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1.
Article in English | WPRIM | ID: wpr-831569

ABSTRACT

Background@#Hodgkin's lymphoma (HL) constitutes 10%–20% of all malignant lymphomas and has a high cure rate (5-year survival, around 90%). Recently, interest has increased concerning preventing secondary complications (secondary cancer, endocrine disorders) in long-term survivors. We aimed to study the epidemiologic features and therapeutic outcomes of HL in children, adolescents, and young adults in Korea. @*Methods@#We performed a multicenter, retrospective study of 224 patients aged < 25 years diagnosed with HL at 22 participating institutes in Korea from January 2007 to August 2016. @*Results@#A higher percentage of males was diagnosed at a younger age. Nodular sclerosis histopathological HL subtype was most common, followed by mixed cellularity subtype.Eighty-one (36.2%), 101 (45.1%), and 42 (18.8%) patients were classified into low, intermediate, and high-risk groups, respectively. Doxorubicin, bleomycin, vinblastine, dacarbazine was the most common protocol (n = 102, 45.5%). Event-free survival rate was 86.0% ± 2.4%, while five-year overall survival (OS) rate was 96.1% ± 1.4%: 98.7% ± 1.3%, 97.7% ± 1.6%, and 86.5% ± 5.6% in the low, intermediate, and high-risk groups, respectively (P = 0.021). Five-year OS was worse in patients with B-symptoms, stage IV disease, highrisk, splenic involvement, extra-nodal lymphoma, and elevated lactate dehydrogenase level.In multivariate analysis, B-symptoms and extra-nodal involvement were prognostic factors for poor OS. Late complications of endocrine disorders and secondary malignancy were observed in 17 and 6 patients, respectively. @*Conclusion@#This is the first study on the epidemiology and treatment outcomes of HL in children, adolescents, and young adults in Korea. Future prospective studies are indicated to develop therapies that minimize treatment toxicity while maximizing cure rates in children, adolescents, and young adults with HL.

2.
Article in English | WPRIM | ID: wpr-763109

ABSTRACT

BACKGROUND: Pilocytic astrocytoma (PA) is a brain tumor that is relatively more common in children and young adults. METHODS: We retrospectively reviewed the medical records of patients with PA treated at a single center between 1988 and 2018. RESULTS: We included 31 subjects with PA. The median age at diagnosis was 13.4 years, and the median follow-up duration was 9.9 years. The total PA group had a 10-year disease-specific survival (DSS) rate of 92.6% [95% confidence interval (CI), 82.6–100] and 10-year progression-free survival (PFS) rate of 52.8% (95% CI, 32.0–73.6). In patients aged <20 years, tumors were more likely to be located in sites in which gross total tumor resection (GTR) was impossible. No statistically significant difference in 10-year DSS was found between the GTR (100%) and non-GTR (89.7%; 95% CI, 76.2–100; p=0.374) groups. However, a statistically significant difference in 10-year PFS was found between the GTR (100%) and non-GTR groups (30.7%; 95% CI, 8.6–52.8; p=0.012). In the non-GTR group, no statistically significant difference in 10-year DSS was found between the patients who received immediate additional chemotherapy and/or radiotherapy (Add-Tx group, 92.9%; 95% CI, 79.4–100) and the non-Add-Tx group (83.3%; 95% CI, 53.5–100; p=0.577). No statistically significant difference in 10-year PFS was found between the Add-Tx group (28.9%; 95% CI, 1.7–56.1) and non-Add-Tx group (33.3%; 95% CI, 0–70.9; p=0.706). CONCLUSION: The PFS of the patients with PA in our study depended only on the degree of surgical excision associated with tumor location. This study is limited by its small number of patients and retrospective nature. A multicenter and prospective study is necessary to confirm these findings.


