Is HbA1c an Indicator of Diabetic Ketoacidosis Severity in the Pediatric Population?

ABSTRACT: Glycosylated hemoglobin (HbA1c) reflects how well blood glucose is controlled and is one of the strongest predictors of chronic complications of diabetes mellitus. The degree of acidosis helps determine the severity of diabetic ketoacidosis (DKA) (mild: pH 7.2-7.3; moderate: pH 7.1-7.2; severe: pH <7.1) and guides the level of care and predicts outcome. Many studies have implicated that higher HbA1c levels lead to recurrent DKA. However, there is no description of the association of higher HbA1c with the severity of DKA. One hundred thirty-eight electronic medical records of patients aged 1 to 21 years admitted to the pediatric intensive care unit with DKA between 2011 and 2015 were analyzed. We excluded 50 patients because the HbA1c level was not available. Spearman correlation analyzed the data for 88 patients included in the study. The mean HbA1c was 13.3, with female patients having more admissions compared with male patients (58% vs 42%). The age group from 13 to 21 years accounted for 77.3% of the patients. The duration of type 1 diabetes mellitus did not affect the HbA1c level. Likewise, the blood glucose and serum creatinine level did not show a statistical correlation with blood pH levels. Mean HbA1c for mild, moderate, and severe DKA groups were 11.4%, 12.2%, and 14.8%, respectively. Blood pH and HbA1c returned a negative correlation (correlation coefficient, -0.557; P = 0.005). The HbA1c level correlated positively with the 3 groups of DKA (correlation coefficient, 0.595; P = 0.01). A higher A 1c was associated with more severe DKA.

Characterization and Outcomes of Hospitalized Children With Coronavirus Disease 2019: A Report From a Multicenter, Viral Infection and Respiratory Illness Universal Study (Coronavirus Disease 2019) Registry.

OBJECTIVES: Multicenter data on the characteristics and outcomes of children hospitalized with coronavirus disease 2019 are limited. Our objective was to describe the characteristics, ICU admissions, and outcomes among children hospitalized with coronavirus disease 2019 using Society of Critical Care Medicine Discovery Viral Infection and Respiratory Illness Universal Study: Coronavirus Disease 2019 registry. DESIGN: Retrospective study. SETTING: Society of Critical Care Medicine Viral Infection and Respiratory Illness Universal Study (Coronavirus Disease 2019) registry. PATIENTS: Children (< 18 yr) hospitalized with coronavirus disease 2019 at participating hospitals from February 2020 to January 2021. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The primary outcome was ICU admission. Secondary outcomes included hospital and ICU duration of stay and ICU, hospital, and 28-day mortality. A total of 874 children with coronavirus disease 2019 were reported to Viral Infection and Respiratory Illness Universal Study registry from 51 participating centers, majority in the United States. Median age was 8 years (interquartile range, 1.25-14 yr) with a male:female ratio of 1:2. A majority were non-Hispanic (492/874; 62.9%). Median body mass index (n = 817) was 19.4 kg/m2 (16-25.8 kg/m2), with 110 (13.4%) overweight and 300 (36.6%) obese. A majority (67%) presented with fever, and 43.2% had comorbidities. A total of 238 of 838 (28.2%) met the Centers for Disease Control and Prevention criteria for multisystem inflammatory syndrome in children, and 404 of 874 (46.2%) were admitted to the ICU. In multivariate logistic regression, age, fever, multisystem inflammatory syndrome in children, and pre-existing seizure disorder were independently associated with a greater odds of ICU admission. Hospital mortality was 16 of 874 (1.8%). Median (interquartile range) duration of ICU (n = 379) and hospital (n = 857) stay were 3.9 days (2-7.7 d) and 4 days (1.9-7.5 d), respectively. For patients with 28-day data, survival was 679 of 787, 86.3% with 13.4% lost to follow-up, and 0.3% deceased. CONCLUSIONS: In this observational, multicenter registry of children with coronavirus disease 2019, ICU admission was common. Older age, fever, multisystem inflammatory syndrome in children, and seizure disorder were independently associated with ICU admission, and mortality was lower among children than mortality reported in adults.

