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.

Status of Alternative Therapies Used by the Children Visiting the 'Growth Clinic' / 대한소아내분비학회지

PURPOSE: Although growth hormone treatment is now widely used to treat some group of children with short stature, it is still expensive and many parents are seeking alternative therapies. We report the current status of alternative therapies for children visiting the 'growth clinic' in a single tertiary center. METHODS: Questionnaire about the usage of alternative therapy was used. Parents were asked whether they used alternative therapies and where they achieved the information. Also, they were asked whether the treatment was effective, and whether they would continue to use the alternative therapy. RESULTS: Two hundred and twenty nine children (127 boys, 102 girls) visiting the growth clinic in Dongsan Medical Center from February 2005 to January 2006 were analyzed. Their ages were between 6.1 years to 15.4 years (mean 10.4 years). Height Standard Deviation Score (SDS) were between -3.2 to 1.0 (mean -1.1). Twelve boys (9.4%) and 7 girls (6.9%) were more than zero in height SDS value. Among 145 children (63.3%) who used the alternative therapy, supplemental foods and oriental herbs were most widely used (43.3% each) followed by over-the-counter drugs. They attained the information most frequently from their neighbors followed by television, newspaper, and internet. More than half (57.9%) of the parents answered that the alternative therapies were not effective. However, 46.9% of the parents answered they would continuously use the therapy though it is ineffective. CONCLUSION: Many patients visiting the 'growth clinic' for short stature had experiences on the alternative therapies. Although these therapies seemed not quite effective, many parents answered that they would continue alternative therapy.

A Case of Meningococcal Infection necessitating Amputation of Both Legs in a Child

Meningococcal sepsis-associated purpura fulminans is a rapidly progressing condition with high morbidity and mortality. There are several reports of amputation of extremities due to gangrenous change in this condition. However, in Korean literature, we found only one case report associated with amputation of one leg due to meningococcal infection. We report a case of meningococcal infection necessitating the amputation of both legs in a previously healthy seven-year-old girl with a review of literatures.

A Case of Meningococcal Infection necessitating Amputation of Both Legs in a Child

Meningococcal sepsis-associated purpura fulminans is a rapidly progressing condition with high morbidity and mortality. There are several reports of amputation of extremities due to gangrenous change in this condition. However, in Korean literature, we found only one case report associated with amputation of one leg due to meningococcal infection. We report a case of meningococcal infection necessitating the amputation of both legs in a previously healthy seven-year-old girl with a review of literatures.

Change of absolute neutrophil count after intravenous immunoglobulin administration for the children with idiopathic thrombocytopenic purpura / 소아과

PURPOSE: Intravenous immunoglobulin (IVIG) is effective for the treatment of idiopathic thrombocytopenic purpura (ITP) in children. Recently, several reports have been published that show its impact on the absolute neutrophil count. The present study was performed to confirm these findings. METHODS: Data on 26 ITP patients were analyzed. Patients with febrile illness or increased C-reactive protein levels at presentation, which would influence the neutrophil counts, were excluded to determine the sole impact of IVIG. In addition, patients who received steroid treatment were also excluded. RESULTS: Sixteen boys and ten girls were analyzed. For patients who received an IVIG dose of 0.4 g/kg/day (n=17), the absolute neutrophil count (ANC) measured next day was significantly decreased. For patients who received an IVIG dose of 1 g/kg/day (n=9), the ANC measured the next day was also significantly decreased. However, the decrease was more profound in the high-dose group compared to the low-dose group. Among six cases with profoundly decreased ANC greater than 1,000/ mm3, four patients (67%) received IVIG at a dose of 1 g/kg/day. All four cases with increased ANC were treated with IVIG dose of 0.4 g/kg/day, and three cases (75%) among them had a febrile reaction during IVIG administration. None of the cases with decreased ANC had a febrile reaction. No cases had infectious complications reported. CONCLUSION: IVIG treatment for ITP patients appears to suppress the ANC. This decrease of ANC was more pronounced when a higher dose of IVIG was used. Some cases with increased ANC counts after IVIG use were found only in low-dose IVIG group, and was associated with febrile reactions during IVIG use.

