Comparison of acute phase reactant levels of Kawasaki disease patients who visited with less than 5 days duration of fever and with 5 days or longer

PURPOSE: Kawasaki disease (KD) is a common, acute systemic vasculitis in children. Acute phase reactants (APRs) have been used to assist diagnosis, and to predict outcome in children with KD. However, it remains unknown on levels of APRs depending on duration of fever. We aimed to compare APR levels of children with KD who visited with < 5 days duration of fever and with ≥ 5 days. METHODS: Children (≤ 15 years) with complete KD who visited the emergency department were enrolled from March 2012 through February 2018. The children were divided into the early (fever < 5 days) and late (fever ≥ 5 days) presenters. The baseline characteristics, APR levels, such as platelet count, and outcomes were compared between the 2 groups. RESULTS: A total of 145 children with complete KD were enrolled. Median age was 27.0 (interquartile range [IQR], 12.0–46.5) months, and boys accounted for 60.0%. The early presenters (63 [43.4%]) had a younger age (17.0 [IQR, 7.0–45.0] vs. 32.5 [IQR, 14.0–48.0] months; P = 0.006), shorter duration of fever (3.0 [IQR, 2.0–4.0] vs. 6.0 [IQR, 5.0–7.0] days; P < 0.001), and a lower platelet count (336.7 ± 105.2 [× 10³/µL] vs. 381.6 ± 121.8 [× 10³/µL], P = 0.02) than the late presenters. The other APR levels, and frequency of resistance to intravenous immunoglobulin and coronary artery abnormalities showed no differences between the 2 groups. CONCLUSION: Children with KD who visited with < 5 days duration of fever had a lower platelet count compared to those with ≥ 5 days. No differences were found in the other APR levels and the outcomes. It may be necessary to consider the differences in APR levels depending on duration of fever when treating children with KD.

Factors Associated with Removal of Impactted Fishbone in Children, Suspected Ingestion / 대한소아소화기영양학회지

PURPOSE: The management and clinical course in pediatric patients who had ingested foreign body were investigated retrospectively to evaluate the frequency and factor associated with successful removal of fishbone foreign body. METHODS: Based on the medical records of patients younger than 15 years old who visited emergency room because of foreign body ingestion from January 1999 to December 2012, the authors reviewed clinical characteristics including type of ingested foreign bodies, time to visits, managements and complications. RESULTS: Fishbone (50.1%) was the most common ingested foreign body in children. Among 416 patients with ingested fishbone, 245 (58.9%) were identified and removed using laryngoscope, rigid or flexible endoscope from pharynx or upper esophagus by otolaryngologists and pediatric gastroenterologists. The kind of ingested fish bone in children was diverse. The mean age of identified and removed fishbone group was 7.39 years old, and higher than that of unidentified fishbone group (5.81 years old, p<0.001). Identified and removed fishbone group had shorter time until hospital visit than the unidentified fishbone group (2.03 vs. 6.47 hours, p<0.001). No complication due to ingested fishbone or procedure occurred. CONCLUSION: Older age and shorter time from accident to hospital visit were the different factors between success and failure on removal of ingested fish bone in children.

Newly Detected PKHD1 Gene Mutation in a Newborn with Fatal Autosomal Recessive Polycystic Kidney Disease

Autosomal recessive polycystic kidney disease is among the most common inherited ciliopathies and is caused by mutations in the polycystic kidney and hepatic disease 1 (PKHD1) gene. Despite its great phenotypic variability, this condition is usually diagnosed during the neonatal and early infantile periods. We report a 37+3 -gestational-week neonate presenting with fatal autosomal recessive polycystic kidney disease who died at 28 hours of life from severe respiratory failure. The familial history is significant because a previous sibling died in utero at 24+2 weeks of gestational age and was diagnosed with polycystic kidney disease based on prenatal ultrasonography and autopsy. Our patient's autopsy revealed findings compatible with polycystic kidney disease. In addition, a PKHD1 gene study of peripheral blood leukocytes identified the compound heterozygote mutation c.274C>T(p.Arg92Trp), as well as the novel heterozygous nonsense mutation c.2770C>T(p.Gln924*).

