Nutcracker Syndrome combined with Superior Mesenteric Artery Syndrome in a Pediatric Patient: A Case Report

Nutcracker syndrome is a phenomenon that the left renal vein (LRV) is pressed between the superior mesenteric artery (SMA) and the aorta. Clinical characteristics include gross or microscopic hematuria, orthostatic proteinuria, abdominal pain, and back pain. It occurs due to LRV squeezing caused by narrowed aortomesenteric angle. SMA syndrome is a disease that the third part of the duodenum is prone to intestinal obstruction by narrowed angle between the SMA and the abdominal aorta. Clinical symptoms include postprandial abdominal distension, epigastric pain, nausea, and vomiting. SMA syndrome and nutcracker syndrome have common features that result from narrowed aortomesenteric angle. However, it is very rare for both syndromes to occur simultaneously, so the two syndromes are regarded as separate diseases. This is a report on a case of nutcracker syndrome with SMA syndrome in a child who presented gross hematuria, recurrent abdominal pain and vomiting. To our knowledge, nutcracker syndrome simultaneous with SMA syndrome has not been previously reported in pediatric patient, especially with an exhibition of gross hematuria. This case suggests that the simultaneous presence of SMA syndrome with the same pathogenesis needs to be considered when nutcracker syndrome is suspected in pediatric patients with hematuria.

Predictors of High-grade Vesicoureteral Reflux in Children with Febrile Urinary Tract Infections

PURPOSE: This study aimed to investigate clinical and radiological factors that may predict high-grade vesicoureteral reflux (VUR) in patients with febrile urinary tract infection (UTI). METHODS: We retrospectively analyzed medical records of 446 patients diagnosed with febrile UTI from March 2008 to February 2017. All patients underwent renal-bladder ultrasonography (RBUS), 99mTc dimercaptosuccinic acid (DMSA) renal scan, and voiding cystourethrography (VCUG), and were divided in to 3 groups: a high-grade VUR group (n=53), a low-grade VUR group (n=28), and a group without VUR (n=365). RESULTS: The recurrence and non-Escherichia coli infection rates in febrile UTI were significantly higher in the high-grade VUR group than in the other two groups (P<0.05). RBUS showed that hydronephrosis and ureter dilatation were more frequent in the high-grade VUR group than in the other groups (P<0.05). In the high-grade VUR group, a renal cortical defect was more likely to appear as multiple defects, and the difference in bilateral renal scan uptake between both kidneys was larger than in the other two groups (P<0.001). CONCLUSION: Recurrent UTI, non-E. coli UTI, abnormal findings on RBUS such as hydronephrosis and ureter dilatation, and abnormal findings in the DMSA renal scan such as multiple renal cortical defects and greater uptake difference were associated with high-grade VUR. VCUG should be selectively performed when RBUS and/or DMSA renal scan reveal significant abnormalities.

Predictors of High-grade Vesicoureteral Reflux in Children with Febrile Urinary Tract Infections

PURPOSE: This study aimed to investigate clinical and radiological factors that may predict high-grade vesicoureteral reflux (VUR) in patients with febrile urinary tract infection (UTI). METHODS: We retrospectively analyzed medical records of 446 patients diagnosed with febrile UTI from March 2008 to February 2017. All patients underwent renal-bladder ultrasonography (RBUS), 99mTc dimercaptosuccinic acid (DMSA) renal scan, and voiding cystourethrography (VCUG), and were divided in to 3 groups: a high-grade VUR group (n=53), a low-grade VUR group (n=28), and a group without VUR (n=365). RESULTS: The recurrence and non-Escherichia coli infection rates in febrile UTI were significantly higher in the high-grade VUR group than in the other two groups (P<0.05). RBUS showed that hydronephrosis and ureter dilatation were more frequent in the high-grade VUR group than in the other groups (P<0.05). In the high-grade VUR group, a renal cortical defect was more likely to appear as multiple defects, and the difference in bilateral renal scan uptake between both kidneys was larger than in the other two groups (P<0.001). CONCLUSION: Recurrent UTI, non-E. coli UTI, abnormal findings on RBUS such as hydronephrosis and ureter dilatation, and abnormal findings in the DMSA renal scan such as multiple renal cortical defects and greater uptake difference were associated with high-grade VUR. VCUG should be selectively performed when RBUS and/or DMSA renal scan reveal significant abnormalities.