Growth Hormone Therapy in Russell-Silver Syndrome / 대한소아내분비학회지

Russell-Silver syndrome is characterized by low birth weight, growth retardation, delayed bone age, asymmetry, abnormal sexual development, cranio-facial disproportion, short little finger and clinodactyly. Short stature is the major concern, so there have been interests treating Russell-Silver syndrome with human growth hormone. However, there are no large-scale controlled studies to confirm the effectiveness of growth hormone therapy until now because of the rarity of this disorder. In Korea, we cannot find reports dealing with growth hormone treatment in Russell-Silver syndrome. We present three children with Russell-Silver syndrome who showed accelerated growth with growth hormone treatment.

Diagnostic effectiveness of fine needle aspiration cytology on pediatric cervical lymphadenopathy / 소아과

PURPOSE: There are few reports in Korean literature on the diagnostic efficacy of fine needle aspiration cytology(FNAC) for pediatric cervical lymphadenopathy. This study examined the diagnostic value of FNAC on cervical lymphadenopathy in children. METHODS: Data from 57 pediatric patients(aged 0.3 to 14 years) who underwent FNAC due to cervical lymphadenopathy between January 2001 and March 2005 was reviewed retrospectively. RESULTS: Reactive cervical lymphadenitis was the most common result of the FNAC(52.5 percent). Malignant disease were revealed in 14 percent of all cases. The sensitivity, specificity was 86 percent and 96 percent, respectively. The positive and negative predictive values for malignant disease was 75 percent and 98 percent, respectively. There were two false-positive cases on FNAC; one case of Langerhans cell histiocytosis, and another with infectious mononucleosis. A false-negative case on FNAC was found to be acute lymphocytic leukemia. In seven cases(12 percent), the final diagnosis was confirmed by an open biopsy after the FNAC. There were four FNAC cases where the specimen was not satisfactory for making a diagnosis(7 percent). There were no serious complications of the FNAC procedure. CONCLUSION: FNAC had a high diagnostic efficacy for evaluating children with cervical lymphadenopathy.

A case of recurrent hepatoblastoma: lung, heart and brain metastasis / 소아과

Hepatoblastoma is a hepatic tumor predominantly occurring in children. The usual site of metastasis is the lung. There are only several reports worldwide on the distant metastasis of hepatoblastoma to the central nervous system in children. Only one reported case showed survival of a patient after multiple resections of a recurrent brain lesion. Involvement of the cardiovascular system has been reported in the medical literature. Lesions almost always involve the right-side of the heart. We report a case of recurrent hepatoblastoma at multiple sites, including brain, left atrium of the heart and lung in a 6-year-old girl who was partially treated in the past at the age of 1.5 years; the patient had been event-free for four and a half years.

Estimation of Bone Age using Ultrasonic Method / 대한소아내분비학회지

PURPOSE: In evaluating the children with short stature, estimation of bone age is the primary step. Conventionally, radiologist estimates the bone age by comparing the radiographic image (left hand) of the child with the age-standardized atlas or by summing up the points given to individual bones of the hand and wrist. This method needs a skilled radiologist, causes radiation exposure, and it may take time. Recently, commercial ultrasonic method to estimate the bone age (BonAge(R), Sunlight Medical Ltd. Israel) was developed. We intended to compare the estimated bone ages using different methods-by radiographic and ultrasonographic methods. METHODS: One hundred and one boys and 108 girls over 5 years (all Koreans) who visited pediatric department from December 2004 to March 2005 for evaluation of short stature were evaluated. The radiologist estimated the bone age with Tanner-Whitehouse 2 method (Korean version). The ultrasonography was done in outpatient base by residents in pediatric department. We calculated the Pearson's correlation coefficients between each age and sex groups and compared the mean of each group by paired t-test. RESULTS: Ultrasonic bone age estimation was well correlated with radiologic method and was concordant with radiologic estimates in boys under age 12 and premenarchal girls of all ages and in postmenarchal girls under age 14. CONCLUSION: BonAge system could be easily performed without radiation. Further observation to more patients will be needed to see the accuracy of bone age estimation between ultrasonography and radiologic methods in older age group of Korean children.

