Thrombotic Thrombocytopenic Purpura as the First Symptom of Systemic Lupus Erythematosus: A Case Report with Review of Literature / 임상소아혈액종양

Thrombotic thrombocytopenic purpura (TTP) can cause serious morbidity and mortality, and differentiating between this disease and systemic lupus erythematosus (SLE) can prove challenging. Although rare, TTP accompanied by SLE is linked to several complications and a higher mortality rate. Herein, we report a case of a 16-year-old boy who presented with systemic symptoms, such as petechiae, and was diagnosed with acquired TTP following a laboratory test. Steroid treatment was initiated and a diagnosis of SLE was reached after the symptoms had improved. Treatment with low-dose prednisone in addition to hydroxychloroquine was continued. The patient did not develop renal failure or neurologic deficit. No specific symptoms were observed after treatment and during the follow-up period. Early treatment of SLE is crucial, but it is difficult to reach an early diagnosis because the symptoms are similar to those of TTP. In the current study, an early diagnosis of TTP led to prompt treatment, thereby avoiding the fatal symptoms that could be caused by SLE.

Analysis of Clinical Manifestations and Laboratory Findings in Children with Influenza B-Associated Myositis: A Single Center Study / 가정의학회지

BACKGROUND: Influenza-associated myositis (IAM) is a rare and poorly recognized complication of influenza infection in children, and is characterized by acute onset of severe pain in the lower extremities and a refusal to ambulate walk. We sought to understand the association between IAM and influenza B infection and to investigate its clinical and laboratory characteristics in affected children. METHODS: Influenza B-associated myositis (IBAM) cases diagnosed in the pediatrics department of Wonkwang University Hospital from January 2010 and March 2016 were analyzed retrospectively. RESULTS: Medical records of affected children were examined, and clinical characteristics and laboratory findings were recorded. Of the 536 children diagnosed with influenza B infection, 47 children complained of bilateral calf pain with or without gait disturbance. All children exhibited elevated serum aspartate aminotransferase (AST) level. The median serum creatine kinase (CK) and lactate dehydrogenase (LDH) levels, reportedly elevated in myositis, were 2,597 IU/L and 678 IU/L, respectively. While the immunofluorescence test results were negative for some patients, the polymerase chain reaction test results indicated influenza B infection in all 47 children. At the time of hospital discharge, the patients' symptoms had resolved, and their CK levels had improved. CONCLUSION: IBAM was generally benign and short, and although the blood AST, CK, and LDH levels were markedly high, the erythrocyte sedimentation rate and C-reactive protein levels were normal. Further, the duration of IBAM symptoms correlated with the duration of fever. The IBAM-associated clinical and laboratory findings are highly characteristic and may allow its rapid diagnosis during the influenza season.

A Case of Mesenteric Cyst in a 4-Year-Old Child with Acute Abdominal Pain / 대한소아소화기영양학회지

Mesenteric cysts are rare intra-abdominal lesions occurring during childhood, which were first described in 1507. Cases of mesenteric cysts have been continuously reported, but these cases were very small in number. They are often asymptomatic and incidentally found while patients are undergoing work-up or receiving treatment for other conditions such as appendicitis, small-bowel obstruction, or diverticulitis; however, patients may still have lower abdominal pain and symptoms that are frequently associated with other abdominal conditions. The symptoms are variable and non-specific, including pain (82%), nausea and vomiting (45%), constipation (27%), and diarrhea (6%). An abdominal mass may be palpable in up to 61% of patients. We are to report the clinical course and literature of a child with mesenteric cysts who complained of acute abdominal pain, distension, and vomiting and were surgically treated after being diagnosed with mesenteric cysts based on radiological examination.

A Case of Mesenteric Cyst in a 4-Year-Old Child with Acute Abdominal Pain / 대한소아소화기영양학회지

Mesenteric cysts are rare intra-abdominal lesions occurring during childhood, which were first described in 1507. Cases of mesenteric cysts have been continuously reported, but these cases were very small in number. They are often asymptomatic and incidentally found while patients are undergoing work-up or receiving treatment for other conditions such as appendicitis, small-bowel obstruction, or diverticulitis; however, patients may still have lower abdominal pain and symptoms that are frequently associated with other abdominal conditions. The symptoms are variable and non-specific, including pain (82%), nausea and vomiting (45%), constipation (27%), and diarrhea (6%). An abdominal mass may be palpable in up to 61% of patients. We are to report the clinical course and literature of a child with mesenteric cysts who complained of acute abdominal pain, distension, and vomiting and were surgically treated after being diagnosed with mesenteric cysts based on radiological examination.

