A 14-year-old male with rhabdomyolysis associated with psychogenic polydipsia and hyponatremia

Rhabdomyolysis associated with psychogenic polydipsia and hyponatremia is a rare condition that can cause substantial morbidity and mortality. We report a 14-year-old boy with psychogenic polydipsia who experienced recurrent hyponatremia and subsequent rhabdomyolysis. Treatment involved intravenous fluids and restriction of oral water intake. This case emphasizes the importance of early recognition and management of this condition. The possibility of rhabdomyolysis should be considered in patients with hyponatremia who have myalgia.

Slower progression of central puberty in overweight girls presenting with precocious breast development

Purpose@#Overweight (OW)/obese girls tend to have an earlier pubertal onset than girls with normal weight. However, only a few studies have reported the progression of puberty in these girls. This study aimed to identify risk factors for rapid pubertal progression in OW/obese girls presenting with precocious breast development. @*Methods@#This retrospective cohort study reviewed the medical records of 110 OW (body mass index [BMI] ≥85th percentile for age and sex) and 213 nonoverweight (NW, BMI <85th percentile for age and sex) girls who presented with breast budding before 8 years of age. OW girls were divided into 2 subgroups: girls with central puberty progression before 9 years of age (OW-RP) and those without (OW-SP). @*Results@#Progression to central puberty before the age of 9 was more common in NW girls than in OW girls (83.8 % vs. 65.2 % in NW vs. OW group, p<0.001), and progression-free survival for 1, 2, and 3 years was higher in the OW group (p<0.001). In a subgroup analysis of OW girls, the OW-RP subgroup had more advanced bone age (BA) at the first visit (p=0.047) and higher initial luteinizing hormone (LH, p=0.010) levels than the OW-SP subgroup. Being NW (p=0.001) and having more advanced BA (p=0.023) at the initial workup were the risk factors for pubertal progression before age 9. @*Conclusion@#Pubertal progression seems to be slower in OW girls than in NW girls presenting with precocious breast development. However, it can progress rapidly in OW girls with particularly pronounced BA advancement and high LH levels at the initial workup.

2022 Clinical practice guidelines for central precocious puberty of Korean children and adolescents

The Committee of Central Precocious Puberty of Korean Pediatrics and Adolescents of the Korean Society of Pediatric Endocrinology has newly developed evidence-based 2022 clinical practice guidelines for central precocious puberty in Korean children and adolescents. These guidelines provide the grade of recommendations, which includes both the strength of recommendations and the level of evidence. In the absence of sufficient evidence, recommendations are based on expert opinion. These guidelines have been revised and supplement the previous guidelines "Clinical Guidelines for Precocious Puberty 2011," and are drawn from a comprehensive review of the latest domestic and international research and the grade of recommendation appropriate to the domestic situation. This review summarizes the newly revised guidelines into 8 key questions and 27 recommendations and consists of 4 sections: screening, diagnosis, treatment, and long-term outcome of central precocious puberty.

Hypertriglyceridemia with acute pancreatitis in a 14-year-old girl with diabetic ketoacidosis

Diabetic ketoacidosis (DKA) is a medically fatal condition in poorly controlled hyperglycemia or newly diagnosed diabetes mellitus. Severe hypertriglyceridemia (HTG) is an uncommon complication of DKA and can be associated with acute pancreatitis (AP). We present the clinical manifestations, laboratory findings, and management of AP associated with HTG in a 14-year-old girl with DKA. The patient, with a 7-year history of type 2 diabetes presented with epigastric pain, 1 month after stopping insulin injection. DKA, severe HTG, and AP were diagnosed based on the laboratory and imaging tests. She recovered from DKA after conventional treatment for DKA, and her triglyceride (TG) level was reduced from 10,867 mg/dL to the normal range after 7 days of admission without antilipid medication. Given that her C-peptide level was not too low and considering her negative diabetes-related antibodies and high TG level, targeted gene panel sequencing was performed on the genes associated with diabetes and HTG. We identified a heterozygous mutation, c.4607C>T (p. Ala1537Val), in ABCC8 related to maturityonset diabetes of the young (MODY) 12. To our knowledge, this is the first reported case of HTG-induced AP with DKA in a patient with MODY. In addition, we reviewed the literature for pediatric cases of HTG with DKA. In patients with DKA, timely awareness of severe HTG related to insulin deficiency is crucial for improving the consequences of AP. We recommend considering AP in all DKA patients presenting with severe HTG to ensure early and proper management.