Development of paediatric critical care in northwestern Nigeria: Initial implementation with a needs assessment model.

INTRODUCTION: There is high paediatric morbidity and mortality in northwestern Nigeria, attributable in part to vaccine-preventable illnesses and lack of comprehensive training of medical and nursing staff in the healthcare delivery of paediatric critical care. Pediatric Universal Life-Saving Effort Inc. (PULSE), a New York-based nonprofit organisation with a mission to develop paediatric critical care in resource-limited settings, collaborated with Aminu Kano University Teaching Hospital to decrease the gaps in knowledge and skills of medical and nursing personnel. The joint effort also aims to address and remove barriers to the delivery of paediatric critical care in northwestern Nigeria. The primary objective was to perform a needs assessment for paediatric intensive care resources in northwestern Nigeria, identify barriers to delivering these services, and designate a hub for the development of paediatric critical care educational programs for healthcare professionals. METHODS: An anonymous survey was designed and distributed using SurveyMonkey® online software to medical and nursing staff from nine healthcare institutions in northwestern Nigeria. RESULTS: Analysis from 67 responses revealed that care delivered to critically ill paediatric patients was by anaesthesiologists (77%), pediatricians (26%), and adult intensive care specialists (10%). The acquisition of clinical skills was perceived to be an essential need (65%), followed by adequate staffing of critical care units (19%), continuing medical and nursing education (13%), and availability of medical equipment (3%). DISCUSSION: There is an identified need for paediatric critical care training and resources in northwestern Nigeria. CONCLUSION: The needs assessment conducted has provided important results that will form the basis for building staff capacity and training programs for paediatric critical care in northwestern Nigeria.

Clinical Manifestations and Outcomes of Critically Ill Children and Adolescents with Coronavirus Disease 2019 in New York City.

OBJECTIVES: To describe the clinical manifestations and outcomes of critically ill children with coronavirus disease-19 (COVID-19) in New York City. STUDY DESIGN: Retrospective observational study of children 1 month to 21 years admitted March 14 to May 2, 2020, to 9 New York City pediatric intensive care units (PICUs) with severe acute respiratory syndrome coronavirus 2 infection. RESULTS: Of 70 children admitted to PICUs, median age was 15 (IQR 9, 19) years; 61.4% male; 38.6% Hispanic; 32.9% black; and 74.3% with comorbidities. Fever (72.9%) and cough (71.4%) were the common presenting symptoms. Twelve patients (17%) met severe sepsis criteria; 14 (20%) required vasopressor support; 21 (30%) developed acute respiratory distress syndrome (ARDS); 9 (12.9%) met acute kidney injury criteria; 1 (1.4%) required renal-replacement therapy, and 2 (2.8%) had cardiac arrest. For treatment, 27 (38.6%) patients received hydroxychloroquine; 13 (18.6%) remdesivir; 23 (32.9%) corticosteroids; 3 (4.3%) tocilizumab; and 1 (1.4%) anakinra; no patient was given immunoglobulin or convalescent plasma. Forty-nine (70%) patients required respiratory support: 14 (20.0%) noninvasive mechanical ventilation, 20 (28.6%) invasive mechanical ventilation (IMV), 7 (10%) prone position, 2 (2.8%) inhaled nitric oxide, and 1 (1.4%) extracorporeal membrane oxygenation. Nine (45%) of the 20 patients requiring IMV were extubated by day 14 with median IMV duration of 218 (IQR 79, 310.4) hours. Presence of ARDS was significantly associated with duration of PICU and hospital stay, and lower probability of PICU and hospital discharge at hospital day 14 (P < .05 for all). CONCLUSIONS: Critically ill children with COVID-19 predominantly are adolescents, have comorbidities, and require some form of respiratory support. The presence of ARDS is significantly associated with prolonged PICU and hospital stay.

Integrating Fat Embolism Syndrome Scoring Indices in Sickle Cell Disease: A Practice Management Review.