Subject(s)
Adolescent , Astrocytoma , Brain Neoplasms , Child , Diagnosis , Disease-Free Survival , Drug Therapy , Follow-Up Studies , Glioma , Humans , Medical Records , Prognosis , Prospective Studies , Radiotherapy , Retrospective Studies , Survivors , Young Adult
3.
Article in English | WPRIM | ID: wpr-762587

ABSTRACT

Idiopathic infantile hypercalcemia is characterized by hypercalcemia, dehydration, vomiting, and failure to thrive, and it is due to mutations in 24-hydroxylase (CYP24A1). Recently, mutations in sodium-phosphate cotransporter (SLC34A1) expressed in the kidney were discovered as an additional cause of idiopathic infantile hypercalcemia. This report describes a female infant admitted for evaluation of nephrocalcinosis. She presented with hypercalcemia, hypercalciuria, low intact parathyroid hormone level, and high 1,25-dihydroxyvitamin D3 level. Exome sequencing identified novel compound heterozygous mutations in SLC34A1 (c.1337G>A, c.1483C>T). The patient was treated with fluids for hydration, furosemide, a corticosteroid, and restriction of calcium/vitamin D intake. At the age of 7 months, the patient's calcium level was within the normal range, and hypercalciuria waxed and waned. Renal echogenicity improved on the follow-up ultrasonogram, and developmental delay was not noted. In cases of hypercalcemia with subsequent hypercalciuria, DNA analysis for SLC34A1 gene mutations and CYP24A1 gene mutations should be performed. Further studies are required to obtain long-term data on hypercalciuria and nephrocalcinosis.


Subject(s)
Calcitriol , Calcium , Dehydration , DNA , Exome , Failure to Thrive , Female , Follow-Up Studies , Furosemide , Humans , Hypercalcemia , Hypercalciuria , Hypophosphatemia , Infant , Kidney , Nephrocalcinosis , Parathyroid Hormone , Reference Values , Sodium-Phosphate Cotransporter Proteins , Ultrasonography , Vitamin D , Vitamin D3 24-Hydroxylase , Vomiting
4.
Article in English | WPRIM | ID: wpr-719418

ABSTRACT

PURPOSE: Dexrazoxane has been used as an effective cardioprotector against anthracycline cardiotoxicity. This study intended to analyze cardioprotective efficacy and secondary malignancy development, and elucidate risk factors for secondary malignancies in dexrazoxane-treated pediatric patients. MATERIALS AND METHODS: Data was collected from 15 hospitals in Korea. Patients who received any anthracyclines, and completed treatment without stem cell transplantation were included. For efficacy evaluation, the incidence of cardiac events and cardiac event-free survival rates were compared. Data about risk factors of secondary malignancies were collected. RESULTS: Data of total 1,453 cases were analyzed; dexrazoxane with every anthracyclines group (D group, 1,035 patients) and no dexrazoxane group (non-D group, 418 patients). Incidence of the reported cardiac events was not statistically different between two groups; however, the cardiac event-free survival rate of patients with more than 400 mg/m2 of anthracyclines was significantly higher in D group (91.2% vs. 80.1%, p=0.04). The 6-year cumulative incidence of secondary malignancy was not different between both groups after considering follow-up duration difference (non-D, 0.52%±0.37%; D, 0.60%±0.28%; p=0.55). The most influential risk factor for secondary malignancy was the duration of anthracycline administration according to multivariate analysis. CONCLUSION: Dexrazoxane had an efficacy in lowering cardiac event-free survival rates in patients with higher cumulative anthracyclines. As a result of multivariate analysis for assessing risk factors of secondary malignancy, the occurrence of secondary malignancy was not related to dexrazoxane administration.


Subject(s)
Anthracyclines , Cardiotoxicity , Dexrazoxane , Disease-Free Survival , Follow-Up Studies , Humans , Incidence , Korea , Multivariate Analysis , Neoplasms, Second Primary , Risk Factors , Stem Cell Transplantation
5.
Article in English | WPRIM | ID: wpr-718501

ABSTRACT

Floating-Harbor syndrome is a rare autosomal dominant genetic disorder associated with SRCAP mutation. To date, approximately 50 cases of Floating-Harbor syndrome have been reported, but none have been reported in Korea yet. Floating-Harbor syndrome is characterized by delayed bony maturation, unique facial features, and language impairment. Here, we present a 6-year-old boy with a triangular face, deep-set protruding eyes, low-set ears, wide nose with narrow nasal bridge, short philtrum, long thin lips, clinodactyly, and developmental delay that was transferred to our pediatric clinic for genetic evaluation. He showed progressive delay in the area of language and cognition-adaption as he grew. He had previously undergone chromosomal analysis at another hospital due to his language delay, but his karyotype was normal. We performed targeted exome sequencing, considering several syndromes with similar phenotypes. Library preparation was performed with the TruSight One sequencing panel, which enriches the sample for about 4,800 genes of clinical relevance. Massively parallel sequencing was conducted with NextSeq. An identified variant was confirmed by Sanger sequencing of the patient and his parents. Finally, the patient was confirmed as the first Korean case of Floating-Harbor syndrome with a novel SRCAP (Snf2 related CREBBP activator protein) mutation (c.7732dupT, p.Ser2578Phefs*6), resulting in early termination of the protein; it was not found in either of his healthy parents or a control population. To our knowledge, this is the first study to describe a boy with Floating-Harbor syndrome with a novel SRCAP mutation diagnosed by targeted exome sequencing in Korea.