Response to vitamin D replacement in overweight and normal weight children with vitamin D deficiency

PURPOSE: It is well known that obesity is related to vitamin D deficiency (VDD). We investigated the response to vitamin D replacement in normal-weight and overweight children. METHODS: This was a prospective study including 62 Korean children with VDD. VDD was defined as a serum 25-hydroxycholecalciferol (25(OH)D) concentration <20 ng/mL. Overweight was defined as a body mass index (BMI)≥the 85th percentile (n=21), and normal weight as a BMI between the 5th and 84th percentiles (n=41). All participants received vitamin D3 supplementation (2,000 IU/day) for 8 weeks. The serum levels of 25(OH)D, PTH and biochemical parameters were measured before and after treatment. RESULTS: The mean age was 10.0±1.4 years in normal-weight children and 10.0±2.1 years in overweight children (P=0.93). After 8 weeks of treatment, 61.9% of normal-weight children and 47.6% of overweight children achieved vitamin D sufficiency (P =0.30). The mean serum 25(OH)D levels after vitamin D replacement were 33.8±7.6 ng/mL and 30.3±6.6 ng/mL in normal-weight and overweight children, respectively (P =0.10). The mean calcium/creatinine ratios after treatment were 0.09±0.07 and 0.08±0.06 in the normal-weight and overweight groups, respectively, and no hypercalciuria was found. In multiple regression analysis, the response to vitamin D replacement was influenced by the BMI (β=-1.0, P=0.03) and sex (β=-4.0, P=0.04). CONCLUSIONS: Eight weeks of vitamin D replacement (2,000 IU/day) is sufficient to overcome vitamin D deficiency in normal-weight and overweight children without any complications.

Delayed diagnosis of 22q11 deletion syndrome due to late onset hypocalcemia in a 11-year-old girl with imperforated anus

Neonatal hypocalcemia and congenital heart defects has been known as the first clinical manifestation of the chromosome 22q11.2 deletion syndrome (22q11DS). However, because of its wide clinical spectrum, diagnosis of 22q11DS can be delayed in children without classic symptoms. We report the case of a girl with the history of imperforate anus but without neonatal hypocalcemia or major cardiac anomaly, who was diagnosed for 22q11DS at the age of 11 after the onset of overt hypocalcemia. She was born uneventfully from phenotypically normal Korean parents. Imperforate anus and partial cleft palate were found at birth, which were surgically repaired thereafter. There was no history of neonatal hypocalcemia, and karyotyping by GTG banding was normal. At the age of 11, hypocalcemia (serum calcium, 5.0 mg/dL) and decreased parathyroid hormone level (10.8 pg/mL) was noted when she visited our Emergency Department for fever and vomiting. The 22q11DS was suspected because of her mild mental retardation and velopharyngeal insufficiency, and a microdeletion on chromosome 22q11.2 was confirmed by fluorescence in situ hybridization. The 22q11DS should be considered in the differential diagnosis of hypocalcemia at any age because of its wide clinical spectrum.

The triglyceride-to-high density lipoprotein cholesterol ratio in overweight Korean children and adolescents

