• Clinical and Experimental Pediatrics
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• v.45 no.4
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• pp.466-472
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• 2002

Purpose : The diagnosis of Meckel's diverticulum is difficult and delayed because it presents with various clinical symptoms. We evaluated clinical, imaging and pathologic findings of Meckel's diverticulum to facilitate detection of Meckel's diverticulum in children. Methods : Review of clinical, imaging, surgical and pathological findings in 10 children aged 7 days to 14 years with Meckel's diverticulum during an 8-year period, 1993-2001, at Ewha Womans University Hospital was undertaken. Results : The male to female ratio was 2.3 : 1. The chief complaint was painless lower gastrointestinal( GI) bleeding; others were abdominal pain, abdominal distention and vomiting, in order of frequency. The diagonsis before surgery were Meckel's diverticulum in 5 patients, non-reducible intussusception in 3 patients and intestinal obstruction in 2 patients. The diverticulum was located between 35 cm to 70 cm proximal to the ileocecal valve. The length of the diverticulum ranged from 4 cm to 12 cm and 80% of it was within 5 cm. A Meckel scan($^{99m}Tc-pertechnetate$ scintigraphy) after cimetidine administration was done in 6 cases. All 5 cases that presented with lower GI bleeding had ectopic gastric mucosa confirmed on pathology. Out of 5 cases of ectopic gastric mucosa, only 4 cases were positive on the Meckel's scan. Conclusion : In cases of unexplained GI bleeding, obstruction, or inflammation diagnostic workup should be carried out to rule out Meckel's diverticulum. Laparoscopy, high resolution ultrasonography and computed tomography of the abdomen may be indicated in the assessment of pediatric patient with lower GI bleeding, especially in patients with suspected bleeding from Meckel's diverticulum showing negative Meckel's scan.

• Clinical and Experimental Pediatrics
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• v.45 no.8
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• pp.1007-1015
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• 2002

Purpose : The aim of this study is to find a method to diagnose and treat children who showed benign hemophagocytic histiocytes in bone marrow examination. Methods : We analyzed the clinical data of thirty patients retrospectively who showed benign hemophagocytic histiocytes in bone marrow examination from January 1995 to November 2001 at Keimyung University Dong-san Hospital. Bone marrow histiocytes were classified into a few, some, and many according to the number of histiocytes comparing with the white cells. Results : The age of thirty patients ranged from two months to 15 years. The median age was 5.6 years with male predominance(2.3 : 1). The most frequent clinical manifestation was fever; others were respiratory symptom, hepatosplenomegaly, lymphadenopathy and skin rash(in order of frequency). Common laboratory findings were leukopenia, thrombocytopenia, anemia and abnormal liver function test. Infection was present in 30 patients; causative organisms were documented in 15 patients, and bacterial infection was more common. Epstein Barr virus was the cause of infection in four patients. Bone marrow examination showed a few(20.0%), some(75.0%) and many(30.0%) hemophagocytic histiocytes. Combination immunochemotherapy including immunoglobulin, steroids and cyclosporine were helpful in 22 out of 30 patients. The mortality rate was high in young patients who showed some to many hemophagocytic histiocytes. Conclusion : Bone marrow examinations and early detection of histiocytes will be helpful in children who have fever, hepatosplenomegaly and abnormal liver function test. Immunochemotherapy were helpful and further investigation will be needed for the detection of the relationship between the bone marrow findings and prognosis.

• Clinical and Experimental Pediatrics
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• v.46 no.1
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• pp.33-36
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• 2003

