A case of lethal kwashiorkor caused by feeding only with cereal grains / 소아과

Kwashiorkor is a syndrome of severe protein malnutrition, which manifests itself in hypoalbuminemia, diarrhea, dermatitis, and edema. It can be life-threatening due to associated immune deficiency and an increased susceptibility to infections. Kwashiorkor should be treated early with nutritional support and the control of infection. Dilated cardiomyopathy may develop during the treatment and in such cases a poor prognosis is expected. Kwashiorkor has been known as a common disease of poor countries. To date, in fact, there has been no report of kwashiorkor leading to death in technically advanced countries. We here report a fatal case of a baby girl admitted with kwashiorkor. She had been fed only with cereal grain mixed with juice, without any protein supplement, for 2 months. This diet was deficient not because of poverty, but due to the illiteracy of her parents. The patient suffered from diarrhea, whole body edema, hypothermia, and dermatitis. Laboratory findings revealed an immune-deficient state featuring leukopenia and decreased immunoglobulin. Blood and urine cultures revealed Alcaligenes Xylosoxidans growth. The patient was fed frequent small amounts of protein-containing formula and intravenous albumin and micronutrients were administered for nutritional support. She was also treated with intravenous immunoglobulin and antibiotics in order to control infection. Nevertheless, she developed dilated cardiomyopathy and multi-organ failure and died. We review this case in light of the literature.

A case of Balantidium coli in the gastric juice of a neonate / 대한주산의학회잡지

Balantidium coli is the only pathogenic ciliate parasitizing humans. Balantidium coli is widely distributed in hogs, particularly in warm and temperate climates, and in monkeys in the tropics. It is infrequently pathogenic for man. Balantidium coli is considered as a possible pathogen in immunocompromised patients. The infection fundamentally affects the colon and cause variable clinical pictures, from asymptomatic to serious dysenteric forms. Apart from intestinal involvement there have been reports on lung, liver, vagina infection by Balantidium coli. Balantidium coli were observed in the gastric juice of neonate. this is first report of asymptomatic balantidiasis in neonate.

A Case of Esophageal Achalasia in a 10-Year-Old Girl with Persistent Asthma / 소아알레르기및호흡기학회지

Achalasia is a primary esophageal motor disorder, which is a rare disease with an estimated prevalence of 0.5-1 per 100,000 general population. The typical symptoms are vomiting, dysphagia and weight loss, but the respiratory symptoms are often presented in the 20-30% of the patients. The respiratory symptoms are known to be caused by the regurgitation or aspiration of the undigested food. Choking, recurrent pneumonia nocturnal cough are common and bronchiectasis, lung abscess and atelectasis also have been reported. The treatment of achalasia aimed at improving esophageal peristalsis by reducing pressure at the lower esophageal sphincter. We experienced a case of esophageal achalasia coincidentally found in a 10-year-old girl who was admitted with the exacerbation of asthma. She had pneumatic dilatation and her asthma symptoms including nocturnal cough much improved.

Neuroprotective effects of geneticin (G418) via apoptosis in perinatal hypoxic-ischemic brain injury / 소아과

PURPOSE: Some antibiotics were known to exert neuroprotective effects in the animal model of hypoxic-ischemic (H-I) brain injury, but the mechanism is still unclear. A recent study reported that geneticin (G418), an aminoglycoside antibiotic, increased survival of human breast cancer cells by suppressing apoptosis. We investigated the neuroprotective effects of systemically administrated geneticin via anti-apoptosis following the H-I brain injury METHODS: Seven-day-old Sprague-Dawley rat pups were subjected to unilateral (left) common carotid artery occlusion followed by 2.5 hours of hypoxic exposure and the cortical cell culture of rat brain was done under a hypoxic incubator. Apoptosis was measured in the injured hemispheres 7 days after H-I insult and in the injured cells from hypoxic chamber using morphologic analysis by Terminal dUTP Nick-end Labeling(TUNEL) assay and immunohistochemistry for caspase-3, and cytologic analysis by western blot and real time PCR for bax, bcl-2, and caspase-3. RESULTS: The gross appearance and hematoxylin and eosin stain revealed increased brain volume in the geneticin-treated animal model of perinatal H-I brain injury. The TUNEL assay revealed decreased apoptotic cells after administration of geneticin in the cell culture model of anoxia. Immunohistochemistry showed decreased caspase-3 expression in geneticin-treated cortical cell culture. Western blot and real-time PCR showed decreased caspase-3 expression and decreased ratio of Bax/Bcl-2 expression in geneticin-treated animal model. CONCLUSION: Geneticin appears to exert a neuroprotective effect against perinatal H-I brain injury at least via anti-apoptosis. However, more experiments are needed in order to demonstrate the usefulness of geneticin as a preventive and rescue treatment for H-I brain injuries of neonatal brain.

