Epicardial Implantation of a Micra™ Pacemaker in a Premature Neonate with Congenital Complete Heart Block.

Pacemaker implantation in neonates can be challenging due to their small size. Even pulse generators adapted for pediatric patients, such as the Microny device (Abbott, Chicago, IL, USA), are proportionately large in comparison to the size of the smallest newborns. Due to anatomic considerations, such as small vascular and ventricular sizes, leadless pacemakers and transvenous implantation in the youngest neonatal population remain unsuitable. Even so, the desire for leadless devices has prompted the industry to create the smallest pacemakers available. Adapting the smaller Micra™ transcatheter leadless pacing system (Medtronic, Minneapolis, MN, USA) for an epicardial pacing application may be advantageous to the smallest patients. This case illustrates the use of a Micra™ device modified with a header block to serve as the pulse generator in a ventricular epicardial pacing system for a 1-day-old, 2.68-kg patient with complete heart block.

Early Infective Endocarditis Associated with an Amplatzer Atrial Septal Occluder Device in a 14-Year-Old Male.

Infective endocarditis (IE) associated with an ASD device, particularly in the early post-procedure period, is extremely rare. We report a case of infective endocarditis presenting with embolic complications and vegetations on the device that were only seen on transesophageal echocardiography, necessitating device removal.

Bronchiolitis obliterans organising pneumonia secondary to tacrolimus toxicity in a pediatric cardiac transplant recipient.

BACKGROUND: Bronchiolitis obliterans organising pneumonia is a rare complication associated with calcineurin inhibitors and mammalian target of rapamycin inhibitors. While bronchiolitis obliterans organising pneumonia in adult transplant patients has been reported, it has not been well described in pediatric transplant patients. CASE DESCRIPTION: We present a case of a 19-month-old male patient with dilated cardiomyopathy who underwent orthotropic heart transplantation at 14 months of life for heart failure refractory to medical therapy. Approximately 4 months post-transplant, he presented with diarrhea and vomiting with acute kidney injury secondary to dehydration. His tacrolimus level on admission and first week of hospitalisation was within target range of 10-12 ng/ml. He was diagnosed with esophagitis and prescribed proton pump inhibitors. Our patient subsequently developed significant respiratory distress with initial chest radiograph showing right lower lobe opacities. Repeat tacrolimus at the time of worsening respiratory status was 84.2 ng/dL and his tacrolimus was held. He required intubation due to significant hypoxia with progression of lung to disease and development of diffuse bilateral opacities consistent with acute respiratory distress syndrome. Despite initiation of steroids and aggressive ventilator management, he continued to be hypoxic on maximal respiratory support. After 28 days post admission, support was withdrawn. On autopsy, his lung biopsy findings were consistent with bronchiolitis obliterans organising pneumonia. CONCLUSION: Life-threatening bronchiolitis obliterans organising pneumonia can be seen in pediatric transplant patients on tacrolimus or when transitioning from tacrolimus to sirolimus, highlighting the need for close monitoring of heart transplant patients on immunosuppressive medications presenting with hypoxia.

Klebsiella Discitis in a 15-Year-Old Male Diagnosed With Plasma Microbial Cell-Free DNA Next-Generation Sequencing Test: A Case Report.

Spinal discitis (SD) is a rare condition, particularly in the pediatric population. The course of SD may be acute or chronic, and the non-specificity of symptoms leads to great delays in diagnosis. The most commonly isolated causative organism is Staphylococcus aureus whereas gram-negative infections are hardly ever reported in the literature. Comorbidities that increase the risk of bacteremia such as diabetes, chronic kidney disease, HIV, and cancer are major risk factors for SD. Hereby, we present an atypical case of SD in a previously healthy 15-year-old male with an unusual organism, Klebsiella aerogenes, diagnosed by plasma microbial cell-free DNA with negative blood cultures. The clinical course was complicated by antibiotic resistance and subsequent development of a ventral epidural abscess requiring readmission followed by surgical drainage of the abscess with a prolonged course of antibiotics.

Obesity as a Risk Factor for Severe Illness From COVID-19 in the Pediatric Population.

In this current outbreak of coronavirus disease 2019 (COVID-19) caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), many studies have been published to determine the spectrum of illness, risk factors, prevention, and treatment strategies. Due to relatively fewer cases among children as compared to adults, there is a paucity of clinical data available to fully understand the risk factors and disease course in the pediatric population. Our understanding is evolving with limited data showing an increased risk of severe or critical disease in children less than one year of age and those with certain underlying medical conditions. Recognition of emerging risk factors for morbidity and mortality is now paramount, to anticipate and provide appropriate clinical care specific to the pediatric population. Obesity has only recently been identified as a risk factor for severe COVID-19 disease in children. Case reports such as this are essential in understanding the pathophysiologic association, associated disease severity, and clinical outcome attributed to obesity and COVID-19 infections in children.

