Molecular Mechanism of Pancreatic Bicarbonate Secretion

Thanks to recent progress in availability of molecular and functional techniques it became possible to search for the basic molecular and cellular processes that mediate and control HCO3- and fluid secretion by the pancreatic duct. The coordinated action of various transporters on the luminal and basolateral membranes of polarized epithelial cells mediates the transepithelial HCO3- transport, which involves HCO3- absorption in the resting state and HCO3- secretion in the stimulated state. The overall process of HCO3- secretion can be divided into two steps. First, HCO3- in the blood enters the ductal epithelial cells across the basolateral membrane either by simple diffusion in the forms of CO2 and H2O or by the action of an Na+-coupled transporter, a Na+-HCO3- cotranporter (NBC) identified as pNBC1. Subsequently, the cells secrete HCO3- to the luminal space using at least two HCO3- exit mechanisms at the luminal membrane. One of the critical transporters needed for all forms of HCO3- secretion across the luminal membrane is the cystic fibrosis transmembrane conductance regulator (CFTR). In the resting state the pancreatic duct, and probably other HCO3- secretory epithelia, absorb HCO3-. Interestingly, CFTR also control this mechanism. In this review, we discuss recent progress in understanding epithelial HCO3- transport, in particular the nature of the luminal transporters and their regulation by CFTR.

Nitroblue Tetrazolium(NBT) Test as a Screening Test of Urinary Tract Infection in Children

PURPOSE: It has been reported that the Nitroblue Tetrazolium(NBT) test is more accurate than the urine pH, leukocyte esterase and nitrite test as a screening test of urinary tract infection (UTI). The purpose of this study is to compare the NBT test with other screening tests and evaluate the clinical usefulness of the NBT test as a screening test. METHODS: We selected 298 results out of the 304 urine cultures which were performed from March, 1999 to July, 1999 and compared them with screening tests such as NBT, urine pH, leukocyte esterase and nitrite tests. We interpreted those results as the urinary tract infection when the screening results were urine NBT(+), pH(>6.5), leukocyte esterase(>or=++) and nitrite(+). RESULTS: Urine NBT, pH and leukocyte esterase tests showed the statistical significance in comparison with the urine culture results(chi-square tests; P<0.001, <0.05, <0.001), while urine nitrite tests did not show statistical significance. As time passing, the sensitivity of 10 min, 30 min, 60 min NBT test was increased to 35%, 72%, 80% respectively but the sensitivity of urine pH and leukocyte esterase was as low as 33%, 16% respectively. But, the specificity of NBT test was reduced from 93% to 53% as the time went by, while the specificity of urine pH and leukocyte esterase tests were as high as each 79%, 96% respectively. Urine NBT tests at 10 min and 30 min showed a higher positive and negative predictive value than those of the other screening tests. CONCLUSION: Urine NBT test as a screening test for UTI was more accurate than the urine pH, nitrite and leukocyte esterase tests. But we think that we should develop a more quick and precise screening test in the future, because of the long time it requires to perform it.

Influence of Environmental Living Standards on Helicobacter pylori Infection in Korean Elementary School Children / 대한소아소화기영양학회지

PURPOSE: We measured anti-H. pylori IgG in Korean elementary school children living in Shinchon area of Seoul, Korea to evaluate the influence of environmental living standards on H. pylori infection. METHODS: IgG antibodies to H. pylori were measured in plasma using a commercial ELISA kit (GAP IgG Helicobacter pylori, Bio-Rad Laboratories Inc., Hercules, CA, USA). Information on environmental status such as place of birth, parental income, type of housing, number of persons in the household, parents' occupation, family history of peptic ulcer disease and gastric cancer was obtained. Statistical analysis was done by Chi-square and logistic regression test using SPSS 7.0(TM) for Windows. RESULTS: Study subjects consisted of 571 children, and the age distribution ranged from 6.0 to 13.6 years with a mean of 9.6+/-1.8 years. Male-to-female ratio was 1.1:1. The seropositive rates of H. pylori infection ranged from 10.4% in children aged 6 years to 30.9% in 12 year-old group, overall 16.8%. The prevalence of H. pylori infection progressively increased with age, but there was no significant difference in seropositive rates among children in different age groups (p=0.06). Seropositive rates of anti-H. pylori IgG on the basis of gender, place of birth, parental income, type of housing, parents' occupation, family history of peptic ulcer disease and gastric cancer showed no statistically significant difference. Interestingly, however, seropositive rate of anti-H. pylori IgG showed statistical significance in relation to number of persons in the household (p=0.003; Odds ratio 1.50 by logistic regression test). CONCLUSION: These results suggest that number of persons in the household is the most important factor among environmental living standards, and that risk of H. pylori infection increases by increment of 1.5 times as the number of persons in the household increases by one.

