Group A Streptococcal Toxic Shock Syndrome With Portal Vein Thrombosis: A Rare Presentation in Newborns.

Invasive disease due to group A Streptococcus infection results in a large spectrum of clinical manifestations. In the neonatal period, the occurrence is rare and potentially serious. We present a case of a term male newborn on the 9th day of life who was admitted to the emergency room with moaning and poor feeding. The patient was hemodynamically unstable needing mechanical ventilation and inotropic support. Mother and father had clinical symptoms of pharyngitis. Blood samples revealed high serum C-reactive protein and procalcitonin, leucopenia, thrombocytopenia, hyponatremia, hepatic cytolysis, and cholestasis. He started on IV ampicillin, gentamicin, and cefotaxime. Due to an abdominal distension, an ultrasound was done showing a heterogenous hepatic lobe. A color Doppler scan completed the study revealing a left hepatic thrombosis. Enoxaparin was started. The newborn's blood culture and mother's milk were positive for the same strain of group A Streptococcus. Intravenous immunoglobulin and clindamycin were added to the treatment. On day 5 of treatment, inotropic support was ceased and extubation took place on day 6. Neonatologists should be aware of rare complications of group A Streptococcus infection such as thrombotic events.

Neonatal Renal Failure Following Intrauterine Exposure to an Angiotensin-Converting Enzyme Inhibitor.

The renin-angiotensin-aldosterone system (RAAS) plays a crucial role in the normal development of the fetal kidney. Late pregnancy blockage of the RAAS, through in-utero exposure to angiotensin-converting enzyme inhibitors (ACEIs) or angiotensin II receptor blockers, is associated with poor fetal outcomes, including oligohydramnios, renal tubular dysplasia, postnatal anuric renal failure, and hypotension. The present case describes a 39-year-old primigravida that was referred to the emergency department, at 37 weeks, for the evaluation of intrauterine growth restriction and suspected coarctation of the aorta (CoA). She was medicated with enalapril since the 35th week of gestation. She delivered a male infant, weighing 2,110 g, with no apparent malformations. CoA was excluded. During his first day of life, the patient developed anuria, acute renal failure, and hypotension, requiring ionotropic support. Renal ultrasound appeared normal. Diuresis was reinitiated at 48 hours of life after continued supportive measures. Kidney function tests progressively normalized. Additional investigations revealed a low concentration of angiotensin-converting enzyme. The patient is currently 12 months old and has had a favorable evolution. This case highlights the fact that even brief exposure to enalapril in the third trimester may cause RAAS blocker fetopathy. As long-term sequelae of ACEI-exposed infants are poorly described, close follow-up of renal complications is essential. Physicians should be aware of the deleterious effects of RAAS blockers in pregnancy.

Neonatal Urinary Tract Infection and Renal Nodular Lesion: A Rare Case of Xanthogranulomatous Pyelonephritis.

Xanthogranulomatous pyelonephritis (XPN) is an uncommon variant of chronic pyelonephritis with a poorly understood pathogenesis and a challenging diagnosis. It is rare in pediatric patients, particularly in the neonatal period. We report the case of an 18-day-old female neonate admitted to the emergency room due to macroscopic hematuria and poor feeding. Urinalysis revealed leukocyturia and she was initially admitted under the clinical suspicion of acute pyelonephritis. Renal ultrasound and magnetic resonance imaging (MRI) revealed a progressive nodular lesion in the middle third of the left kidney. Given the suspicion of renal abscess or neoplasm, the patient was transferred to our tertiary hospital. Urinary catecholamines and tumor markers had normal values. Percutaneous kidney biopsy confirmed XPN. Posterior computed tomography scan excluded extension to neighboring structures. A conservative management with systemic antibiotic therapy was decided. She completed 7 weeks of systemic antibiotic therapy (ampicillin and cefotaxime) with progressive reduction of lesion size and posterior calcification. Follow-up at 3 years was uneventful. The lipid profile and study of neutrophil function were normal. Voiding cystourethrography excluded vesicoureteral reflux. The authors intend to highlight the importance of a high index of suspicion of XPN to allow preoperative diagnosis. Histopathological assessment is mandatory to confirm XPN and exclude other entities mimicked by focal and unilateral progressive disease. There are only a few published cases of optimal clinical evolution solely with broad-spectrum antibiotics; however, this may allow a beneficial nephron-sparing approach in selected patients.