Preliminary Study on Neurodevelopmental Outcome and Placental Pathology among Extremely Low Birth Weight Infants / 대한주산의학회잡지

PURPOSE: To investigate the relationship between placental pathology and neurodevelopmental outcomes among extremely low birth weight (ELBW) infants. METHODS: Pathology of placentas from ELBW infants born at a tertiary neonatal intensive care unit from January 2007 to December 2012 were reviewed and placental histology was grouped into 3 categories by a designated pathologist: acute chorioamnionitis (ACA), maternal vascular underperfusion (MVU), and control group. Matched ELBW infants were tested for significant neurodevelopmental delays defined as mental developmental index (MDI) or psychomotor developmental index (PDI) <70, using Bayley Scales of Infant Development-II (BSID-II). RESULTS: The mean gestational age and birth weight of 175 infants were 27.1+/-2.5 weeks and 764.7+/-152.3 g respectively. Placental histology revealed MVU (48.0%), ACA (25.1%) and control (26.9%) in distribution. There were less significant patent ductus arteriosus in MVU group than in control group [adjusted odds ratio (OR)=0.331, P=0.011]. The frequencies of other neonatal diseases and mortality were similar in 3 groups. Sixty four of 175 infants were examined for BSID-II at mean corrected 19.9+/-3.2 months. MVU was associated with significant mental developmental delay (OR=5.185, P=0.036), but after adjustment for head circumference/weight at birth, the statistically significance of association disappeared (adjusted OR=4.391, P=0.075). ACA did not affect neonatal and neurodevelopmental outcomes. CONCLUSION: The result of placenta biopsy could be a useful tool in counseling parents for future neurodevelopmental outcome, however, further studies are required to define definitive association in between placenta biopsy and neurodevelopmental outcomes.

Early Sodium and Fluid Intake and Severe Intraventricular Hemorrhage in Extremely Low Birth Weight Infants

Hypernatremic dehydration is an important cause of intracranial hemorrhage. A possible association of intraventricular hemorrhage (IVH) with hypernatremia and/or high sodium intake has been suggested in preterm infants. To investigate the associations of early fluid and sodium intake or serum sodium concentrations with severe intraventricular hemorrhage (IVH) in extremely low birth weight (ELBW) infants, we reviewed the medical records of 169 inborn ELBW infants. Daily fluid and sodium intake, urine output, weight loss and serum sodium concentration during the first 4 days of life were obtained. Patients were divided into the severe IVH (grade 3/4) and the control (no or grade 1/2 IVH) group. The maximum serum sodium concentration and the incidence of hypernatremia did not differ between the two groups. Related to the fluid balance and sodium intake, the risk for severe IVH was strongly associated with total fluid and sodium intake during the initial four days of life. With respect to the fluids other than transfusion, severe IVH can be discriminated only by sodium intake but not by fluid intake. Large randomized controlled trials are required to clarify the causal relationship between the early sodium intake and severe IVH in ELBW infants.

Early Sodium and Fluid Intake and Severe Intraventricular Hemorrhage in Extremely Low Birth Weight Infants

Hypernatremic dehydration is an important cause of intracranial hemorrhage. A possible association of intraventricular hemorrhage (IVH) with hypernatremia and/or high sodium intake has been suggested in preterm infants. To investigate the associations of early fluid and sodium intake or serum sodium concentrations with severe intraventricular hemorrhage (IVH) in extremely low birth weight (ELBW) infants, we reviewed the medical records of 169 inborn ELBW infants. Daily fluid and sodium intake, urine output, weight loss and serum sodium concentration during the first 4 days of life were obtained. Patients were divided into the severe IVH (grade 3/4) and the control (no or grade 1/2 IVH) group. The maximum serum sodium concentration and the incidence of hypernatremia did not differ between the two groups. Related to the fluid balance and sodium intake, the risk for severe IVH was strongly associated with total fluid and sodium intake during the initial four days of life. With respect to the fluids other than transfusion, severe IVH can be discriminated only by sodium intake but not by fluid intake. Large randomized controlled trials are required to clarify the causal relationship between the early sodium intake and severe IVH in ELBW infants.

Uric Acid as a Predictor of Severe Intraventricular Hemorrhage in Very Low Birth Weight Infants