Bacteremia in pediatric cancer patients: A single center study / 소아과

PURPOSE: Bacteremia is one of the major concerns in the treatment of pediatric cancer patients. This study was to determine the etiologic agents and the pattern of antibiotic susceptibilities in a single tertiary medical center. METHODS: We retrospectively reviewed the medical records of the cases of bacteremia in pediatric cancer patients from 1998 to 2005 in Keimyung University Dongsan Medical Center. RESULTS: There were 62 cases of bacteremia from 44 patients. Gram-positive organisms(48.3%) were more common than gram-negative organisms(38.7%) or fungi(13%). Among gram-positive organisms, Staphylococcus epidermidis was the most common etiologic agent(63.3%), followed by Staphylococcus aureus(16.7%), alpha-hemolytic Streptococcus(16.7%), and Streptococcus mitis(3.3%). Among gram-negative organisms, Alcaligenes xylosoxidans was the most common agent(41.7%) and the other organisms were Klebsiella pneumoniae(20.8%), Stenotrophomonas maltophilia(12.5%), Acinetobacter baumanii(8.2%), etc. In febrile neutropenic patients, however, K. pneumoniae was the most common cause of gram-negative bacteremia. All of the isolated K. pneumoniae in our center produced extended-spectrum beta-lactamase and were related with high mortality. S. aureus, S. epidermidis, and Streptococcus species were all susceptible to vancomycin and teicoplanin. Most staphylococci were resistant to penicillin and oxacillin. Most of the gram-negative organisms were susceptible to imipenem. CONCLUSION: Gram-positive organisms were more commonly isolated than gram-negative organisms in pediatric cancer patients like other studies. We could obtained valuable information on the choice of proper antibiotics in our institution. Further studies will be needed to explain the prevalence of A. xylosoxidans in our center.

A Suggested New Clinical Classification for Pediatric Intussusception / 대한소아소화기영양학회지

PURPOSE: We proposed a new classification of pediatric intussusception based on clinical and radiologic findings. METHODS: Data from 88 consecutive patients with intussusception were reviewed. We retrospectively analyzed six factors; patient age, sites of intussusception, symptoms, therapeutic methods, existence of enlarged mesenteric lymph nodes, and ultrasonographic (US) findings from clinical records. RESULTS: 1) There was one neonatal case (1.1%), the others (98.9%) were infants and children. 2) These 87 infant and child cases consisted of 14 cases (16.1%) of small bowel intussusception (SBI) and 73 cases (83.9%) of ileo-colic intussusception (ICI). Of the 14 SBI cases, 12 cases were symptomatic and 2 cases were asymptomatic. The symptomatic group comprised 8 transient cases (66.7%), 3 operative cases (25.0%), and 1 enema-reduction case (8.3%). Two asymptomatic cases were incidentally captured by computed tomography. Of the 73 ICI cases, 19 cases (26.0%) required operation, and 54 (74.0%) enema-reduction. 3) When transient SBI cases were compared with operated SBI cases, enema-reduced and operated ICI cases, the age (38.0+/-22.9 months) of transient SBI cases were significantly higher than those of the others (p=0.003). Mean mass size (20.8+/-2.7 mm) in transient SBI was significantly smaller than in the others (p=0.0001). 4) No correlation was found between the existence of enlarged mesenteric lymph nodes and therapeutic method or concomitant illness. 5) Most of the target types observed by US were in transient SBI cases, the remainder were in the enema-reduced ICI cases. In terms of the doughnuts type, all 8 cases (34.8%) with an external hypoechoic rim thickness of >8.9 mm were treated surgically. CONCLUSION: Pediatric intussusception may be classified based on clinical and radiologic findings, which are likely to indicate appropriate therapies.

