Revista Chilena de Pediatria-Chile最新文献

Pediatric cardiomyopathies are infrequent diseases of the cardiac muscle, with an annual inciden ce of 1.1 to 1.2 per 100,000 children. Dilated cardiomyopathy (DCM) is the predominant form, characterized by ventricular dilatation and systolic dysfunction. Etiologies are multiple, with at least 50%-70% of cases being idiopathic. When assessing a child with DCM, secondary potentially reversible causes must be ruled out. The main diagnostic tool is the echocardiogram which allows the identification of cardiac phenotype, to establish the degree of functional compromise, and res ponse to medical therapy. Prognosis is limited but more favorable in infants younger than 1 year at the onset, post myocarditis, or with a lesser degree of ventricular dysfunction. At least 20% of patients may recover ventricular function in the first 2 years after the onset and 40%-50% may die or need heart transplant in the first 5 years. Medical therapy is mainly based on adult experience with limited scientific evidence in children. Heart transplant is the therapy of choice in patients with end-stage disease, with excellent short- and medium-term survival. A significant proportion of patients may require stabilization on the waiting list, including the use of mechanical circulatory support as a bridge to transplantation. The purpose of this revision is to update the available infor mation on etiology, physiopathological mechanisms, prognostic factors, and management of DCM in children.

Introduction: The hypernatremic neonatal dehydration is a severe condition whose incidence has increased in recent years resulting in complications leading to the hospitalization of the newborn.

Objective: Describe the clinical and laboratory characteristics of term-newborns with Hypernatremic Dehy dration diagnosis.

Patients and method: Descriptive observational study of hospitalized term- newborns due to hypernatremic dehydration between a period from 2014 to 2016. Term newborns over 37 weeks with clinical signs of dehydration (dry mucous membranes, depressed fontanel, tear less crying, signs of the cutaneous pleat), and/or excessive weight loss greater than 7% and serum sodium greater than 145 mEq/L were included. Sociodemographic and biochemical variables were recorded for analysis.

Results: 43 neonates were included. 60.5 percent of their mothers were pri- miparous, 90 percent of neonates received exclusive breastfeeding, mothers reported breastfeeding problems in 76.7 percent. Incoming neonates reported weight loss compared to birth weight at 15.3% on average. 83.3% had public health insurance. 65.1% had dehydration clinical signs at entry and 83.5% transient neurological signs. The average sodium was 155 mEq/L at revenue. The sodium decrease in the first 24 hours of handling was 7.74 mEq/L (0.32mEq/L per hour). The correction of the hypernatremia was 55.8% by oral intake and 4 days hospital stay on average.

Conclusions: The feeding's problems came up in a (76%), primiparous mothers in an (88.4%). 90.6 percent of this population administered exclusive breastfeeding, results that can help to alert the health professional to timely identification, warning signs, and early post-discharge control and preventive measures.

Introduction: The prepubertal stage is a critical period of body fat development, in which leptin and insulin re sistance has been associated, however, there are few studies in normal-weight prepubescents. Ob jective: To assess the relationship between leptin and body composition and insulin resistance in a group of normal-weight prepubescents.

Patients and method: Analytical cross-sectional study with 128 healthy prepubescents of normal weight, aged between 6 and 10 years. Height, weight, body mass index (BMI), body fat percentage (BFP), waist circumference (WC), and hip circumference (HC) were measured. Plasma leptin (ng/mL) and insulin (mU/L) were evaluated by immunoassay and glycemia (mmol/L) by enzymatic method. HOMA-IR was calculated. A comparison study and correlation analysis by sex were performed.

Results: Females presented higher values than males of leptin (6.8 ± 5 vs 3.3 ± 3.7; p = 0.000), insulin (7.1 ± 4.5 vs 5.2 ± 2.5; p = 0.016), BFP (22.4 ± 4.3 vs 18.6 ± 3.9; p = 0.000), and HC (67 ± 5.7 vs 65.0 ± 4.5; p = 0.019), and a lower waist/hip ratio (0.84 ± 0.04 vs 0.88 ± 0.04; p = 0.000). Leptin correlations with anthropometric variables were significant in both sexes, with greater association in females. The association of HOMA-IR with leptin was similar in both sexes.