A Case of Addition of Chromosome 3 associated with Multiple Anomalies

Chromosomal anomalies are associated with various congenital malformations and impaired development. The addition or duplication of chromosome 3 is a very rare chromosomal anomaly, in comparison to the deletion of chromosome 3. To date, only one case of the duplication of chromosome 3p has been reported, but an addition or duplication of chromosome 3p was not reported in Korea. We experienced a case of the addition of chromosome 3 in a male newborn infant who had suffered from multiple anomalies and congenital heart disease, atrioventricular septal defects and coarctation of the aorta. The karyotype of this patient was 46, XY, add(3)(p25). We report the case with the review of the associated literatures.

The Expression of Isoforms of Nitric Oxide Synthase (NOS) and Subunits of N-methyl-D-aspartate (NMDA) Receptor according to Administration of Mycophenolic Acid before or after Perinatal Hypoxic Ischemic Brain Injury

PURPOSE: Mycophenolic acid (MPA), a potent inhibitor of inosine-monophosphate dehydrogenase (IMPDH), was used as a new immunosuppressive drug since 1990s. It was reported that MPA increased neuronal survival after excitotoxic injury, induced apoptosis in microglial cells, inhibited the induction of inducible nitric oxide synthase (iNOS) in astrocytes. and inhibited microglial cell proliferation in N-methyl-D-aspartate (NMDA) induced hippocampal cells. However, the effects of MPA on the perinatal hypoxic-ischemic (HI) brain injury had not been yet evaluated. Therefore, we examined whether MPA could be neuroprotective in the HI brain injury. METHODS: Cortical cells were cultured using a 18-day-pregnant Sprague-Dawley (SD) rats and incubated in 1% O2 incubator for hypoxia. MPA (10 ug/mL) before or after a HI insult were treated. Seven-day-old SD rat pups were subjected to left carotid occlusion followed by 2.5 hours of hypoxic exposure (8% O2). MPA (10 mg/kg) were administrated intraperitoneally before or after a HI insult. Nitric oxide (NO) activity and expression of N-methyl-D-aspartate (NMDA) receptors also measured using Real-time PCR with primer pairs of isoforms of NOS; iNOS, endothelial NOS (eNOS), neuronal NOS (nNOS), and subunits of NMDA receptors; NR1, NR2A, NR2B, NR2C, NR2D. RESULTS: The expression of iNOS was decreased in the hypoxia group but increased in the MPA-treated group. However express or that eNOS and nNOS were inversed. The expression of all NMDA receptor subunits except NR2B was decreased in the hypoxia group but increased in the MPA-treated group. CONCLUSION: This study indicates that the administration of MPA before a HI insult could significantly protect against perinatal HI brain injury via some parts of NO-mediated or excitotoxic mechanisms.

The Cardioprotective Effects of Resveratrol via Anti-Apoptosis in Hypoxic Injury of Myocardial Cells

BACKGROUND AND OBJECTIVES: Resveratrol (trans-3, 4', 5-trihydroxy-stilbene), a naturally occurring polyphenolic phytoalexin found abundantly in grape skins and red wines, has been reported to protect heart cells from ischemia/reperfusion (I/R) injury through its significant antioxidant properties. Apoptosis of cardiac myocytes is also involved in several cardiovascular diseases, but it remains unknown whether the protective effects of resveratrol in hypoxic myocardial cell injury are mediated via suppression of apoptosis. In this study, we investigated whether resveratrol confers cardioprotection against hypoxia via anti-apoptosis in a hypoxic model of cultured H9c2 cardiomyoblasts. MATERIALS AND METHODS: H9c2 cardiomyoblasts were obtained from the Korean Cell Line Bank. The cultured cells were divided into four groups: a normal control group, a hypoxia group, a group treated with resveratrol (10 microgram/mL) before hypoxic insult, and a group treated with resveratrol (10 microgram/mL) after hypoxic insult. The control group was placed in 5% CO2 incubators, and the hypoxia and resveratrol-treated groups were placed in 1% O2 incubators. Apoptosis was assayed by cytological analysis with Western blotting and real-time PCR for Bcl-2, Bax, and caspase-3. RESULTS: The expression of Bcl-2 was significantly decreased in the hypoxia group compared with the control group, and resveratrol treatment inhibited the hypoxia-induced decline of Bcl-2 in hypoxic myocardial cells. Conversely, the expressions of Bax and caspase-3 were significantly increased in the hypoxia group, while resveratrol inhibited the hypoxia-induced increase of Bax and caspase. In addition, hypoxia significantly increased the ratio of Bax/Bcl-2 expression, but it was significantly decreased in the resveratrol-treated group. CONCLUSION: The present study demonstrates that the cardioprotective effects of resveratrol in hypoxic injury are mediated via the mechanisms of anti-apoptosis.