Purpose : We evaluated clinical manifestations and laboratory findings in patients with measles according to age distribution. Methods : Retrospective analyses were performed using medical records of patients with measles admitted to The Catholic University of Korea, Daejon St. Mary's Hospital from October 2000 to May 2001. We divided the patients with measles into three age groups, i.e., those who were under two years of age(159 patients), those between 9-11 years of age(39 patients), and those older than 16 years of age who were hospitalized in the department of internal medicine(young adult group; 23 patients). We compared clinical and laboratory characteristics among these three groups. Results : Almost all patients with measles were presented with fever, skin rash and cough. No statistical differences were present between the three groups in total fever duration, number of hospitalization days, complications determined with longer hospitalization for more than eight days, and positive values of anti-measles IgM. Patients under 2 years of age showed statistically higher levels of white bood cell and lymphocyte counts. However, neutropenia and lymphopenia were observed in all age groups compared with age-matched standard values. Campared with the other two age groups, the young adult group showed a higher mean level of liver enzymes(AST/ALT) and more patients with a level twice as high as the normal values. Conclusion : Clinical manifestations including complications according to age groups showed no significant differences in patients with measles. Hepatic involvement was more prevalent in the young adult group.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.15 no.1
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• pp.1-8
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• 2015

Purpose: 3-methylcrotonyl CoA carboxylase deficiency (3MCCD) is leucine metabolic disorder caused by mutation in MCCC1 or MCCC2 gene. Clinical manifestations are variable, ranging from fatal neonatal onset to asymptomatic individuals. There is no retrospective study of Korean patients undergoing long-term treatment for 3MCCD. We reported this study to find out clinical symptoms and gene analysis of 3MCCD patients. Methods: This study was based on data of patients diagnosed with 3MCCD in Soonchunhyang university hospital between April 2009 and September 2013. We report clinical, enzymatic and mutation data of 3MCCD patients found by newborn screening. Results: In tandem mass spectrometry, 3-OH-isovalerylcarnitine (C5OH) of all patients increased. And all 7 patients were elevated 3-methylcrotonylglycine (3MCG) and 3-hydroxyisovaleric acid (3HIVA) in urine. MCCC mutation was identified in 2 patients and MCCC2 was mutated in 5 patients. We found mutation occurred in 8 different parts of nucleotide and such mutation caused 7 different types of changes in amino acid. All patients are on medication of L-carnitine and L-glycine. 4 patients are taking biotin. And 4 patients are eating leucine free formula. After starting treatment, there were no significant changes of urine 3MCG and 3HIVA levels. Conclusions: According to our data, MCCC2 gene mutation was more common than MCCC1 gene mutation. But the level of 3HIVA or 3MCG in urine has no correlation with phenotype. All patients has no symptoms and are shown normal development.

• Pediatric Infection and Vaccine
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• v.23 no.1
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• pp.46-53
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• 2016

Purpose: Enterovirus (EV) infection in children can manifest various diseases from asymptomatic infection to nonspecific febrile illness, hand-foot-mouth disease, and aseptic meningitis. This study was aimed to investigate epidemiology and clinical significance of various genotypes of EV infections in pediatric inpatient. Methods: We collected the stool samples from the admitted pediatric patients in Inha University Hospital from March 2014 to March 2015. EV detection and genotype identification were performed by real-time RT-PCR and semi-nested RT-PCR. Phylogenetic trees were constructed by neighbor joining method. Results: A total of 400 samples were collected during study period and 112 patients (28%) were diagnosed with EV infections. The mean age of EV positive patients was 2.66 years (0.1-14) and sex ratio was 1.73:1. Genetic sequences of EVs were identified; coxsackievirus B5 (17, 15.2%), coxsackievirus A16 (13, 11.6%), enterovirus 71 (10, 8.9%), and coxsackievirus A2 (9, 8.0%). Nonspecific febrile illness (96, 86%) was the most common clinical manifestation and the duration of fever was 0-11 days (mean 3.1 days). Rash (44, 39%) and meningitis (43, 38%) were followed. Patients who were attending daycare center or had siblings accounted for 82.1%. Phylogenetic relationship tree revealed 6 distinct genogroups among 56 types of EVs. Conclusions: This study is the report of epidemiology, serotype distribution and clinical manifestations of children with EV infection in Incheon. This data will be helpful for further study about the epidemiology of EV infection in Korea.