PURPOSE: The triglyceride-to-high-density lipoprotein cholesterol (TG/HDL-C) ratio has recently been reported as a biomarker of cardiometabolic risk in obese children and adolescents. The purpose of this study is to describe the TG/HDL-C ratio and related factors in overweight and normal weight Korean children and to evaluate whether the high TG/HDL-C ratio is associated with insulin resistance in overweight children and adolescents. METHODS: Data from 255 overweight (aged 8.7±2.0 years) and 514 normal weight (aged 8.9±1.8 years) children and adolescents were evaluated. Glucose, insulin, total cholesterol (TC), HDL-C and TG levels were measured after overnight fasting, and the TG/HDL-C ratio, non–HDL-C and the homeostasis model assessment of insulin resistance (HOMA-IR) were calculated. RESULTS: The TG/HDL-C ratio was higher in overweight group compared to normal weight group (P < 0.001). Among overweight children and adolescents, alanine aminotransferase (P=0.018), non–HDL-C (P < 0.001), and HOMA-IR (P=0.004) were different between the TG/HDL-C ratio tertile groups. The prevalence of elevated HOMA-IR was increased with increasing TG/HDL-C ratio tertiles (P for trend=0.003). On regression analysis adjusted for age and sex, the BMI (β=0.402, P=0.001) and TG/HDL-C ratio (β=0.251, P=0.014) were independently associated with HOMA-IR (adjusted R2=0.324). The TG/HDL-C ratio of 2.0 or more showed higher sensitivity (55.6%) and specificity (72.9%), when compared to TC (≥200 mg/dL), non–HDL-C (≥145 mg/dL), and LDL-C (≥130 mg/dL) for identifying overweight children with elevated HOMA-IR. CONCLUSION: The TG/HDL-C ratio is independently associated with insulin resistance in overweight children and adolescents, and it can be useful in identifying those at higher cardiometabolic risk.

Diurnal variation of gonadotropin levels in girls with early stages of puberty

PURPOSE: Pubertal gonadotropin secretion shows circadian pattern and the luteinizing hormone (LH) levels tend to rise in later stages of puberty in girls. We studied the usefulness of basal LH in the evaluation of central precocious puberty with emphasis on the influence of sampling time. METHODS: Medical records of 334 girls that underwent gonadotropin-releasing hormone stimulation test (GnRHST) were reviewed. Auxological and laboratory data were compared between those with early morning (EM, before 10 AM) and late morning/afternoon (LM/A, after 10 AM) basal samples. RESULTS: Among those in sexual maturity rating (SMR) 2, EM samples showed higher basal LH (P=0.004) compare to LM/A samples, whereas those in SMR 3 showed no difference in LH levels between EM and LM/A samples. Among girls with pubertal response, EM group showed higher basal LH (P=0.031) and follicular stimulating hormone (P=0.008) than LM/A group. The EM basal LH was more closely related with the peak stimulated LH than the LM/A basal LH did (rs=0.871 vs. rs=0.524). The optimal basal LH cutoffs to predict a pubertal response to GnRHST were 0.11 IU/L with a sensitivity of 66.7% and a specificity of 78.7% in EM group, and 0.07 IU/L with a sensitivity of 60.0% and a specificity of 78.9% in LM/A group, respectively. CONCLUSION: In girls with early stages of puberty, EM basal LH is a more sensitive screening tool than the LM/A basal LH. Diurnal variation should be considered in evaluating children with precocious puberty.

Waist-to-height ratio as a screening tool for obesity and cardiometabolic risk / 소아과

The waist-to-height ratio (WHtR), calculated by dividing the waist circumference (WC) by height, has recently gained attention as an anthropometric index for central adiposity. It is an easy-to-use and less age-dependent index to identify individuals with increased cardiometabolic risk. A WHtR cutoff of 0.5 can be used in different sex and ethnic groups and is generally accepted as a universal cutoff for central obesity in children (aged ≥6 years) and adults. However, the WHtR has not been validated in preschool children, and the routine use of WHtR in children under age 6 is not recommended. Prospective studies and meta-analysis in adults revealed that the WHtR is equivalent to or slightly better than WC and superior to body mass index (BMI) in predicting higher cardiometabolic risk. In children and adolescents, studies have shown that the WHtR is similar to both BMI and WC in identifying those at an increased cardiometabolic risk. Additional use of WHtR with BMI or WC may be helpful because WHtR considers both height and central obesity. WHtR may be preferred because of its simplicity and because it does not require sex- and age-dependent cutoffs; additionally, the simple message 'keep your WC to less than half your height' may be particularly useful. This review article summarizes recent publications on the usefulness of using WHtR especially when compared to BMI and WC as a screening tool for obesity and related cardiometabolic risks, and recommends the use of WHtR in clinical practice for obesity screening in children and adolescents.