A Case of Isolated Rice Allergy / 대한소아소화기영양학회지

Rice allergen has low antigenicity, and thus, anaphylactoid reactions to rice are exceedingly rare. We experienced a case of isolated rice allergy in a 5 month-old girl who had been fed a milk formula without incident. However, after feeding a powdered weaning milk formula containing rice, she developed symptoms of projectile vomiting and diarrhea, at this time rice specific antigen tests were all negative. One month later a challenge test was performed using a rice gruel, and her symptoms recurred. Endoscopic and microscopic findings showed hyperemic mucosa in the duodenum and subtotal villous atrophy. Thereafter, she showed no adverse reaction to almost all foods appropriate for her age, but after feeding rice gruel at 10 months, she developed symptoms of cyanosis and vomiting. However, none of the allergic symptoms were demonstrated at 13 months upon repeated challenge test. Currently, she is 28 months old and tolerates all foods including rice.

A Case of PFAPA Syndrome Mimicking Cyclic Vomiting Syndrome / 대한소아소화기영양학회지

PFAPA syndrome is characterized by periodic fevers associated with aphthous stomatitis, pharyngitis, and cervical adenitis and is unusual in infants and children. We report on a case of PFAPA syndrome mimicking cyclic vomiting syndrome in a 42-month-old girl. She had experienced multiple episodes of cyclic vomiting with abdominal pain from age 20 to 30 months. When she was 30 months old, periodic fever with pharyngitis was combined with cyclic vomiting, and when 40 months old, aphthous stomatitis and cervical adenitis were added. These periodic symptoms and signs were not treated with prokinetics or antibiotics. Symptom duration of an episode was 3 days. After cimetidine therapy (150 mg three times daily for 6 months), her febrile and cyclic vomiting episodes ceased. At the time of writing she had not received therapy for 10 months and has remained well without periodic attack.

Risk Factors for the Early Recognition of Cow's Milk Protein-induced Enterocolitis / 소아과

PURPOSE: Cow's milk protein-induced enterocolitis (CMPIE) is a symptom complex of vomiting and/ or diarrhea caused by delayed hypersensitivity and may result in serious complications. This study was undertaken to identify high risk factors to facilitate the early recognition of CMPIE. METHODS: We reviewed the data of 101 patients, aged 15 to 45 days, admitted due to vomiting and/ or diarrhea between 2003 and 2004. After excluding 13 patients absolutely breast-fed and 2 patients transferred from other hospitals with the impression of CMPIE, the 86 study subjects were divided into three groups based on the underlying etiologies; CMPIE, infectious and non-infectious group. RESULTS: CMPIE was diagnosed in 11 patients (12.8%). On admission, failure to gain weight (P= 0.003), hypoalbuminemia (P=0.003), peripheral leukocytosis (P=0.015), and metabolic acidosis (P=0.014) were more significant in the CMPIE group than in the others. Multiple logistic regression analysis showed that the independent predictors of high risks for CMPIE were failure to gain weight < 10 g/day (OR, 10.25[95% CI, 1.62-65.06]) and serum hypoalbuminemia < 3.5 g/dL (OR, 9.18[95% CI, 1.69- 49.74]). Cow's milk challenges were performed in the 11 CMPIE patients; vomiting (81.8%), abnormal stool test (80.0%), peripheral leukocyte count and absolute neutrophil count (ANC) increase (100.0%) (P< 0.05), and enteropathy (100.0%). CONCLUSION: CMPIE is not a rare clinical disease in early infancy. The high risk factors of CMPIE were identified as follow: failure to gain weight below 10 g/day, hypoalbuminemia on admission and a rapid decrease during admission. Cow's milk challenge test with endoscopic duodenal biopsy was helpful to confirm CMPIE.

