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. Therefore, we determined the association between clinical history, physical, and radiological examination in diagnosing DDH in children with DS. Referral centers for children with DS were interviewed to explore variety of hip examination throughout the Netherlands. Clinical features of 96 outclinic children were retrospectively collected. Clinical history was taken, physical examination was performed, and X-ray of the hip was analyzed. All the referral centers performed physical examination and clinical history; however, 20% performed X-ray. Following physical examination according to Galeazzi test 26.9% and to limited abduction 10.8% of the outclinic-studied children were at risk for DDH. Radiological examination showed moderate or severe abnormal deviating migration rate of 14.6% resp. 11.5% in the right and left hip. However, no association between clinical history, physical examination, and radiological examination was found.Conclusion: Clinical history and physical examination are insufficient to timely detect DDH in children with Down syndrome. Thereby regular radiological examination of the hip is advised. What is Known: • Developmental dysplasia of the hip (DDH) in people with Down syndrome (DS) develops during childhood. • Guidelines for medical support of children with DS suggest an annual hip screening to enable early detection of hip damaging. How to perform this annual screening is not described. What is New: • This study shows no association between clinical history, physical and radiological examination of the hip. • We recommend regular radiological examination of the hip in children with DS in order to identify DDH early up to 16 years of age.

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. Thus, the high intra-thoracic pressures resulting from CC may make the newborns more vulnerable for air-embolisms. OBJECTIVES: We studied the postmortem prevalence of air-embolism in neonates that have received CPR. METHODS: Prospective cohort analysis of newborns receiving CC during CPR. CPR was performed by trained staff according to ILCOR guidelines, in a tertiary hospital. Air-embolisms were sought after using CT/MRI and autopsy. RESULTS: During a 61/2 year period (2007-2014), n = 56 newborns received CC. Newborns were resuscitated following severe perinatal hypoxia, or due to complications during NICU treatment. In n = 14 (25.0%) circulation could not be restored (mean CPR duration: 32.7 ± 15.2 min). Post-mortem CT/MRI was performed in n = 9, of whom n = 8 (88.9%) had air-embolisms. Autopsy was performed in n = 9. The air-embolisms could not be retraced on autopsy except for n = 1 patient. In patients with CPR resulting in restored circulation (n = 42), no CT or MRI was performed for comparison due to radiation and/or hemodynamic instability. Cerebral ultrasound could not identify or exclude air-embolisms in this subgroup. CONCLUSIONS: Post-mortem CT after prolonged resuscitation showed a high prevalence of intravascular air-embolism. Autopsy was not suited to detect air-embolism. The clinical importance of air-embolisms on the lethal outcome needs further research.

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. Long-term follow-up of children with symptomatic vacuum extraction-associated intracranial hemorrhage was retrospectively analyzed using Bayley Scales of Infant Development. Twenty-five newborns were identified with symptomatic intracranial hemorrhage after vacuum extraction. Motor development was severely impaired in 4 children (16%, Bayley Scale score <55), moderately impaired in 5 children (20%, Bayley Scale score 55-69) and mildly impaired in 2 children (8%, Bayley Scale score 70-84). Mental development was severely impaired in 2 children (8%), moderately impaired in 3 children (12%) and mildly impaired in 5 children (20%). Impaired outcome was associated with parenchymal injury and seems to be a higher reported outcome in non-vacuum extraction-associated intracranial hemorrhage. The high prevalence of impaired development in symptomatic vacuum extraction-associated intracranial hemorrhage necessitates long-term follow-up.

[Procedural sedation with propofol in non-painful interventions in children]. / Sedatie met propofol voor niet-pijnlijke procedures bij kinderen.

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. Patient characteristics, American Society of Anesthesiologists (ASA) classification, vital parameters and propofol dosage were registered on specially designed forms. Patient data were analyzed and compared with data from a non-matched historical cohort of patients who in the past had undergone PSA with chloral hydrate. RESULTS: 204 procedural sedations with intravenous propofol were performed in 196 patients. The mean cumulative induction dose was 3.39 mg/kg (SD: 1.34) and the mean maintenance dose was 4.05 mg/kg/h (SD: 2.23). The success rate was 99.5%, compared to 88.6% in the cohort that had received PSA with chloral hydrate. 1 procedure was aborted because of desaturation due to an obstructed airway, for which a jaw thrust was performed. No complications were observed in 199 procedures (97.5%). In 4 procedures a mild and transient desaturation (85-89%) occurred. CONCLUSION: The results suggest that propofol can be used safely and is effective for procedural sedation in selected children, provided that PSA is performed by experienced and trained staff.