PURPOSE: To determine whether serum uric acid levels in the first 7 days of life can predict development of severe intraventricular hemorrhage (IVH) among very low birth weight (VLBW) infants. METHODS: VLBW infants admitted to the neonatal intensive care unit of Asan Medical Center between January 2009 and December 2012 were selected for chart review. Infants were divided into groups with and without severe IVH (grade> or =3). To determine whether uric acid is a predictor of severe IVH, uric acid levels on the first day (within 24 hours of birth), peak uric acid levels (during the first 7 days for infants without severe IVH, prior to IVH documentation by cranial sonogram for infants with severe IVH, and trend in uric acid levels were analyzed for both groups. Various antenatal and postnatal factors were compared between the groups, and risk factors associated with severe IVH were identified. RESULTS: A total of 397 VLBW infants were included, with mean birth weight of 1,075+/-292 g and a mean gestational age of 29.6+/-3.3 weeks. Higher levels of uric acid on day 1, higher peak levels, and rising uric acid levels were all found to be associated with the development of severe IVH on univariate analysis. Multivariate analysis confirmed that rising uric acid levels predicted subsequent development of severe IVH. Other factors associated with development of severe IVH included higher sodium, higher potassium, higher PaCO2, higher lactic acid, and lower PaO2. CONCLUSION: Careful attention to uric acid levels, which are easily measured, may be useful in predicting subsequent development of severe IVH among VLBW infants.

Continuous Renal Replacement Therapy in the Neonatal Intensive Care Unit: A Single-Center Study

PURPOSE: Continuous renal replacement therapy (CRRT) has become an essential modality for the care of critically ill pediatric patients who require renal support. However, experience with CRRT in the neonatal population is not common in Korea. In this study, we aimed to investigate the clinical features, outcomes, and complications of CRRT in neonates in a single neonatal intensive care unit (NICU). METHODS: We reviewed the medical records of 17 neonates who underwent CRRT at a NICU of a tertiary hospital. The data included demographic characteristics, diagnosis, complications, and laboratory and CRRT parameters. RESULTS: The median age at initiation of CRRT was 6 days after birth. All patients were treated with CRRT in continuous venovenous hemodiafiltration mode, with a median treatment duration of 57 hours. The main indication for CRRT was an inborn error of metabolism (IEM), followed by congenital renal disease and multiorgan failure. In patients with an IEM, the median plasma ammonia level at the CRRT initiation was 1,232 micromol/L, and the mean duration until the ammonia level decreased to half of the peak ammonia level was 7.3+/-2.5 hours. The overall hospital mortality rate was 41.2%. The outcomes of the 10 survivors after discharge included death (n=2), loss to follow-up (n=3), and survival with developmental delay (n=4). CONCLUSION: Although CRRT was effective in lowering the plasma ammonia level of neonates with IEM, the associated mortality and morbidity were high. Hence, further studies are needed to optimize the CRRT protocol and to establish an effective patient referral system in Korea.

Neonatal Late-onset Hypocalcemia: Is There Any Relationship with Maternal Hypovitaminosis D? / 대한소아소화기영양학회지

PURPOSE: Neonatal late-onset hypocalcemia is defined as hypocalcemia developed after postnatal 3 days and associated with hypoparathyroidism, high phosphate diets and vitamin D deficiency. We experienced the increment of neonatal late onset hypocalcemia over 1 year. We tried to evaluate the relationship between late onset hypocalcemia and maternal hypovitaminosis D. METHODS: The medical records in the neonates with late-onset hypocalcemia during January 2007 to July 2008 were retrospectively reviewed. Among those patients, 17 paired sera of mothers and neonates had collected. The levels of 25-OH vitamin D (25OHD) and intact parathyroid hormone (iPTH) were measured and were compared with neonate and the mother. RESULTS: The mean gestational age was 38+1 weeks, and the mean body weight was 2,980 g. The onset time of hypocalcemia was 5.9 days of age. Most of them (88.2%) were feeding with formula and no one was only breast milk feeding. Of the 17 patients, 13 were born in spring or in winter. The median levels of calcium, phosphorus, alkaline phosphatase, iPTH and 25OHD were 7.0 mg/dL, 8.6 mg/dL, 191.0 U/L, 57.2 pg/mL and 24.0 ng/mL in neonates. The levels of 25OHD of 6 neonates were <20 ng/mL. A total of 16 mothers were considered vitamin D-deficient (<20 ng/mL), and vitamin D insufficient (20<25OHD<30 ng/mL). CONCLUSION: Neonatal late-onset hypocalcemia in our study seems to be influenced by maternal vitamin D deficiency and insufficiency. Sun tanning and vitamin D supplements from winter to spring would be helpful to prevent maternal vitamin D deficiency, one of the causes of neonatal late-onset hypocalcemia.