Fat embolism syndrome (FES) has been described in the literature as a rare complication of sickle cell disease (SCD). A review article published in 2005 reported 24 cases of FES associated with SCD. In many cases, a definitive diagnosis of FES in SCD is made on autopsy because of the lack of early recognition and the paucity of sensitive and specific testing for this syndrome. Patients with FES usually have a fulminant, rapidly deteriorating clinical course with mortality occurring within the first 24 hours. We postulate that FES is not well recognized in SCD and that FES scores are useful diagnostic tools in patients with SCD. We queried the electronic medical records with the diagnostic codes for SCD with acute chest syndrome (ACS), pulmonary embolism, or acute respiratory distress syndrome admitted to our hospital from 2008 to 2016 to identify patients suspected of having FES. In addition, we performed an extensive literature review to evaluate the management practice of pediatric patients with FES and SCD from 1966 to 2016. Six patients met our selection criteria from the hospital records, and 4 case reports from the literature search were also included. We applied the Gurd and Wilson criteria and the Schonfeld Fat Embolism Index to identify patients who met the criteria for FES. Nine patients fulfilled Gurd and Wilson criteria, and 9 patients who were evaluable met the Schonfeld criteria for FES. A rapidly deteriorating clinical course in a patient with SCD presenting with ACS or severe vaso-occlusive crisis should trigger a high index of suspicion for FES. Gurd and Wilson criteria or the Schonfeld Fat Embolism Index are useful diagnostic tools for FES in SCD.

The Cause of Severe Metabolic Acidosis With Vomiting in a Neonate.

We present a 22-day-old male born full term who presented with worsening non-projectile, non-bilious vomiting and failure to thrive (FTT) and was admitted to the pediatric intensive care unit (PICU) for severe metabolic acidosis with an elevated anion gap. Despite changing the formula, the patient continued to have spit-ups after feeds since birth. Before this admission, his vomiting worsened with every feed, which was now forceful along with two days of loose stools. Obstructive causes of emesis were ruled out with an upper gastrointestinal series, and a decision was made to evaluate for organic causes of FTT. Transient resolution of symptoms was noticed when the patient was placed NPO (nothing by os/mouth) briefly. His symptoms returned on resuming cow milk-based formula feeds. At this time, a presumptive diagnosis of cow milk protein allergy (CMPA) was made. Positive fecal occult blood supported the diagnosis, and his formula was changed to an extensively hydrolyzed formula (eHF). This is a case of severe CMPA with prolonged vomiting and FTT presenting with severe metabolic acidosis with an elevated anion gap. This case report highlights how CMPA can lead to severe dehydration with metabolic acidosis and increased anion gap.

Acute Pediatric Pancreatitis Presenting with Splenic Vein Thrombosis.

Acute pancreatitis among the pediatric population can result from genetic disorders, anatomic anomalies, gallstones, trauma, and medications; trauma and idiopathic causes being the most common. Although chronic pancreatitis presents with increased severe long-term complications, acute pancreatitis presents with its share of complications such as fistulas, pseudocysts, and venous abnormalities. With an increase in hospitalization rates of acute pancreatitis among the pediatric population, the importance of understanding rare complications and how to further recognize these complications can aid in the diagnosis, medical management, and intervention necessary to optimize a patient's outcome. Our patient presented with a rare complication of splenic vein thrombosis (SVT), which is a complication that can also be observed in adults with acute pancreatitis. SVTs are uncommon in both the adult and pediatric populations, and they have received little attention or research in the pediatric population. We report a case that will highlight a rare case of SVT presenting in a pediatric patient with acute necrotizing gallstone pancreatitis.

COVID 19 infection: Pediatric perspectives.