A Study of Home Therapy in Hemophilia Patients / 임상소아혈액종양

BACKGROUND: Home therapy has merits, enabling rapid treatment thereby reducing complications, but difficulty with venous access is a barrier to treatment. There is a paucity of data on this. This study investigated the current status of home therapy for patients with hemophilia, and examined problems and potential solutions.METHODS: Patients and their families who were performing home therapy and who attended the camp of the Korea Hemophilia Association in 2014 were asked to complete questionnaires. The questionnaires pertained to complications of infusion and whether (or not) factor infusion was properly performed. Responses were scored on a scale from 1-5, with a high frequency of complications and adequate performance of infusion being allocated relatively higher scores.RESULTS: The mean score of complications arising from infusion was 1.56±0.46. This was relatively low and was not correlated with the factor infusion training method. The performance of home therapy obtained a relatively high score: 4.46±0.56. The performance score was significantly higher for patients who had practiced infusion with medical personnel, an injection simulator or a video clip.CONCLUSION: Although most patients properly performed home therapy, further improvement is needed in training of infusion and keeping records of bleeds. It is essential to establish guidelines on home therapy, develop a standardized patient and family training program, and reinforce the role of hemophilia treatment centers in educating patients and their families.

A Study of Home Therapy in Hemophilia Patients / 임상소아혈액종양

BACKGROUND: Home therapy has merits, enabling rapid treatment thereby reducing complications, but difficulty with venous access is a barrier to treatment. There is a paucity of data on this. This study investigated the current status of home therapy for patients with hemophilia, and examined problems and potential solutions. METHODS: Patients and their families who were performing home therapy and who attended the camp of the Korea Hemophilia Association in 2014 were asked to complete questionnaires. The questionnaires pertained to complications of infusion and whether (or not) factor infusion was properly performed. Responses were scored on a scale from 1-5, with a high frequency of complications and adequate performance of infusion being allocated relatively higher scores. RESULTS: The mean score of complications arising from infusion was 1.56±0.46. This was relatively low and was not correlated with the factor infusion training method. The performance of home therapy obtained a relatively high score: 4.46±0.56. The performance score was significantly higher for patients who had practiced infusion with medical personnel, an injection simulator or a video clip. CONCLUSION: Although most patients properly performed home therapy, further improvement is needed in training of infusion and keeping records of bleeds. It is essential to establish guidelines on home therapy, develop a standardized patient and family training program, and reinforce the role of hemophilia treatment centers in educating patients and their families.

Factors Influencing on Perinatal Outcomes of Asian Marriage Immigrant Women: Ten-year Experience in a Single Center / 대한주산의학회잡지

PURPOSE: We aimed to analyze the maternal and perinatal factors associated with perinatal outcomes by examining families comprised of Korean fathers, Asian immigrant mothers, and their newborns. METHODS: Medical records of newborn infants admitted to Jeonju Jesus Hospital nursery or ne-onatal intensive care unit (NICU) from January 2004 to June 2013 and their Asian immigrant mothers were reviewed retrospectively. The newborns were divided into two groups depending on whether they were admitted NICU or not, and factors influencing on perinatal outcomes were compared between the two groups. The newborn were divided into the two groups, including those who did not receive inpatient care and those treated in the NICU. The differences between the two groups were analyzed. RESULTS: The study included 180 newborns and 172 mothers, and 94 (52.3%) and 86 (47.7%) newborns were classified as the nursery group and the NICU group, respectively. There were no statistically significant differences between the two groups in terms of the mothers' nationality, maternal age, maternal education level, maternal occupation, residential area, maternal height and weight, maternal weight gain during pregnancy, maternal hepatitis B antigen positivity, maternal parity, paternal age, and age gap between spouses. However, underweight maternal prepregnancy body mass index (BMI) and hemoglobin level over 11.0 g/dL were significantly more frequent in the NICU group in the comparative analysis. The NICU group showed significantly more frequent no iron supplements during pregnancy (OR=4.06) and gestational disease (OR=3.81). CONCLUSION: In cases where married immigrant mothers had underweight prepregnancy BMI, gestational disease, or no iron supplements during pregnancy, their newborns were more likely to have NICU care. Therefore, married immigrant women should have appropriate perinatal care including education about a balanced diet to maintain an appropriate body weight with ensuring an adequate iron supplements intake.