Subject(s)
Child , Ear , Exome , High-Throughput Nucleotide Sequencing , Humans , Karyotype , Korea , Language Development Disorders , Lip , Male , Nose , Parents , Phenotype
6.
Blood Research ; : 110-116, 2018.
Article in English | WPRIM | ID: wpr-714934

ABSTRACT

BACKGROUND: Cytomegalovirus (CMV) causes severe diseases in premature infants and immunocompromised hosts, and antiviral therapy is often required for disease control. However, the clinical manifestations and treatment courses for CMV-associated thrombocytopenia in immunocompetent children are unclear. METHODS: Medical records of the children who suffered from thrombocytopenia, and showed positive CMV polymerase chain reaction and CMV-like symptoms were retrospectively analyzed at three university hospitals in Daegu from January 2000 to March 2017. Patients suffering from leukemia, immunodeficiency, and other infections were excluded. RESULTS: Among 1,065 children with thrombocytopenia, 29 (2.7%) displayed CMV-associated thrombocytopenia. The median age at diagnosis was 15 months and the median platelet count was 26,000/µL. They were classified into the CMV-induced thrombocytopenia (23/29) and CMV-related secondary immune thrombocytopenia (ITP, 6/29) groups. Fourteen subjects had hepatic dysfunction, four had Evans syndrome, two had pneumonitis, and one had gastritis. IVIG was used for 21 patients, and six patients among them showed recurrence, for whom IVIG or antiviral therapy was used. All, except one, recurrent or chronic cases belonged to the CMV-induced thrombocytopenia group. Antiviral therapy was used more frequently for the CMV-induced thrombocytopenia group (8/23, 34.8%) than for the CMV-related secondary ITP group (0/6); however, the results were not statistically significant (P=0.148). CONCLUSION: CMV is a rare but unique etiology of thrombocytopenia, and observed even in healthy children after the neonatal period. About one-third patients need antiviral therapy for disease control. Further, CMV-induced thrombocytopenia is more complex than CMV-related secondary ITP.


Subject(s)
Child , Cytomegalovirus , Diagnosis , Ganciclovir , Gastritis , Hospitals, University , Humans , Immunocompromised Host , Immunoglobulins, Intravenous , Infant, Newborn , Infant, Premature , Leukemia , Medical Records , Platelet Count , Pneumonia , Polymerase Chain Reaction , Purpura, Thrombocytopenic, Idiopathic , Recurrence , Retrospective Studies , Thrombocytopenia
7.
Article in English | WPRIM | ID: wpr-714199

ABSTRACT

BACKGROUND: Venous thromboembolism (VTE) is rare in pediatric patients compared to adults, but it's incidence is gradually increasing. The purpose of this study was to analyze the incidence, risk factors, and prognosis of pediatric patients with VTE in Korea. METHODS: Between January 2000 and July 2017, 249,312 medical records of the patients older than 1 year who were hospitalized in the department of pediatrics of 10 university hospitals in Yeungnam region were retrospectively reviewed. RESULTS: The overall incidence of VTE was 4.9 per 10,000 admissions. Of the total 123 patients, 80 (65.0%) were male and the median age was 10.8 years (range, 1.0–23.5 years). Magnetic resonance imaging was performed most frequently to confirm the diagnosis of VTE (43.1%). Thrombosis occurred in the cerebral vessels (46.3%), lower extremities (23.8%), pulmonary (19.5%), abdomen (9.8%), and upper extremities (4.1%). One hundred and six patients had underlying causes such as cancer (27.6%), infection (26.8%), intravenous catheter insertion (17.9%), and surgery (14.6%). Protein C was evaluated in 39 patients (31.7%), protein S in 40 (32.5%), antithrombin (AT) III in 52 (42.3%), and homocysteine in 21 (17.1%). Among them, one patient with a family history of AT III deficiency had SERPINC gene mutation. Seventy-seven patients (62.6%) started anticoagulation treatment. Most (52.0%) were treated for more than 90 days. CONCLUSION: Healthcare providers must be aware of the potential for VTE development in childhood. In the near future, a nationwide survey should be investigated to determine the incidence rate and the trends in VTE among Korean children.


