Pediatr Nephrol
November 2022
Background: Inborn errors of metabolism (IEM), including organic acidemias and urea cycle defects, are characterized by systemic accumulation of toxic metabolites with deleterious effect on the developing brain. While hemodialysis (HD) is most efficient in clearing IEM-induced metabolic toxins, data regarding its use during the neonatal period is scarce.
Methods: We retrospectively summarize our experience with HD in 20 neonates with IEM-induced metabolic intoxication (seven with maple syrup urine disease, 13 with primary hyperammonia), over a 16-year period, between 2004 and 2020.
H syndrome (OMIM 612391) is a recently described autosomal recessive genodermatosis characterized by indurated, hyperpigmented, and hypertrichotic skin and systemic manifestations including hepatosplenomegaly, cardiac anomalies, hearing loss, hypogonadism, low height, hypertriglyceridemia, hallux valgus, and flexion contractures. H syndrome results from mutations in the SLC29A3 gene, which encodes the human equilibrative nucleoside transporter hENT3. The cutaneous histopathology is characterized by a striking mononuclear cell infiltrate in the dermis consisting of CD68+ monocyte-derived cells and CD34+ and factor XIIIa+ dendrocytes.
View Article and Find Full Text PDFAnaerobic meningitis may occur alone, but is usually encountered as a complication of a brain abscess. In either case it is rare in a normal host. We present a 6-year old boy with anaerobic meningitis after missed penetrating trauma, stressing the need for a thorough investigation after head trauma.
View Article and Find Full Text PDFAlthough uncommon, mycotic aneurysms in infants can be lethal because of the high risk of rapid expansion and rupture. Most catheter-associated mycotic aneurysms reported in the first year of life develop following umbilical artery catheterizations. We describe the sonographic detection of an early stage mycotic aneurysm in a 4-month-old following femoral artery catheterization complicated by methicillin-resistant Staphylococcus aureus (MRSA) septicemia.
View Article and Find Full Text PDFAims: Brain abscess is rare in children. Predisposing factors are found in almost 85% of cases. Overall, 25% of brain abscesses develop in children, mostly in the 4-7 years age group.
View Article and Find Full Text PDFIn Israel, there have been increasing reports of Bordetella pertussis infection among adolescents and adults, but the peak incidence and highest mortality occur among infants. The authors report four cases involving the likely transmission of pertussis from parents to their offspring in two hospitals in Israel. The adoption of proper infection control measures and targeted screening of parents may reduce the potential risk for such transmission.
View Article and Find Full Text PDFPediatr Crit Care Med
July 2009
Objective: To report a rare case of an entrapped subclavian venous catheter and to describe an anatomically based maneuver to remove it.
Design: Case report.
Setting: Pediatric critical care unit in a tertiary care hospital.
Object: Deepening sedation is often needed in patients with intracranial hypertension. All widely used sedative and anesthetic agents (opioids, benzodiazepines, propofol, and barbiturates) decrease blood pressure and may therefore decrease cerebral perfusion pressure (CPP). Ketamine is a potent, safe, rapid-onset anesthetic agent that does not decrease blood pressure.
View Article and Find Full Text PDFObjective: To draw attention to a rare, life threatening complication of a rather common procedure, namely medullary injury following adenoidectomy and local anesthetic infiltration of the operative bed.
Design: Case report.
Setting: A tertiary pediatric critical care unit.
Background: In the western world, trauma is the leading cause of disability and mortality in the 1-39 years age group. Road accidents constitute the most frequent cause of mortality among children older than 1 year and falls from heights are the most frequent cause of injuries requiring hospitalization.
Objectives: To analyze the epidemiology and characteristics of severe pediatric trauma due to falls from a height in northern Israel.
A 5-week-old baby with coagulopathy due to hepatic mitochondrial disorder developed a bladder haematoma secondary to suprapubic urine aspiration. Unlike previously reported cases, the haematoma manifested itself only as concentric thickening of the bladder wall without an intraluminal component. The ultrasound and CT features are described.
View Article and Find Full Text PDFPediatr Hematol Oncol
September 2007
Anaplastic large cell lymphoma (ALCL) is a well-known entity, but there are no data on prognosis according to the age of the patient, especially in infants. A 2-month-old girl was admitted with a 2-week history of coughing, fever, and lymphadenopathy. Physical examination revealed mild respiratory distress, an erythematous macular rash on her trunk, massive cervical lymphadenopathy, splenomegaly, and very mild ascites.
View Article and Find Full Text PDFPurpose Of Review: The issue of mass casualty associated with terrorism has gained tremendous public interest, but only modest medical interest, in the past 2 years. This review examines recent medical literature and outlines a practical approach to managing the mass casualty situation on the basis of a doctrine developed and extensively practised in Israeli hospitals.
Recent Findings: A large portion of recently published articles deal with the preparedness for 'conventional', biological or chemical mass casualty situations.
We report 4 cases involving the likely transmission of pertussis from parents to newborns in a hospital setting. The adoption of proper infection control measures and targeted screening of parents may reduce the potential for such transmission.
View Article and Find Full Text PDFThe aim of this study is to report our experience with a child who developed extrapyramidal perturbations complicating acute organophosphate insecticides poisoning and to review the literature reporting on basal ganglia impairment associated with this poisoning. Our patient had developed overt parkinsonism presenting with a resting tremor, expressionless face, and lack of blinking along with marked cogwheel rigidity and a stooped, slow gait. He was alert, coherent, and cooperative, yet agitated.
View Article and Find Full Text PDFBackground: The use of sodium bicarbonate (SB) in cardiopulmonary resuscitation (CPR) is controversial. This study analyzes the effects of SB use on CPR outcome in the Brain Resuscitation Clinical Trial III (BRCT III), which was a multicenter randomized trial comparing high-dose to standard-dose epinephrine during CPR. Sodium bicarbonate use in BRCT III was optional.
View Article and Find Full Text PDFOBJECTIVE: To investigate the frequency, predisposing factors, clinical presentation, and outcome of abdominal compartment syndrome (ACS) in critically ill pediatric patients. DESIGN: A prospective study over a 5-yr period. SETTING: Pediatric intensive care unit of a tertiary care, university hospital.
View Article and Find Full Text PDFArch Orthop Trauma Surg
September 2002
The use of external fixation in the immobilization of diaphyseal and metaphyseal fractures in children is still controversial, as these fractures are generally managed by immediate plaster casting, by traction followed by casting, by various methods of internal fixation, including the recently developed flexible rods, and by plating. Between 1982 and 1998, we treated 64 children with fractures of the long bones of the lower limb using external fixation, 44 of whom were available for follow-up (46 fractures). Their average age on the day of injury was 8.
View Article and Find Full Text PDFThis study retrospectively analyzed the pattern of sodium bicarbonate (SB) use during cardiopulmonary resuscitation (CPR) in the Brain Resuscitation Clinical Trial III (BRCT III). BRCT III was a prospective clinical trial, which compared high-dose to standard-dose epinephrine during CPR. SB use was left optional in the study protocol.
View Article and Find Full Text PDFPediatr Nephrol
January 2001
Atypical hemolytic uremic syndrome (HUS) is a heterogeneous group of disorders, the pathogenesis of which is unclear. Plasma transfusions and plasmapheresis are widely used modes of therapy for adults with this life-threatening syndrome. There is very limited experience in using plasmapheresis therapy in children and infants with atypical HUS.
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