A Case of Partial Trisomy 14q derived from Paternal Balanced Translocation / 대한주산의학회잡지

Partial trisomy 14q is an extremely rare disorder. Approximately 50 cases have been reported around the world. This disorder results from a malsegregation or non-disjunction of balanced translocated chromosome, thus the offspring inherits such a chromosome. In this report, the father's chromosomal arrangement was 46, XY, t(11;14)(q25;q32.1) and the mother had a normal chromosomal arrangement. We experienced a case of partial trisomy 14q in a male neonate who had craniofacial abnormalities, frontal bossing, bulgin fontanelle, hypertelorism, low-set ears, wide and flat nasal bridge, thin cupid bow lip and micrognathia. The karyotype of his chromosome was 46,XY,der(11)t(11;14)(q25;q32.1). We report the case with a review of the literature.

The Changes of Incidence of Childhood Diabetes in Jeollabuk-do for 26 Years / 대한소아내분비학회지

PURPOSE: The purpose of this study was to know the changes and types of diabetes mellitus (DM) in children who lived in Jeollabuk-do for last 26 years. METHODS: We subdivided 26 years (January 1981-December 2006) as group 1 (January 1981-December 1993) and group 2 (January 1994-December 2006). Patients under 15 years old who had been newly diagnosed with DM in these periods in general hospitals situated in Jeollabuk-do were reviewed retrospectively about clinical conditions at the onset. RESULTS: 141 patients were enrolled; group 1 was 43 (30.5%) and group 2, 98 (69.5%). Patients number in same period increased over twice. For types of diabetes, group 1 was all type 1 diabetes and group 2 was 75.5% in type 1 diabetes, 21.4% type 2 diabetes and 3.1% clinically suspected maturity onset diabetes of the youth (cMODY). The incidence of diabetes was 0.12 patients per 100,000 under 15 years old in 1980 and increased upto 4.73 ones in 2005. The diagnosed mean age increased from 8.9 years (group 1) to 10.3 years (group 2), but type 1 diabetes had no significant difference. Diabetes occurred in girls more than boys. Two-thirds of all patients were diagnosed in the winter (December-February) and the spring (March-May). CONCLUSION: The incidence of childhood diabetes in Jeollabuk-do increased rapidly and type 2 diabetes was remarkable. We think these aspect would be not only the problem in this province but also the national one. So, we suggest that the practicing of the dynamic survey nationwide is important for the treatment and the management of childhood diabetes.

Twins / 소아과

Recently, twinning rate increases in Korea since the early 1990s by delayed marriage and prevailing of assisted reproductive technology. But twin and higher-order multiples are at increased risk for perinatal and neonatal mortality over 5 fold despite of dramatic improvement of perinatal and neonatal care. Because twins are born more prematurely and have lower birth weights than singleton. In addition, twins are at increased risk for cerebral palsy mainly in monochorionic twins due to co-twin fetal death, twin to twin transfusion and congenital anomaly. So, this article reviews the factors contributing to the mortality and morbidity of the twins and the efforts to decrease the neonatal mortality of twins.

Spontaneously Resolved Neonatal Hemochromatosis

Neonatal hemochromatosis is a very rare disorder with an unclear etiology with an extremely poor outcome. We report a case of female newborn who presented with direct hyperbilirubinemia without any evidence of congenital infection, immune related hemolysis or exogenous iron uptake. Diagnostic studies revealed highly elevated ferritin level. T2 weighted image of abdominal Magnetic Resonance Imaging (MRI) showed decreased signal intensity of entire left lobe and a part of right lobe of liver with normal spleen. Liver biopsy showed a large amount of hemosiderin in the hepatocytes and Kupffer cells. All the biochemical and excretory liver functions normalized with conservative managements.