Subject(s)
Abdomen , Adult , Catheters , Child , Diagnosis , Epidemiology , Health Personnel , Homocysteine , Hospitals, University , Humans , Incidence , Korea , Lower Extremity , Magnetic Resonance Imaging , Male , Medical Records , Pediatrics , Prognosis , Protein C , Protein S , Retrospective Studies , Risk Factors , Thrombosis , Upper Extremity , Venous Thromboembolism
8.
Article in English | WPRIM | ID: wpr-788592

ABSTRACT

A 9-year-old boy presented with increased sweating and abdominal pain. His mother and uncle had been diagnosed with bilateral pheochromocytoma and medullary thyroid carcinoma. Magnetic resonance imaging of the boy's abdomen revealed a 7.5 cm×7.0 cm×6.0 cm mass with a thick peripheral enhancing wall and fluid-fluid level at the right suprarenal region. His ¹²³I-meta-iodobenzylguanidine (MIBG) scan showed a large mass with increased MIBG uptake in the right adrenal gland. The levels of serum norepinephrine, urine epinephrine/norepinephrine, metanephrine, and vanillylmandelic acid were elevated. He, his mother, and two sisters tested positive for the known mutation of multiple endocrine neoplasia type 2A, Cys634Tyr in RET proto-oncogene. Laparoscopic tumor excision and right adrenalectomy were performed. Final diagnosis was pheochromocytoma with malignant behavior, based on adrenal gland scoring scale. However, there was no overt metastasis. After surgery, his symptoms resolved and abnormal laboratory tests were normalized.


Subject(s)
3-Iodobenzylguanidine , Abdomen , Abdominal Pain , Adrenal Glands , Adrenalectomy , Child , Diagnosis , Humans , Magnetic Resonance Imaging , Male , Metanephrine , Mothers , Multiple Endocrine Neoplasia Type 2a , Multiple Endocrine Neoplasia , Neoplasm Metastasis , Norepinephrine , Pheochromocytoma , Proto-Oncogenes , Siblings , Sweat , Sweating , Thyroid Neoplasms , Vanilmandelic Acid
9.
Article in English | WPRIM | ID: wpr-197952

ABSTRACT

A 9-year-old boy presented with increased sweating and abdominal pain. His mother and uncle had been diagnosed with bilateral pheochromocytoma and medullary thyroid carcinoma. Magnetic resonance imaging of the boy's abdomen revealed a 7.5 cm×7.0 cm×6.0 cm mass with a thick peripheral enhancing wall and fluid-fluid level at the right suprarenal region. His ¹²³I-meta-iodobenzylguanidine (MIBG) scan showed a large mass with increased MIBG uptake in the right adrenal gland. The levels of serum norepinephrine, urine epinephrine/norepinephrine, metanephrine, and vanillylmandelic acid were elevated. He, his mother, and two sisters tested positive for the known mutation of multiple endocrine neoplasia type 2A, Cys634Tyr in RET proto-oncogene. Laparoscopic tumor excision and right adrenalectomy were performed. Final diagnosis was pheochromocytoma with malignant behavior, based on adrenal gland scoring scale. However, there was no overt metastasis. After surgery, his symptoms resolved and abnormal laboratory tests were normalized.