Sitosterolemia: a review and update of pathophysiology, clinical spectrum, diagnosis, and management

Sitosterolemia is an autosomal recessive disorder characterized by increased plant sterol levels, xanthomas, and accelerated atherosclerosis. Although it was originally reported in patients with normolipemic xanthomas, severe hypercholesterolemia have been reported in patients with sitosterolemia, especially in children. Sitosterolemia is caused by increased intestinal absorption and decreased biliary excretion of sterols resulting from biallelic mutations in either ABCG5 or ABCG8, which encode the sterol efflux transporter ABCG5 and ABCG8. Patients with sitosterolemia show extreme phenotypic heterogeneity, ranging from almost asymptomatic individuals to those with severe hypercholesterolemia leading to accelerated atherosclerosis and premature cardiac death. Hematologic manifestations include hemolytic anemia with stomatocytosis, macrothrombocytopenia, splenomegaly, and abnormal bleeding. The mainstay of therapy includes dietary restriction of both cholesterol and plant sterols and the sterol absorption inhibitor, ezetimibe. Foods rich in plant sterols include vegetable oils, wheat germs, nuts, seeds, avocado, shortening, margarine and chocolate. Hypercholesterolemia in patients with sitosterolemia is dramatically responsive to low cholesterol diet and bile acid sequestrants. Plant sterol assay should be performed in patients with normocholesterolemic xanthomas, hypercholesterolemia with unexpectedly good response to dietary modifications or to cholesterol absorption inhibitors, or hypercholesterolemia with poor response to statins, or those with unexplained hemolytic anemia and macrothrombocytopenia. Because prognosis can be improved by proper management, it is important to find these patients out and diagnose correctly. This review article aimed to summarize recent publications on sitosterolemia, and to suggest clinical indications for plant sterol assay.

Waist-to-Height Ratio as an Index for Cardiometabolic Risk in Adolescents: Results from the 1998-2008 KNHANES

PURPOSE: To describe the relationship between the waist-to-height ratio (WHtR) and cardiometabolic risk factors (CMRFs) and to evaluate the validity of WHtR in identifying adolescents with metabolic syndrome. MATERIALS AND METHODS: We analyzed data from a pooled population of 4068 adolescents aged 10-19 years from the Korean National Health and Nutrition Examination Surveys conducted between 1998 and 2008. Overweight individuals were defined by body mass index (BMI) ≥85th percentile. Those with at least 2 CMRFs among hypertension, hyperglycemia, hypertriglyceridemia, and decreased high-density lipoprotein cholesterol (HDL-C) were classified as having multiple CMRFs. RESULTS: WHtR was significantly related to systolic blood pressure, HDL-C, and triglycerides in both non-overweight and overweight adolescents (all p<0.01). Among overweight adolescents, the area under the curve (AUC) for WHtR in identifying multiple CMRFs was significantly greater than that for BMI (p=0.014). Metabolic syndrome was more common in overweight adolescents with a WHtR of ≥0.5 than in those with a WHtR of <0.5 (p<0.001). In non-overweight adolescents, the prevalences of multiple CMRFs (p=0.001) and metabolic syndrome (p<0.001) were higher in those with a WHtR of ≥0.5 than in those with a WHtR of <0.5. Among those without central obesity, the prevalence of multiple CMRFs was higher in those with a WHtR of ≥0.5 than in those with a WHtR of <0.5 (p=0.021). CONCLUSION: WHtR is a simple and valid index for identifying adolescents with increased cardiometabolic risk and is related to CMRFs even in non-overweight adolescents. In adolescents already screened via BMI and waist circumference (WC), WHtR seems to be of additional help in discriminating those at higher cardiometabolic risk.