A Recurred Case of Subacute Necrotizing Lymphadenitis(Kikuchi's Disease) During Childhood

Subacute necrotizing lymphadenitis, or Kikuchi's disease, primarily affects the cervical lyrnph nodes of lung adults and has a self-limited clinical course. Differential diagnoses are malignant lymphoma and systemic lupus erythematosus. Recurrence rate is low as 3.3%, and there have been a few reports describing the recurrence of the disease. The symptoms in almost all recurrent cases were similar to those of the early stages of the disease. The etiology of the disease remains unknown, but various viral infections and autoimmune processes have been postulated to be the cause. A 6-year-old boy presented with a fever of approximately 40C degrees, painless lumps on both sides of the neck, and intermittent abdominal pain for 2 weeks. Subacute necrotizing lymphadenitis was diagnosed 3 years before admission, which was reported by us in 1996. Diagnosis of recurred subacute necrotizing lymphadenitis was confirmed by histological examination of the lymph node. We report a recurred case of subacute necrotizing lymphadenitis during childhood.

A Case of Facial Nerve Palsy Associated with Epstein-Barr Virus Infection in a Child

Facial palsy is a rare complication of infectious mononucleosis of Epstein-Barr virus infection, which can be easily overlooked by clinicians if one is unnoticed about it. We report a 2 year and 11 month old boy who was diagnosed by facial nerve palsy caused by Epstein-Barr virus infection. Pseudothrombocytopenia was also associated with him. Steroid (prednisolone 2mg/kg) was administered daily for 2 weeks and then maintained with alternative tapering schedule (2mg/kg at once QOD) in order to decompress facial nerve. He was completely recovered at 2 months of follow-up.

A Case of Greig Cephalopolysyndactyly Syndrome

Greig cephalopolysyndactyly syndrome (GCPS) is a disorder characterized by postaxial polydactyly of the hand, broad or occasionally bifid thumbs, preaxial polydactyly of the feet, broad halluces, syndactyly of the fingers or toes, macrocephaly, frontal bossing, hypertelorism and a broad nasal bridge. Intelligence is usually normal, although borderline IQ has been reported. Advanced bone age, mild hydrocephalus, craniosynostosis and agenesis of the corpus callosum are occasionally associated abnormalities. We report here a 10-day-old male infant with GCPS. Birth Weight was 2,400kg and gestational age was 39 wks. He had a wide broad high forehead, hypertelorism, broad nose base and cryptorchidism. He had preaxial polysyndactyly due to duplication of the right thumb and left accessory thumb, duplication of both halluces and syndactyly of both toes and fingers. His brain MRI showed corpus callosum agenesis, mild hydrocephalus and small choroid plexus cyst. High resolution chromosomal analysis showed a de novo balanced translocation 46, XY, t (7;8) (p22;q24.1). We report the first GCPS case in Korea with brief literature.

Chest Pain

No abstract available.

A Case of Severe Air Leak Syndrome Treated with High - Frequency Oscillatory Ventilation ( HFOV )

In air leak syndrome, a significant portion of the volume delivered during a positive pressure breath can be lost through the leak. HFOV can achieve adequate ventilation at lower peak and/or mean intrapulmonary pressure than conventional mechanical ventilation (CMV) and has been an effective treatment of already established air leak syndrome. We report a 1-day-old male infant with severe respiratory failure from pneumothorax and pneumomediastinum, who was refractory to CMV with chest tube drainage. HFOV was applied to this patient for 114 hours, and improvement of oxygenation and ventilation as well as significant reduction of pneumothorax followed.