The coronavirus disease 2019 (COVID-19) pandemic caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has rapidly spread across the globe, causing innumerable deaths and a massive economic catastrophe. Exposure to household members with confirmed COVID-19 is the most common source of infection among children. Children are just as likely as adults to get infected with SARS-CoV-2. Most children are asymptomatic and when symptoms occur, they are usually mild. Infants <12 months old are at a higher risk for severe or critical disease. COVID-19 is diagnosed the same way in pediatric population as adults by testing specimen obtained from upper respiratory tract for nucleic acid amplification test (NAAT) using reverse transcriptase viral polymerase chain reaction (RT-PCR). The common laboratory findings in hospitalized patient include leukopenia, lymphopenia, and increased levels of inflammatory markers. Chest X-ray findings are variable and computed tomography scans of the chest may show ground glass opacities similar to adults or non-specific findings. Prevention is the primary intervention strategy. Recently the U.S. Food and Drug Administration (FDA) has provided emergency authorization of the Pfizer-BioNTech COVID-19 vaccine and many other vaccine candidates are in the investigational stage. There is limited data in children on the use of antivirals, hydroxychloroquine, azithromycin, monoclonal antibody, and convalescent plasma. Oxygen therapy is required in hypoxic children (saturation <92%). Similar to adults, other measures to maintain oxygenation such as high flow nasal cannula, CPAP, or ventilatory support may be needed. Ventilatory management strategies should include use of low tidal volumes (5-6 cc/kg), high positive expiratory pressure, adequate sedation, paralysis, and prone positioning. Recently, a new entity associated with COVID-19 called multisystem inflammatory syndrome in children (MIS-C) has emerged. Clinical, laboratory, and epidemiological criteria are the basis for this diagnosis. Management options include ICU admission, steroids, intravenous gamma globulin, aspirin, anakinra, and anticoagulants. Vasoactive-inotropic score (VIS) is used to guide vasopressor support.

A Rare Cause of Post-traumatic Right Lower Extremity Swelling in an Adolescent Male.

Unilateral extremity swelling after trauma usually results from acute musculoskeletal or orthopedic injuries. Worsening of swelling raises concern for compartment syndrome or vascular injury. Time-sensitive diagnosis and interventions are needed to avoid life- or limb-threatening consequences. In this report, we highlight the case of a 16-year-old male who presented with unilateral lower extremity pain and swelling, one week after a motor vehicle accident. Thorough evaluation and appropriate imaging detected the presence of an abnormal communication between the muscular branch of the anterior tibial artery and the vein. Arteriovenous fistulas (AVFs) are usually acquired and caused by penetrating trauma or iatrogenic procedures. They are rarely associated with blunt trauma. It is important to determine the degree of flow within the communication, as high flow lesions are associated with severe complications such as limb ischemia and heart failure. This report highlights the evaluation and management of a patient with delayed post-traumatic unilateral extremity swelling that eventually resulted in the diagnosis of a low-flow AVF amenable to conservative management, resulting in complete resolution of his symptoms.

Aversion to Hospital Admission Due to Fear of COVID Infection Leading to Fatality From Diabetic Ketoacidosis.

Diabetic ketoacidosis (DKA) is a potentially life-threatening condition that occurs in patients with diabetes mellitus (DM) where the decrease in the insulin level leads to a state of metabolic acidosis and hyperglycemia. Based on the literature review, the risk of severity of DKA in children was significantly associated with coronavirus disease-2019 (COVID-19) cases during the first wave of the pandemic. This could be attributed to social distancing restrictions which delayed hospital presentation and timely treatment and interventions. We present the case of a 15-year-old female, with non-insulin-dependent diabetes (type 2), who presented during the COVID-19 pandemic with severe DKA from another hospital. She had elevated glucose level at home for three days that was worsening but her parents continue to manage the patient at home out of fear of the patient contracting COVID-19 if she was brought to the hospital. After she deteriorated, the parents took her to the nearest hospital which did not have a pediatric intensive care unit (PICU). She was immediately transferred to our facility. The patient was intubated immediately on arrival because of altered mental status possibly due to cerebral edema from severe metabolic acidosis and elevated glucose level. The patient rapidly progressed into shock, acute respiratory distress syndrome (ARDS), and multiple organ dysfunction syndrome (MODS). She was managed aggressively with vasopressors, fluid resuscitation, and insulin drip. She had four cardiac arrests for which she was resuscitated. Despite all efforts, she subsequently expired less than 24 hours after admission. We intend on shedding light on an emerging phenomenon due to the ongoing COVID-19 pandemic, wherein due to the fear of contracting COVID-19, many parents opt to keep and manage sick children at home. This report highlights the important role that the aversion of presenting to medical establishments out of fear of contracting COVID-19 may have led to the untimely and preventable death of our patient. It also outlines the importance of future educational reforms toward changing the patient and family's perception of hospitals and medical institutions, especially in children with pre-existing chronic medical conditions.

