.8% of the patients had cervical lymph node metastases; 13.3% had distant metastases. All patients underwent total thyroidectomy. Radioiodine was administered to 97.1%, with a median cumulative activity of 5.66 (range 0.74–35.15) GBq. Lifelong postoperative complications (permanent hypoparathyroidism and/or recurrent laryngeal nerve injury) were present in 32.4% of the patients. At last known follow-up, 8.6% of the patients had persistent disease and 7.6% experienced a recurrence. TSH suppression was not associated with recurrences (OR 2.00, 95% CI 0.78 to 5.17, P = 0.152). Conclusions: Survival of pediatric DTC is excellent. Therefore, minimizing treatment-related morbidity takes major priority. Our study shows a frequent occurrence of lifelong postoperative complications. Adverse effects may be reduced by centralization of care, which is crucial for children with DTC

Neurological soft signs discriminating mood disorders from first episode schizophrenia

Objective: To investigate the specificity of neurological soft signs (NSS) for first episode schizophrenia compared with mood disorders. Method: We assessed NSS in a sample of 60 healthy controls, 191 first episode psychosis patients and 81 mood disorder patients. We used a principle component analysis to identify dimensions of NSS. We subsequently investigated the specificity of these dimensions for schizophrenia and their relationships with medication and symptom scores. Results: We identified five dimensions; coordination disorders, movement disorders, increased reflexes, dyskinesia and catatonia. These dimensions were related to neural circuits associated with schizophrenia and mood disorders and included the fronto-striatal-thalamic and the fronto-cerebellar pathway. The movement disorder dimension, which was suggestive for the involvement of the fronto-striatal-thalamic pathway, was specific for first episode schizophrenia independent from medication. Conclusion: NSS are the result of circuitry dysfunctions rather than overall dysfunction and a particular set of NSS shows specificity for schizophrenia

Neurological soft signs discriminating mood disorders from first episode schizophrenia

Objective: To investigate the specificity of neurological soft signs (NSS) for first episode schizophrenia compared with mood disorders. Method: We assessed NSS in a sample of 60 healthy controls, 191 first episode psychosis patients and 81 mood disorder patients. We used a principle component analysis to identify dimensions of NSS. We subsequently investigated the specificity of these dimensions for schizophrenia and their relationships with medication and symptom scores. Results: We identified five dimensions; coordination disorders, movement disorders, increased reflexes, dyskinesia and catatonia. These dimensions were related to neural circuits associated with schizophrenia and mood disorders and included the fronto-striatal-thalamic and the fronto-cerebellar pathway. The movement disorder dimension, which was suggestive for the involvement of the fronto-striatal-thalamic pathway, was specific for first episode schizophrenia independent from medication. Conclusion: NSS are the result of circuitry dysfunctions rather than overall dysfunction and a particular set of NSS shows specificity for schizophrenia

The association between atherosclerotic risk factors and renal function in the general population

The association between atherosclerotic risk factors and renal function in the general population.BackgroundGeneralized atherosclerosis is increasingly recognized as an important cause of end-stage renal disease (ESRD). We questioned to what extent atherosclerotic risk factors determine renal function in the general population.MethodsWe used baseline data of the Prevention of Renal and Vascular End-Stage Disease (PREVEND) Study. A total of 8592 subjects, aged 28 to 75 years old, visited the outpatient clinic for blood pressure and anthropometric measurements, fasting blood sampling, and delivery of two 24-hour urine collections for creatinine clearance and albuminuria calculations. Design-based multivariate linear regression was used to estimate renal function.ResultsIn a multivariate model, male gender and body mass index (BMI) were associated with a higher renal function, while increasing diastolic blood pressure, serum triglycerides, use of antihypertensive or lipid-lowering medication were associated with a lower renal function. Age, systolic blood pressure, and plasma glucose showed an inverse U-shaped relationship with renal function. Cholesterol/high-density lipoprotein (HDL) ratio, smoking, and antidiabetic medication did not contribute to explain renal function. The atherosclerotic risk factors were related to renal function independently of albuminuria or C-reactive protein (CRP). Albuminuria and CRP itself were also related to renal function. Following gender and age, BMI, urinary albumin excretion (UAE), and plasma glucose had the strongest relation with renal function.ConclusionWe conclude that differences in renal function in the general population are (partly) explained by various atherosclerotic risk factors. Some risk factors are associated with elevated filtration, some with an impaired filtration, and others with both a higher and a lower renal function

C-reactive protein modifies the relationship between blood pressure and microalbuminuria

C-reactive protein (CRP) and microalbuminuria reflect intimately related components of the atherosclerotic disease process. Epidemiological studies found only modest associations between CRP and microalbuminuria. Blood pressure, one of the components of the metabolic syndrome in the general population, is the main determinant of microalbuminuria in diabetes and hypertension. We questioned whether CRP modifies the relationship of blood pressure and other cardiovascular risk factors with microalbuminuria in a cross-sectional study in 8592 inhabitants from Groningen, The Netherlands. The crude data showed an increase in the prevalence of microalbuminuria with increasing CRP quartiles (4.8, 9.6, 14.5, and 18.6%,