Establishment and Characteristics of the B Cell Line (KEB1) from the Bone Marrow Cells of the Patient Infected with Epstein-Barr Virus / 대한혈액학회지

BACKGROUND: Cell lines can be established when the cells are clonally selected and propagated continuously in vitro culture system. Recently we established a B cell line (KEB1) from the bone marrow cells of the patient infected with Epstein-Barr virus (EBV). METHODS: The patient's initial platelet count was 1,000/microliter and peripheral blood smear showed atypical lymphocytes accounting 20% of the differentials of WBC. Antibodies to EBV and PCR for EBV were positive but heterophil antibody was negative. Mononuclear cells were obtained by Ficoll-paque separation and suspended in RPMI media with 10% FCS. After incubation in 37degrees C, 5% CO2 incubator, cells grew continuously and finally immortalized to B cell line. RESULTS: Cells showed abundant, clear basophilic cytoplasms and a few vacuoles. Cells had granular reaction in PAS stain and were positive to B cell antibodies. Immunohistochemical stain showed positive expression for EBV antibody. Electron microscopic finding of cultured cells showed several viral particles, and immunoelectron microscopic finding showed electron dense expression. Immunophenotyping of cultured cells was positive for B lymphoid lineage, and karyotypings had hypotetraploidy. Cells expressed MAGE and SSX gene. Cytotoxicity showed relative resistance to mistletoe and several chemotherapeutic agents compared to leukemic cell line. CONCLUSION: KEB1 cell line was established from the bone marrow cells of the patient with infectious mononucleosis. The characteristics of the cell lines including morphology, immunophenotype, karyotype, gene analysis (MAGE, SSX) and chemosensitivity were analyzed. There should be further studies of these cell lines including gene analysis, telomerase activity and cytokine production. This cell line might be helpful to establish another normal lymphocyte cell line and to predict the toxicity of chemotherapy.

A Case of Childhood Acute Lymphoblastic Leukemia with Monosomy 20 and Parotid Gland Involvement / 소아과

Hypodiploidy exists in 3-15% of patients with childhood acute lymphoblastic leukemia(ALL) and is associated with a poor prognosis. Monosomy 7 and monosomy 20 account for most karyotypic abnormalities in patients in whom whole chromosomes are lost and their incidences are rare. Parotid tumors in the pediatric age group are unusual and in 1996, there was a case of invasion of the parotid glands of ALL in a 6-year-old boy with swelling of both parotid glands. But invasion of acute lymphocytic leukemia to the parotid gland has not yet been reported in Korea. Here, we report a 33 month-old boy with swelling of both parotid glands who was diagnosed to have ALL with monosomy 20 & parotid gland invasion.

Thrombopoietin Level and Bone Marrow Megakaryocyte Colony Formation in Various Diseases with Thrombocytopenia / 대한소아혈액종양학회지

PURPOSE: Thrombocytopenia is a serious life threatening consequence in patients with bone marrow failure syndrome. Thrombopoietin (TPO), recently cloned by several groups has been shown to be a key regulation of megakaryopoiesis and thrombopoiesis. Recent studies have demonstrated a positive or negative relationship between TPO levels and platelet counts due to underlying disease states. To clarify the role of TPO in thrombocytopenic condition we determined plasma TPO levels and megakaryocyte colony assay. METHPDS: TPO levels were measured in thrombocytopenic patient with aplastic anemia, chemotherapy induced bone marrow failure, idiopathic thrombocytopenic purpura (ITP) and in newborn by ELISA (QuantikineTM, R&D System, USA). Controls were short statured normal children with normal platelet counts. Plasma was preserved in 20oC until test. CFU-mega was determined by MegaCultTM (Stem Cell Tech. Inc., Canada). Ficoll separated mononuclear cells were cultured for 10~12 days with TPO or stem cell factor (SCF) in 37degrees C 5% CO2 atmosphere, colonies were fixed, stained and examined with inverted microscope. Results were analysed by Student-t test. RESULTS: TPO levels were markedly increased in aplastic anemia and chemotherapy induced thrombocytopenia compared to those of normal controls. Patients with ITP had decreased level of plasma TPO. There was inverse relationship between platelet count and TPO levels for patients with aplastic anemia and chemotherapy induced thrombocytopenia. There was no definite relationship between platelet counts and TPO levels but inverse relationship between platelet counts and PDW levels in neonates was noted. The levels of TPO were increased after improvement of platelet in thrombocytopenic neonate. Megakaryocyte colonies were increased in the mononuclear cells of the patients with ITP and chemotherapy induced thrombocytopenia. There was little colony formation in aplastic anemia. TPO had no definite effect in megakaryocyte colony formation but SCF increased colony formation. CONCLUSION: TPO levels were increased in aplastic anemia and chemotherapy induced thrombocytopenia but decreased in ITP. There was inverse relationship between platelet count and TPO levels in aplastic anemia and chemotherapy induced thrombocytopenia. Thus TPO could be useful for differentiate the etiology of thrombocytopenia. Megakaryocyte colony was increased in ITP and chemotherapy induced thrombocytopenia, but decreased in aplastic anemia. SCF was effective in megakaryocyte colony formation. TPO and SCF will be helpful to increase platelet in thrombocytopenic patients. However, further study will be needed.

