Revista Chilena de Pediatria-Chile最新文献

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.

Craniosynostosis is defined as the premature fusion of one or more skull sutures, characterized by an abnormal shape of the head. It is a rare condition but should be recognized and timely referred to Neurosurgery in order to prevent complications. The objective of this review is to describe the most frequent clinical and genetic characteristics of this pathology, its classification according to the shape of the skull, and the most characteristic signs to achieve timely recognition. A search for scientific articles in Pubmed, Scielo, and EMBASE databases was performed using the terms craniosynostosis, plagiocephaly, scaphocephaly, and brachycephaly. We selected articles in Spanish and English that described the characteristics of the pathology and about its management, choosing systematic reviews or recommendations from scientific societies when available. Craniosynostosis may occur in isola tion or associated with other deformities. Its classification depends on the affected suture(s), leading to the characteristic shape of the skull and the presence of other malformations. This condition is usually diagnosed and referred late, which is associated with complications such as intracranial hy pertension and impaired brain development. Early surgery has less comorbidity and better esthetic results. In conclusion, the abnormal shape of the skull must raise the suspicion of craniosynostosis, even if it occurs in isolation. Surgical management before one year of life is associated with a better prognosis.

Introduction: Acute neonatal appendicitis is a rare pathology, with few reports in the last 30 years. Since its clinical presentation and imaging studies are non-specific, most cases are diagnosed during a surgical pro cedure.

Objective: To describe a neonatal case of acute perforated appendicitis associated with later appearance of inguinal hernia, with pre-surgical diagnosis and treated through laparoscopy.

Clinical case: A 17-day-old preterm female newborn presented with fever, irritability, and increased milk intolerance. Physical examination showed abdominal distention, tenderness and both, abdominal X- ray and ultrasound showed compatible images with acute perforated appendicitis. Once the diagnosis was confirmed, we performed an appendicectomy through laparoscopy. Two weeks later, during an outpatient visit, we observed a bilateral inguinal hernia which was confirmed by ultrasound, and then it was surgically corrected.

Conclusion: Acute appendicitis should be considered within the differential diagnosis in neonates with acute abdominal symptoms, mainly in premature infants. In this case, ultrasound scan allowed us to make the pre-operative diagnosis. The possible association with inguinal hernias should motivate to examine inguinal ducts during imaging assessment and surgical procedure.

Introduction: There is a close relationship between chronic kidney disease (CKD) and cardiovascular disease. One of its clinical manifestations is left ventricular hypertrophy (LVH), expressed as Left Ventricular Mass Index (LVMI gr/m27). In CKD patients with growth retardation, the LVMI calculation should be adjusted by correcting age for length/height.

Objective: To compare the age-corrected LVMI for length/height with the value calculated by chronological age in CKD children on dialysis.

Patients and method: Cross-sectional study. We analyzed echocardiographies of CKD children on dialysis aged between 1 and 18, from January 2016 to July 2017. LVMI was evaluated by adjusting the value expressed in gr/m27 to the percentile for the chronological child's age, and then the value was adjusted to the age-corrected length/height. We used descriptive statistics and concordance study for LVMI assessments calculating by chronological age and for age-corrected length/height.

Results: 26 patients were included and 75 echocardiograms. 56% had left ventricular hypertrophy using chronological age versus 46.6% age-corrected LVMI for length/height. When comparing the percentile groups of LVMI-chronological age vs. age-adjusted LVMI for actual length/height, it was observed that 18.6% of the sample changed percentile groups, 100% of them to a lower percentile group. The agreement evaluated based on the Kappa coefficient was 0.72 (perfect agreement > 0.8), confirming differences when adjusting the LVMI for age-corrected length/height.

Conclusion: Calculating LVMI by chro nological age overestimates the cardiovascular involvement in children with CKD who are charac teristically stunted. The results suggest that the age-adjusted, length/height-corrected calculation of LVMI gives greater accuracy to the diagnosis of left ventricular hypertrophy in this group of patients.