Neonatal Atypical Hemolytic Uremic Syndrome in the Eculizumab Era.

The atypical hemolytic uremic syndrome (aHUS) in the newborn is a rare disease, with high morbidity. Eculizumab, considered a first-line drug in older children, is not approved in neonates and in children weighing less than 5 kg. We present a 5-day-old female newborn, born at 36 weeks' twin gestation, by emergency cesarean section due to cord prolapse, with birth weight of 2,035 g and Apgar score of 7/7/7, who develops microangiopathic hemolytic anemia, thrombocytopenia, and progressive acute renal failure. In day 5, after diagnosis of aHUS, a daily infusion of fresh frozen plasma begins, with improvement of thrombocytopenia and very slight improvement in renal function. The etiologic study (congenital infection, Shiga toxin, ADAMTS13 activity, directed metabolic study) was normal. C3c was slightly decreased. On day 16 for maintenance of anemia and severe renal failure, she started 300 mg/dose eculizumab. Anemia resolves in 10 weeks and creatinine has normal values after 13 weeks of treatment. The genetic study was normal. In this case, eculizumab is effective in controlling microangiopathy and in the recovery of renal function. Diagnosis of neonatal aHUS can be challenging because of phenotypic heterogeneity and potential overlap with other manifestations that may confound it, such as perinatal asphyxia or sepsis/disseminated intravascular coagulation.

Assessment of the renal angina index for the prediction of acute kidney injury in patients admitted to a European pediatric intensive care unit.

BACKGROUND: Acute kidney injury (AKI) is associated with worse outcomes and increased morbidity and mortality in pediatric intensive care unit (PICU) patients. The renal angina index (RAI) has been proposed as an early prediction tool for AKI development. OBJECTIVES: The objective was to evaluate outcomes of RAI-positive patients and to compare RAI performance with traditional AKI markers across different patient groups (medical/post-surgical). This was an observational retrospective study. All children admitted to a tertiary hospital PICU over a 3-year period were included. Electronic medical records were reviewed. Day 1 RAI was calculated, as was the presence and staging of day 3 AKI. RESULTS: A total of 593 patients were included; 56% were male, the mean age was 55 months, and 17% had a positive RAI. This was associated with day 3 AKI development and worse outcomes, such as greater need for kidney replacement therapy, longer duration of mechanical ventilation, vasoactive support and PICU stay, and higher mortality. For all-stage kidney injury, RAI presented a sensitivity of 87.5% and a specificity of 88.1%. Prediction of day 3 all-stage AKI by RAI had an AUC=0.878; its performance increased for severe AKI (AUC = 0.93). RAI was superior to serum creatinine increase and KDIGO AKI staging on day 1 in predicting severe AKI development. The performance remained high irrespective of the type of admission. CONCLUSIONS: The RAI is a simple and inexpensive tool that can be used with medical and post-surgical PICU patients to predict AKI development and anticipate complications, allowing for the adoption of preventive measures.

Performance of PRISM III and PELOD-2 scores in a pediatric intensive care unit.