COVID-19 Infection Presenting With Cheilitis and Fever in a Toddler.

Coronavirus disease 2019 (COVID-19) is a newly found infectious disease caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), first observed in Wuhan, China, in December 2019. An otherwise healthy 13-month-old male presented with persistent fever and cheilitis as his initial findings of COVID-19 in April 2020 prior to the discovery and classification of the multisystem inflammatory syndrome in children (MIS-C). Clinical symptoms of COVID-19 are still evolving in the pediatric population, ranging from being asymptomatic to varied symptoms, such as fever, abdominal pain, and myocarditis. Other manifestations such as conjunctivitis and cheilitis can offer clues. We speculate that cheilitis can be a sign of the hyperinflammatory state, as seen in MIS-C.

Splenic rupture in a newborn with severe hemophilia--case report and review.

SUMMARY: Life-threatening splenic rupture is rare in neonates with severe hemophilia. There are only 3 cases of splenic rupture in neonates with hemophilia reported in the literature. We present the case of an infant, born to a hemophilia A carrier mother. The infant was asymptomatic until discharge at 48 hours of age, but presented on the third day of life with shock, abdominal distension, and severe anemia. Computed tomography of the abdomen confirmed the diagnosis of splenic rupture with hemoperitoneum. The infant recovered after extensive supportive care surgery and factor replacement.

Compressive Neuropathy of the Facial Nerve Presenting as Bell's Palsy in a Pediatric Patient on High-Frequency Oscillatory Ventilation.

A three-year eight-month-old female with Werdnig Hoffman disease presented with an acute onset of respiratory failure secondary to influenza infection. The patient required conventional mechanical ventilation (CMV). Due to worsening hypoxemia on maximal support, high-frequency oscillatory ventilation (HFOV) was initiated. On recovery from her respiratory failure, she was noted to have developed a left-sided Bell's palsy. A pressure ulcer in the left mastoid area through which the facial nerve transverses was noted, with no evidence of mastoiditis. The patient fully recovered after a course of oral steroid therapy. We postulate that compression pressure might have contributed to the palsy. However, it is unclear what role the acute viral illness played in this case.

Duodenal Stenosis: A Diagnostic Challenge in a Neonate With Poor Weight Gain.

Cases of isolated duodenal stenosis in the neonatal period are minimally reported in pediatric literature. Causes of small bowel obstruction such as duodenal atresia or malrotation with midgut volvulus have been well documented and are often diagnosed due to their acute clinical presentation. Duodenal stenosis, however, causes an incomplete intestinal obstruction with a more indolent and varying clinical presentation thus making it a diagnostic challenge. We present a neonate with a unique case of congenital duodenal stenosis. The neonate presented with poor weight gain and frequent "spit-ups" as per the mother at the initial newborn visit. The clinical presentation was masked as the patient was being fed infrequently and with concentrated formula. We postulate that this may be due to the fact that the mother was an adolescent and relatively inexperienced with newborn care. During the hospital course, the patient had recurrent episodes of emesis with notable electrolyte abnormalities including hypochloremia and metabolic alkalosis. Further investigation with an abdominal X-ray showed dilated loops of bowel. Pyloric stenosis was ruled out via abdominal ultrasound. An upper gastrointestinal (GI) series ultimately confirmed a diagnosis of duodenal stenosis and the infant underwent surgical repair with full recovery. Congenital duodenal stenosis may have atypical presentations in neonates requiring pediatricians to have a high index of suspicion for diagnosis and to ensure timely therapy.

Reversible posterior leukoencephalopathy syndrome in a child treated with bevacizumab.

Bevacizumab is a monoclonal antibody targeting vascular endothelial growth factor (VEGF). Hypertension is a well-recognized, common side effect of VEGF blocking agents. The reversible posterior leukoencephalopathy syndrome (RPLS) has been described as a rare but serious consequence of bevacizumab administration. We present a case of a 6-year-old child with refractory hepatoblastoma who developed hypertensive crisis, seizures and MRI changes consistent with RPLS while receiving bevacizumab with gemcitabine and oxaliplatin. Findings completely resolved without neurologic sequelae with stringent blood-pressure control. Better understanding of risk for RPLS, prompt recognition and aggressive management will be required as bevacizumab gains wider use in pediatrics.