Autoimmune Hemolytic Anemia after Intravenous Immunoglobulin Therapy in a Child with Kawasaki Disease / 임상소아혈액종양

Kawasaki disease (KD) can cause acquired heart disease and systemic vasculitis in children. It is treated with intravenous immunoglobulin (IVIG). A significant complication is development of coronary artery lesions such as dilatations or aneurysms. However, uncommon complications can occur, like autoimmune hemolytic anemia when IVIG is used. We present a case of autoimmune hemolytic anemia associated with KD. Dilatation of right coronary artery was found at echocardiography and he was treated twice with IVIG (2 g/kg) with interval of 48 hours. Laboratory finding showed hemoglobin 7.1 g/dL, hematocrit 20.8%, corrected reticulocyte 5.86%, total bilirubin 0.29 mg/dL, lactate dehydrogenase 425 IU/L, and haptoglobin 5 mg/dL. Normocytic, normochromic anemia with anisopoikilocytosis was found on peripheral blood smear, and direct antiglobulin test was positive. The patient was started on oral prednisolone for 3 weeks, with which all symptoms resolved. We report this rare case, prompting consideration of IVIG associated complications when treating KD.

Autoimmune Hemolytic Anemia after Intravenous Immunoglobulin Therapy in a Child with Kawasaki Disease / 임상소아혈액종양

Kawasaki disease (KD) can cause acquired heart disease and systemic vasculitis in children. It is treated with intravenous immunoglobulin (IVIG). A significant complication is development of coronary artery lesions such as dilatations or aneurysms. However, uncommon complications can occur, like autoimmune hemolytic anemia when IVIG is used. We present a case of autoimmune hemolytic anemia associated with KD. Dilatation of right coronary artery was found at echocardiography and he was treated twice with IVIG (2 g/kg) with interval of 48 hours. Laboratory finding showed hemoglobin 7.1 g/dL, hematocrit 20.8%, corrected reticulocyte 5.86%, total bilirubin 0.29 mg/dL, lactate dehydrogenase 425 IU/L, and haptoglobin 5 mg/dL. Normocytic, normochromic anemia with anisopoikilocytosis was found on peripheral blood smear, and direct antiglobulin test was positive. The patient was started on oral prednisolone for 3 weeks, with which all symptoms resolved. We report this rare case, prompting consideration of IVIG associated complications when treating KD.

The usefulness of soluble transferrin receptor in the diagnosis and treatment of iron deficiency anemia in children / 소아과

PURPOSE: Soluble transferrin receptor (sTfR) is a truncated extracellular form of the membrane transferrin receptor produced by proteolysis. Concentrations of serum sTfR are related to iron status and erythropoiesis in the body. We investigated whether serum sTfR levels can aid in diagnosis and treatment of iron deficiency anemia (IDA) in children. METHODS: Ninety-eight patients with IDA were enrolled and were classified according to age at diagnosis. Group 1 comprised 78 children, aged 6-59 months, and group 2 comprised 20 adolescents, aged 12-16 years. RESULTS: In group 1, patients' serum sTfR levels correlated negatively with mean corpuscular volume; hemoglobin (Hb), ferritin, and serum iron levels; and transferrin saturation and positively with total iron binding capacity (TIBC) and red cell distribution width. In group 2, patients' serum sTfR levels did not correlate with ferritin levels and TIBC, but had a significant relationship with other iron indices. Hb and serum sTfR levels had a significant inverse relationship in both groups; however, in group 1, there was no correlation between Hb and serum ferritin levels. In 30 patients of group 1, serum sTfR levels were significantly decreased with an increase in Hb levels after iron supplementation for 1 month. CONCLUSION: Serum sTfR levels significantly correlated with other diagnostic iron parameters of IDA and inversely correlated with an increase in Hb levels following iron supplementation. Therefore, serum sTfR levels can be a useful marker for the diagnosis and treatment of IDA in children.