Risk factors for the deterioration of oxygenation ratio in ventilated intensive care unit patients: a retrospective cohort study.

PURPOSE: The aim of the study is to determine which factors are associated with the deterioration of Pao(2)/fraction of inspired oxygen (Fio(2)) ratio in patients with normal oxygenation at admission and ventilated according to a lung protective ventilation strategy. MATERIALS AND METHODS: Retrospective cohort study of ventilated (>/=3 days) intensive care unit patients with an admission Pao(2)/Fio(2) ratio of 300 mm Hg or higher (n = 105). Patients who developed lung injury (Pao(2)/Fio(2) ratio, <300 mm Hg) on day 7 (n = 37) were compared to those who did not (n = 68), with regard to ventilator settings, gas exchange variables, and lung injury risk factors. RESULTS: Mean +/- SD of administered tidal volume was 7.9 +/- 1.3 mL/kg. Patients who developed lung injury were older (P = .019), had lower Pao(2) (P = .009), higher Paco(2) (P = .045), and lower Pao(2)/Fio(2) ratio (P = .002) at admission. Postoperative state (Hazard risk [HR], 5.1) and controlled ventilation mode (HR, 4.3) were identified as independent risk factors. Lung injury-free time was shorter in patients with low initial Pao(2)/Fio(2) ratio (odds ratio, 1.7; P = .039). This effect was not only caused by the baseline difference, as the decrease in Pao(2)/Fio(2) ratio was more pronounced in patients who developed lung injury compared to those who did not (P = .008). CONCLUSIONS: Lung injury exacerbates during mechanical ventilation. In patients treated with a mean tidal volume of 7.9 mL/kg, controlled ventilation is a major risk factor.

A case of juvenile bullous pemphigoid--successful treatment with diaminodiphenylsulfone and prednisone.

Blistering skin diseases can be difficult to diagnose, particularly in children. Because of the wide variety of bullous disorders and the considerable clinical overlap between them, it is difficult to differentiate one from the other on clinical features alone. Appropriate additional investigations are required to confirm the diagnosis. These include routine histologic examination of the skin, in addition to immunohistochemical staining and immune serology. Here, we present a rare case of juvenile bullous pemphigoid, which we will use to illustrate the difficulties encountered in the diagnostic process and to show how acquired blistering disorders of childhood should be approached.

The oxygenation ratio during mechanical ventilation in children: the role of tidal volume and positive end-expiratory pressure.

OBJECTIVE: The objective of this study is to analyze the role of tidal volume (Vt) and positive end-expiratory pressure on the oxygenation ratio (OR) (Pao(2)/Fio(2)) during mechanical ventilation (MV) in children with a normal pulmonary gas exchange on admission. METHODS: A retrospective cohort study of children with an admission OR greater than 300 mm Hg and duration of MV greater than 48 hours (n = 96) was done. We analyzed Vt, Fio(2), Pao(2), and positive end-expiratory pressure and calculated Vt (mL/kg) and Pao(2)/Fio(2) based on the measured Vt and weight. Patients were divided into group 1, Vt less than 9 mL/kg (n = 24); 2, Vt 9 to 12 mL/kg (n = 58); and 3, Vt 12 mL/kg or higher (n = 14). RESULTS: Baseline characteristics and OR were comparable. Forty-one percent of patients developed OR less than 300 mm Hg. The proportion of patients developing an OR less than 300 mm Hg was lowest in group 1 and highest in group 3, and differences became more pronounced with longer MV duration: 56%, 58%, and 89% on day 5; 29%, 65%, and 100% on day 7 (P = .05); 0%, 40%, and 100% on day 10 (P = .03). In patients maintaining an OR greater than 300 mm Hg during 10 days of MV, Vt was 9.3 +/- 1.0 vs 12.7 +/- 4.8 mL/kg in patients developing an OR less than 300 mm Hg (P = .05). Mechanical ventilation duration was longer in children developing OR less than 300 mm Hg (P < .01). Positive end-expiratory pressure levels were not significantly different between groups. CONCLUSION: In ventilated children, Vt was greater than 9 mL/kg were associated with increased development of an OR less than 300 mm Hg and longer duration of MV.