An Unusual Cause of Recurrent Urinary Retention in an Adolescent Female.

Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare, combined Mullerian and Mesonephric duct anomaly characterized by the triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis. We present the case of an otherwise healthy 16-year-old female with acute urinary retention secondary to HWWS. The diagnosis was established with abdominal ultrasound and Magnetic Resonance Imaging (MRI). The patient subsequently underwent surgical resection of the vaginal septum resulting in relief of obstruction. Clinical symptoms in patients with HWWS typically present after menarche with progressive hematometra causing pain and compression of localized structures. Even though ultrasound can help in the diagnosis, MRI is the best choice of imaging for the visualization of these anomalies. The diagnosis of HWWS is important to consider in young females of reproductive age presenting with symptoms of obstruction of adjacent structures. Our patient presented with acute urinary retention which is a rare symptom in this entity. A high index of clinical suspicion and awareness of the syndrome are required to make a speedy diagnosis and prevent future complications.

A Unique Pathogen Causing Infective Endocarditis in a Three-year-old Girl.

The overall incidence of infective endocarditis (IE) in adults has been reported to be 1.5 to 6.0 per 100,000 patient-years. In children, the incidence of IE in the general population is approximately three times lower. The presence of cyanotic congenital heart disease is considered to be the most strongly associated risk factor to develop IE. In approximately 8% to 10% of pediatric cases, IE develops without structural heart disease or any other readily identifiable risk factors. In these situations, the infection usually involves the aortic or mitral valve secondary to Staphylococcus aureus bacteremia. Streptococcus pneumoniae endocarditis in a female with no known risk factors is extremely rare and has no established optimal therapy. We hereby present a case of a three-year-old girl, with no identifiable risk factors diagnosed with IE caused by S.  pneumoniae.

Primary non-typhoidal Salmonella infection presenting as a splenic abscess in a healthy adolescent male.

Isolated splenic abscess in a previously healthy patient is a rare clinical condition and remains a diagnostic dilemma. Clinical presentation is often non-specific and leads to a delay in diagnosis. Imaging studies help to elucidate the condition. Despite advances in medical diagnostics and therapeutics, splenic abscesses can cause significant morbidity and can be fatal. Although splenectomy was considered the treatment of choice in the past, recent trends have seen a shift towards more conservative management. We present the clinical case of a patient who presented to our emergency room with a chief complaint of left shoulder and left upper quadrant abdominal pain. Abdominal imaging showed an intrasplenic collection suspicious for a hemorrhage or an abscess. Percutaneous drainage was successfully performed, followed by conservative management with intravenous antibiotics. The culture of the fluid drained from the spleen was positive for Salmonella Saintpaul. The patient improved and was discharged. A high degree of clinical suspicion is necessary for early identification of a splenic abscess. Splenectomy can be avoided with the use of interventional radiological drainage.

Performance comparison in Pediatric Fundamental Critical Care Support among staff from the USA versus those from resource-limited countries.

OBJECTIVE: This study aimed to evaluate the performance of participants in the USA compared with international participants taking the Pediatric Fundamental Critical Care Support (PFCCS) course, and the significance of training for resource-limited environments. METHODS: PFCCS courses were conducted in the USA, El Salvador, Haiti, Kenya, and Nepal between January 2011 and July 2013. All of the participants took pre- and post-tests. We compared the performance of these tests between international and USA participants. All participants answered a post-course survey to evaluate the didactic lectures and skill stations. RESULTS: A total of 244 participants took the PFCCS course, comprising 71 from the USA, 68 from Kenya, 37 from Haiti, 48 from Nepal, and 20 from El Salvador. The mean pre-test score of USA participants (50.6%) was significantly higher than that of international participants (44.7%). There was no significant difference in the post-test score between USA and international participants (78.6% versus 81.4%). There was a significant difference between pre- and post-test scores. There was better appreciation of the course content by the USA participants. CONCLUSION: International course takers without prior pediatric intensive care training have similar test scores to USA participants suggesting comparable efficacy.