Developmental dysplasia of the hip in children with Down syndrome: comparison of clinical and radiological examinations in a local cohort.

Guidelines for children with Down syndrome (DS) suggest to perform an annual hip screening to enable early detection of developmental dysplasia of the hip (DDH). How to perform this screening is not described. Delayed detection can result in disabling osteoarthritis of the hip.

Urine flow cytometry is an adequate screening tool for urinary tract infections in children.

Diagnosing a urinary tract infection in children is often difficult due to non-specific symptoms and requires invasive and time-consuming procedures. Flow cytometry is a new and rapid method of analyzing urine to confirm or exclude UTIs. We have investigated the sensitivity and specificity of urine flow cytometry (Sysmex UF1000i) compared to conventional diagnostic techniques in a prospective study from January 1, 2014 until January 1, 2015.

Brain imaging can predict neurodevelopmental outcome of Group B streptococcal meningitis in neonates.

Aim: The association between cranial ultrasound (CUS) or magnetic resonance imaging (MRI) lesions and neonatal Group B streptococcal (GBS) meningitis outcome has not been studied in detail.

Methods: This retrospective study assessed CUS, cranial MRI and neurodevelopmental outcome in 50 neonates with GBS meningitis admitted to three neonatal intensive care units in the Netherlands between 1992 and 2014. Death, cognitive outcome and motor outcome below -1 SD were considered as adverse outcomes.

Pyridoxine dependent epilepsy: Is late onset a predictor for favorable outcome?

Aim: In pyridoxine dependent epilepsy (PDE), patients usually present with neonatal seizures. A small subgroup is characterized by late-onset beyond 2 months of age. We aim to analyze the observation of relatively good cognitive outcome in this subgroup of late-onset PDE patients.

Parry Romberg syndrome presenting with a giant intracranial aneurysm: a case report.

A giant intracranial aneurysm was diagnosed in a 10-year-old girl when she developed a right abducens nerve palsy. The aneurysm was treated successfully. Six years later, however, she presented with a progressive deformity, leading to the diagnosis Parry Romberg Syndrome (PRS), a rare diagnosis characterized by hemifacial atrophy of skin, subcutaneous tissue, skeletal muscle and bones and often associated with various non-specific intracerebral abnormalities.

Prevalence of systemic air-embolism after prolonged cardiopulmonary resuscitation in newborns: A pilot study.

Background: Chest compressions (CC) during cardiopulmonary resuscitation (CPR) are the cornerstone of adult CPR protocols and are meant to restore circulation and improve outcome. Although adverse effects such as air-embolisms have been reported, these are rare and considered to be outweighed by beneficial effect. In newborns, however, the lung tissue is more fragile.

Subsequent Pregnancy after Preterm Prelabor Rupture of Membranes before 27 Weeks' Gestation.

Objective Midtrimester preterm prelabor rupture of membranes (PPROM) has a high rate of neonatal mortality and morbidity. The aim of this study was to study outcomes of subsequent pregnancies after a pregnancy with PPROM before 27 weeks' gestation. Study Design Retrospective study of subsequent pregnancies of women who suffered PPROM before 27 weeks' gestation from 1994 to 2009.

Acute neonatal presentation of a lymphatic malformation.

Oropharyngeal lymphatic malformations usually present with a mass either at birth or in the first 2 years of life. Rarely, lymphatic malformations present with extremely progressive respiratory problems shortly after birth, and usually occur in cases which have remained undetected in the absence of antenatal ultrasound. We report the case of a newborn that required tracheostomy and gastrostomy due to a rapidly expansive lymphatic malformation.

Outcome of pregnancies with preterm prelabor rupture of membranes before 27 weeks' gestation: a retrospective cohort study.

Objective: Preterm prelabor rupture of membranes (PPROM) before 27 weeks' gestation is associated with severe perinatal complications, but quantitative estimates are lacking. The aim of this study was to report and predict outcomes of pregnancies complicated by early PPROM and to study antepartum risk factors that might predict perinatal death in future patients.

Study Design: We performed a retrospective cohort study of women with PPROM between 13(+0) weeks and 27(+0) weeks' gestation between 1994 and 2009 in three perinatal centers.

Value of an automatic external defibrillator printout as a diagnostic tool after successful AED use on a child.

A 6-year-old girl without any medical history experienced a drowning incident for a duration of 2 min, according to witnesses. This was followed by cardiopulmonary resuscitation, during which the automatic external defibrillator (AED) detected a shockable rhythm and subsequently delivered a single electroshock. At the time of admission, her medical history was unclear, and as her chest had been wet, it was not clear if the AED had been capable of correctly analysing the rhythm.

Long-term outcome in pyridoxine-dependent epilepsy.

Aim: The long-term outcome of the Dutch pyridoxine-dependent epilepsy cohort and correlations between patient characteristics and follow-up data were retrospectively studied.

