Effect of various plasma expanders on blood group serological study results]

Plasma expanders can disturb in serological blood typing tests to varying amounts. In order to avoid possibly fatal errors in index reading, it is, therefore, necessary when demanding this type of examination to inform about any infusion of plasma expanders carried through before the blood test and to take it into consideration.     

New dosing strategies for antibacterial agents in the neonate

Dosing of antibiotics in neonates requires finding a delicate balance between maximal efficacy and minimal toxicity. There is a lack of data on efficacy of currently used antibiotics in neonates, and rational dosing therefore needs to be based on gestational- and postnatal-age-dependent pharmacokinetics in combination with surrogate markers. These surrogate markers are: (i) the area-under-the serum concentration time curve to minimum inhibitory concentration ratio (AUC/MIC); (ii) peak concentration to MIC ratio (Cmax/MIC); and (iii) the time the concentration remains above the MIC (T>MIC). Whereas the efficacy of beta-lactam antibiotics (including carbapenems) depends on T>MIC, the efficacy of most other antimicrobials (including aminoglycosides and fluoroquinolones) is related to AUC/MIC and Cmax/MIC. Most modern dosing regimens are adequate when these concentration effect relationships are taken into account. Dosing adjustments in neonates are suggested, based on these relationships. Several antimicrobial combinations for treatment of meningitis and necrotizing enterocolitis exist. Empiric treatment should be based on efficacy, concerns about resistance as well as information from institutional microbiological surveillance.     

Undiagnosed obstructive sleep apnea syndrome in children with syndromal craniofacial synostosis

Children with syndromal craniofacial synostosis have a high risk for obstructive sleep apnea syndrome. Early diagnosis and treatment can relieve symptoms and morbidity. Little is known about the development and natural history of obstructive sleep apnea syndrome through life. The aim of this study was to investigate our experience of clinical history and treatment modalities concerning obstructive sleep apnea syndrome from birth until the current age in children with syndromal craniofacial synostosis. Children with one of the three syndromal craniofacial synostoses (Apert, Crouzon, or Pfeiffer) born between 1984 and 2001 were evaluated. The medical history and symptoms of obstructive sleep apnea syndrome were assessed by retrospective analysis of the medical records. The present and past complaints were explored by means of a questionnaire. Retrospective analysis of the medical records showed a suspicion for obstructive sleep apnea syndrome in 26% of the children compared with 53% in the questionnaire. The severity and presentation of obstructive sleep apnea syndrome were not related to the age of the child. Obstructive sleep apnea syndrome symptoms occurred in almost half of the children during colds. Several symptoms were significantly more common in children with a high suspicion for obstructive sleep apnea syndrome. Treatment modalities consisted of adenotonsillectomies, continuous positive airway pressure, and Le Fort III surgery. Use of a standard questionnaire showed that the suspicion for obstructive sleep apnea syndrome in children with syndromal craniofacial synostosis is much higher than reported in the medical records. Regular screening for obstructive sleep apnea syndrome with a standard questionnaire could be of additional value for the detection of obstructive sleep apnea syndrome in children with syndromal craniofacial synostosis.     

Congenital disorder of glycosylation (CDG) type Ie. A new patient

CDG Ie is caused by a deficiency of dolichol-phosphate-mannose synthase 1 (DPM1), an enzyme involved in N-glycan assembly in the endoplasmic reticulum. Three proteins are known to be part of the synthase complex: DPM 1, DPM2 and DPM3. Only mutations in DPM1, the catalytic subunit, have been described in three families. One was homozygous for the c274C>G (R92G) mutation in DPM1 and two others were compound heterozygous for R92G and a c628delC deletion or a c331-343del13, respectively. Clinical features were a severe infantile encephalopathy, early intractable seizures, acquired microcephaly, and some dysmorphic features. We report a patient with milder symptoms: microcephaly, dysmorphic features, developmental delay, optic atrophy, and cerebellar dysfunction without cerebellar atrophy. The patient is homozygous for a new mutation in exon 9 of the DPM1 gene (c742T>C (S248P)). Our findings extend the spectrum of CDG Ie.     

Molecular adsorbents recirculating system dialysis for liver insufficiency and sepsis following right ventricular assist device after cardiac surgery

We report a case of right heart failure (RHF) and sepsis with liver insufficiency in a 70-year-old patient after coronary artery bypass graft surgery. Three hours after surgery the patient suddenly developed therapy refractory cardiac arrest caused by RHF. He had to have emergency surgery, under which the graft to the right coronary artery was revised and a right ventricular assist device was implanted. Heart function recovered and the assist device was explanted on day 1 after surgery. Thoracic closure was performed on day 5 after surgery. The patient went into septic shock on day 11. Liver dysfunction developed postoperatively and worsened the course of sepsis. Therefore, MARS (molecular adsorbents recirculating system) dialysis was performed once on day 20 after surgery. Liver function improved after MARS therapy and the patient recovered from sepsis. On day 46 the patient was transferred from the ICU of another hospital to one of the peripheral wards, to be finally discharged on day 67.     

Rapidly lethal progression of a therapy-resistant status epilepticus

We present a case of death after first manifestation of generalised convulsive status epilepticus in a young man. A previously healthy 23-year-old man was admitted to our emergency department by ambulance service with approximately 20 min of generalised convulsive seizures. First line treatment in the emergency ward with benzodiazepines failed. The patient was cardiopulmonary stable until, after more than 30 min of status epilepticus, he developed tachycardia and became bradypnoeic. Intubation and ventilation was performed and anticonvulsive treatment was escalated with thiopental. Fifteen minutes later he developed ventricular fibrillation. CPR was started. The patient became asystolic after 90 min CPR following the ILCOR (International Liaison Committee on Resuscitation) Instructions. CPR was continued for another 30 min without success. The patient died after 120 min of maximal efforts. Autopsy and toxicology were performed, neuropathologic examination showed general brain edema and neuronal cell loss in purkinje cell layers of the cerebellum and olive knots which may be the consequence of generalised convulsive status epilepticus. We conclude: status epilepticus becomes refractory in approximately 30 % of cases. Until now, there are no randomised trials on the optimal treatment of refractory status epilepticus. Better treatment algorithms are urgently needed.     

Cerebral white matter lesions and the risk of dementia

OBJECTIVE: To study the association between white matter lesions (WML) in specific locations and the risk of dementia. DESIGN: The Rotterdam Scan Study, a prospective population-based cohort study. We scored periventricular and subcortical WML on magnetic resonance imaging and observed participants until January 2002 for incident dementia. SETTING: General population. PARTICIPANTS: We included 1077 people aged 60 to 90 years who did not have dementia at baseline. MAIN OUTCOME MEASURE: Incident dementia by Diagnostic and Statistical Manual of Mental Disorders, Third Edition (DSM III-R) criteria. RESULTS: During a mean follow-up of 5.2 years, 45 participants developed dementia. Higher severity of periventricular WML increased the risk of dementia, whereas the association between subcortical WML and dementia was less prominent. The adjusted hazard ratio of dementia for each standard deviation increase in periventricular WML severity was 1.67 (95% confidence interval, 1.25-2.24). This increased risk was independent of other risk factors for dementia and partly independent of other structural brain changes on magnetic resonance imaging. CONCLUSION: White matter lesions, especially in the periventricular region, increase the risk of dementia in elderly people.