Subject(s)
3-Iodobenzylguanidine , Abdomen , Abdominal Pain , Adrenal Glands , Adrenalectomy , Child , Diagnosis , Humans , Magnetic Resonance Imaging , Male , Metanephrine , Mothers , Multiple Endocrine Neoplasia Type 2a , Multiple Endocrine Neoplasia , Neoplasm Metastasis , Norepinephrine , Pheochromocytoma , Proto-Oncogenes , Siblings , Sweat , Sweating , Thyroid Neoplasms , Vanilmandelic Acid
10.
Article in English | WPRIM | ID: wpr-30880

ABSTRACT

A 3-year-old previously healthy boy was admitted because of a 1-week history of fever, abdominal pain, vomiting, and diarrhea. The initial laboratory tests showed hepatic dysfunction with disseminated intravascular coagulation. There was a large amount of pleural effusion, periportal edema, minimal ascites, and splenomegaly. He was initially managed with broad spectrum antibiotics with transfusion. Despite 2 days of treatment, the fever persisted and the results of the laboratory tests had worsened. Bacterial cultures from the blood, urine, pleural effusion, and ascites were all negative. He was finally diagnosed with hemophagocytic lymphohistiocytosis (HLH) based on the diagnostic criteria. Adenovirus was detected in the initial diarrhea and nasal swab specimens using polymerase chain reaction-based method. One year after chemotherapy with dexamethasone, cyclosporine, and etoposide, he is now healthy without evidence of disease recurrence. This is the first Korean case report of adenovirus-induced HLH in a previously healthy child.


Subject(s)
Abdominal Pain , Adenoviridae , Anti-Bacterial Agents , Ascites , Child , Child, Preschool , Cyclosporine , Dexamethasone , Diarrhea , Disseminated Intravascular Coagulation , Drug Therapy , Edema , Etoposide , Fever , Humans , Lymphohistiocytosis, Hemophagocytic , Male , Methods , Pleural Effusion , Recurrence , Splenomegaly , Vomiting
11.
Article in English | WPRIM | ID: wpr-110511

ABSTRACT

To analyze the infectious causes and clinical symptoms of febrile infants aged less than 3 months presenting to a Pediatric Emergency Medical Center (PEMC) and to propose more efficient, evidence-based management and treatment. We conducted a retrospective study of 462 febrile infants aged less than 3 months who visited PEMC at Keimyung University Dongsan Medical Center from January 2015 to June 2016. Infants' sex, age, fever duration, and laboratory findings, including bacterial or viral pathogens, were recorded. To evaluate clinical signs, one point per sign was given for grunting, decreased activity, and the presence of cyanosis; total scores were compared between the bacterial infection (BI) and non-bacterial infection (NBI) groups. BI was diagnosed in 118 (25.5%) infants, and no BI was diagnosed in 344 (74.5%) infants. Escherichia coli was the most frequently isolated pathogen, accounting for 80.5% (n = 95) of all infections (n = 118). Statistically significant differences in sex, the duration of fever, sign scores, white blood cell count, neutrophil count, lymphocyte count, and C-reactive protein (CRP) level were found between the BI and NBI groups. The cut-off value for CRP was 1.445 mg/dL, with sensitivity and specificity values of 76.3% and 75.9%, respectively, in BI patients, as determined by the receiver operating characteristic curve. As more PEMCs are being built in Korea, hospital accessibility is better; thus, it may be possible to perform outpatient management of young, febrile infants aged younger than 3 months without antibiotics and lumbar puncture using individual sign scores and laboratory findings.


Subject(s)
Anti-Bacterial Agents , Bacterial Infections , C-Reactive Protein , Cyanosis , Emergencies , Escherichia coli , Fever , Humans , Infant , Korea , Leukocyte Count , Lymphocyte Count , Neutrophils , Outpatients , Retrospective Studies , ROC Curve , Sensitivity and Specificity , Spinal Puncture
12.
Article in English | WPRIM | ID: wpr-788576

ABSTRACT

A 3-year-old previously healthy boy was admitted because of a 1-week history of fever, abdominal pain, vomiting, and diarrhea. The initial laboratory tests showed hepatic dysfunction with disseminated intravascular coagulation. There was a large amount of pleural effusion, periportal edema, minimal ascites, and splenomegaly. He was initially managed with broad spectrum antibiotics with transfusion. Despite 2 days of treatment, the fever persisted and the results of the laboratory tests had worsened. Bacterial cultures from the blood, urine, pleural effusion, and ascites were all negative. He was finally diagnosed with hemophagocytic lymphohistiocytosis (HLH) based on the diagnostic criteria. Adenovirus was detected in the initial diarrhea and nasal swab specimens using polymerase chain reaction-based method. One year after chemotherapy with dexamethasone, cyclosporine, and etoposide, he is now healthy without evidence of disease recurrence. This is the first Korean case report of adenovirus-induced HLH in a previously healthy child.