Iron and vitamin D status in breastfed infants and their mothers / 소아과

PURPOSE: We assessed the relationships between iron and vitamin D statuses in breastfed infants and their mothers and evaluated the determinants of iron and vitamin D deficiencies in breastfed infants. METHODS: Seventy breastfed infants aged 4-24 months and their mothers participated in this study from February 2012 to May 2013. Complete blood counts, total iron binding capacity, and levels of C-reactive protein, iron, ferritin, calcium, phosphate, alkaline phosphatase, and 25-hydroxyvitamin D (25(OH)D) in infants and their mothers were measured. RESULTS: A history of maternal prepregnancy anemia was associated with lower ferritin and 25(OH)D levels in both infants and their mothers. The 25(OH)D level of infants correlated with maternal 25(OH) D levels. The independent risk factors for iron deficiency in breastfed infants were the duration of breastfeeding (odds ratio [OR], 6.54; 95% confidence interval [CI], 1.09-39.2; P=0.04) and infant body weight (OR, 2.65; 95% CI, 1.07-6.56; P=0.04). The determinants for vitamin D deficiency were the infant's age (OR, 0.15; 95% CI, 0.02-0.97; P=0.046) and maternal 25(OH)D level (OR, 0.74; 95% CI, 0.59-0.92; P=0.01). CONCLUSION: A maternal history of prepregnancy anemia requiring iron therapy was associated with lower current ferritin and 25(OH)D levels in both infants and their mothers. Therefore, physicians should monitor not only iron but also vitamin D levels in infants who are breastfed by mothers who had prepregnancy anemia.

Vitamin D deficiency in Korean children: prevalence, risk factors, and the relationship with parathyroid hormone levels

PURPOSE: This study was performed to investigate the relationship between serum vitamin D and parathyroid hormone (PTH) levels as well as to describe the prevalence and the risk factors of vitamin D deficiency (VDD) in Korean children. METHODS: Participants were 1,212 children aged 4 to 15 years, who visited Bundang CHA Medical Center (located at 37degreesN) between March 2012 and February 2013. Overweight was defined as body mass index> or =85th percentile. Participants were divided into 4 age groups and 2 seasonal groups. VDD was defined by serum 25-hydroxyvitamin D (25OHD) <20 ng/mL. RESULTS: The level of 25OHD was significantly lower in overweight group than in normal weight group (17.1+/-5.1 ng/mL vs. 19.1+/-6.1 ng/mL, P<0.001). Winter-spring season (odds ratio [OR], 4.46; 95% confidence interval [CI], 3.45-5.77), older age group (OR, 1.60; 95% CI, 1.36-1.88), and overweight (OR, 2.21; 95% CI, 1.62-3.01) were independently related with VDD. The PTH levels were significantly higher in VDD group compared to vitamin D insufficiency and sufficiency group (P<0.001). In normal weight children, 25OHD (beta=-0.007, P<0.001) and ionized calcium (beta=-0.594, P=0.007) were independently related with PTH, however, these associations were not significant in overweight children. CONCLUSION: VDD is very common in Korean children and its prevalence increases in winter-spring season, in overweight children and in older age groups. Further investigation on the vitamin D and PTH metabolism according to adiposity is required.

Insulin pump therapy in transient neonatal diabetes mellitus

Neonatal diabetes mellitus (NDM) is a rare disease requiring insulin treatment. Its treatment is primarily focused on maintaining adequate glycemic control and avoiding hypoglycemia. Although insulin pump therapy is frequently administered to adults and children, there is no consensus on the use of insulin pumps in NDM. A 10 day-old female infant was referred to us with intrauterine growth retardation and poor weight gain. Hyperglycemia was noted, and continuous intravenous insulin infusion was initiated. However, the patient's serum glucose levels fluctuated widely, and maintaining the intravenous route became difficult within the following weeks. Continuous subcutaneous insulin infusion with an insulin pump was introduced on the twenty-fifth day of life, and good glycemic control was achieved without any notable adverse effects including hypoglycemia. We suggest that the insulin pump is a safe and effective mode for treating NDM and its early adoption may shorten the length of hospital stays in patients with NDM.