Fulminant Clinical Course on Listeria Sepsis in Preterm and Late Preterm Neonates / 대한주산의학회잡지

Listeria monocytogenes is a facultative anaerobic, gram-positive bacillus. Listeria is commonly found in processed and prepared foods and listeriosis is associated with high morbidity and mortality. This bacterium is an important cause of severe infection in neonates and pregnant women. Pregnant women are at high risk for listeriosis, but symptoms are non-specific and diagnosis is difficult. Neonatal Listeriosis is generally a severe and fatal disease. There are two forms of the disease in the neonate, early- and late-onset, suggesting different modes of transmission. Generally, early detection and empirical treatment of listeriosis help to improve survival and prognosis. We explained two cases of L. monocytogenes sepsis with culture-proven in preterm and late preterm newborns from asymptomatic mothers. These neonates had fulminant clinical course even if standard antibiotics treatments were administrated.

Hematologic Complication of Respiratory Virus Infection / 소아감염

OBJECTIVE: Cytopenia is a common hematologic complication of viral infections. However, information regarding hematologic effects of common respiratory virus infections is scarce. This study aimed to evaluate hematologic complications and the clinical course of patients infected with common respiratory viruses. METHODS: We retrospectively analyzed 496 patients with respiratory tract infections admitted to the Department of Pediatrics, Wonkwang University Hospital from November 2011 to March 2012 using multiplex real-time polymerase chain reaction to detect the presence of respiratory viruses and hematologic abnormalities. RESULTS: Respiratory viruses were identified in 379 patients. Respiratory syncytial virus (RSV) was most frequently detected (55.7%), followed by influenza A (Flu-A, 23.0%). Further, cytopenia was observed in 35.5% of RSV-infected patients, 25.0% of Flu-A-infected patients, and 34% of patients infected by other viruses. Each virus caused a decrease in 3 blood cell component values, which corresponded with cytopenia frequency. Of the 379 infected patients, 83 had anemia (9.71+/-1.09 g/dL); 46 had neutropenia (803.70+/-263.09 cells/mm3); and 23 had transient thrombocytopenia (142,434.78+/-86,835.18 cells/mm3). However, no patient required treatment. A comparison of clinical characteristics between RSV- and Flu-A-positive patients with anemia revealed that RSV-infected patients had significantly longer duration of hospitalization. RSV was detected more commonly in young neutropenic patients, who had a shorter duration of fever. CONCLUSIONS: Our findings suggest that infections, particularly RSV and Flu-A, result in varying degrees of cytopenia, which usually improves without treatment and does not affect the clinical course of the infection.

A Case of Acute Myopericarditis Associated With Mycoplasma Pneumoniae Infection in a Child

Mycoplasma pneumoniae (M. pneumoniae) primarily causes respiratory tract infections in persons aged 5-20 years. Tracheobronchitis and bronchopneumonia are the most commonly recognized clinical symptoms associated with M. pneumoniae infection. Complications of this infection are unusual; in particular, cardiac involvement is very rare and is generally accompanied by pneumonia. Nonrespiratory illness can therefore involve direct invasion by M. pneumoniae or autoimmune mechanisms, as suggested by the frequency of cross reaction between human antigens and M. pneumoniae. Herein, we report a case of severe acute myopericarditis with pneumonia caused by M. pneumoniae in a healthy young child who presented with fever, lethargy, oliguria and dyspnea. She survived with aggressive therapy including clarithromycin, intravenous immunoglobulin, inotropics, and diuretics. The patient was discharged on the 19th day after admission and followed up 1 month thereafter at the outpatient clinic without sequelae.