Subject(s)
Abdominal Pain , Adenoviridae , Anti-Bacterial Agents , Ascites , Child , Child, Preschool , Cyclosporine , Dexamethasone , Diarrhea , Disseminated Intravascular Coagulation , Drug Therapy , Edema , Etoposide , Fever , Humans , Lymphohistiocytosis, Hemophagocytic , Male , Methods , Pleural Effusion , Recurrence , Splenomegaly , Vomiting
13.
Article in English | WPRIM | ID: wpr-145157

ABSTRACT

PURPOSE: To determine whether serum insulin and glucagon levels of umbilical cord blood correlate with subsequent postnatal hypoglycemia in appropriate for gestational age (AGA) - preterm infants at different gestational ages (GAs). METHODS: The serum insulin and glucagon levels of umbilical cord blood were measured using magnetic bead based multiplex immunoassay in 69 AGA - premature infants, stratified according to GA: GA 23-30 weeks, early preterm (EP, n=31); GA 31-34 weeks, late preterm (LP, n=38). Postnatal hypoglycemia was defined as a capillary glucose level <40 mg/dL within the first 60 minutes of life, regardless of GA. RESULTS: The capillary glucose concentration in EP infants (65.5±21.2 mg/dL) was significantly higher than that of LP infants (55.9±17.3 mg/dL) (P=0.043). The serum glucagon level in EP infants (44.3±28.7 pg/mL) was significantly higher than that in LP infants (28.1±13.6 pg/mL) (P=0.006). There was not a significant difference in serum insulin level between EP and LP infants (372.7±254.2 pg/mL vs. 372.4±209.1 pg/mL, P=0.996). There was a significant difference in the serum glucagon level between infants with and without hypoglycemia (27.7±8.9 mg/dL vs. 36.8±24.6 mg/dL, P=0.036), but not in the serum insulin level (451.9±256.9 pg/mL vs. 357.4±222.2 pg/mL, P=0.211). Postnatal glucose concentration within the first 60 minutes of life had a significant positive correlation with serum glucagon levels (r=0.256, P=0.034), but not with serum insulin levels (r=-0.020, P=0.867). CONCLUSION: Lower glucagon levels of cord blood were seen in premature infants with higher GA, which might contribute to the occurrence of postnatal hypoglycemia.


Subject(s)
Capillaries , Fetal Blood , Gestational Age , Glucagon , Glucose , Humans , Hypoglycemia , Immunoassay , Infant , Infant, Newborn , Infant, Premature , Insulin , Umbilical Cord
14.
Article in English | WPRIM | ID: wpr-145153

ABSTRACT

The incidence of type 1 diabetes is increasing worldwide, and the greatest increase has been observed in very young children under 4 years of age. A case of infantile diabetic ketoacidosis in a 10-month-old male infant was encountered by these authors. The infant's fasting glucose level was 490 mg/dL, his PH was 7.13, his pCO₂ was 15 mmHg, and his bicarbonate level was 5.0 mmol/L. The glycosylated hemoglobin level had increased to 9.4%. Ketonuria and glucosuria were detected in the urinalysis. The fasting C-peptide and insulin levels had decreased. The infant was positive for anti-insulin and antiglutamic acid decarboxylase antibodies. Immediately after the infant's admission, fluid therapy and intravenous insulin infusion therapy were started. On the second day of the infant's hospitalization and after fluid therapy, he recovered from his lethargic condition, and his general condition improved. Feeding was started on the third day, and he was fed a formula 5 to 7 times a day and ate rice, vegetables, and lean meat. Due to the frequent feeding, the frequency of rapid-acting insulin injection was increased from 3 times before feeding to 5 times, adjusted according to the feeding frequency. The total dose of insulin that was injected was 0.8-1.1 IU/kg/day, and the infant was discharged on the 12th day of his hospitalization. The case is presented herein with a brief review of the relevant literature.


