Macrophage activation syndrome in neonatal lupus presenting with fever and rash

Neonatal lupus can occur in infants born to mother with autoimmune disorders through transplacental auto-antibodies. Clinical manifestations in neonatal lupus include cutaneous lesions and hematologic or hepatobiliary findings resembling those seen in systemic lupus erythematosus. In autoimmune state, macrophage activation syndrome (MAS) represent a critical and potentially fatal complication that can result in mortality if not immediately identified and managed with the appropriate care. Here we present a 33-day-old girl diagnosed with neonatal lupus and serious MAS. She was delivered by a primipara mother who did not exhibit any autoimmune symptoms. The patient visited the hospital due to fever and pancytopenia. Laboratory data were compatible with MAS, including pancytopenia, high level of ferritin, soluble interleukin-2, and decreased natural killer cell activity. In addition, autoimmune study showed positive results for anti-nuclear antibody (ANA), anti-Sjogren syndrome antigen A (SSA), and SSB, The autoimmune study for mother also showed positive results for ANA, anti-SSA, and SSB. The patient recovered after she received high dose steroid and supportive care. Our case indicates that neonatal lupus should be taken into consideration when fever, erythematous skin rash, and pancytopenia are observed in infants, even if their mothers have no prior history of autoimmune conditions.

A Pediatric Case of Bannayan–Riley–Ruvalcaba Syndrome with Recurrent Iron Deficiency Anemia / 임상소아혈액종양

We present a case of Bannayan-Riley-Ruvalcaba syndrome (BRRS) diagnosed during the treatment of recurrent iron deficiency anemia (IDA). IDA is the most common hematological disorder in children and is often caused by dietary problem or obesity.However, in relatively rare cases, it is caused by gastrointestinal (GI) disease. BRRS is a rare genetic disorder characterized by macrocephaly, multiple noncancerous tumors, intestinal hamartomatous polyps, and penile freckling due to a defect in the PTEN gene. GI polyps can cause chronic GI bleeding, leading to IDA development.We recommend active GI evaluation, including esophagogastroduodenoscopy, for children with recurrent IDA.

Three Case Reports of Pediatric Collagenous Gastritis with Recurrent Iron Deficiency Anemia / 임상소아혈액종양

Iron deficiency is the most common nutritional deficiency in children and adolescents. Pediatric collagenous gastritis (CG) is a rare gastrointestinal disorder. Abdominal pain associated with iron deficiency anemia (IDA) is a common clinical symptom.We present three cases of pediatric CG diagnosed during the treatment of recurrent IDA. During esophagogastroduodenoscopy (EGD), nodularity of the gastric corpus was observed, which is a characteristic finding in pediatric patients with CG.Although pediatric CG could not be diagnosed by general EGD biopsy and pathological examination, it was diagnosed after suspecting pediatric CG and performing appropriate diagnostic tests. We recommend EGD for pediatric patients with recurrent IDA, especially for those with gastrointestinal symptoms. Moreover, when performing EGD, conducting appropriate examinations for pediatric CG is necessary.

Proven Cytomegalovirus Colitis Associated with Dasatinib Administration in Two Pediatric Allogeneic Hematopoietic Stem Cell Transplantation Recipients / 대한소아소화기영양학회지

Gastrointestinal (GI) bleeding is a rare adverse event of dasatinib, which is known to be caused by dasatinib-induced colitis, severe thrombocytopenia, and platelet dysfunction.We present two cases of pediatric patients who developed hematochezia during treatment with dasatinib after hematopoietic stem cell transplantation (HSCT). A colonic tissue biopsy was performed to differentiate the cause of GI bleeding. Both patients were diagnosed with proven cytomegalovirus (CMV) colitis, but only one was treated with ganciclovir. The patient who did not receive antiviral therapy experienced recurrent GI bleeding during dasatinib administration, leading to multiple treatment interruptions. During dasatinib therapy after HSCT, patients with GI bleeding and confirmed CMV colitis may benefit from antiviral therapy to reduce interruptions in dasatinib therapy.

Changes in the Incidence of Intussusception and Infectious Diseases After the COVID-19 Pandemic in Korea

Background@#Intussusception refers to the invagination of a part of the intestine into itself. The exact cause for this condition is unknown in most cases. The active implementation of coronavirus disease 2019 (COVID-19) infection control guidelines has reduced the spread of COVID-19 and the incidence of other infectious diseases in children. The current study aimed to identify changes in pediatric intussusception and infectious diseases after the implementation of infection control guidelines and confirm the association between intussusception and contagious diseases. @*Methods@#We analyzed the electronic medical records of pediatric patients diagnosed with intussusception from seven hospitals in Korea between January 2017 and December 2020.We used open data from the Korea Disease Control and Prevention Agency to investigate changes in infectious diseases over the same period. @*Results@#Altogether, we evaluated 390 children with intussusception. There was a statistically significant decrease in the incidence of monthly visits with intussusception in the COVID-19 period group (9.0 vs. 3.5, P < 0.001). When the monthly incidence of infectious diseases was compared between the pre-COVID-19 and the COVID-19 periods, a statistically significant decrease in respiratory viruses (7979.0 vs. 815.2, P < 0.001), enterovirus infection (262.2 vs. 6.6, P < 0.001), and viral enteritis (916.2 vs. 197.8, P< 0.001) were confirmed in the COVID-19 period. Through interrupted time series analysis, it was confirmed that the incidence of intussusception and viral infectious diseases have drastically decreased since March 2020, when COVID-19 infection control guidelines were actively implemented. @*Conclusion@#We confirmed that implementing infection control guidelines during the COVID-19 pandemic resulted in a decrease in intussusception and viral infectious diseases.Through this result, it was possible to indirectly confirm the existing hypothesis that viral infections play a significant role in the pathophysiologic mechanism of intussusception.