Relationship between iron deficiency anemia and febrile convulsion in infants / 소아과

PURPOSE: The association between iron deficiency anemia and febrile convulsion in infants has been examined in several studies with conflicting results. Therefore, the authors aimed to evaluate the precise relationship involved. METHODS: In this case-control study, the authors assessed 100 children with a diagnosis of febrile convulsion, aged between 9 months and 2 years, during January 2007 to July 2009. The control group consisted of 100 febrile children without convulsion; controls were closely matched to the cases by age, gender, and underlying disease. RESULTS: The mean ages of the febrile convulsion and control group were 16.3+/-7.4 and 15.8+/-6.1 months, respectively, and the two groups had no differences in clinical features. Iron deficiency anemia (Hb <10.5 gm/dL) was more frequent in the febrile convulsion group than in the control group, although there was no statistical significance. Unexpectably, the RDW (red blood cell distribution width) was significantly lower and the MCNC (mean corpuscular hemoglobin concentration) was significantly higher among seizure cases than among the controls (P<0.05). There is no statistical difference between simple and complex febrile groups in the clinical and laboratory profiles. On multiple logistic regression analysis, iron deficiency anemia was more frequent, but the RDW was lower, among the cases with febrile convulsion, compared with the controls. Conclusions: Our study suggests that the iron deficiency anemia is associated with febrile convulsion, and screening for iron deficiency anemia should be considered in children with febrile convulsions.

A Case of Neuroendocrine Carcinoma and Childhood Myelodysplastic Syndrome in Hyper-IgM Syndrome

Congenital immunodeficiency is one or combined immune defect in immunoglobulin, leukocyte, and complement. These patients have increased susceptibility to respiratory infection. Hence, their infection must be taken care of, tried to gene therapy and stem cell transplantation. We present here a case of hyper-IgM syndrome in an 11-year-old male patient who complained of abdominal distension and abdominal pain. Multiple abdominal masses were detected by abdominal computed tomography (CT) and he was diagnosed with neuroendocrine carcinoma by mass biopsy. There was no evidence of metastasis of cancer cells to the bone marrow, but a dysgranulopoietic feature was noted and he was diagnosed with childhood myelodysplastic syndrome. This is the first report that neuroendocrine carcinoma is associated with childhood myelodysplastic syndrome in hyper-IgM syndrome.

Anti-tumor effect of new compound, 127, through the induction of apoptosis / 소아과

PURPOSE: We screened more than 350 compounds with an endoperoxide ring structure in search of an anti-leukemic drug and found that compound 127 (c-127) could induce significant cytotoxicity in HL-60 cells. In this study, we investigated the molecular mechanisms of compound 127-induced antitumor activity on HL-60 cells. METHODS: HL-60 cells were cultured in Rosewell Park Memorial Institute 1640 and cell viability was measured by MTT [3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide], a tetrazole assay. Apoptosis was assessed by a DNA fragmentation test. Apoptotic machineries were determined by Western blot analysis. RESULTS: C-127 could induce a cytotoxic effect at 24 h and apoptosis at 6 h, which was demonstrated with MTT assay and DNA fragmentation test, respectively. The apoptotic effect of this drug was caused by the activation of the intracellular caspase-8,3 activation, the cleavage of pro-apoptotic Bid, and the increase of c-Jun expression accompanied with JNK (Jun N-terminal kinases) phosphorylation. On the contrary, it increased the expression of anti-apoptotic Bcl-2 levels, leading to the induction of the induction of anti-apoptotic effect. Taken together, the present study demonstrated that c-127 was a potent inducer of cytotoxicity on HL-60 cells through apoptotic mechanisms, which included the activation of caspase family, the regulation of Bcl-2 family, and the activation of JNK signaling pathway. CONCLUSION: Our results suggest that c-127 has a strong antitumor activity through the regulation of various apoptotic machineries on HL-60 cells. The compound may be utilized as an effective and potentially therapeutic drug in leukemia.