Subject(s)
Antibodies , C-Peptide , Child , Diabetes Mellitus, Type 1 , Diabetic Ketoacidosis , Fasting , Fluid Therapy , Glucose , Glycated Hemoglobin A , Hospitalization , Humans , Hydrogen-Ion Concentration , Incidence , Infant , Insulin , Insulin, Short-Acting , Ketosis , Male , Meat , Urinalysis , Vegetables
15.
Article in English | WPRIM | ID: wpr-145144

ABSTRACT

PURPOSE: To determine whether serum insulin and glucagon levels of umbilical cord blood correlate with subsequent postnatal hypoglycemia in appropriate for gestational age (AGA) - preterm infants at different gestational ages (GAs). METHODS: The serum insulin and glucagon levels of umbilical cord blood were measured using magnetic bead based multiplex immunoassay in 69 AGA - premature infants, stratified according to GA: GA 23-30 weeks, early preterm (EP, n=31); GA 31-34 weeks, late preterm (LP, n=38). Postnatal hypoglycemia was defined as a capillary glucose level <40 mg/dL within the first 60 minutes of life, regardless of GA. RESULTS: The capillary glucose concentration in EP infants (65.5±21.2 mg/dL) was significantly higher than that of LP infants (55.9±17.3 mg/dL) (P=0.043). The serum glucagon level in EP infants (44.3±28.7 pg/mL) was significantly higher than that in LP infants (28.1±13.6 pg/mL) (P=0.006). There was not a significant difference in serum insulin level between EP and LP infants (372.7±254.2 pg/mL vs. 372.4±209.1 pg/mL, P=0.996). There was a significant difference in the serum glucagon level between infants with and without hypoglycemia (27.7±8.9 mg/dL vs. 36.8±24.6 mg/dL, P=0.036), but not in the serum insulin level (451.9±256.9 pg/mL vs. 357.4±222.2 pg/mL, P=0.211). Postnatal glucose concentration within the first 60 minutes of life had a significant positive correlation with serum glucagon levels (r=0.256, P=0.034), but not with serum insulin levels (r=-0.020, P=0.867). CONCLUSION: Lower glucagon levels of cord blood were seen in premature infants with higher GA, which might contribute to the occurrence of postnatal hypoglycemia.


Subject(s)
Capillaries , Fetal Blood , Gestational Age , Glucagon , Glucose , Humans , Hypoglycemia , Immunoassay , Infant , Infant, Newborn , Infant, Premature , Insulin , Umbilical Cord
16.
Article in English | WPRIM | ID: wpr-145141

ABSTRACT

The incidence of type 1 diabetes is increasing worldwide, and the greatest increase has been observed in very young children under 4 years of age. A case of infantile diabetic ketoacidosis in a 10-month-old male infant was encountered by these authors. The infant's fasting glucose level was 490 mg/dL, his PH was 7.13, his pCO₂ was 15 mmHg, and his bicarbonate level was 5.0 mmol/L. The glycosylated hemoglobin level had increased to 9.4%. Ketonuria and glucosuria were detected in the urinalysis. The fasting C-peptide and insulin levels had decreased. The infant was positive for anti-insulin and antiglutamic acid decarboxylase antibodies. Immediately after the infant's admission, fluid therapy and intravenous insulin infusion therapy were started. On the second day of the infant's hospitalization and after fluid therapy, he recovered from his lethargic condition, and his general condition improved. Feeding was started on the third day, and he was fed a formula 5 to 7 times a day and ate rice, vegetables, and lean meat. Due to the frequent feeding, the frequency of rapid-acting insulin injection was increased from 3 times before feeding to 5 times, adjusted according to the feeding frequency. The total dose of insulin that was injected was 0.8-1.1 IU/kg/day, and the infant was discharged on the 12th day of his hospitalization. The case is presented herein with a brief review of the relevant literature.