• Childhood Kidney Diseases
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• v.6 no.2
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• pp.155-168
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• 2002

Purpose; Systemic lupus erythematosus(SLE) is an autoimmune disease with multi-system involvement and renal damage is a major cause of morbidity and mortality in children. Renal involvement is more common and severe in children than in adults. Therefore, renal biopsy plays a crucial role in planning effective therapy. In this study, we investigated the clinical and pathological findings of lupus nephritis in children to aid clinical care of the disease. Methods: The clinical and pathological data of 40 patients who were diagnosed as SLE with renal involvement in Shinchon Severance Hospital from Jan. 1990 to Sep. 2002 were analyzed retrospectively. Results: The ratio of male to female patients was 1:3 and the median age at diagnosis was 12.1(2-18) years old. FANA(95.0%), anti-ds DNA antibody(87.5%), malar rash(80.0%) were the most common findings among the classification criteria by ARA. Microscopic hematuria with proteinuria(75.0%), nephrotic syndrome(55.0%), and microscopic hematuria alone(15.0%) were the most common renal presentations in the respective order at diagnosis. There were 27 cases with WHO class IV lupus nephritis confirmed by renal biopsy and 3 cases with pathological changes of WHO class type. Different treatment modalities were carried out : prednisolone only in 5 cases, prednisol-one+azat-hioprine in 9 cases, prednisolone+azathioprine+intravenous cyclophosphamide in 14 cases, prednisolone+cyclosporine A+intravenous cyclophosphamide in 12 cases, plasma exchange in 9 cases and intravenous gamma-globulin in 2 cases. The average follow-up period was $51.8{\pm}40.5$ months. During $51.8{\pm}40.5$ months. During follow-up, 4 patients expired. The risk factors associated with mortality were male, WHO class IV and acute renal failure at diagnosis. Conclusion: Renal involvement was noted in 63.5% of childhood SLE, and 67.5% of renal lesion was WHO class IV lupus nephritis which is known to be associated with a poor prognosis. Therefore aggressive treatment employing immunosuppressant during the early stages of disease could be helpful in improving long-term prognosis. But careful attention should be given to optimize the treatment due to unique problems associated with growth, psychosocial development and gonadal toxicity, especially in children.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.3 no.1
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• pp.68-74
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• 2000

Purpose: This study was to investigate the clinical manifestations of FTT in children. Methods: From March 1997 to July 1999, clinical observations were made on patients with FTT who had visited to Samsung Medical Center. Detailed histories and through physical examinations were taken, and when suspected organic FTT, basic laboratory studies were done. Results: Upon the review of medical records, we investigated the clinical manifestations of 74 children, aged 1 month and 13 year 1 month. The causes of FTT were composed of either physiologic (47.8%) or pathologic (52.2%) ones. Among the physiologic FTT, were there familial short stature (FSS, 14.5%), intrauterine growth retardation (IUGR, 14.5%), constitutional growth delay (CGD, 11.6%), idiosyncrasy and prematurity. Among pathologic causes, neurologic disorders (20%) are the most common causes of FTT, and then follow by GI (13.4%), allergic and infectious disorders in decreasing order. The data showed that average caloric intake in patients with FTT was 76,2% of recommended amount. FTT patients with CGD, IUGR, and idiosyncrasy had tendency to take small foods. The FTT children with prematurity, IUGR and pathologic FTT, were short and thin for their ages. However FTT children with CGD and FSS had tendency to be thin with relatively normal heights for their ages, in comparison with those of the children with prematurity, IUGR and pathologic FTT. Conclusion: The diagnosis of FTT was easily obtained with simple and through medical history, physical examination, and minimal laboratory tests. In this study, organic FTT was more prevalent than physiologic one. This results indicate that early intervention is mandatory, because children may develop significant long-term sequelae from nutritional deficiency.

• Clinical and Experimental Pediatrics
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• v.45 no.11
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• pp.1373-1380
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• 2002