A Case of Dieulafoy's Disease in a Child / 대한소아소화기영양학회지

Dieulafoy's disease, a vascular anomaly mainly in the upper stomach, is a rare but potentially life-threatening cause of upper gastrointestinal bleeding. Pathogenesis is still controversial, but the most accepted theory is that a persistent caliber vessel in the submucosa is exposed by a small mucosal erosion leading to massive bleeding. The bleeding site is usually within 6 cm of the esophagogastric junction in the cardia or fundus of the stomach. The treatment of choice is therapeutic endoscopy or surgery. The age of patients reported is mainly between 50 and 70 years, and patients of pediatric age are extremely rare. We are reporting a 5-year-old male patient who had Dieulafoy's disease which was diagnosed by emergency upper gastrointestinal endoscopy. Endoscopic finding was a nodular lesion with an adherent clot on the lessor curvature of the stomach 2 cm below the esophagogastric junction. Epinephrine and Beriplast(R) was injected in the lesion. On the second day after endoscopic sclerotherapy, the patient had recurred massive hematemesis and accompanying shock. So we performed gastrotomy and ligation. After the operation, he showed an improved general condition and was discharged at the 12th hospital day.

A Study of Factors Affecting Time of First Stool in Premature Infants

PURPOSE: To assess the effect of gestational age and illness severity, and the effect of antenatal exposure to magnesium sulfate, glucocorticoids, and antibiotics, on the timing of the first stool in preterm infants. METHODS: Medical records of all preterm infants admitted to the neonatal ward at Kangnam Sacred Heart Hospital between March 1998 and August 1998 were reviewed. We studied the time of the first stool in 55 infants. RESULTS: The median age of the infant at the time of first stool was 18 hours, and 90% of the infants passed stool by 50 hours. Both the gestational age and the illness severity, as measured by the score for neonatal acute physiology(SNAP), correlated significantly with the timing of the first stool(r=0.47 and P<0.001 for SNAP; r=0.29 and P<0.05 for gestational age). An analysis of covariance showed that the relationship between SNAP and the timing of the first stool was significant even after adjustment for gestational age(P<0.01), but the relationship between the gestational age and the timing of the first stool was not significant after adjustment for SNAP (P=0.14). Antenatal exposure to magnesium sulfate for tocolysis, glucocorticoids for enhancing fetal lung maturity, and antibiotics, had no effect on the timing of the first stool. CONCLUSION: Delayed passage of first stool is a function of illness severity, not of gestational immaturity. Antenatal exposure to magnisium sulfate, dexamethasone, and antibiotics, does not affect the timing of first stool in premature infants.

A Study of Factors Affecting Time of First Stool in Premature Infants

PURPOSE: To assess the effect of gestational age and illness severity, and the effect of antenatal exposure to magnesium sulfate, glucocorticoids, and antibiotics, on the timing of the first stool in preterm infants. METHODS: Medical records of all preterm infants admitted to the neonatal ward at Kangnam Sacred Heart Hospital between March 1998 and August 1998 were reviewed. We studied the time of the first stool in 55 infants. RESULTS: The median age of the infant at the time of first stool was 18 hours, and 90% of the infants passed stool by 50 hours. Both the gestational age and the illness severity, as measured by the score for neonatal acute physiology(SNAP), correlated significantly with the timing of the first stool(r=0.47 and P<0.001 for SNAP; r=0.29 and P<0.05 for gestational age). An analysis of covariance showed that the relationship between SNAP and the timing of the first stool was significant even after adjustment for gestational age(P<0.01), but the relationship between the gestational age and the timing of the first stool was not significant after adjustment for SNAP (P=0.14). Antenatal exposure to magnesium sulfate for tocolysis, glucocorticoids for enhancing fetal lung maturity, and antibiotics, had no effect on the timing of the first stool. CONCLUSION: Delayed passage of first stool is a function of illness severity, not of gestational immaturity. Antenatal exposure to magnisium sulfate, dexamethasone, and antibiotics, does not affect the timing of first stool in premature infants.

Colonic transit patterns and plasma cholecystokinin levels in children with recurrent abdominal pain