Developmental Changes in Immune Responses During Respiratory Syncytial Virus Bronchiolitis / 소아알레르기및호흡기학회지

PURPOSE: This study was performed to examine the developmental changes in the innate or adaptive immune responses in serum during naturally occurring acute respiratory syncytial virus (RSV) bronchiolitis. METHODS: Forty-five children who were admitted with acute RSV bronchiolitis were enrolled. Two patient groups were defined: Group 1: young infants under 6 months of age; and Group 2: young children of less than 2 years of age. Immune responses in serum were determined in these two patient groups and compared with two age-matched control groups (Control 1 and 2), respectively. Serum samples were obtained on admission and analyzed for sCD14, a receptor for innate immune response, and the cytokines, IFN-gamma and IL-13 using ELISA. RESULTS: Serum sCD14 levels were significantly higher but IFN-gamma and IL-13 levels were lower in control 2 compared with control 1. During RSV bronchiolitis, serum sCD14 increased significantly in both patient groups comparing each age-matched control group, which was even greater in group 2. IFN-gamma significantly increased in group 2, but showed a trend to decrease in group 1. IL-13 levels were significantly higher in group 2, but showed no significant difference in group 1 compared with age-matched controls. CONCLUSION: Our study showed that innate immunity plays an important role in the pathophysiology of acute RSV bronchiolitis during early life and suggests that developmental changes in the innate and adaptive immunity may be considered in treatment of the disease.

A Case of Membranoproliferative Glomerulonephritis Associated with Complement Deficiency and Meningococcal Meningitis

Hypocomplementemia is found in all types of membranoproliferative glomerulonephritis (MPGN) but not in all patients. Hypocomplementemia can be ascribed to at least two circulating complement reactive modalities. The activation of the classical pathway produced by circulating immune complexes and the presence in the blood of anticomplement autoantibodies, called "nephritic factor"(NF). The activation of the classical pathway by circulating immune complexes is probably the major mechanism responsible for hypocomplementemia in idiopathic MPGN type I. Nephritic factors have been shown to be responsible for the hypocomplementemia in both MPGN type II and III. NFa is probably the major mechanism responsible for the hypocomplementemia of idiopathic MPGN type II. NFt appears to be solely responsible for the hypocomplementemia in MPGN type III. Judging from the complement profile, NFt also may be present in some patients with MPGN type I. Although infection by meningococcus has been associated with deficiency of any of the plasmatic proteins of complement, it more commonly involves deficiency of the terminal components of the complement pathway(C5-C9). We experienced a patient who had MPGN and meningococcal meningitis. We examined the complement level and significantly lower levels of C3, C5 were found persistently. C7 was low at first and it returned to normal range after 2 months. C9 was normal at first, and was low after 2 months. This is the first reported case in which MPGN with meningococcal meningitis occurred.

A Case of Ring Chromosome 20 with Mental Retardation and Epilepsy / 소아과

Ring chromosome 20 mosaicism [r(20)] is a rare chromosomal anomaly associated with minor dysmorphism, mental retardation, autistic behavior, and intractable epilepsy. The proposed mechanism of ring formation is breakage of both short and long arms of a chromosome with subsequent end-to- end fusion. We encountered an 18-month-old boy who presented with developmental delay and mental retardation with seizure episodes, but showed normal brain magnetic resonance imaging. Chromosome study from peripheral blood showed 46,XY, r(20)(p13q13.3) karyotype. The authors report a case of ring chromosome 20 with mental retardation and epilepsy, with a review of the literature.

