COVID-19 Infection in Patients with Gaucher Disease

Aim: Coronavirus disease 2019 (COVID-19) is a severe acute respiratory syndrome with a high mortality rate and has been labeled a global pandemic in March 2020. Gaucher Disease (GD) is one of the rare inherited lysosomal storage diseases (LSDs). We aimed to call attention to the frequency, susceptibility of COVID-19 infection, and the factors that prevent this infection in patients with GD as compared to other LSDs. Material and Methods: The study was conducted retrospectively between September and December 2020. Participants were divided into two groups: GD group (19 patients) and the control group (19 patients, those with other LSDs). All patients were contacted by phone to collect data about their health status, and any possible contact with Covid-19 patients. Results: Six of the GD patients (36.8%) had contacted a confirmed COVID-19 infected person but only three (15.8%) had developed a mild COVID-19 with fever and fatigue that did not require hospital admission. Four of the control group patients (21.1%) had experienced contact with a person with a confirmed COVID-19 infection. Three of the control group patients, that comprised of patients with various LSDs other than GD (15.8%) were positive on COVID-19 PCR tests and two of them had developed a mild COVID-19 infection. One of these (with Mucopolysaccharidosis type 1) had severe symptoms and required hospitalization. Conclusion: There is no consensus on the management of rare diseases such as lysosomal storage diseases during the COVID-19 pandemic. Developing plans regarding the management of COVID-19 infections in LSDs will be useful when drawing up consensus guidelines.

Loneliness, internalizing symptoms, and inflammatory markers in adolescent COVID-19 survivors.

BACKGROUND: Concerns about the psychiatric sequelae after COVID-19 infection have increased as the pandemic spreads worldwide. The increase in self-isolation during this pandemic period has also revealed the importance of feelings of loneliness. This study aimed to examine the relationship between baseline inflammation levels, internalizing symptoms, and feelings of loneliness in adolescent COVID-19 survivors in the long term. METHODS: A total of 74 adolescents (41 girls, 55.4%, mean age 14.88) and their parents were included in the study. This cross-sectional study assessed internalizing symptoms via Revised Children's Anxiety and Depression Scale (RCADS) and feelings of loneliness using the UCLA-loneliness scale. Baseline inflammatory markers at COVID-19 diagnosis were collected. Logistic regression analysis was used to determine predictors for depression in adolescents. RESULTS: The most common disorder was Major Depressive Disorder (MDD) (25.7%), and 33.8% of the adolescents were in the clinical range in at least one internalizing domain. Baseline C-Reactive Protein (CRP) levels correlated weakly with MDD scores. Loneliness scores correlated with all internalizing symptoms, strong association with MDD scores. Loneliness, anxiety, and parental anxiety were associated with an increased likelihood of MDD. Baseline CRP positivity did not predict MDD in adolescent COVID-19 survivors. CONCLUSIONS: This study indicates that anxiety, loneliness, and parental anxiety play an important role in adolescents' experience of depressive symptoms after COVID-19 infection. Thus, screening parental psychopathology and loneliness in COVID-19 survivors seems to be preventive for adolescent mental health problems.

COVID-19 Enfeksiyonuna Bağlı Gelişen Serum Transaminaz Yüksekliği

Although COVID-19 was first described as a respiratory disease, current data has shown that it is a disease with multisystemic involvement including respiratory, cardiovascular, gastrointestinal, neurological, hematological and immune systems. COVID-19 associated liver injury may be due to various potential mechanisms. Direct viral cytotoxic effect, immun mediated injury, drugs, ischemic injury due to hypoxia-hypoperfusion are among these mechanisms. Here we present a five year-old male patient who had no known history of liver disease admitted to our clinic due to elevated transaminase during the course of COVID-19 infection.

High Serum Transaminase Due to COVID-19 Infection: Journal of Pediatric Infection

The liver can be damaged due to the virus's direct cytotoxic effect, immune-mediated damage, medication side effects, stasis due to myocardial damage, hypoxia, and ischemic damage due to hypoperfusion (6). The laboratory results were as follows: hemoglobin 14.3 g/dL, leukocytes 6.2 109/L, platelets 175 109/L (200-445 109), CRP 0.005 g/L (0-0.005), procalcitonin 0.04 pg/L, aspartate aminotransferase (AST) 393 U/L (0-46), alanine aminotransferase (ALT) 726 U/L (0-32), total bilirubin 0.5 mg/dL, direct bilirubin 0.2 mg/dL, lactate dehydrogenase (LDH) 548 U/L, gamma glutamyl transferase (GGT) 35 U/L. The biopsy, however, showed no virus particles. Since the patient had no known underlying disease, pathological findings in the liver were thought to be related to SARS-CoV-2 infection or the drugs used (11). According to reports, an increase in liver enzymes has no prognostic significance.

