Andes pediatrica : revista Chilena de pediatria最新文献

In pediatrics, a process called Pediatric Inflammatory Multisystem Syndrome (PIMS) associated with recent infection by SARS-CoV-2 virus has been observed. One of its variants presents similarities with Kawasaki disease (KD).

Objective: to compare the clinical presentation, laboratory testing, and evolution of KD with PIMS Kawasaki phenotype (PIMS-KD) in patients hospitalized before the pandemic, compared with the pandemic period.

Patients and method: Cross-sectional study in two groups of patients at the Hospital Exequiel González Cortés: typical KD (group 1) and PIMS-KD (group 2). Data on demographic, clinical, and biochemical details were collected, as well as echocardiogram, treatment, and evolution records. IgG and IgM serology for SARS-CoV-2 was performed in both groups.

Results: In the KD group and the PIMS-KD group, 20 and 33 patients were analyzed, respectively. There were differences in age, days of fever, count of leukocytes, lymphocytes, and platelets, erythrocyte sedimentation rate (ESR), and hospital stay. In 25% of the KD group, there were alterations in the echocardiogram and, in the PIMS-K group, all patients received corticosteroids and 25 patients received intravenous immunoglobulin (IVIG). In both groups, a favorable clinical evolution was observed, characterized by the absence of complications and mortality.

Conclusions: Based on the data obtained in our study, the importance of the epidemiological link is emphasized as an essential factor in differentiating between both pathologies, highlighting the need to consider factors such as age, duration of fever, count of leukocytes, lymphocytes, and platelets, and degree of cardiac involvement, for a differential evaluation between patients with PIMS-KD versus KD.

Diabetic ketoacidosis (DKA) is one of the most serious complications of type 1 diabetes mellitus. Its treatment requires fluid and electrolyte replacement and insulin. Hypophosphatemia as a complication of treatment has been scarcely evaluated.

Objectives: To estimate the incidence of hypophosphatemia in children with DKA, treated with subcutaneous regular insulin (IRS), and to explore factors associated with this complication.

Patients and method: Prospective, observational study. Patients diagnosed with DKA hospitalized in the general care ward were included. Data on phosphatemia, glycemia, acid-base status, and IRS amount (U/kg) received were recorded at baseline and after 24 h of treatment. Hypophosphatemia was defined as values below 2.5 mg/dl. The correlation between initial phosphate and at 24 h of treatment was evaluated; the incidence of hypophosphatemia at 24 h was expressed as a percentage of the total number of patients.

Results: 30 patients were included, 15 were female, mean age 11.4 ± 3.2 years. At 24 h of treatment with IRS, 36.7% (95%CI 22-55%) presented hypophosphatemia, mean value 1.9 ± 1.5 mg/dl. Initial bicarbonate < 10 mmol/L acted as a predictor of hypophosphatemia (OR 7.5; 95%CI 1.4-39.8%; p = 0.01). No patient required intravenous phosphate correction, and no associated clinical complications were observed.

Conclusion: In the group studied, the incidence of hypophosphatemia reached 36.7% at 24 hours of treatment. Initial bicarbonate lower than 10 mmol/L was significantly associated with hypophosphatemia. No complications associated with hypophosphatemia were observed.

Alagille syndrome (ALGS) is an autosomal dominant, multisystem disorder that typically presents with cholestasis, cardiac, ocular, skeletal, vascular and renal abnormalities, and distinct facial features. Most cases are due to variants in the JAG1 gene, with only a small percentage involving a complete gene deletion.

Objective: to contribute to the phenotype delineation and interpretation of a microdeletion not previously described in the literature on chromosome 20.

Clinical case: A 4-month-old female patient was diagnosed with a heart murmur. An echocardiogram revealed pulmonary artery stenosis, which, combined with a prominent forehead observed on physical examination, determined her referral to clinical genetics. Because ALGS was suspected, complementary studies were performed, revealing butterfly vertebras and a genetic panel identified a pathogenic heterozygous deletion, encompassing the entire coding sequence of the JAG1 gene. To rule out a more extensive deletion, a chromosome microarray was performed, confirming a pathogenic microdeletion on chromosome 20 of 378 kb (arr[GRCh37] 20p12.2(10414643_10792802)x1).

Conclusions: A targeted sequencing panel followed by confirmation with a chromosome microarray allowed the identification and delineation of a pathogenic microdeletion not previously reported in the literature, including the complete JAG1 gene in a Chilean patient whose phenotype is consistent with ALGS.

Bacteremia is a major cause of morbidity and mortality in patients with cancer and episodes of high-risk febrile neutropenia (HRFN).

Objective: To identify the frequency of microorganisms isolated from blood cultures (BC) and their antimicrobial resistance (R) profile in children with HRFN, compared with the same data from previous studies of the same group.

Method: Prospective, multicenter, epidemiological surveillance study of microorganisms isolated from BC in patients under 18 years of age, from 7 PINDA network hospitals, between 2016 and 2021.

Results: 284 episodes of HRFN with positive BC were analyzed out of 1091 enrolled episodes (26%). Median age 7.2 years [3.0-12.3]. The main isolates were gram-negative bacilli (GNB) 49.2%, gram-positive cocci (GPC) 43.8%, and fungi 3.6%. The most frequently isolated microorganisms were viridans group Streptococci (VGS) (25.8%), Escherichia coli (19.8%), Pseudomonas spp. (11.2%), Klebsiella spp. (10.9%), and coagulase negative Staphylococci (CoNS) (10.9%). There was an increase in R to third-generation cephalosporins (p = 0.011) in GNB and to oxacillin in CoNS (p = 0.00), as well as a decrease in R to amikacin in non-fermenting GNB (p = 0.02) and to penicillin in VGS (p = 0.04).