Usefulness of Endoscopic Hill Grade in Evaluating Children Suspected of Having Gastroesophageal Reflux Disease

Background/Aims@#This study aim to evaluate the relationship between the Hill grade confirmed by esophagogastroduodenoscopy (EGD) and the degree of gastroesophageal reflux (GER) by 24-hour multichannel intraluminal impedance-pH monitoring (MII-pH) in children suspected of having gastroesophageal reflux disease (GERD). @*Methods@#A retrospective review of 105 children and adolescents who underwent EGD and MII-pH for the evaluation of GERD from March 2013 to July 2019 was performed. Clinical features and results of EGD and 24-hour MII-pH were collected and statistically analyzed. @*Results@#Hill grades 1, 2, 3, and 4 were identified using EGD in 56 (53.3%), 22 (22.0%), 16 (15.2%), and 11 (10.5%) patients, respectively. As the Hill grade increased, the proportion of neurological diseases (P < 0.001) and endoscopic erosive esophagitis (P < 0.001) increased significantly. The acid exposure index, bolus exposure index, number of reflux episodes, and number of GER reaching proximal extent on MII-pH increased significantly as the endoscopic Hill grade increased (all P < 0.001). Linear regression analysis revealed an increase in the Hill grade by 1 increased the acid exposure index by 2.0%, bolus exposure index by 0.7%, number of reflux episodes by 18.9 episodes, and the number of GER reaching the proximal esophagus increased by 10.5 episodes on average (all P < 0.001). @*Conclusions@#Hill grade on EGD was associated with GER in children. Estimating the degree of GER by applying Hill grade in the retroflexion view may be useful in practice when evaluating children suspected with GERD.

Usefulness of Endoscopic Hill Grade in Evaluating Children Suspected of Having Gastroesophageal Reflux Disease

Background/Aims@#This study aim to evaluate the relationship between the Hill grade confirmed by esophagogastroduodenoscopy (EGD) and the degree of gastroesophageal reflux (GER) by 24-hour multichannel intraluminal impedance-pH monitoring (MII-pH) in children suspected of having gastroesophageal reflux disease (GERD). @*Methods@#A retrospective review of 105 children and adolescents who underwent EGD and MII-pH for the evaluation of GERD from March 2013 to July 2019 was performed. Clinical features and results of EGD and 24-hour MII-pH were collected and statistically analyzed. @*Results@#Hill grades 1, 2, 3, and 4 were identified using EGD in 56 (53.3%), 22 (22.0%), 16 (15.2%), and 11 (10.5%) patients, respectively. As the Hill grade increased, the proportion of neurological diseases (P < 0.001) and endoscopic erosive esophagitis (P < 0.001) increased significantly. The acid exposure index, bolus exposure index, number of reflux episodes, and number of GER reaching proximal extent on MII-pH increased significantly as the endoscopic Hill grade increased (all P < 0.001). Linear regression analysis revealed an increase in the Hill grade by 1 increased the acid exposure index by 2.0%, bolus exposure index by 0.7%, number of reflux episodes by 18.9 episodes, and the number of GER reaching the proximal esophagus increased by 10.5 episodes on average (all P < 0.001). @*Conclusions@#Hill grade on EGD was associated with GER in children. Estimating the degree of GER by applying Hill grade in the retroflexion view may be useful in practice when evaluating children suspected with GERD.

Fecal Calprotectin as a Useful Non-Invasive Screening Marker for Eosinophilic Gastrointestinal Disorder in Korean Children

Background@#Eosinophilic gastrointestinal disorder (EoGID) is an emerging disease condition in Korean children, but its diagnosis requires invasive endoscopic biopsies. Fecal calprotectin (FCal) is a noninvasive biomarker for intestinal inflammation to differentiate organic gastrointestinal diseases from functional abdominal pain disorder. This study aimed to evaluate the diagnostic accuracy of FCal and to determine the optimal cutoff to differentiate EoGID from functional abdominal pain disorder. @*Methods@#A total of 253 children (122 boys, 131 girls; mean age 12.2 ± 3.6, range 2.9–17.8 years) who underwent endoscopy with biopsies for chronic gastrointestinal symptoms were recruited, except for 38 children diagnosed with inflammatory bowel disease, and divided into EoGID (n = 67) and functional abdominal pain disorder (n = 186). FCal, white blood cell (WBC) counts, eosinophil counts, erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP) levels were measured in all subjects at initial diagnosis. @*Results@#FCal levels weakly correlated with WBC (r = 0.127, P = 0.044) and CRP (r = 0.126, P = 0.040) but not with ESR and eosinophil count. FCal levels were significantly higher in the EoGID group than in the functional abdominal pain disorder group (mean 179.5 ± 242.9 mg/kg vs. 44.3 ± 68.1 mg/kg; P 0.05). An optimal cutoff of FCal 73.2 mg/kg distinguished EoGID from functional abdominal pain disorder with a sensitivity of 50.7% and a specificity of 84.6%. @*Conclusion@#FCal is a useful and reliable noninvasive marker for differentiating EoGID from functional abdominal pain disorder in Korean children with chronic gastrointestinal symptoms when optimal cutoffs are applied.