Stem Cell Therapy for Type 1 Diabetes Mellitus

Type 1 diabetes mellitus (T1DM) is caused by autoimmune destruction of insulin producing beta cells, and theoretically, it can be cured by replacement of the lost beta cells. The limitations along with successes of pancreas transplantation opened the door for stem cell therapy for T1DM. For clinical application, regenerated beta cells have to show robust glucose-responsiveness to maintain euglycemia. It is also important to protect the new beta cells from autoimmune destruction as well as graft rejection. Studies using embryonic stem cells (ESCs) are encouraging, but there are ethical concerns on destroying human embryo to harvest ESCs. Researches using bone marrow stem cells (BMSCs), induced pluripotent stem cells (iPSCs), umbilical cord blood, pancreatic and hepatic cells are also promising, and they can circumvent the ethical issues on using human ESCs. BMSCs are probably the most promising because of their proven ability to promote the regeneration of beta cells, and immune modulating effect that may prevent autoimmune destruction of beta cells. IPSCs, reprogrammed from skin fibroblasts or spermatogonial stem cells, are also good candidates for stem cell therapy. Although there are major challenges to overcome, with rapid progress of science, stem cell therapy has the potential to realize a permanent cure for T1DM in the future.

Stem Cell Therapy for Type 1 Diabetes Mellitus

Type 1 diabetes mellitus (T1DM) is caused by autoimmune destruction of insulin producing beta cells, and theoretically, it can be cured by replacement of the lost beta cells. The limitations along with successes of pancreas transplantation opened the door for stem cell therapy for T1DM. For clinical application, regenerated beta cells have to show robust glucose-responsiveness to maintain euglycemia. It is also important to protect the new beta cells from autoimmune destruction as well as graft rejection. Studies using embryonic stem cells (ESCs) are encouraging, but there are ethical concerns on destroying human embryo to harvest ESCs. Researches using bone marrow stem cells (BMSCs), induced pluripotent stem cells (iPSCs), umbilical cord blood, pancreatic and hepatic cells are also promising, and they can circumvent the ethical issues on using human ESCs. BMSCs are probably the most promising because of their proven ability to promote the regeneration of beta cells, and immune modulating effect that may prevent autoimmune destruction of beta cells. IPSCs, reprogrammed from skin fibroblasts or spermatogonial stem cells, are also good candidates for stem cell therapy. Although there are major challenges to overcome, with rapid progress of science, stem cell therapy has the potential to realize a permanent cure for T1DM in the future.

Prevalence and risk factors for vitamin D deficiency in children with iron deficiency anemia / 소아과

PURPOSE: The increasing prevalence of breast feeding has led to concerns about vitamin D deficiency (VDD) and iron deficiency anemia (IDA) in children. We evaluated the prevalence of VDD in a population of Korean children with IDA and assessed the risk factors for VDD in these children. METHODS: A total of 79 children who were diagnosed with IDA were prospectively surveyed from April 2010 to March 2011. Data were collected by questionnaire, medical assessment, and laboratory tests, including measurement of 25-hydroxyvitamin D (25OHD), hemoglobin, and wrist radiography. RESULTS: The median age was 22 months and 30% of the subjects were female. Over a half of subjects (58%) had subnormal vitamin D level (25OHD<30 ng/mL), and VDD (25OHD<20 ng/mL) was present in 39% of children. There was no difference in serum hemoglobin level between IDA patients with VDD and those without VDD. Most subjects (89%) were currently or had recently been breastfed and almost all subjects (97%) who had VDD received breastfeeding. Children with VDD were more likely to be younger than 2 years, to have been breastfed, and to have been tested in winter or spring. Multivariable analysis indicated seasonal variation was a significant independent risk factor for VDD in our IDA patients. CONCLUSION: Our results demonstrated that VDD has a high prevalence in Korean children with IDA. Primary care physicians should be aware of the possibility of VDD in children with IDA and should supplement the vitamin D as well as iron.