A Case of Acute Leukemia Remitted by Adding Cyclosporin-A Previously Failed with Induction Therapy

Clinical chemotherapy refractoriness is characterized by resistance to multiple drugs. Multidrug resistance(MDR) is caused by over-reactivity of a unidirectional drug efflux pump, transmembrane glycoprotein(P-glycoprotein), which is encoded by the MDR1 gene. P-glycoprotein leads to increased drug efflux and decreased intracellular drug concentration. Clinical trials that attempt to reverse or modulate MDR have been done. Cyclosporin-A and verapamil are the most extensively studied agents and several trials of cyclosporin-A as a MDR modulator have been reported. We report a case of an 8-year-old girl with acute mixed type leukemia who failed to respond 3 times to remission-induction therapy. It led us to conclude she had multidrug resistance. We tried a fourth induction chemotherapy including cytarabine, idarubicin and 6-thioguanine to which cyclosporin-A was added. Then, she showed signs of severe bone marrow depression and fulminant perianal cellulitis. But she recovered and successfully achieved complete remission. The addition of cyclosporine could be useful in achieving complete remission for cases of acute leukemia that resist to usual chemotherapy. Futher observation including more cases will be needed to assess long-term survival and efficacy of adding cyclosporine.

Seizure Patterns of Temporal Lobe and Extratemporal Lobe Epilepsies in Childhood Intractable Epilepsy

BACKGROUND: The clinical characteristics of seizures in adults with localization-related epilepsy have been clearly described and classified. But few researches and data are available in childhood intractable epilepsy. METHODS: We analyzed 334 videotaped seizures from 41 pediatric patients who underwent epilepsy surgery at Dong San Medical Center between 1993 and 1997. Twenty- one of 41 patients had temporal lobe epilepsy, and the remaining 20 patients had extratemporal lobe epilepsy. We divided the patient sample into two groups : (a) those with total cases, (b) those with good outcome (seizure-free or more than 90% reduction of seizures after surgery). We compared temporal lobe epilepsy (TLE) with extratemporal lobe epilepsy (ETE) by clinical symptoms and initial ictal symptoms. In addition, we analyzed the lateralizing value of ictal dystonia, head turning, head deviation and automatism. RESULTS: The aura and head deviation of TLE and the motor arrest, head deviation and dystonic posture of ETE were observed more frequently in total group (P<0.05). In contrast, the vocalization and secondary generalization of TLE and the motor arrest, dystonic posture and dysarthria of ETE were observed more frequently in good outcome group (P<0.05). In cases of the initial ictal symptoms, the aura and hand automatism of TLE and the motor arrest and dystonic posture of ETE were observed more frequently in both groups (P<0.05). Of the ictal motor phenomena, ipsilateral hand automatism and contralateral dystonic posture were useful in lateralizing the seizure focus (P<0.05). CONCLUSION: The clinical ictal manifestations in children are very helpful to localize or lateralize the epileptic focus, like in adults case. Because of the lack of younger age group in our study, however, we suggest more rigorous studies based on more comprehensive data.