Angiodysplasia is an important vascular lesion of the gastointestinal tract , located at the site of ectasia of intestinal submucosal veins and overlying mucosal capillaries. It is a major cause of morbidity from gastrointestinal bleeding in old age, but rather rare in children. Its cause is still unknown, though is mostly considered to be acquired as a result of a degenerative process associated with aging. We experienced a case of colonic angiodysplasia in 14-year old boy, diagnosed by colonoscopy. The patient had a history of repeated hematochezia for a year. This report summarized our case and reviewed the literature.
Aging ; Angiodysplasia ; Capillaries ; Child ; Colon ; Colonoscopy ; Dilatation, Pathologic ; Gastrointestinal Hemorrhage ; Hemorrhage ; Humans ; Male ; Veins

Achalasia is very uncommon in children, and cases accompanied with alacrima and adrenal insufficiency is even more uncommon. When these three disorders are seen altogether, it is called triple A syndrome. It is inherited in an autosomal recessive manner and has potentially life-threatening sequelae. So, pediatricians should always consider the possibility of triple A syndrome when seeing children with achalasia. Neurological abnormalities such as autonomic neuropathy, peripheral neuropathy, sensory impairment and mental retardation occasionally accompany. We report a 2-year-old girl who presented with repeated vomiting, short stature and alacrima. Diagnosis of achalasia was made after perfoming esophagogram and endoscopy and was confirmed with esophageal manometry. After pneumatic dilatation, she became asymptomatic.
Adrenal Insufficiency ; Child ; Child, Preschool ; Diagnosis ; Dilatation ; Endoscopy ; Esophageal Achalasia ; Female ; Humans ; Intellectual Disability ; Manometry ; Peripheral Nervous System Diseases ; Vomiting

BACKGROUND: Neutrophil gelatinase-associated lipocalin (NGAL), a bacteriostatic agent, is known to inhibit erythropoiesis leading to anemia. We aimed to investigate the associations of NGAL, anemia, and renal scarring in children with febrile urinary tract infections (UTIs).METHODS: We retrospectively reviewed the medical records of 261 children with febrile UTIs. The relationship between the presence of anemia and plasma NGAL levels was investigated. NGAL performance in comparison with serum C-reactive protein (CRP) at admission and after 72 hours of treatment was also evaluated for the prediction of renal scarring as well as acute pyelonephritis (APN) and vesicoureteral reflux (VUR).RESULTS: Plasma NGAL levels were elevated in patients with anemia compared with those without anemia. Multiple linear regression analysis showed an inverse relationship between NGAL levels and erythrocyte counts (standard β = −0.397, P < 0.001). Increased NGAL, but not CRP, was independently associated with the presence of anemia (odds ratio [OR], 2.37; 95% confidence interval [CI], 1.07–5.27; P < 0.05). Receiver operating curve analyses showed good diagnostic profiles of pre- and post-treatment NGAL for identifying APN, VUR, and renal scarring (all P < 0.05). For detecting renal scars, the area under the curve of post-treatment NGAL (0.730; 95% CI, 0.591–0.843) was higher than that of post-treatment CRP (0.520; 95% CI, 0.395–0.643; P < 0.05). The presence of anemia and elevated NGAL at admission (> 150 ng/mL) were independent risk factors for renal scarring in children with febrile UTIs. With anemia, NGAL levels increased consecutively in children with febrile UTI without renal involvement, with APN without scar, and with APN with renal scarring.CONCLUSION: Increased plasma NGAL levels may be associated with the presence of anemia and renal scarring in children with febrile UTIs.

BACKGROUND: Neutrophil gelatinase-associated lipocalin (NGAL), a bacteriostatic agent, is known to inhibit erythropoiesis leading to anemia. We aimed to investigate the associations of NGAL, anemia, and renal scarring in children with febrile urinary tract infections (UTIs). METHODS: We retrospectively reviewed the medical records of 261 children with febrile UTIs. The relationship between the presence of anemia and plasma NGAL levels was investigated. NGAL performance in comparison with serum C-reactive protein (CRP) at admission and after 72 hours of treatment was also evaluated for the prediction of renal scarring as well as acute pyelonephritis (APN) and vesicoureteral reflux (VUR). RESULTS: Plasma NGAL levels were elevated in patients with anemia compared with those without anemia. Multiple linear regression analysis showed an inverse relationship between NGAL levels and erythrocyte counts (standard β = −0.397, P < 0.001). Increased NGAL, but not CRP, was independently associated with the presence of anemia (odds ratio [OR], 2.37; 95% confidence interval [CI], 1.07–5.27; P < 0.05). Receiver operating curve analyses showed good diagnostic profiles of pre- and post-treatment NGAL for identifying APN, VUR, and renal scarring (all P < 0.05). For detecting renal scars, the area under the curve of post-treatment NGAL (0.730; 95% CI, 0.591–0.843) was higher than that of post-treatment CRP (0.520; 95% CI, 0.395–0.643; P < 0.05). The presence of anemia and elevated NGAL at admission (> 150 ng/mL) were independent risk factors for renal scarring in children with febrile UTIs. With anemia, NGAL levels increased consecutively in children with febrile UTI without renal involvement, with APN without scar, and with APN with renal scarring. CONCLUSION Increased plasma NGAL levels may be associated with the presence of anemia and renal scarring in children with febrile UTIs.