Prevalence and risk factors for vitamin D deficiency in children with iron deficiency anemia / 소아과

PURPOSE: The increasing prevalence of breast feeding has led to concerns about vitamin D deficiency (VDD) and iron deficiency anemia (IDA) in children. We evaluated the prevalence of VDD in a population of Korean children with IDA and assessed the risk factors for VDD in these children. METHODS: A total of 79 children who were diagnosed with IDA were prospectively surveyed from April 2010 to March 2011. Data were collected by questionnaire, medical assessment, and laboratory tests, including measurement of 25-hydroxyvitamin D (25OHD), hemoglobin, and wrist radiography. RESULTS: The median age was 22 months and 30% of the subjects were female. Over a half of subjects (58%) had subnormal vitamin D level (25OHD<30 ng/mL), and VDD (25OHD<20 ng/mL) was present in 39% of children. There was no difference in serum hemoglobin level between IDA patients with VDD and those without VDD. Most subjects (89%) were currently or had recently been breastfed and almost all subjects (97%) who had VDD received breastfeeding. Children with VDD were more likely to be younger than 2 years, to have been breastfed, and to have been tested in winter or spring. Multivariable analysis indicated seasonal variation was a significant independent risk factor for VDD in our IDA patients. CONCLUSION: Our results demonstrated that VDD has a high prevalence in Korean children with IDA. Primary care physicians should be aware of the possibility of VDD in children with IDA and should supplement the vitamin D as well as iron.

Clinical Features of Human Metapneumovirus and Respiratory Syncytial Virus Infection in Hospitalized Children / 소아알레르기및호흡기학회지

PURPOSE:To identify the clinical features of human metapneumovirus (hMPV) and the respiratory syncytial virus (RSV) infection in children. METHODS:The participants of our study were 1,104 children who were admitted to Bungdang CHA hospital for lower respiratory infection from August 2006 through July 2007. Nasopharyngeal swabs were taken from the patients, and viruses were identified by RT-PCR. The clinical features of 51 patients with hMPV infection and 138 patients with RSV infection were compared by retrospective review of their medical records. RESULTS:The peak incidence of hMPV infection was noted in April, and that of RSV was noted in November. Both viruses had the highest incidence in patients age or =2 years, while in the RSV group, 19% were age > or =2 years. In both groups, pneumonia was the most common clinical diagnosis, followed by acute bronchiolitis, acute bronchitis, and asthma. The white blood cell counts were higher in the RSV group, and fever was more frequent on admission in the hMPV group. CONCLUSION:hMPV was the fourth most common virus causing lower respiratory tract infections in children. The clinical features of hMPV infection were similar to those of RSV infection. This study may be helpful for the effective treatment of lower respiratory tract infection in children.

Liver Dysfunction due to Hepatic Glycogenosis in a Girl with Type 1 Diabetes / 대한소아내분비학회지

Hepatic glycogenosis is an under-recognized cause of hepatomegaly and elevated serum aminotransferase levels in patients with type 1 diabetes; further, most cases of hepatic glycogenosis are reported to be associated with poor glycemic control. In this report, we describe the case of a 12-year-old girl with hepatic glycogenosis, who presented with elevated liver enzymes. She was diagnosed with type 1 diabetes at the age of 6 years, and her diabetes was very "brittle" with recurrent episodes of hypoglycemic attacks. Her recent hemoglobin A1C (HbA1c) level was 8.4%, and the average HbA1c level during the last 2 years was 8.7%. Her aspartate aminotransferase (AST) and alanine aminotransferase (ALT) levels were persistently elevated during the last year, up to 700 and 258 U/L, respectively. Her growth rate and pubertal development were normal. Her serum was negative for hepatitis viral markers, and the plasma levels of ceruloplasmin and ferritin were also normal. Ultrasound examination revealed hepatomegaly with increased hepatic echogenicity. Liver biopsy demonstrated irregular glycogen deposition in hepatocytes. Recurrent hypoglycemia was resolved with continuous subcutaneous insulin infusion, and after 3 weeks, her AST and ALT levels decreased to 47 and 33 U/L, respectively. We conclude that hepatic glycogenosis should be suspected as a cause of abnormal liver function tests in patients with poorly controlled or brittle type 1 diabetes. After excluding other causes of hepatic dysfunction, a 1-month trial for achieving improved glycemic control, while avoiding hypoglycemia, is recommended before proceeding with invasive investigation of the patient.