Hypercapnic acidosis attenuates the pulmonary innate immune response in ventilated healthy mice.

BACKGROUND: Mechanical ventilation with small tidal volumes reduces the development of ventilator-induced lung injury and mortality, but may increase PaCO2. It is not clear whether the beneficial effect of a lung-protective strategy results from reduced ventilation pressures/tidal volumes or is mediated by the effects of hypercapnic acidosis on the inflammatory response involved in the pathogenesis of ventilator-induced lung injury. OBJECTIVE: To analyze whether hypercapnic acidosis affects lung tissue cytokine levels and leukocyte influx in healthy ventilated mice. STUDY DESIGN: Analysis of lung tissue and plasma concentrations of interleukin (IL)-1beta, tumor necrosis factor (TNF)-alpha, IL-6, IL-10, and keratocyte-derived chemokine after 2 hrs of mechanical ventilation (V(t) 8 mL/kg, positive end-expiratory pressure 4 cm H2O) with 0.06% CO2 (room air), 2% CO2, or 4% CO2. SUBJECTS: Healthy C57BL6 mice (n = 40). MEASUREMENTS/RESULTS: PaCO2 and pH were within normal range when ventilated with 0.06% CO2 and significantly changed with 2% and 4% CO2: (mean +/- SD) pH 7.23 +/- 0.06 and 7.15 +/- 0.04, PaCO2 7.9 +/- 1.4 and 10.8 +/- 0.7 kPa, respectively (p < 0.005). Blood pressure remained within normal limits in all animals. Quantitative microscopic analysis showed a 4.7 +/- 3.7-fold increase (p < 0.01) in pulmonary leukocyte influx in normocapnic ventilated animals and a significant reduction in leukocyte influx of 57 +/- 32% (p < 0.01) and 67 +/- 22% (p < 0.01) when ventilated with 2% and 4% CO2, respectively. Normocapnic ventilation induced a significant elevation of lung tissue IL-1beta (1516 +/- 119 ng/mL), TNF-alpha (344 +/- 88 ng/mL), IL-6 (6310 +/- 807 ng/mL), IL-10 (995 +/- 152 ng/mL), and keratocyte-derived chemokine (36,966 +/- 15,294 ng/mL) (all p-values <0.01). Hypercapnic acidosis with 2% respectively 4% CO2 significantly attenuated this increase with 25 +/- 32% and 54 +/- 32% (IL-1beta, p < 0.01); 17 +/- 36% and 58 +/- 33% (TNF-alpha, p < 0.02); 22 +/- 34% and 89 +/- 6% (IL-6, p < 0.01); 20 +/- 31% and 67 +/- 17% (IL-10, p < 0.01) and 16 +/- 44% and 45 +/- 30% (keratocyte-derived chemokine, p = 0.07). CONCLUSION: Hypercapnic acidosis attenuates the mechanical ventilation-induced immune response independent from reduced tidal volumes/pressures and may protect the lung from ventilator induced lung injury.

Cardiac diverticulum and omphalocele: Cantrell's pentalogy or syndrome.

Omphaloceles and left ventricular diverticulums are rare disorders. Although either is known to occur on its own, the combination is highly suggestive of the so-called pentalogy of Cantrell. This syndrome is a combination of deformities involving midline structures, with exteriorisation of the heart, or 'ectopia cordis', as the most severe malformation. A cause has yet to be identified, though genes located on the X-chromosome may be involved. We discuss a neonate who presented with an omphalocele and a palpable diverticulum of the left ventricle. An omphalocele, especially when above the umbilicus, is an indication for further investigation for deformities as seen in the spectrum of Cantrell's pentalogy, especially cardiac malformations and anterior diaphragmatic herniation. A left ventricular diverticulum is usually associated with Cantrell's syndrome. When found, it is usually accompanied by other intracardiac malformations, so that again further examination is indicated. In our patient, there was an atrial septal defect within the oval fossa, along with a ventricular septal defect and unobstructed albeit anomalous venous pulmonary return to the left atrium. Early surgical intervention seems to be indicated, as spontaneous rupture, arrhythmias, and thromobogenicity of the ventricular diverticulum have all been reported.