Rehospitalization of Low-birth-weight Infants Who Were Discharged from NICU

PURPOSE: We tried to compare normal term infants with low-birth-weight infants discharged from NICU by evaluating morbidity. So we studied rates of rehospitalization, reasons for rehospitalization and neonatal risk factors that affect rehospitalization of normal term infants and low-birth-weight infants discharged from NICU. METHODS: This study was performed on 217 low-birth-weight infants discharged from NICU and 126 normal term infants born in Presbyterian Medical Center from January 1992 to December 1994. RESULTS: Low-birth-weight infants discharged from NICU had higher rates of rehospitalization than normal term infants. Twenty-nine percent of low-birth-weight infants discharged from NICU were rehospitalized, compared with 12.7% of normal term infants(P<0.001). Rates of rehospitalization by birth weight was 45.5% in infants of 1500g or less and 31.2% in infants of 1500-1999g of birth weight. The rates of rehospitalization increased as birth weight decreased (P<0.001), but there was no significant difference between 16.4% in infants of 2000-2500g of birth weight and 12.7% of normal term infants(P=0.442). Reasons for rehospitalization of low bith weight infants were respiratory problem(63.1%), congenital/developmental problem(10.8%), surgical problem(9.2%), G-I problem(7.7%), ophthalmic problem(3.1%), and cardiac problem(1.5%). Neonatal risk factors related to rehospitalization of low birth weight infants were mechanical ventilation, duration of mechanical ventilation, bronchopulmonary dysplasia and duration of hospitalization(P<0.01). CONCLUSION: Rates of rehospitalization increased accordingly to decreasing birth weight. The most common cause of rehospitalization was respirartory problem. Neonatal risk factors related to rehospitalization were mechanical ventilation, bronchopulmonary dysplasia and duration of hospitalization. The data suggest that high risk infants of rehospitalization require close follow up.

Clincial Study on Very Low Birth Weight Infants

Medical records of very low birth weight infants weighing less than 1500 grams at birth were reviewed and analyzed. One hundred and forty three infants who were admitted to neonatal intensive care unit of Presbyterian Medical Center, including those who were born and transferred from other hospitals, from January 1987 to December 1991 were examined and the following results were obtained; 1) The incidence of very low birth weight infant was 1.21% 2) The most common maternal risk factor was premature labor and the next was toxemia. 3) The most common disease of very low birth weight infant was neonatal hyperbilirubinemia and the next was septicemia. 4) Twenty five infants (38.5%) of intensive care group and 67 infants (85.9%) of Not-ventilated group survived with overall survival rate of 64.6%. 5) The heavier birth weight and longer intrauterine period were factors offering better chance for survival. 6) Among 43 (30%) infants expired, 7 (16.3%) died within 24 hours after birth, 12 (27.9%) died in second or third day, 4 (9.3%) died between fourth and seventh day, and 20 (46.5%) died between eighth.and twenty eighth day.

Diagnostic Value and Relationship of the between Stable Microbubble Rating Test and Shake Test for the Prediction of Neonatal Respiratory Distress Syndrome

Respiratory distress syndrome (RDS) in the newborn infants remains a major cause of mortality and morbidity in the newborn period despite much improvements in neonatal intensive care and artificial ventilatory techniques. Gastric fluid was obtained from 151 patients within 6 hours after delivery. The sensitivity, specificity, and predictive value of the simple shake test (133 cases) and stable microbubble rating (SMR) test (151 cases) were assessed in the diagnosis of RDS, as well as the relation between both tests and RDS. We carried out both tests of on gastric aspirates all newborn who admitted to NICU of Presbyterian Medical Center from June 1991 to August 1992. The results were summarized as follows: 1) Among the total 151 cases, RDS were found in 41 cases(27.2%). 2) RDS occurence rate of the simple shake test was 11/11 in 0 group, 17/26 in +1 group, 8/28 in +2 group, 2/41 in +3 group, and 2/27 in +4 group. RDS occurence rate was high the 0 and +1 group. 3) RDS occurence rate of the SMR test was 4/4 in very weak group, 32/36 in weak group, 1/33 in medium group, and 4/78 in strong group. RDS occurence rate was high in the very weak and weak group. 4) Among the positive group of the SMR test 95 cases, positive group of the shake test were found in 87 cases. Among negative group of the SMR test 38 cases, negative of the shake test were found in 29 cases (correlation coefficient=0.763). 5) Sensitivity of the shake test and SMR test were 70%, 87.8% respectively. Specificity of the shake test and SMR test were 93.3%, 96.4% respectively. Positive predictability were 75.7%, 90% respectively and negative predictability were 87.5%, 95.5% respectively. The shake test, as Well as SMR test, has significant value to diagnosis of the RDS. We predict RDS occurence rate of the SMR test was significantly higher than shake test.