Introduction: The main role of Vitamin D is to regulate calcium metabolism, whose main source is vitamin D3 ob tained mostly from the action of ultraviolet (UV) light on the skin.

Objective: To evaluate the seaso nal differences in the concentrations of 25-hydroxy-vitamin D3 (25OHVitD3), parathyroid hormone (PTH), alkaline phosphatase (ALP), and calcium in school-age children.

Subjects and method: The concentrations of 25OHVitD3, PTH, ALP, and calcium were measured in children from Santiago, Chile (latitude -33.4372), aged 5 to 8 years, without Vitamin D supplementation, in different seasons of the year. VitD status was defined as sufficient with concentrations of 25OHVitD3 >20 ng/mL (50 nmol/L), insufficient 12-20 ng/mL (30-50 nmol/L) and deficient <12 ng/mL (30 nmol/L) based on the recommendations of the expert group of the "Global Consensus for the Prevention and Mana gement of Nutritional Rickets".

Results: 133 children participated (89 preterms under or equal to 32 weeks), 41 during summer, 28 in fall, 35 in winter, and 29 in spring. The difference of means between summer and winter was 9.6 ng/mL for 25OHVitD3 (p <0.0001), -11.1 pg/mL for PTH (p <0.0001), and -47.5 IU/mL for ALP (p= 0.01). There were no differences in calcium concentrations. In sum mer, 97.6% of the subjects were classified with sufficiency status (> 20 ng/mL), which decreased significantly in winter to 54.3% (p <0.0001).

Conclusions: In winter, 25OHVitD3 concentrations decreased in approximately half of the children, which was associated with an increase in PTH and ALP, and normal calcium concentrations. According to our results, children may need VitD supple mentation during fall and winter.

Introduction: Orbital myositis (OM) is a serious inflammation of extraocular muscles with unknown etiology. Pe diatric presentation is rare and often affects more than one individual in a family, suggesting a genetic predisposition.

Objective: To describe a pediatric case of orbital myositis, its clinical characteristics, and the usefulness of MRI for confirming the diagnosis.

Clinical case: A 13-year-old female patient presenting with acute headache, right periorbital pain, exacerbated by eye movements, and blurred vision. We ruled out thyrotoxic myopathy, infectious diseases, autoimmunity, and malignancy. An MRI showed right medial rectus muscle myositis and no evidence of optic neuritis. She was treated with intravenous systemic glucocorticoids followed by oral steroids with complete clinical resolution.

Conclusions: OM has unknown etiology and can present a malignant course. Due to its unspecific clinical presentation, a comprehensive differential diagnosis should be made and it should consider performing MRI. Early treatment avoids permanent damage of extraocular muscles.

Introduction: In the last decades, Chile has experienced a triple transition regarding demographic, nutritional, and economic issues.

Objective: To explore the relationship between childhood weight and two dimen sions of socioeconomic status, family income, and maternal educational level, analyzing the effect of each one by itself and how they operate together to determine childhood weight and comparing their effect on obese and non-obese children.

Subjects and method: Study based on data from the 2012 Encuesta Longitudinal de Primera Infancia (Early Childhood Longitudinal Survey), evaluating children between 2 and 6 years old (n=11,399). We estimated multivariate quantile regression models for the z-score of the body mass index (BMI-z).

Results: We found that in children aged 2-3 years, income and weight had a positive association, while maternal educational level and weight had a negative one. In children aged 4-6 years, income and weight were negatively associated among chil dren whose mothers have a higher educational level but positive among those with lower educational levels.

Conclusion: Family income and maternal educational level have opposite effects on childhood weight. The positive effect of income on BMI-z is diminished when mothers have high educational levels. We recommend studying the effects of income and education on child weight separately and exploring the causal mechanisms that explain the relations between socioeconomic determinants and childhood weight.