Weaning food practice in children with iron deficiency anemia / 소아과

PURPOSE: Iron deficiency anemia (IDA) is one of the most common nutritional deficiencies in children on a weaning diet. We investigated weaning practices in infants and children, as well as their mothers' knowledge about weaning. METHODS: We investigated 129 children with IDA and 166 without IDA (aged 6-36 months) who had visited 10 university hospitals between March 2006 and July 2007. We investigated the hematologic values of both groups. A questionnaire on weaning was answered by the mothers of these children. RESULTS: The hematologic values in the IDA group showed a significant difference from those in the comparison group (P0.05). Rice gruel, boiled rice, and fruit juice accounted for approximately 8 0% of the starting foods in both groups (P>0.05). Only 40% of the children in the IDA group had a balanced diet within a month, versus 38% in the comparison group. In response to questions about the necessity of iron-fortified foods for breast-fed infants, less than 50% of mothers in both groups answered correctly. In the IDA group, 42% showed serum ferritin less than 10 ng/ mL, while 92% showed serum MCV less than 72 fL. CONCLUSION: In conclusion, collection of information on history should be thorough for feeding and selective examinations for IDA in high-risk groups. Considering the adaptation period, we suggest beginning children on a weaning diet at 45 months. In addition, we need to educate mothers on weaning practice, especially on the necessity of iron-fortified foods for breast-fed infants.

A Case of Peritoneal Loose Body in a Child / 대한소아소화기영양학회지

A peritoneal loose body is reported to develop because of torsion and separation of the epiploic appendages. The condition is usually symptomless and may be incidentally during abdominal surgery or autopsy. It usually occurs in middle-aged and elderly adults and is very rare in children. In this paper, we report a case of a peritoneal loose body in the pelvic cavity of a 10-year old-girl who presented with urinary frequency and left lower abdominal discomfort. A second plain X-ray film of the abdomen, obtained before surgery, in a different view than the first, revealed that the calcified mass had migrated to a lower position. The mass was laparoscopically resected, and histological examination revealed it to be a fibrotic nodule with central liquefaction and calcification.

Clinical features of vaccination-associated thrombocytopenic purpura in children / 소아과

PURPOSE: Idiopathic thrombocytopenic purpura (ITP) is a relatively common hematological disease in children. It generally occurs after exposure to a common viral infection episode; however, it may occasionally follow immunization with measles, measles-mumps-rubella (MMR), hepatitis B (HBV), influenza, diphtheria-tetanus-pertussis (DTP), or chickenpox vaccines. In this study, the incidence, clinical characteristics, and treatment outcome of vaccination-associated ITP were investigated and compared with non-vaccination-associated ITP. METHODS: The admission records of 105 pediatric ITP patients between 0-14 years of age admitted to Department of Pediatrics, Wonkwang University Hospital from January 1994 to July 2007 were retrospecitively reviewed. Patients were grouped into a vaccination-associated group and a non-vaccination-associated group according to vaccination history within the previous 1 month, and various clinical features between the two groups were statistically analyzed. RESULTS: Thirteen patients (12%) had a preceding vaccination. Eight had received DTP vaccination, 2 had received hepatitis B, and 1 each had received influenza, MMR, and Japanese B encephalitis vaccination. However, none of the patients had a recurrent thrombocytopenia after subsequent vaccinations. In the vaccination-associated group, the age was significantly lower, anemia was more common, and the risk period with blood platelet count <20x10(9)/L was significantly shorter than for the in non-vaccination-associated group. Also, wet purpura was less prominent and the remission within 1month was more frequently achieved in the vaccination-associated ITP group. CONCLUSION: Vaccination-associated ITP patients showed mild symptoms with a more benign and shorter lasting course than non-vaccination-associated ITP patients. Moreover, platelet count assessment at the time of the next immunization may not be necessary.

A case of dapsone syndrome / 소아과

Diamino-diphenyl-sulfone (Dapsone) is widely used in the treatment of leprosy and a variety of blistering skin diseases. It sometimes has adverse side effects with common usual doses, such as skin, nervous system, gastrointestinal tract, liver, kidney and hematologic toxicity. One of these side effects is a rare but serious hypersensitivity reaction called dapsone syndrome, which occurs several weeks after the initial administration of the drug and results in unpredictable, sometimes fatal outcomes. This report deals with a 13-year-old girl's case with typical features of dapsone syndrome that included fever, exfoliative dermatitis, jaundice, hemolytic anemia and pleural effusion after being treated with dapsone for four weeks.