Comparison of Rehospitalization during the First Year of Life in Normal and Low Birth Weight Infants Discharged from NICU

PURPOSE: Although the short- and long-term outcomes of low birth weight(LBW) neonatal intensive care unit(NICU) survivors have been extensively studied, much less information is available for normal birth weight(NBW) infants(greater than 2,500 gm) who require NICU care. METHODS: We retrospectively examined the neonatal hospitalizations and one year health status of 302 NBW and 131 LBW admissions to our NICU. Information on the neonatal hospitalization was obtained from a review of medical records. Postdischarge health status was collected by using telephone surveys and medical records. RESULTS: After initial discharge, 21.2% of the NBW infants and 23% of the LBW infants required rehospitalization during the first year of life and there was no significant difference between the two groups. The reasons for rehospitalization of the NBW infants included respiratory disorders (32.1%), G-I problems(26.2%), genitourinary problems(11.9%), surgery(10.7%), cardiac problems(7.1 %), and congenital/developmental problems(1.2%). For the LBW infants, the order of frequency was the same, with the percentages slightly different. Neonatal risk factors related to the rehospitalization of the NBW infants included mechanical ventilation, duration of mechanical ventilation, and congenital anomaly. But no positive significant correlation of neonatal risk factors with rehospitalization of LBW infants was found. CONCLUSIONS: Low and normal birthweight NICU survivors were rehospitalized at similar rates. The most common cause of rehospitalization was respiratory problems. Neonatal risk factors related to rehospitalization of NBW infants were mechanical ventilation, duration of mechanical ventilation, and congenital anomaly. However, no positive significant correlation of neonatal risk factors with rehospitalization of LBW infants was found. The data suggests that NBW infant survivors, as well as LBW infant NICU survivors, require close follow up.

The Statistical Study of Patient with Acute Poisoning

PURPOSE: The incidence of infectious disease, which has been the cause of death in pediatric period, was decreased. But, the opportunistic exposure to toxic substance shows a tendency to increase. We performed this study in order to find out preventive measures and the latest inclination of acute poisoning in childhood. METHODS: From January 1985 to July 1996, 88 children with acute drug and chemical poisoning, who diagnosed at Presbyterian Medical Center, were analyzed retrospectively. RESULTS: 1) It occurred more frequently in male than female (1.7:1) and high incidence was noted in 0-4 year-old age group (75%). 2) Annual distribution of poisoning shows a tendency to increased. 3) There was no significant seasonal difference in frequency of poisoning. 4) The vast majority (85%) of poison exposure were accidental and therapeutic intent was present in 7.5% of cases and suicidal intent was 7.5% of cases. 5) We noticed an hourly predominance with most accidents occurring from 9 a.m. to 1 p.m. and from 5 p.m. to 9 p.m., 35% respectively. 6) Categories with the largest numbers of total exposure, in descending order by exposure frequently, include the following : drug (30.1%), insecticide (20.4), rodenticide (12.9%), glacial acetic acid (13.9%), herbicide (4.3%), Carbon monoxide (3.2%), others (13%). So, food and household material are leading cause of poisoning, except drug. Of drug, DDS was the most common and then psychotherapeutic drugs were the next. 7) The chief complaints on admission were nausea and vomiting (32%), mental change (26%) irritability (9%), peripheral cyanosis (8%), seizure (5%), and in case of 20%, the patient were found incidentally without symptoms. 8) There were complications which were pneumonia, pulmonary fibrosis, esophageal stricture and pneumothorax. And death occurred in 4 cases, due to insecticide (2 cases), herbicide and rodenticide, 1 case respectively. CONCLUSIONS: In previous report in Korea, causative substance of poisoning were drug, carbone monoxide, insecticide, rodenticide in descending order. In our study, drug was the most common cause of poisoning. But it' s proportion was decreased compared with previous report, and carbone monoxide intoxication was markedly decreased. But, we noticed that the proportion of household material, such as glacial acetic acid and other household chemical, was increased. So, we should be pay more attention to poisoning and try to prevent them.