Conclusion: VGS is the main agent isolated in BC from pediatric patients with cancer and episodes of HRFN, followed by E. coli, Pseudomonas spp., and Klebsiella spp. Having epidemiological surveillance of microorganisms isolated from BC and their antimicrobial R profile is essential to favor the rational use of antimicrobials.

Growth hormone (GH) is effective in improving height in several conditions.

Objective: To describe the evolution of a group of children who received GH in a tertiary center between 2012-2022.

Patients and method: Descriptive, retrospective study. We analyzed the impact on height after GH use with Z-score according to etiology, age at onset and bone age. Patients under 15 years old at baseline and receiving GH for at least 12 months, with diagnoses of GH deficiency (GHD), idiopathic short stature (ISS), small for gestational age (SGA), SHOX Haploinsufficiency (SHOX) and Turner syndrome (TS) were included. Height was expressed as Z-score for age and sex, according to NCHS curves.

Results: 145 children received GH. Sixty patients were excluded due to irregular administration, incomplete data, less than 12 months of GH, change of hospital, and associated comorbidities. Seventy-three patients were analyzed, 23 GHD, 15 ISS, 20 SGA, 9 SHOX and 6 TS patients. Significant improvement in height (Z-score for age and sex) was observed in SGA (1.4 ± 0.8 gain; p < 0.001), GHD (1.1 ± 1.0; p < 0.001), ISS (1.1 ± 0.8; p < 0.001) and SHOX (0.8 ± 0.7; p = 0.007) patients. In TS, a non-statistically significant improvement was observed (0.7 ± 0.8; p = 0.085). In GHD, onset before 3 years showed a gain of 1.9 ± 1.1, vs 0.7 ± 0.6 (p = 0.083) and in ISS onset with bone age less than 9 years increased it by 1.7 ± 0.5 vs 0.5 ± 0.5 (p < 0.001).

Adverse events: 27/73 (37%) headache, 18/73 (24%) lower extremity pain, 1/73 (1.5%) dizziness, 1/73 (1.5%) scoliosis, 1/73 (1.5%) epiphysiolysis and 1/73 (1.5%) craniopharyngioma recurrence.

Conclusions: Children with GHD, ISS, SHOX mutation and SGA significantly improved their height, highlighting in GHD and ISS the importance of early treatment. Treatment was well tolerated in the 5 groups analyzed.

Sepsis is one of the main causes of admission to Intensive Care Units (ICU). The hemodynamic objectives usually sought during the resuscitation of the patient in septic shock correspond to macrohemodynamic parameters (heart rate, blood pressure, central venous pressure). However, persistent alterations in microcirculation, despite the restoration of macrohemodynamic parameters, can cause organ failure. This dissociation between the macrocirculation and microcirculation originates the need to evaluate organ tissue perfusion, the most commonly used being urinary output, lactatemia, central venous oxygen saturation (ScvO2), and veno-arterial pCO2 gap. Because peripheral tissues, such as the skin, are sensitive to disturbances in perfusion, noninvasive monitoring of peripheral circulation, such as skin temperature gradient, capillary refill time, mottling score, and peripheral perfusion index may be helpful as early markers of the existence of systemic hemodynamic alterations. Peripheral circulation monitoring techniques are relatively easy to interpret and can be used directly at the patient's bedside. This approach can be quickly applied in the intra- or extra-ICU setting. The objective of this narrative review is to analyze the various existing tissue perfusion markers and to update the evidence that allows guiding hemodynamic support in a more individualized therapy for each patient.

In patients with complete double renal system with the involvement of only one system, there are several surgical alternatives for its resolution. Uretero-ureteral anastomosis has been presented as a good alternative, even in cases with atrophy of the affected system.

Objective: To report our experience in patients with complete double renal system with only one system affected, with the surgical technique of uretero-ureteral anastomosis.

Patients and method: Retrospective study of patients with double renal system with involvement of one of the systems, treated with uretero-ureteral anastomosis technique between January 2015 and May 2022. The variables of age, specific pathology of the affected system, preoperative study, days of hospitalization, postoperative complications (leakage, obstruction, infection), and follow-up time were evaluated.

Results: We analyzed 26 procedures in 25 patients, mean age 36.8 months (range: 8-80); 53.8% had ectopic ureter, 23% ureterocele, 11.5% sphincteric ureterocele, and 11.5% VUR of the lower system. All were studied preoperatively with urethrocystography and 65% with scintigraphy. 50% of the operated systems showed signs of renal atrophy. The average hospital stay was 2.2 days (range: 1-7). In an average follow-up of 26.5 months (range: 3-77), one patient presented leakage, no patient presented signs suggestive of obstruction, and one patient presented febrile urinary tract infection with persistent lower-grade reflux.

Conclusion: In our experience, the uretero-ureteral anastomosis technique proved to be an easy and safe alternative to reproduce, with a success rate of 96%, 11% of grade I complications, and 4% of grade II complications according to the Clavien-Dindo classification.