Plasma cholecystokinin levels were measured in children with recurrent abdominal pain to investigate the relationship of plasma cholecystokinin levels with colonic transit patterns and clinical symptoms. Subjects consisted of 120 children (mean age 9.6 +/- 2.6 years) for whom colonic transit study had also been done. Plasma cholecystokinin levels were 79.2 +/- 58.7 pg/mL in children with colonic inertia, 70.7 +/- 47.0 pg/mL in hindgut dysfunction, 57.4 +/- 53.1 pg/mL in pelvic outlet obstruction, and 67.6 +/- 47.9 pg/mL in normal colonic transit. These data showed that there was a tendency of increasing plasma cholecystokinin levels in children with proximal colon transit delay, although there was no significant difference among four groups. Plasma cholecystokinin levels in children of 10 years of age and under (54.5 +/- 40.4 pg/mL) were significantly lower (p = 0.01) than in children over 10 years (79.1 +/- 59.8 pg/mL). Plasma cholecystokinin levels based on colonic transit patterns, however, were not significantly different between the two age groups. There was no significant difference in plasma cholecystokinin levels between groups based on defecation frequency per week, presence of defecation pain, symptoms of milk intolerance, or the presence of emotional stress. These results suggested that there was a tendency of increasing plasma cholecystokinin levels in the younger age group and in children with delay in proximal colonic transit, but further study is required in relation to plasma cholecystokinin levels based on colonic transit patterns in a large number of patients.

A Case of Segmental Nectotizing Jejunitis / 대한소아소화기영양학회지

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.

A Case of Isolated Adenomatous Polyp of Rectum in a Child: Tubulovillous adenoma / 대한소아소화기영양학회지

Isolated rectal adenomatous polyp without genetic background is rarely found in children. A 4-year and 5 month-old girl was admitted for intermittent bloody stools lasting 4 months. A 1.5x1.2 cm sized rectal polyp was found by air contrast barium enema. Endoscopic polypectomy was performed without complications. In histopathologic examination, it was found to be a tubulovillous adenoma. Typical radiologic, colonoscopic, and pathological pictures are presented.

Serum gastrin and pepsinogen I, II concentrations in children with Helicobacter pylori infection: the role of CagA and VacA

Serum gastrin and pepsinogen concentrations were measured in 51 children infected with Helicobacter pylori, to investigate the clinical significance and influence of CagA and VacA on serum concentrations of these peptides. CagA+ was 44/51 (86%) and VacA+ was 42/51 (82%). Type I (CagA+/VacA+) included 39/51 (76%), type II (CagA-/VacA-) was 4/51 (8%), and intermediate (CagA-/VacA+, CagA+/VacA-) was 8/51 (16%). There was no significant correlation between endoscopic diagnosis and the state of CagA/VacA. Serum gastrin concentrations were not significantly correlated with the state of CagA/VacA. Serum pepsinogen I and II concentrations were significantly higher in CagA+ than in CagA-, but there was no significant difference between VacA+ and VacA-, Serum pepsinogen I/II ratio was not significantly correlated with the state of CagA/VacA. There was no significant difference between serum concentrations of gastrin, pepsinogen I and H. pylori phenotypes. However, pepsinogen II concentration was significantly higher in type I than type II. Pepsinogen I/II ratio was significantly lower in type I and intermediate than in type II. These findings suggest that CagA positively and phenotype of H. pylori could play a role in the development of upper gastrointestinal diseases in children.

Effect of Cyclosporine A on Plasma and Urine Levels of Endothelin-1 in Steroid Dependent Minimal Change Nephrotic Syndrome

A fine needle aspiration biopsy specimen of a retroperitoneal mass in a 26-year-old man who had had an orchiectomy for seminoma was submitted for cytologic evaluation. Cytologic features of the specimen included uniform neoplastic cells found singly or in groups of several cells intermingled with lymphocytes in a characteristic foamy, lacelike background. These cells varied from 10 to 20 m in diameter. The nuclei were round to ovoid with fine or reticular chromatin and one or more prominent nucleoli. The poorly defined cytoplasm stained pale-blue or blue with cytoplasmic vacuoles. The cytologic appearance was consistent with seminoma. Documented reports of the cytological appearance of seminoma are rare. The diagnosis of primary gonadal seminoma by fine needle aspiration biopsy is probably not indicated since the treatment of a primary gonadal tumor, regardless of its histogenesis, requires surgical resection. However, fine needle aspiration biopsy is extremely valuable in the diagnosis of extragonadal as well as metastic and recurrent seminoma.

A Case of Hypocortisolemia Presented by Short Stature / 대한소아내분비학회지

Isolated ACTH deficiency is a rare cause of secondary adrenocortical insufficiency. The clinical presentation can be similar to that of primary adrenal insufficiency, but most of them may be nonspecific. A female patient of 25 months of age, complainig short stature, showed hypocortisolemia without ACTH & cortisol stimulation by insulin-induced hypoglycemia test. All the other hormone state was normal. Left hand AP view revealed delayed bone age(3 month) compared with chronological age. No radiologic abnormality was found in sella MRI and adrenal CT. Here we report a case of isolated ACTH deficiency presented by short stature.