Helicobacter pylori Infection and Duodenal Gastric Metaplasia in Healthy Young Adults / 대한소화기학회지

BACKGROUND/AIMS: Duodenal ulcers occur relatively frequently in adolescents, like in adults, and may relate to Helicobacter pylori infection and duodenal gastric metaplasia (DGM). This study investigated the association between H. pylori infection and DGM in healthy adults aged 20-29. METHODS: Between 1995 and 2005, endoscopic biopsies of the duodenum, antrum and body were taken from healthy, young volunteers, who were first-year medical students, faculty staff, residents, and research assistants of Gyeongsang National University in Jinju, Korea. Urease tests were performed and the extent of DGM and histopathological grades according to the Updated Sydney System were determined. RESULTS: In total, 662 subjects were enrolled (429 males and 233 females). The median age was 22.3 years. The overall incidence of DGM was 11.5% but DGM was more frequent in males (15.4%) than in females (4.3%) (p<0.0001). While H. pylori positivity rates changed significantly during the 1995-2005 period (p<0.01), the incidences of DGM did not. DGM was observed in 7.2% and 14.9% of subjects who were and were not colonized with H. pylori, respectively. DGM was also associated with less severe chronic gastritis and the absence of active gastritis in both the antrum and body, and the absence of follicles in the antrum (p<0.05). CONCLUSIONS: These findings suggested that DGM is not rare in healthy young adults and is unrelated to gastric H. pylori infection.

A Case of Idiopathic Congenital Neonatal Cholestasis in a Patient with Down Syndrome / 대한소아소화기영양학회지

Down syndrome is a rare cause of neonatal cholestasis. Neonatal cholestasis in a patient with Down syndrome is usually associated with severe liver diseases, such as neonatal hemochromatosis, myeloproliferative disorder and intrahepatic bile duct paucity. We experienced a case of idiopathic neonatal cholestasis in a patient with Down syndrome, which resolved spontaneously.

Regional outbreak of staphylococcal scalded skin syndrome in healthy children / 소아과

PURPOSE: Staphylococcal scalded skin syndrome (SSSS) is a relatively uncommon superficial blistering skin disease that is due to Staphylococcus aureus. We had experienced a regional outbreak of SSSS over 3 years in healthy children. METHODS: We retrospectively reviewed the medical records of those patients diagnosed as SSSS. Most of neonatal cases were nosocomial infections and excluded from the analysis. The clinical features, laboratory findings, the isolation and antibiotic resistance of S. aureus, the antibiotic management and other supportive treatments were analyzed. RESULTS: Fifty-five patients with SSSS were admitted to our hospital from October 2001 to September 2004. The median age of patients was 3.0 years. Of the 55 patients, 9 were the generalized type, 13 were the intermediate type and 33 were the scarletiniform rash. All the patients were living in neighborhood of the Jinju area. S. aureus were isolated from 9 of the patients and all of the isolated S. aureus were methicillin resistant. All the patients except two were treated with intravenous flocloxacillin or nafcillin and/or cefotaxime. All the patients recovered during the follow-up period of 2 to 3 weeks. CONCLUSION: We experienced a regional outbreak of SSSS in previous healthy children. Further study for finding the carriers of S. aureus caused SSSS and preventing the spread of this disease is needed. Additionally, guidelines for treating SSSS due to methicillin resistant S. aureus should be established.

Kawasaki Disease Presenting as Parotitis in a 3-Month-Old Infant

A male infant aged 3 months and 1 week had persistently high fever with parotitis that was unresponsive to antibiotics. Mumps was identified by serologic study, but he was finally diagnosed by clinical features as having Kawasaki disease and echocardiographic findings on the 9th day of fever. Parotitis, which is unresponsive to antibiotics, should be considered Kawasaki disease even though typical symptoms are not present.

A Case of Encephalitis Caused by Epstein-Barr Virus Infection / 대한소아신경학회지

An 11-year-old girl was diagnosed with encephalitis due to Epstein-Barr virus(EBV). She was admitted to our hospital due to convulsion and decreased consciousness after several days of fever, sore throat and headache. Cerebrospinal fluid analysis showed lymphocyte-dominant pleocytosis and markedly elevated levels of protein. Magnetic resonance imaging(MRI) finding was normal, except for marked leptomeningeal enhancement. Even though she had signs and symptoms of infectious mononucleosis, EBV infection could not be considered as the underlying cause of the encephalitis until IgM for the viral capsid antigen of EBV had been detected in her blood. After treatment with acyclovir, a high dose of methylprednisolone and intravenous immunoglobulin, the neurological symptoms improved rapidly. She has not suffered from any neurological complications in the four months since being discharged. EBV infection should be considered as the main etiology in cases of childhood encephalitis, although there is no evidence of infectious mononucleosis existed.