Role of Theophylline in Management of Bradycardia Secondary to High Cervical Spinal Cord Injury in a Seven-Year-Old Child: Case Report and a Review of Literature.

High-level cervical spinal cord injury (SCI) frequently leads to the development of severe sinus bradycardia and asystole. Conventionally, owing to their chronotropic effects, medical management has largely relied on the use of atropine and/or infusion of pressors such as epinephrine or dopamine as the first-line treatment. However, for severe symptomatic events refractory to medical therapy, cardiac pacemaker implantation may be required. In light of the limited data, found in the adult literature, use of methylxanthines such as theophylline has been suggested for the treatment of bradycardia or asystole in the setting of cervical SCI, but to our knowledge, this treatment approach has not been reported in very young children. We present a case of medical management of bradycardia-asystole episodes in a seven-year-old child who sustained cervical SCI after a motor vehicle accident (MVA). His clinical course was complicated by frequent episodes of symptomatic sinus bradycardia progressing to asystole. Episodes were responsive to atropine, but his events were recurrent and feared to be life-threatening if unobserved, and so pacemaker implantation was being considered. In the hope of averting the need for pacemaker implantation, he was started on enteral theophylline, with blood level monitoring and had remained in normal sinus rhythm without recurrence of severe bradycardic or asystole events for a latent period of 74 days. Subsequently, however, he underwent pacemaker placement.

Ectopic follicular variant of papillary thyroid carcinoma in anterior mediastinum with a normal thyroid gland. A case report.

INTRODUCTION: Ectopic thyroid is rare, especially in the anterior mediastinum. Furthermore, true malignant transformation in ectopic location with normal orthotopic gland is exceptionally unusual. The authors have tried to put forward a challenging case of a malignant mediastinal mass managed successfully via a multidisciplinary approach. PRESENTATION OF CASE: We report the case of a 55 year old female with ectopic follicular variant of papillary thyroid cancer in the anterior mediastinum with no disease in the thyroid gland. Subsequently, complete excision of the mediastinal mass and total thyroidectomy was done followed by radio-iodine ablation with radiotherapy. Patient showed good uneventful recovery and remains tumor free till date. CONCLUSION: The aim of this report is to bring forward malignant ectopic thyroid carcinoma as a differential for malignant mediastinal masses. It is emphasized that the diagnosis is made essentially through physical and pathological examination and, in most cases, only after surgery. A Multidisciplinary approach is recommended in such cases.

Childhood-Onset Systemic Lupus Erythematosus: A Cohort Study.

OBJECTIVE: To determine the clinical and immunological characteristics and short-term outcome of children with systemic lupus erythematosus (SLE). STUDY DESIGN: A descriptive cohort study. PLACE AND DURATION OF STUDY: Paediatric Rheumatology Clinic, The Aga Khan University Hospital, Karachi, from January 2011 to December 2015. METHODOLOGY: Clinical and immunological profile and short-term outcome of children less than 16 years of age admitted in the paediatric ward, with the diagnosis of SLE was studied. Demographic data, clinical presentation, laboratory findings, immunological profile and treatment regimens of these children were evaluated. RESULTS: Thirty-two children, satisfying the criteria of American College of Rheumatology (ACR) for SLE, were enrolled during the study period of five consecutive years. A female predominance was observed with 28 (87.5%) patients being female (F:M 7:1). Mean age at symptom onset was 10.5 +2.7 years; and 8.8 +2.1 years in females and males, respectively. The mean age at diagnosis was 11.3 +2.8 years in females and 9.4 +1.9 years in males. Prolonged fever was the most common non-specific symptom found in 27 (84%), followed by pallor in 13 (41%) patients. Twenty-two (69%) children were found to be anemic and 18 patients (56%) having signs of arthritis at presentation. Renal involvement was observed in 15 (47%) patients. The most common laboratory finding was anemia, found in 22 (69%) of cases. The most common immunological markers were serum anti-neutrophil antibodies (ANA), positive in 28 (88%) patients, followed by anti double-stranded DNA antibodies, raised in 26 (81%) of cases. Out of 32, 12 patients were lost to follow-up. Of the remaining 20 children who were followed for four years, ten (50%) went into remission. CONCLUSION: Childhood-onset SLE encompasses a wide variety of manifestations with a female preponderance. Fever, arthralgia and pallor are the most frequent clinical manifestations among the children. Hemolytic anemia (HA) is the most common laboratory abnormality, with ANA and anti ds-DNA antibodies positivity in the majority of padiatric patients.