BACKGROUND: Pharmacological ACTH test provide information only about the ability of the adrenal gland to respond to unusual stimuli and may not reflect the daily cortisol secretion. There were some controversies in the diagnosis of mild adrenal insufficiency by using a pharmacological dose of ACTH. The purpose of the present study was to assess and compare the effect of low dose 1 pg ACTH and high dose 249 pg ACTH in the evaluation of adrenal function. The intravenous injection of a pharmacological dose(250 pg) of ACTH has been used as a standard test in the initial assessment of adrenal function. So we low dose ACTH to evaluate the adrenal function and compare the result of high dose ACTH. METHOD: Basal serum cortisol sampling was done at 2:00 p.m.. And after 1 pg ACTH iv bolus injection, cortisol sampling was done at 20, 30, 45 and 60 min respectively. On the next day same procedure was repeated at same time, but 249 mg ACTH was given instead of the low dose. Normal adrenal function was diagnosed with the criteria of stimulated serum cortisol concentration over 20 mg/dL. Total 65 patients took part in this study. Three hypopituitarism patients and one Addisons disease were included. Sixty-one patients had the history of glucocorticoid ingestion or had physical findings of cushingoid features with symptoms suggest the adrenal insufficiency. RESULTS: Sixteen patients showed normal response to both low dose and high dose ACTH. Thirty-six patients were not stimulated to both low dose and high dose ACTH. The remaining thirteen patients revealed normal response to high dose ACTH, but not to low dose ACTH. CONCLUSION: It appears that low dose ACTH stimulation test is more sensitive and specific than high dose ACTH and is capable of revealing mild adrenal insufficiency.
Addison Disease ; Adrenal Glands ; Adrenal Insufficiency ; Adrenocorticotropic Hormone* ; Diagnosis ; Eating ; Humans ; Hydrocortisone ; Hypopituitarism ; Injections, Intravenous

Schinzel-Giedion syndrome is a rare, distinct dysmorphic syndrome characterized by congenital hydronephrosis, skeletal dysplasia, and severe developmental retardation, likely to be inherited as an autosomal recessive trait, but not yet confirmed. This syndrome is characterized by coarse facial features such as midfacial retraction, bulging forehead, short nose with anteverted nostrils, low-set malformed ears, protruding large tongue, and hypertelorism. Skeletal and limb defects, choanal stenosis, simian creases, hypospadias, microphallus, hypertrichosis, and intractable seizures are the frequently associated clinical findings. Urogenital involvement is a major component of the syndrome, and this problem sometimes is associated with nephrocalcinosis and urinary tract infection in the clinical course of the disease. We report a 22 month-old girl with Schinzel-Giedion syndrome complicated by medullary nephrocalcinosis and urinary tract infection due to Klebsiella pneumoniae. This patient had also been suffering from postnatal growth deficiency, intractable seizure, spastic tetraplegia, delayed development and severe mental retardation.
Constriction, Pathologic ; Ear ; Extremities ; Female ; Forehead ; Humans ; Hydronephrosis ; Hypertelorism ; Hypertrichosis ; Hypospadias ; Infant ; Intellectual Disability ; Klebsiella pneumoniae ; Male ; Nephrocalcinosis ; Nose ; Quadriplegia ; Seizures ; Tongue ; Urinary Tract Infections

PURPOSE: Long-term prognosis of Henoch-Schonlein purpura(HSP) is determined by the existence and severity of renal involvement. We evaluated the relationship between various clinical features of HSP and the development of renal involvement using univariate and multivariate analyses for early detection and proper management of HSP nephritis. METHODS: We performed a retrospective study of 200 children who were diagnosed as HSP and were initially free from renal involvement, from 1998-2003. Clinical features of each patient as risk factors of renal involvement were evaluated by univariate and multivariate analysis. Significant risk factors proven by univariate analysis were paired and re-analyzed to see if there are any interactions between them. RESULTS: A univariate analysis showed that sex, previous disease, severe abdominal symptoms, persistent purpura and steroid administration were associated with developing renal involvement. Among them, severe abdominal symptoms, persistent purpura, and male sex were statistically significant when they were re-analyzed after pairing, and they also increased the risk of renal involvement in a multivariate analysis. CONCLUSION: Severe abdominal symptoms, persistent purpura, and male sex are independent risk factors of renal involvement in HSP and it is presumed that interaction between them could be another factor of HSP nephritis.
Abdominal Pain ; Child ; Humans ; Male ; Multivariate Analysis* ; Nephritis ; Prognosis ; Purpura ; Purpura, Schoenlein-Henoch* ; Retrospective Studies ; Risk Factors*