A Life-Threatening Case of Tubular Esophageal Duplication Complicated with Aneurysm of the Aorta / 소아과

Esophageal duplication cysts are rare congenital lesions that occur as a result of a failure in the tubulation of the esophagus. They are most frequently single, tubular, or cystic. They may cause compressive symptoms or may be discovered incidentally on chest radiographs. They become symptomatic when complications develop. Symptoms often are related to the location of the duplication; esophageal lesions can create respiratory difficulties. The definitive diagnosis of esophageal duplication cysts requires the pathological evaluation of the cyst after surgical removal. We experienced a rare tubular esophageal duplication, in a 2-month old girl who presented with fever and grunting. This is the first reported case in which the sequence of events of ruptured tubular esophageal duplication with empyema, mediastinitis and aneurysm occured.

The Effectiveness of Intravenous Immunoglobulin for Clinically Suspected Neonatal Sepsis / 소아과

PURPOSE: The purpose of this study is to determine the effectiveness of intravenous immunoglobuin (IVIG) administration in fullterm neonates having clinically suspected neonatal sepsis. METHODS: Forty full-term neonates admitted to the neonatal intensive care unit with clinically suspected neonatal sepsis, who had at least two positive diagnostic criteria were enrolled. Twenty neonates were enrolled into the IVIG arm and 20 in the placebo arm. Neonates with a gestational age of less than 36 weeks and those with any major congenital malformation were excluded. The neonates were randomized to receive 1 g/kg of IVIG or equivalent amount of normal saline. The treatments including antibiotics and supportive care were administered. RESULTS: The neonates in the therapy and placebo groups were comparable in terms of birth weight, gestational age, sex distribution, duration of antibiotics therapy and admission, elevation of serum IgG level, mortality rate, change of CBC, and serum level of acute phase reactants etc. CONCLUSION: Serum IgG values increased significantly 5 days after administration of IVIG in the IVIG-treated group and decreased significantly 5 days after administration of normal saline in the placebo group. However, there was no significant difference in the duration of antibiotics therapy and admission, or of mortality between the IVIG-treated and placebo groups. No adverse reactions to the IVIG infusions were noted during the study. Our preliminary observations suggest that the administration of 1 g/kg IVIG to neonates had some effect on augmentation of humural immune status in neonates with clinically suspected sepsis. But further study is needed to verify the benefit of IVIG infusion to neonatal sepsis.

Increased Serum Endothelin (ET) -1 in Children with Mycoplasma pneumoniae Pneumonia / 소아알레르기및호흡기학회지

PURPOSE: Mycoplasma pneumoniae frequently causes lower respiratory illnesses in children, and is known to be associated with acute wheezing and the exacerbation of asthma. However, the mechanism by which this pathogen contributes to the development of wheeze- related symptoms is not fully understood. Previous studies have shown that ET-1 is a potent bronchoconstrictor and implicated in the pathogenesis of bronchial asthma. The aim of our study was to examine the serum ET-1 and other cytokines in M. pneumoniae pneumonia and investigate if there is any difference in relation to the presence of wheezing. METHODS: Patients admitted with pneumonia were divided into three groups: M. pneumoniae pneumonia with wheeze (group 1) and without wheeze (group 2), and patients with pneumonia due to other pathogens (group 3). The serum levels of ET-1, IL-18, IL-5 were measured by ELISA in patient groups and controls. RESULTS: Serum ET-1 increased significantly in the patients with M. pneumoniae pneumonia (groups 1 and 2) compared with group 3 and controls. ET-1 in group 1 was significantly higher than in group 2. IL-5 and IL-18 were higher in patient groups than in controls with no difference between groups. Serum total IgE was significantly higher in group 1 than in group 2. A positive correlation was observed between serum ET-1 and total IgE. CONCLUSION: Our results suggest that ET-1 may be implicated in the pathogenesis of bronchoconstriction and allergic inflammation in the airways of the patients with M. pneumoniae pneumonia.

A Case of Mesodiverticular Band Causing Small Intestinal Obstruction / 대한소아소화기영양학회지

Meckel's diverticulum is found in about 3% of the population, often incidentally during laparotomy or at autopsy. Over 50% of patients who develop symptoms from this anomaly are younger than 2 years of age. The most common symptom of this lesion is intestinal obstruction. Rarely Meckel's diverticulum is complicated by a mesodiverticular band, which is believed to be a remnant of a vitelline artery. We report a 11-year-old girl with small bowel obstruction because of an intestinal hernia beneath the mesodiverticular band. The causative factor was a stenotic area in the terminal ileum caused by a ringlike lipovascular mesenteric band encroaching externally on the lumen. Although the incidence of mesodiverticular bands complicating Meckel's diverticulum is quite low, the rapid clinical course and the associated high mortality rate make this an important disease.