IMPACT OF COVID-19 PANDEMIC ON DELAY OF CHILDHOOD CANCER DIAGNOSIS AND THE OUTCOMES IN A PEDIATRIC HEMATOLOGY/ONCOLOGY DEPARTMENT

Objective Restriction of access to healthcare during COVID-19 pandemic is undoubtedly a major problem for patients with cancer. Although childhood cancers are highly curable, it is obvious that diagnostic and treatment disruptions will lead to poor Results. In this study we investigated the effects of pandemic on diagnosis and treatment delays of children with cancer along with their consequences. Methodology We searched all pediatric patients treated for cancer between March 2020 and January 2022 for COVID-19 infection. Data were collected collected from medical files of patients diagnosed with COVID-19, confirmed by polymerase chain reaction (PCR), who received active antineoplastic treatment. Results Fifty-eight patients developed COVID-19 infection at different stages of their anticancer treatment. Twenty-five had an asymptomatic COVID-19 infection, twenty-six had mild symptoms, three had moderate symptoms and four had severe disease. All of them recovered from COVID-19 infection. Chemotherapy courses were continued during active infection in four patients and interrupted in other patients. Conclusion While strict measures are required to control the pandemic, patients with severe critical illness such as cancer should be carefully evaluated and treatment delays that may have vital consequences should be avoided. In pediatric patients with cancer whom infected by COVID-19, continuation of anticancer treatment may be considered by evaluating the clinical status of the patient.

Multisystem inflammatory syndrome in children: clinical presentation, management, and short- and long-term outcomes.

OBJECTIVE: In this study, it was aimed to evaluate the demographic, clinical and laboratory characteristics of MIS-C patients in our hospital, to share our treatment approach, and to assess the outcomes of short- and long-term follow-up. METHODS: MIS-C patients who were admitted and treated in our hospital between July 2020 and July 2021 were evaluated. Demographic, clinical, laboratory, and follow-up data were collected from patient records retrospectively. RESULTS: A total of 123 patients with MIS-C (median age, 9.6 years) were included the study. Nineteen (15.4%) were mild, 56 (45.6%) were moderate, and 48 (39%) were severe MIS-C. High CRP, ferritin, pro-BNP, troponin, IL-6, and D-dimer values were found in proportion to the severity of the disease (p < 0.001, p < 0.001, p < 0.001, p < 0.001, p = 0.005, p < 0.001), respectively. Two (1.6%) patients died. The mean follow-up period was 7.8 months. Valve failure, left ventricular dysfunction/hypertrophy, coronary involvement, and pericardial effusion were the most common cardiac pathologies in the short- and long-term follow-up of the patients. In the long-term follow-up, the most common reasons for admission to the hospital were recurrent abdominal pain (14.2%), cardiac findings (14.2%), pulmonary symptoms (8%), fever (7.1%), neuropsychiatric findings (6.2%) and hypertension (3.5%). Neuropsychiatric abnormalities were observed significantly more common in severe MIS-C patients at follow-up (p = 0.016). In the follow-up, 6.2% of the patients required recurrent hospitalization. CONCLUSION: MIS-C is a serious and life-threatening disease, according to short-term outcomes. In addition to the cardiac findings of patients with MIS-C, long-term outcomes such as neuropsychiatric findings, persistent gastrointestinal symptoms, fever and pulmonary symptoms should be monitored. Key Points • In MIS-C patients, attention should be paid not only to cardiac findings, but also to symptoms related to other systems. • Patients should be followed up in terms of neuropsychiatric findings, persistent gastrointestinal symptoms, fever and pulmonary symptoms that may occur during follow-up.

Determination of Factors to Distinguish MIS-C from Acute Appendicitis in Children with Acute Abdominal Pain.

INTRODUCTION: The aim of this study was to make the differential diagnosis between acute appendicitis and multisystem inflammatory syndrome in children (MIS-C) for patients presenting with the complaint of acute abdominal pain (AAP) and to identify the determining factors for the diagnosis of MIS-C. MATERIALS AND METHODS: Eighty-one children presenting with AAP/suspected AAP were evaluated. Of these, 24 (29.6%) were included in the MIS-C group (MIS-C/g) and 57 were included in the suspected appendicitis group (S-A/g), which consisted of two subgroups: appendicitis group (A/g) and control observation group (CO/g). RESULTS: Comparing MIS-C/g, A/g, and CO/g, duration of abdominal pain (2.4, 1.5, 1.8 days), high-grade fever (38.8, 36.7, 37°C), severe vomiting, and severe diarrhea were higher in MIS-C/g. Lymphocytes count (LC) was lower, while values of C-reactive protein (CRP), ferritin, and coagulopathy were higher in MIS-C/g (p < 0.05). The optimal cutoffs for the duration of abdominal pain was 2.5 days; the duration of fever, 1.5 days; peak value of fever, 39°C; neutrophil count, 13,225 × 1,000 cell/µMoL; LC, 600 × 1,000 cell/µMoL; ferritin, 233 µg/L; and D-dimer, 16.4 mg/L (p < 0.05). The optimal cutoff for CRP was 130 mg/L (sensitivity 88.9, specificity 100%, positive predictive value 100%, NPV, negative predictive value 92.5%, p < 0.001). All patients in MIS-C/g tested positive by serology by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). CONCLUSION: The duration of abdominal pain, presence of high-grade and prolonged fever, and evaluation of hemogram in terms of high neutrophil count and low LC exhibit high sensitivity and negative predictive value for MIS-C presenting with AAP. In case of doubt, inflammatory markers such as CRP, ferritin, D-dimer, and serology for SARS-CoV-2 should be studied to confirm the diagnosis.