Treatment of chronic hepatitis B in children with prednisolone withdrawal followed by recombinant interferon alpha

Steroid withdrawal followed by interferon therapy is an alternative approach for treating chronic hepatitis B virus infection when there has been no therapeutic response to interferon alone. The effectiveness of steroid withdrawal followed by interferon therapy and factors predictive of the response were evaluated in 35 children with biopsy-proven chronic hepatitis B. Patients had received a 1-month course of prednisolone, 1 mg/kg per day orally, followed by a 2-week rest, and then were treated with interferon alpha 3 MU three times per week for 4-6 months. The serum aminotransferase values normalized in 80%, and negative seroconversion rates of HBeAg and HBV-DNA were 69% and 66%. The good response rate was associated with a pretreatment HBV-DNA level lower than 100 pg/ml and a posttreatment ALT level more than 200 IU/L. Normalization of ALT values usually took 5 months, and the clearance of HBV-DNA and HBeAg took 7.8 and 6.7 months, respectively. These results suggest that steroid withdrawal followed by interferon therapy is useful in the treatment of chronic hepatitis B in children, and that a good response rate can be expected in children with lower pretreatment HBV-DNA levels (< 100 pg/ml).

Therapeutic Outcome and Recurrence Rate of Helicobacter pylori Infection in Children / 대한소아소화기영양학회지

PURPOSE: This study aimed at evaluating the therapeutic outcome, cost effectiveness and recurrence rate in children with H. pylori infection after the treatment using various medications. METHODS: Seventy five children (mean age 11.4+/-2.5 years) were given an endoscopy with biopsy and H. pylori status assessed by CLO test and histologic examination (Warthin Starry stain). Children were given one of following medications such as DA (Denol(R) and amoxycillin), OA (omeprazole and amoxycillin), DC (Denol(R) and clarithromycin) as primary treatment. And one of following medications such as DAM (Denol(R), amoxycillin and metronidazole), DC, OA, OC (omeprazole and clarithromycin) were used in children who failed the eradication of H. pylori. RESULTS: The endoscopic diagnoses were: nodular gastritis (46 cases), gastric ulcer (9), duodenal ulcer (6), superficial gastritis (6), and normal (8). H. pylori eradication rate was 91% (63 of 69 children) on 4 weeks course of DA, 50% (1 of 2 cases who had treatment failure on DA) on DAM, and 75% (3 of 4 cases who treated on DC primarily) and 50% (1 of 2 cases who had treatment failure on DA) on DC, and 100% on OA (all of 2) and on OC (all of 1 who failed on DA). In 3 of 7 children in whom H. pylori had not been eradicated by primary medications (DA 6 and DC 1 case), H. pylori was re-eradicated by secondary medications (DA 1, DAM 1 and DC 1 case). But in remaining 4 cases, H. pylori infection persisted. Reinfection of H. pylori was found in 4 of 75 children between 3 months and 3 years after completion of the treatment of DA, yielding recurrence rate of 5.3%. In 2 of 4 cases who had relapsed, H. pylori was re-eradicated by secondary medications (OC 1 and DA 1 case). But in remaining 2 cases, H. pylori infection persisted. CONCLUSION: These results suggest that dual therapy with Denol(R) and amoxycillin is the effective medications in treating H. pylori infection in children. Concerning the cost effectiveness, it can Austrabe recommended as first line treatment of choice as well.

Midgut Volvulus of Infant Simulating Intussusception Diagnosis with Color Doppler Ultrasonography / 대한소아소화기영양학회지

Although midgut volvulus is clinically characterized by bilous vomiting and abdominal distention, plain abdominal X-ray is usually non-specific and therefore it can be misdiagnosed to other diseases. Upper gastrointestinal contrast study and computed tomography have been used as a routine diagnostic tool but it takes cost and time. Abdominal ultrasonography is a relatively good alternatives in diagnosing midgut volvulus and it relatively saves cost and time. But case presentation of midgut volvulus diagnosed with abdominal ultrasonography are rarely found in literature. We experienced a 6 month old girl who had come to our hospital with bilous vomiting and was diagnosed as midgut volvulus with ultrasonography. Thus we report this case with the presentation of typical ultrasonographic findings of midgut volvulus.