A Case of Hinman Syndrome Complicated by Chronic Renal Failure

Although there have been a few reports of cases in which cancer cells of extrauterine origin were observed in vaginal smears, such findings are relatively uncommon. We recently experienced a case of ovarian serous cystadenocarcinoma diagnosed by cervicovaginal smear in a 56-year-old woman in routine work-up of carcinoma peritonei. The cellular features were several scattered cellular clusters of adenocarcinoma cells in clear background without tumor diathesis. Psammoma body was not present. Exploratory laparotomy confirmed the diagnosis of bilateral ovarian serous cystadenocarcinoma with multiple metastases.

Dynamic Contrast-Enhanced T2*-Weighted Imaging in Acute Cerebral Infarction: Usefulness in Assessment of Cerebral Hemodynamics

PURPOSE: To evaluate the usefulness of dynamic contrast-enhanced T2*-weighted MR imaging for assessing cerebral hemodynamics in acute cerebral infarction. MATERIALS AND METHODS: We performed dynamic T2*-weighted imaging(TR/TE/flip angle : 40/26/10 degrees, 64 x 128 matrix, 5 sec scan time) in conjunction with conventional T1- andT2-weighted imaging and MR angiography in 20 patients with acute cerebral infarction. The regions of ischemia seenon T2-weighted image(high signal intensity), MR angiogram(vascular stenosis or occlusion) and dynamic contrast-enhanced T2*-weighted image(perfusion defect) were topographically compared with each other. Relative cerebral blood volumes(rCBVs) of the ischemic regions and of contralateral normal regions were calculated fromsignal intensity data measured on dynamic contrast-enhanced T2*-weighted images, and were compared. RESULTS: Regions of ischemia were topographically well correlated on T2-weighted image, MR angiogram and dynamic contrast-enhanced T2*-weighted image in all but two patients, of whom one had recanalization of the occluded artery and the other had a small infarction. rCBVs were asymmetrically reduced in the ischemic regions in all butone patient who had recanalization of the occluded artery. CONCLUSION: Dynamic contrast-enhanced T2*-weighted imaging is a useful method in the qualitative and quantitative assessment of cerebral hemodynamics in acutecerebral infarction. The use of this imaging technique along with conventional MR imaging and MR angiography provides better hemodynamic information in acute cerebral infarction.

CT Findings of Mucinous Adenocarcinoma in Gastrointestinal Tract

PURPOSE: To evaluate CT findings of mucinous adenocarcinoma in the gastrointestinal tract. MATERIALS AND METHODS : CT scans of 24 gastric and five colorectal mucinous adenocarcinomas, proven by histology, were retrospectively analysed; the patients consisted of 18 men and 11 women (age range, 27-76; mean, 59). CT findings were analysed, with emphasis on : (a) tumor size and maximal wall thickness ; (b) the presence of a low attenuation area, suggestive of a mucin poll within the tumor ; (c) the presence, shape and location of calcification, and (d) correlation between primary tumor (T) staging and CT findings. RESULTS: The mean tumorsize of gastric mucinous adenocarcinoma was 8.2cm (range, 1.4 - 17cm) and the mean maximal wall thickness was2.3cm (range, 1-4.5cm). Low attenuation areas on enhanced CT were seen in 12 cases (50%). Mottled, punctate, diffuse calcifications were demonstrated in nine cases(38%), and were located in low attenuation areas in eight cases. The T staging could be determined in 22 cases. Of there, low attenuation areas were demonstrated in tencases and calcification in seven. Of those ten cases with low atteuation area T staging was T2 in two cases, T3 intwo, and T4 in six. Of the cases showing calcification, T staging was T3 in one case and T4 in six. The mean sizeof colorectal mucinous adenocarcinoma was 6cm(range, 3-13cm) and the mean maximal wall thickness was 3.6cm (range,1.5-7cm). Low attenuation area were seen in three cases. Mottled calcification within the low sttenuation was detected in one case. The T staging of three cases which showed a low attenuation area was T3 in tow cases and T4in one case. One case with calcification was T3 stage. CONCLUSION: The CT finding of mucinous adenocarcinoma inthe gastrointestinal tract was a relatively thick-walled mass containing an area of low attenuation or calcification. Although calcification is believed to be a pathognomonic finding for the specific diagnosis of mucinous adenocarcinoma, a low attenuation area may be an important CT finding because it can be detected at lower T staging and more frequently.