Conclusions: in normal-weight prepubescents aged between 6-10 years, there are sex differences in adiposity and leptin levels not associated with differences in BMI or insulin resistance. The greater association of leptin with adiposity in girls could be related to a high rate of adipogenesis induced by this hormone.

Stridor is an abnormal respiratory sound caused by obstruction or collapse of the laryngotracheal airway, either acutely or chronically. There are different causes, both congenital and acquired, that can produce shortness of breath which may be severe and potentially life-threatening. The clini cal diagnosis must be complemented with an endoscopic airway assessment and sometimes with imaging, to try to determine the areas involved and possible associated malformations. Treatment should be individualized, considering the patient's overall condition, stridor etiology, its impact on breathing and swallowing, prognosis, and technical capacity of the managing team, among others. Alternatives may include observation, non-pharmacological measures, local or systemic medications, endoscopic and open surgeries, or a temporary or long-term tracheostomy. A thorough understan ding of the pathophysiology and etiopathogenesis of persistent pediatric stridor is essential for the correct management of these complex patients, ideally in a multidisciplinary manner.

Introduction: Acute osteoarticular infections in children are rare pathologies, therefore early diagnosis and prompt treatment are crucial to avoid acute and long-term complications. Brodie's abscess (BA) is an un common type of subacute osteomyelitis, difficult to diagnose, so clinical suspicion is essential. Ob jective: To describe a case of Brodie's abscess and its etiological and clinical features.

Clinical case: A 14-year-old patient was seen at our clinic, who reported a one-month pain in the right thigh, with no history of fever or trauma. Physical examination revealed no volume increase, painful right hip range of motion, and increased sensitivity on superficial palpation of the right iliotibial band. X-rays where normal. Because of the pain persistence, an ultrasound was requested which showed a cortical irregularity. Magnetic resonance imaging (MRI) was performed and revealed a right femoral diaphysis, due to a possible bone tumor or an infectious process. Lab tests were normal. Biopsy and cultures were collected, identifying multi-sensitive Staphylococcus aureus. He was managed with debridement and intravenous antibiotics, responding positively.

Conclusions: The BA's clinical features and lab tests are unspecific, therefore the non-specialist physician should strongly suspect this pathology as a possible differential diagnosis in patients who persist with pain and present imaging alterations, even when there are no other symptoms or normal inflammatory parameters. A bone biopsy is essential for the differential diagnosis of tumor pathologies.

Introduction: Acute necrotizing encephalopathy of childhood (ANEC) is a rare disease characterized by alteration of consciousness and multiple symmetric brain lesions mainly involving the thalamus. It presents a high mortality rate and severe sequelae.

Objective: To describe a school-age patient with influenza A-related ANEC with favorable evolution.

Clinical case: Six-year-old boy with 3 days history of upper respiratory symptoms and fever (39 °C). One day previous to admission, he presented altered state of consciousness. A lumbar puncture was performed, showing a mild increase of protein level in CSF. MRI showed bilateral foci of symmetric restricted signal in the thalamus, mammillary bodies, periaqueductal gray, ventral tegmentum, hippocampus, and in both external capsules, which was compatible with ANEC. The patient received empirical treatment with methylprednisolone and oseltamivir. Subsequently, a positive result was received for influenza. Considering diagnosis and severity of illness, it was decided to administer immunoglobulin. The patient got better slowly but favorably. At discharge, he still was mildly bradypsychic with decreased visual acuity, spontaneous speech and walking with assistance. At 6 months of follow-up, the patient presented normal speech and gait, with persistent visual impairment in the right eye.

Conclusions: Our patient presented ANEC, whose timely diagnosis and management were associated with a favorable neurological evolution in the long term. Although ANEC is an infrequent pathology, it has very high morbidity and mortality rates, so it is very important to have a high degree of suspicion in order to request a targeted imaging study, search for related infectious causes, and start proper treatment.