Purpose : Acute measles encephalitis(ME) is characterized by an abrupt onset of fever and obtundation, frequently accompanied by seizures and multifocal neurological signs. The aim of this study was to clarify the clinical manifestation, progression and the brain SPECT patterns in patients with acute ME. Methods : This study included 11 children with acute ME admitted to Chonbuk National University Hospital. Ten patients received a first dose of measles vaccine, one patient did not receive a first dose, and no patients received a second dose. ME was diagnosed based on characteristic clinical pictures, measles antibodies by ELISA and abnormal CSF findings. Brain MRI and brain SPECT were performed in 11 patients with acute ME. Results : There were four males and seven females whose ages at onset ranged between 18 months and 14 years(mean : 10.5 years). The main clinical neurologic pictures were loss of consciousness( 10) and seizure(five). The titer of IgG and IgM antimeasles antibodies in serum were positive in 10 patients. In CSF, nine patients had IgG antibodies and one patient had IgM antibodies. The concentration of protein(mean : $124{\pm}60mg/dL$) and WBC counts(mean : $158{\pm}157/{\mu}L$) in CSF were elevated in all patients. In electroencephalographic examination, nine patients showed increased slow waves. Seven of 11 patients(63.6%) revealed high signal intensity on the brain MRI. In contrast, all patients showed hypoperfusion in brain SPECT examination. According to brain SPECT, the perfusion deficits were frequently observed in the frontal lobe(nine), temporal (nine), parietal(eight) and thalamus(eight). Conclusion : Brain SPECT is more sensitive than MRI for the evaluation of brain damage in early stages of acute ME.

• Clinical and Experimental Pediatrics
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• v.51 no.11
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• pp.1172-1178
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• 2008

Purpose : Congenital chylothorax is an accumulation of lymphatic fluid within the pleural space. It is a common cause of unidentified hydrops fetalis. We examined the perinatal history, clinical manifestation, diagnosis, treatment, and outcome in 6 newborns diagnosed to have congenital chylothorax with hydrops fetalis. We also studied the effect of octreotide therapy for congenital chylothorax in relation to conservative treatment. Methods : We retrospectively reviewed the medical records of 6 patients diagnosed to have congenital chylothorax with hydrops fetalis among 27,907 newborns who were born at the Cheil General Hospital and Womens Healthcare Center between January 2004 and July 2007. The diagnosis of chylothorax is based on the analysis of pleural fluid before and after milk feeding. Results : Incidence of congenital chylothorax in this study was 0.021%. All 6 cases were noted in over the 92% lymphocyte in pleural analysis. Transudate was changed into chyle with increasing triglyceride levels above 200 mg/dL after milk feeding. Three of 6 infants improved with conservative treatment, including thoracostomy and assisted ventilation. The others had persistent symptoms despite conservative treatment and responded to octreotide therapy. A complication, specifically vomiting was noted in 1 case during octreotide therapy. Conclusion : In this study, octreotide therapy resulted in a safe and excellent outcome. Therefore, octreotide therapy is considered in severe refractory congenital chylothorax in conservative treatment. Further studies are required to determine appropriate guidelines for octreotide therapy.

• Clinical and Experimental Pediatrics
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• v.51 no.11
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• pp.1217-1221
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• 2008

Purpose : An association between idiopathic thrombocytopenic purpura (ITP) and systemic lupus erythematosus (SLE) has been recognized for decades because thrombocytopenia is the first manifestation in some patients with SLE. However, the risk of later development of SLE in childhood ITP is currently unknown. We retrospectively evaluated the incidence and clinical significance of the positive antinuclear antibody (ANA) in children with acute ITP. Methods : This study was retrospectively performed to review the clinical and laboratory characteristics in 77 children diagnosed to have acute ITP and admitted to the Pusan National University Hospital between January 2003 and December 2006. Patients tested positive for ANA were regularly followed-up for at least 12 months for symptoms indicative of SLE. Results : Seventy-seven children were included in the study; 38 males (49.4%) and 39 females (50.5%), the mean age was 4.5 years. Sixteen (20.8%) ITP patients had a positive ANA, with a median titer of 1:320. The mean age of the patients with positive ANA was 9.3 years, which is much older than 3.3 years for patients with negative ANA (P<0.05). The positive ANA group was predominantly female (81.3%) compared to the negative ANA group (P<0.05). There was no statistically significant difference in mean platelet counts between both groups. No statistically significant difference was found in ANA positivity and progression to chronic ITP or SLE. After the median follow-up of 32 months, SLE was diagnosed only in one ITP patient with positive ANA. Conclusion : Our data demonstrated that ANA positivity is often found in children with acute ITP. Large-scale studies should be considered to determine the significance of ANA positivity in childhood ITP for the later development of SLE.