Method: Fourteen patients recruited from a national reference laboratory were included (four males, 10 females, from 11 families; median age at assessment 6y; range 2y 6mo-16y). The following data were retrieved: sex; age at seizure onset; age at the start of pyridoxine therapy; level of urinary alpha-aminoadipic semialdehyde; antiquitin mutations; developmental milestones; evaluation of neurocognitive functioning and school career; magnetic resonance imaging (MRI) and electroencephalography (EEG) assessments.

Development of children with symptomatic intracranial hemorrhage born after vacuum extraction.

Vacuum extraction significantly reduces perinatal morbidity/mortality. Increased occurrence of intracranial hemorrhage has been associated with vacuum extraction and is multifactorial; a causative effect is not assumed. Long-term developmental outcome data in this specific subpopulation are lacking and may differ from non-vacuum extraction-associated intracranial hemorrhage.

Artefacts in the amplitude-integrated EEG background pattern of a full-term asphyxiated neonate caused by diaphragm spasms.

The amplitude-integrated EEG (aEEG) is used to detect neonatal seizures in neonates with asphyxia, and despite limited data-information compared with standard EEG, aEEG is increasingly used as routine monitoring in neonatal intensive care units due to the logistic advantages. In addition, the aEEG background is of prognostic value in these infants. However, aEEG artefacts can lead to an erroneous interpretation of the background pattern.

Spontaneous perinatal epidural haemorrhage in a newborn.

A full-term neonate, born by caesarean section, presents with focal seizures. EEG and cranial ultrasound are normal. MRI of the cerebrum shows an epidural haematoma.

Septic arthritis and osteomyelitis in a 10-year-old boy, caused by Fusobacterium nucleatum, diagnosed with PCR/16S ribosomal bacterial DNA amplification.

A 10-year-old boy presented with an atypical non-febrile septic arthritis/osteomyelitis. He was unresponsive to routine antibiotic treatment with flucloxacillin/gentamicin as the pain and fluid collection increased. Synovial fluid cultures are negative and gram stain remained negative.

[Procedural sedation with propofol in non-painful interventions in children].

Unlabelled: Propofol is the sedative of choice in our hospital for all procedural sedations in children older than 3 months. Data were collected from all patients who underwent PSA with propofol in the period from November 2007 to December 2009. The procedure was performed by a paediatrician experienced in airway management, sedation and paediatric IC, and a specialized nurse.

[A girl with rapidly progressive ataxia].

A 4-year-old girl came to the emergency department with a headache since 4 weeks and ataxia since 1 day. Before she had had stomatitis. The girl tested positive for coxsackievirus.

Acquired progressive hypotonia in infancy: consider compressive cervical myelopathy.

Unlabelled: This case report presents a rare cause of progressive hypotonia due to a congenital bony defect of the atlas in a 2-month-old girl. The patient was initially referred to the paediatric department with feeding problems. Within days after admission she developed progressive hypotonia and showed decreased reflexes of the upper extremities.

Roth spots in pyridoxine dependent epilepsy.

Pyridoxine dependent epilepsy (PDE) is a rare metabolic defect in the degradation of lysine. The authors report a patient with metabolic and DNA confirmed PDE, on the fifth day of life ophthalmoscopy showed bilateral multiple white centred retinal haemorrhages, so called Roth spots. Roth spots are non-specific haemorrhagic signs that occur in a variety of conditions of acute systemic insults in homeostasis - most often infections- which relate to retinal capillary damage and the ensuing reparative process.

Group-A-streptococcal meningitis in a 7-year-old child--a rare pathogen in a non-immune compromised patient.

A case is presented of meningitis in a 7-year-old female child caused by Group A streptococcus (GAS), a rare bacterial cause of meningitis, with a high rate of morbidity (46%) and mortality (10%). GAS is susceptible for empiric antibiotic therapy aimed at the most prevalent pathogens of meningitis. As GAS meningitis is typically associated with ear-nose-throat (ENT) infections, specific search for a reservoir is advised.

Group-A-streptococcal meningitis in a 7-year-old child--a rare pathogen in a non-immune compromised patient.

A case is presented of meningitis in a 7-year-old female child caused by Group A streptococcus (GAS), a rare bacterial cause of meningitis, with a high rate of morbidity (46%) and mortality (10%). GAS is susceptible for empiric antibiotic therapy aimed at the most prevalent pathogens of meningitis. As GAS meningitis is typically associated with ear-nose-throat (ENT) infections, specific search for a reservoir is advised.

A child with a abnormal neck posture after doing a head-over-heels.

Torticollis in children is a frequently encountered problem. Whereas non-traumatic torticollis usually allows a non-urgent work-up, traumatic torticollis-even in the absence of neurologic abnormalities-needs prompt analysis for structural damage of the spinal column and myelum. Even a trauma as mild as a head-over-heels may result in atlanto-axial subluxation as demonstrated by this patient as described in this case report.