PURPOSE: Disruption of normal renal development can lead to congenital anomalies of the kidney and urinary tract, including renal hypodysplasia. We aimed to clarify whether small kidney size affects clinical manifestations in children with urinary tract infection (UTI). METHODS: One hundred fifty-four patients who had their first symptomatic UTI between January 2014 and June 2015 were enrolled in this study. Differences in kidney size were estimated based on percent uptake of (99m)Tc-dimercaptosuccinic acid (DMSA) in scintigraphy. The patients who showed more than 10% difference in kidney size on DMSA scintigraphy with none or minimal cortical defects were included in group A. (group A, n=17). Laboratory, clinical, and imaging results were compared with those of the other patients (group B, n=137). RESULTS: Group A had a relatively higher incidence of vesicoureteral reflux than group B (44% vs 20%, P<0.05). The levels of plasma neutrophil gelatinase-associated lipocalin (NGAL) and serum C-reactive protein were significantly higher in group A (193 [64-337] vs 91 [59-211] ng/mL and 4.1 [0.5-11.9] vs 2.1 [0.7-5.3] ng/mL, respectively; all P<0.05). Linear regression analysis revealed that plasma NGAL level strongly correlated with the difference in renal uptake in DMSA scintigraphy in group A (R²=0.505). CONCLUSION: The difference in kidney size could influence the clinical course and severity of pediatric UTI.
C-Reactive Protein ; Child ; Humans ; Incidence ; Kidney ; Linear Models ; Lipocalins ; Neutrophils ; Plasma ; Radionuclide Imaging* ; Succimer* ; Urinary Tract Infections* ; Urinary Tract* ; Vesico-Ureteral Reflux

PURPOSE: Although the American Academy of Pediatrics provides clinical guidelines for urinary tract infection (UTI) infants, guidelines are not appropriate for neonates and infants less than 2 months of age due to insufficient data. The aim of this study was to evaluate the characteristics of neonates and young infants less than 2 months old (group 1) with UTI compared to older infants from 2 to 12 months old (group 2). METHODS: We reviewed UTI patients aged 0 to 12 months admitted to the pediatric department in the last 5 years. Clinical characteristics such as age, sex, fever duration, recurrence, progression to acute pyelonephritis (APN), malformations like hydronephrosis and vesicoureteral reflux (VUR), and laboratory results were compared between group 1 and group 2. RESULTS: 615 patients were included in this study. Group 1 had 94 cases and group 2 had 521 cases. Escherichia coli was the most commonly isolated pathogen in urine cultures. Fever duration was shorter in group 1 (vs.) 2 (1.91+/-1.43 days vs. 3.42+/-2.40 days, P<0.05). As compared to group 2, group 1 had a higher proportion of patients with antenatal hydronephrosis and hydronephrosis found after admission (10.6% vs. 3.6% and 75.5% vs. 55.9%, P<0.05). There were differences between two groups in white blood cell (WBC) count (Group 1: 13,694+/-5,315/microL, Group 2: 15,271+/-6,130/microL, P<0.05) and C-reactive protein (Group 1: 32.02+/-35.17 mg/L, Group 2: 46.51+/-46.63 mg/L, P<0.05). CONCLUSION: Compared to older infants, UTI in neonates and young infants shows milder clinical manifestations except higher rates of hydronephrosis but outcome is alike.
C-Reactive Protein ; Escherichia coli ; Fever ; Humans ; Hydronephrosis ; Infant* ; Infant, Newborn* ; Leukocytes ; Pediatrics ; Pyelonephritis ; Recurrence ; Urinary Tract Infections* ; Urinary Tract* ; Vesico-Ureteral Reflux

Segmental necrotizing jejunitis is characterized by severe abdominal pain of acute onset, bilious vomitings and foul smelling loose stools containing blood. Pathologic features include circumferential intestinal wall inflammation ranging from edema with minimal congestion to severe congestion, hemorrhage with necrosis, ulceration, and gangrene with perforation. Early diagnosis and suitable supportive measures prevent unnecessary laparatomy and complications. There was no report of this disease entity in children in Korea. We experienced a case of segmental necrotizing jejunitis with fever, abdominal pain and bloody stools, which was diagnosed by exploration and was treated successfully by antibiotics and supportive measures.
Abdominal Pain ; Anti-Bacterial Agents ; Child ; Early Diagnosis ; Edema ; Estrogens, Conjugated (USP) ; Fever ; Gangrene ; Hemorrhage ; Humans ; Inflammation ; Korea ; Necrosis ; Smell ; Ulcer