UNLABELLED: The study aims were to compare two models (The Pediatric Risk of Mortality III (PRISM III) and Pediatric Logistic Organ Dysfunction (PELOD-2)) for prediction of mortality in a pediatric intensive care unit (PICU) and recalibrate PELOD-2 in a Portuguese population. To achieve the previous goal, a prospective cohort study to evaluate score performance (standardized mortality ratio, discrimination, and calibration) for both models was performed. A total of 556 patients consecutively admitted to our PICU between January 2011 and December 2012 were included in the analysis. The median age was 65 months, with an interquartile range of 1 month to 17 years. The male-to-female ratio was 1.5. The median length of PICU stay was 3 days. The overall predicted number of deaths using PRISM III score was 30.8 patients whereas that by PELOD-2 was 22.1 patients. The observed mortality was 29 patients. The area under the receiver operating characteristics curve for the two models was 0.92 and 0.94, respectively. The Hosmer and Lemeshow goodness-of-fit test showed a good calibration only for PRISM III (PRISM III: χ (2) = 3.820, p = 0.282; PELOD-2: χ (2) = 9.576, p = 0.022). CONCLUSIONS: Both scores had good discrimination. PELOD-2 needs recalibration to be a better reliable prediction tool. WHAT IS KNOWN: • PRISM III (Pediatric Risk of Mortality III) and PELOD (Pediatric Logistic Organ Dysfunction) scores are frequently used to assess the performance of intensive care units and also for mortality prediction in the pediatric population. • Pediatric Logistic Organ Dysfunction 2 is the newer version of PELOD and has recently been validated with good discrimination and calibration. What is New: • In our population, both scores had good discrimination. • PELOD-2 needs recalibration to be a better reliable prediction tool.

Accidental Ingestion of Dentistry Material - Report of Cases and Challenges from the Pediatrician Point of View.

INTRODUCTION: Aspiration or ingestion of foreign bodies may occur during dental procedures. Diagnosis and management of these accidents is sometimes challenging. The authors present a small series of clinical cases. CASE 1: Adolescent observed due to suspected accidental bracket ingestion, not visible on x-ray, removed by upper digestive endoscopy. CASE 2: Adolescent observed after accidental ingestion of a dental file. Conflicting results in image exams and absence of object progression led to enteroscopy for extraction. CASE 3: Adolescent observed due to accidental ingestion of a surgical blade, visualized on image study but not accessible by endoscopy, resulting in latter spontaneous elimination. DISCUSSION: Image study is frequently useful when metallic object ingestion is suspected, but has some limitations. In some cases, mucosal protections must be used during removal procedures. Prevention of such accidents is the best approach, using appropriate protections to secure airway and digestive tract during dental procedures.

INTRODUÇÃO: A aspiração ou ingestão de corpos estranhos pode ocorrer durante qualquer procedimento dentário. O diagnóstico e a resolução destas situações podem colocar desafios. Os autores apresentam três casos clínicos. CASO 1: Adolescente observada por suspeita de ingestão acidental de um bracket de aparelho fixo de ortodontia, não visualizado na radiografia e removido por endoscopia digestiva alta. CASO 2: Adolescente com história de ingestão acidental de uma lima de dentista. Os exames complementares realizados não foram esclarecedores e, dado não haver progressão do corpo estranho, foi necessária a sua remoção por enteroscopia. CASO 3: Adolescente que recorre à urgência por ingestão acidental de uma lâmina de dentista, inacessível por endoscopia e que acabou por ser eliminada espontaneamente. DISCUSSÃO: Os exames imagiológicos são habitualmente úteis na suspeita de ingestão de corpos estranhos metálicos mas a informação que fornecem é limitada. A natureza cortante do material dentário deglutido exige que se tomem cuidados adicionais na sua remoção, utilizando auxiliares protectores. A prevenção destes acidentes deve ser promovida, recorrendo a protecções da via aérea e digestiva durante a realização de procedimentos dentários.

Follicular bronchiolitis: A rare cause of persistent atelectasis in children.

Although atelectasis is common in children, its persistence or refractoriness to treatment should lead prompt evaluation to identify causal mechanism. We describe the case of a child presenting in first year of life with persistent left upper lobe atelectasis, recurrent wheezing and respiratory infections refractory to medical therapy, submitted to partial lobectomy when he was 3 years old age. Histopathological examination revealed follicular bronchiolitis. Systemic underlying diseases were excluded. Clinical improvement was initially achieved using inhaled corticosteroids, but oral therapy was needed due to clinical relapse, with favorable response. Follicular bronchiolitis, a rare pulmonary primary lymphoid lesion, consists of numerous reactive lymphoid follicles in a peribonchiolar distribution. Its precise cause is unknown, particularly in children, in which few cases have been reported. Treatment usually includes steroids and prognosis is generally good.