Factors Associated with Pneumothorax in Hyaline Membrane Disease In fants Receiving Mechanical Ventilation

No abstract available.

Impact of Perinatal Risk Factors on Mental or Psychomotor Developmental Outcome of Very Low Birth Weight Infants

PURPOSE: To determine which perinatal risk factors influence neurodevelopmental outcome, we try to investigate which perinatal risk factors are correlated with Baley mental or psychomotor developmental index using BSID-II test (Baley Scales of Infant Development, II) in long term follow up of very low birth weight who received neonatal intensive care. METHODS: This study was performed on 56 very low birth weight infants with corrected age 24 months who were admitted to the neonatal intensive care unit. We analyzed correlation between perinatal risk factors and neurodevelopmental outcome assessed by mental and psychomotor developmental index. RESULTS: Mean birth weight was 1317.8+/-168.5g : Small for gestational age was 11 cases and appropriate for gestational age was 45 cases : Mean mental developmental index was 93.8+/-10.3 and psychomotor developmental index was 99.9+/-13.2. Mean developmental scale did not show any significant difference between perinatal risk factors, such as sex, ventilator care and its duration, hospitalization days, birth weight, small for gestational age. Mental or psychomotor developmental score was significantly delayed in the subgroup with grade III intraventricular hemorrhages and periventricular cyst. On cranial ultrasonogram, sinificantly low mental, psychomotor developmental score was found on the subgroup with grade III intraventricular hemorrhages and periventricular cyst formation larger than 3mm size (P<0.01). CONCLUSIONS: These findings suggest that grade III intraventricular hemorrhage and periventricular cyst larger than 3mm size are associated with poor prognostic risk factors regarding to low neurodevelopmental index score.

Unbalanced Translocations of Chromosome 2 and Chromosome 20 in a Two-Generation Family

An unbalanced translocation is frequently the result of inheritance of an unbalanced haploid set from a parent with a balanced translocation. Families in which one parent is a balanced translocation carrier fall into the following classes : Those in which none of the possible abnormal offsprings is viable; Those in which one type of offspring, usually the one with the smaller deletion, is born alive; Those in which two types of abnormal offspring are viable. We report a neonate whose karyotype was 46,XX,der(2)t(2;7)(q21;p21.2),der(20)t(2;20)(q21;p13). She was small for her gestational age and had multiple anomalies such as exophthalmos, corneal opacity, short neck, tongue tie, clinodactyly, atrial septal defect, patent ductus arteriosus and ventriculomegaly. Moreover, her mother's karyotype was 46,XX,der(2)t(2;7)(q21;p21.2),del(16)(q22.1),der(20)t(2;20)(q21;p13) but her father had normal karyotype. The same derivative chrosomes were found between mother and her infant, except for del(16)(q22.1) in her mother and these same unbalanced translocations in a two-generation family are extremely rare.

A case of Ellis-van Creveld syndrome

No abstract available.

A Case of Primary Multiple Intracerebral spergillosis Abscess in Neonate:Treatment with Amphotericin B, Flucytosine and Itraconazole

Fungal infection of the central nervous system tends to occur mostly in immunosuppressed patients. In the pediatric population, it is usually seen in severely immunocompromised patients, particulary in children with chronic granulomatous disease and hematopoietic malignancies. Although aspergillosis is considered one of the most frequent agents of mycotic infection of the brain, it is especially rare in the neonatal period, and overwhelming multisystem infection is usually diagnosed postmortem. Manifestations include meningitis, meningoencephalitis, granulomata formation, brain abscess, vasculitis, aneurysm formation, infarct and intracranial hemorrhage. We present a neonate who had brain abscess diagnosed by MRI, and aspergillus was found at surgical exploration. There are very few reported survivors of neonatal aspergillosis. We reported a case of primary multiple brain abscess caused by aspergillus associated with severe hypernatremic dehydration and prerenal azotemia. The patient was treated with amphotericin B combined with flucytosine and itraconazole.