Subject(s)
Antibodies , C-Peptide , Child , Diabetes Mellitus, Type 1 , Diabetic Ketoacidosis , Fasting , Fluid Therapy , Glucose , Glycated Hemoglobin A , Hospitalization , Humans , Hydrogen-Ion Concentration , Incidence , Infant , Insulin , Insulin, Short-Acting , Ketosis , Male , Meat , Urinalysis , Vegetables
17.
Article in English | WPRIM | ID: wpr-143123

ABSTRACT

A 9-year-old Tajikistani girl presented to Keimyung University Dongsan Medical Center for evaluation of a skin lesion on her left eyelid, focal alopecia, unilateral ventricular dilatation, and aortic coarctation. She was diagnosed with encephalocraniocutaneous lipomatosis (ECCL) according to Moog's diagnostic criteria. Café-au-lait spots were found on the left side of her trunk. Multiple nonossifying fibromas were found on her left proximal humerus, left distal femur, both proximal tibias, and left proximal fibula, suggesting Jaffe-Campanacci syndrome (JCS), following imaging of the extremities. Many JCS cases with multiple Café-au-lait macules, multiple nonossifying fibromas may actually have Neurofibromatosis type-1 (NF1). Thus, comprehensive molecular analysis to exclude NF1 mutation was performed using her blood sample. The NF1 mutation was not found. Her height was under the 3rd percentile and her bone age was delayed as compared with her chronological age. Baseline growth hormone (GH) level was below the normal range. Using the insulin stimulation and levo-dihydroxyphenylalanine tests, GH deficiency was confirmed. We present a case of GH deficiency with typical features of ECCL and JCS.

18.
Article in English | WPRIM | ID: wpr-143118

ABSTRACT

A 9-year-old Tajikistani girl presented to Keimyung University Dongsan Medical Center for evaluation of a skin lesion on her left eyelid, focal alopecia, unilateral ventricular dilatation, and aortic coarctation. She was diagnosed with encephalocraniocutaneous lipomatosis (ECCL) according to Moog's diagnostic criteria. Café-au-lait spots were found on the left side of her trunk. Multiple nonossifying fibromas were found on her left proximal humerus, left distal femur, both proximal tibias, and left proximal fibula, suggesting Jaffe-Campanacci syndrome (JCS), following imaging of the extremities. Many JCS cases with multiple Café-au-lait macules, multiple nonossifying fibromas may actually have Neurofibromatosis type-1 (NF1). Thus, comprehensive molecular analysis to exclude NF1 mutation was performed using her blood sample. The NF1 mutation was not found. Her height was under the 3rd percentile and her bone age was delayed as compared with her chronological age. Baseline growth hormone (GH) level was below the normal range. Using the insulin stimulation and levo-dihydroxyphenylalanine tests, GH deficiency was confirmed. We present a case of GH deficiency with typical features of ECCL and JCS.

19.
Article in Korean | WPRIM | ID: wpr-44479

ABSTRACT

Phosphate is an essential ion and plays a vital role in many physiological processes. Carpopedal spasm is known as a symptom of hypocalcemia, or rarely, hypomagnesemia. We present an unusual case of carpal spasm seen in a patient with hypophosphatemia. A fourteen year old boy was admitted with acute onset of both carpal spasm after vomiting and diarrhea. Biochemical investigation revealed only moderate hypophosphatemia without hypocalcemia. Other laboratory findings including hormone tests were normal. Carpal spasm improved with correction of hypophosphatemia. The phathophysiology of neurologic dysfunction related to hypophosphatemia is not clear yet. Careful observation of serum electrolytes including phosphate is necessary when the patient has neurologic symptoms.


Subject(s)
Diarrhea , Electrolytes , Humans , Hypocalcemia , Hypophosphatemia , Male , Neurologic Manifestations , Physiological Phenomena , Spasm , Vomiting
20.
Article in Korean | WPRIM | ID: wpr-44475

ABSTRACT

A 23 months-old girl visited the hospital because of fever and left neck mass. She was diagnosed as acute suppurative thyroiditis with piriform sinus fistula. Thyroid sonography showed perithyroidal abscess formation and thyroid scan showed decreased uptake of Tc-99m pertechnate of both thyroid glands. Magnetic resonance imaging of neck showed abscess cavity extending to the swollen left thyroid gland. And there was tiny fistula between thyroid and piriform sinus in the barium esophagogram. Streptococcus gordonii was isolated on needle aspiration culture. We report a case of piriform sinus fistula of the neck complicated with suppurative thyroiditis. The fistula was treated with chemocauterization using trichloroacetic acid.


Subject(s)
Abscess , Barium , Female , Fever , Fistula , Humans , Magnetic Resonance Imaging , Neck , Needles , Pyriform Sinus , Streptococcus gordonii , Thyroid Gland , Thyroiditis, Suppurative , Trichloroacetic Acid
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