Clinical factors causing hyperbilirubinemia in patients with Kawasaki disease / 소아과

PURPOSE: Hyperbilirubinemia, jaundice and gallbladder hydrops are unusual manifestations of Kawasaki disease (KD). In this case, abdominal pain, anorexia and abdominal distension may follow eventfully. We reviewed the clinical and laboratory data to investigate the causative factors of hyperbilirubinemia in patients with KD. METHODS: Two-hundred eighty two children diagnosed and hospitalized with KD were identified by searching patients' charts. Cases were included in the study if diagnosed between January 1997 and December 2004. We reviewed clinical data, age, sex, duration from the onset of fever to admission and duration from start of treatment to defervescence. Clinical and laboratory data were compared between normal (A) and hyperbilirubinemia (B) groups. RESULTS: Thirteen patients (4.6 percent) showed hyperbilirubinemia. In the hyperbilirubinemia group, age and initial ALT value were higher than group A (P=0.003, 0.018 respectively). Duration from the onset of fever to admission and age were relative risk factors in hyperbilirubinemia (P=0.007, 0.003 respectively) in patients with KD. CONCLUSION: Shorter duration from the onset of fever to admission and older age group were relative risk factors of hyperbilirubinemia in patients with KD.

Lower respiratory tract infection of positive antigen test for respiratory syncytial virus on children under 2 years of age / 소아과

PURPOSE: This study was design and performed for evaluations of resent clinical pattern of bronchiolitis caused by RSV infection with children under 2 year of age for 5 years, who were admitted to pediatric ward. METHODS: The inclusion criteria of the patients were children under 24 month-of-age, clinical manifestations of lower respiratory tract infection, and RSV antigen that was detected by a direct immunofluorescence test from the nasal secretions. The additional laboratory and simple chest X-ray findings were reviewed from the medical records of children who were admitted Wonkwang university hospital from Jan. 1999 to Dec. 2003. RESULTS: In the 5 year study duration, 127 patients were enrolled and outbreak of RSV bronchiolitis took place in 2001. The 80 cases(63 percent) of RSV infection were concentrated in later autumn and winter. Number of the cases show coughing were 120(94.5 percent), but rale was audible in 78 cases(61.4 percent). Dyspnea, wheezing, and intercostal retraction were noticed in 27(21.3 percent), 21(16.5 percent), and 4(3 percent) cases respectively. The most common chest X-ray finding was hyperinflation of the lung that was noticed in 110 cases(86.6 percent). Care with mechanical ventilator for more than 2 days required in 5 cases. CONCLUSION: Lower respiratory tract infection by RSV was common in late autumn and winter season but year-round infection was noticed. The severity of RSV respiratory tract infectiontakes in some degree a grave course. So we suggest that population-based surveillance of acute respiratory infection due to RSV is necessary for assessment of prevalence and epidemiology of this disease.

The study on cytotoxicity of cytokines produced by the activated human NKT cells on neuroblastoma / 소아과

PURPOSE: alpha-Galactosylceramide (alpha-GalCer)-stimulated human Valpha24 natural killer T (NKT) cells exert antitumor activity against some leukemia in a CD1d dependent and TCR-mediated manner, but could not kill CD1d-negative neuroblastoma (NB) cells. There are few reports about the direct antitumor effect of highly secreted cytokines by these cells on activation. In this study, using a cell-free supernatant (SPN) collected from plate bound hCD1d/alphaGalCer tetramers-stimulated NKT cells, we examined whether they could be helpful in the immunotherapeutic treatment of NB. METHODS: Cells were cultured in IMDM. The cytokines produced by NKT cells were measured with Cytometric Bead Array (CBA) analysis. Cell viability was evaluated by calcein-AM fluorescence with digital image microscopy scanning (DIMSCAN). The percentage of specific apoptosis was calculated by flow cytometric detection of apoptosis using annexin V and 7-AAD. RESULTS: The activated NKT cells secreted high levels of IL-2, INF-gamma, TNF-alpha. The SPN was significantly cytotoxic against four out of eight tested NB cell lines, through mainly apoptosis as evidenced by annexin-V staining and inhibition with the pretreatment of pancaspase blocker. This apoptosis was significantly inhibited when anti-TNF-alpha and anti-IFN-gamma neutralizing mAbs were used separately and it was completely abolished when the two mAbs were combined. CONCLUSION: IFN-gamma and TNF-alpha produced by NKT cells could exert synergistically direct anti-tumor activity through apoptosis on some NB cell lines.