Microvillous inclusion disease (MID) or congenital microvillous atrophy is a rare cause of intractable, persistent secretory diarrhea shortly after birth or at birth. The prognosis of MID is very poor and most of the reported patients have died within 6.5 years. Diagnosis is based on the ultrastructural demonstration of intracytoplasmic inclusion of microvilli. There have been several cases reported in the literature. To evaluate the clinicopathologic features of MID, we have reviewed 4 cases of MID which was confirmed by the ultrastructural study of the duodenal biopsy. All patients suffered from life-threatening diarrhea since birth. Many other radiologic or laboratory findings were of no value in the evaluation of causative agents. One of the patients died of the disease and remaining patients have been alive on intravenous fluids or total parenteral nutrition. Histological findings of all cases were similar and characterized by varying degrees of mucosal atrophy and chronic inflammatory cell infiltration in the duodenal mucosa. PAS stain revealed a discontinuous brush border over the atrophic villous surface with or without small vacuoles in the cytoplasm of the surface epithelium. Ultrastructural changes were found mainly in the surface epithelium of the duodenal mucosa and characterized by the presence of membrane bound inclusions lined by intact or degenerating microvilli, as well as degeneration of surface epithelial cells with loss of microvilli, or with sparse, short microvilli.
Atrophy ; Biopsy ; Child ; Cytomegalovirus Infections* ; Cytoplasm ; Diagnosis ; Diarrhea ; Epithelial Cells ; Epithelium ; Humans ; Membranes ; Microvilli ; Mucous Membrane ; Parenteral Nutrition, Total ; Parturition ; Prognosis ; Vacuoles

PURPOSE: Cyclic vomiting syndrome is characterized by repeated, unpredictable, explosive and unexplained bouts of vomiting and recur after intervals of uncertain length. In the absence of either known etiology or pathogenesis, deciphering the mystery of cyclic vomiting syndrome presents us with a daunting clinical challenge. As we seek to understand this mysterious vomiting disorder of unknown etiology and pathogenic mechanism, we perfomed electrogastrography (EGG) and measured radionuclide gastric emptying time and compared the data with that of control. METHODS: Total 13 patients with cyclic vomiting (ages from 1 yr 6 m to 13 yr 3 m : mean 7 yr 2 m ) and 9 normal controls (ages from to 1 yr 8 m to 13 y 4 m, mean; 7 y 6 m) were enrolled in this study. Among them 16 children (9 patients and 7 controls) undertook gastric emptying time and 13 children (7 patients and 5 controls) undertook EGG. EGG : after midnight NPO, total 3 triplet of electrodes (two for the recoding of gastric electricity along the antral axis, and the other one for references) were used. After 1 hour recording of EGG we analysed the dominant frequencies. Gastric emptying: gastric emptying was evaluated by a radiolabeled (1mCi [99mTc] sulfur colloid incorporated into egg albumin) solid phase meal after an overnight fast of at least 8 hr. Radioisotope counts were obtained immediately after ingestion and continued for 10-min intervals for 120 min. The half emptying time (T1/2) and percent gastric residual at 2 hr were calculated. RESULTS: EGG: the dominant frequencies were significanlty different between case and control group (p=0.005, median values; 4.1 Hz and 3.1 Hz in case group and control group respectively ). Gastric emptying time: the gastic emptying rates at 120 min were 53.1+/-16% and 66.9+/-7.0% in patients and control groups respectively, which showed the gastric emptying was significantly delayed in patient groups (p=0.042) CONCLUSIONS: With the results of our study, we felt that the patients with cyclic vomiting has a some sort of motility disorders, like as a variant of chronic idiopathic intestinal pseudoobstruction or gastroparesis. But further sophisticated designed studies are needed to confirm this.
Axis, Cervical Vertebra ; Child ; Colloids ; Eating ; Electricity ; Electrodes ; Gastric Emptying* ; Gastroparesis ; Humans ; Intestinal Pseudo-Obstruction ; Meals ; Ovum ; Sulfur ; Triplets ; Vomiting*

PURPOSE: Endoscopic variceal sclerotherapy(EVS) has been considered the mainstay therapy for bleeding esophageal varices in adults. However, recent data have shown that endoscopic variceal ligation is just as efficacious and has fewer complications than EVS. Although there are many reports concerning EVL in adults, only a few studies have been done of children. METHODS: We performed EVL in 9 children under 6 years of age(mear age, 2.5 year) with esophageal varices. Outcome was assessed with respect to survival, rebleeding, and complications. RESULTS: The causes of portal hypertension were 3 cases of congenital hepatic fibrosis, 3 cases of biliary atresia, 2 cases of portal vein thrornbosis, and one case of portal vein fibromuscular dysplasia. The age at diagnosis ranged from 10 months to 6 years. The patients underwent a mean of 2.6+/- 0.7 sessions of EVL(ranging from two to four). The numbers of bands per person were 5.6+/-1.8, and the numbers of bands per session were 2.2+/-1.0 Two complications of esophageal rebleeding were noted, and none of the cases experienced symptoms of esophageal stenosis, nor gastroesophageal reflux. CONCLUSION: EVL is safe and effective in controlling variceal hemorrhage in children with portal hypertension, regardless of etiology. The cornplication rate is low and EVL is an acceptable and perhaps preferable altemative to EVS in children with esophageal varices. But regular periodic examination for recurrence of varices after eradication should be required.
Adult ; Biliary Atresia ; Child* ; Diagnosis ; Esophageal and Gastric Varices* ; Esophageal Stenosis ; Fibromuscular Dysplasia ; Fibrosis ; Gastroesophageal Reflux ; Hemorrhage ; Humans ; Hypertension, Portal ; Ligation* ; Portal Vein ; Recurrence ; Varicose Veins

No abstract available.
Hypoglycemia*

PURPOSE: Anorectal manometry is a way of investigation for anti-rectal sphincters. In this paper we evaluated the usefulness of anorectal manometry in constipation patients and compared the anal spnincter function in control, constipation and encopresis patients. METHOD: We analysed the data of anorectal function studies in normal children (control, n=11), children with constipation (constipation group, n=20) and children with encopresis (encopresis group, n=16). RESULTS: The specific manometric parameters in normal children were like as follows; external anal sphinter pressure 21.0+/-8.00 mmHg, internal anal sphicter pressure 30.0+/- 14.57 mmHg, conscious rectal sensitivity threshold 11.4+/-4.52 mmHg. The above results were not different from that of previous studies except conscious rectal sensitivity threshold, which was slightly lower than that of others. Internal and external anal sphincter pressure were elevated significantly in constipation and encopresis groups than in control, which results was the same in conscious rectal sensitivity threshold. But the values of rectoanal inhibitory threshold and percent relaxation of rectoanal inhibitory reflex were not different among control group, constipation group and encopresis group. External sphincter activity was increased during the act of bearing down for defecation in none of the child in control group, in 6 of 17 children in constipation group and 5 of 12 children in encopresis group. CONCLUSION: With the results of above we could say that complete history taking and physical examination are important in diagnosis of constipation, and we could say also that the anorectal manometry was a valuable tool to understand the physiology of normal defecation and the pathophysiology of constipation and encopresis.
Anal Canal ; Child* ; Constipation* ; Defecation ; Diagnosis ; Encopresis ; Humans ; Manometry* ; Physical Examination ; Physiology ; Reflex ; Relaxation

PURPOSE: Recurrent abdominal pain(RAP) is a common problem in childhood, and has a reported prevalence of 10 to 20% in children. Functional abdominal pain is classified into nonulcer dyspepsia(NUD) and irritable bowel(IB) in adults. We designed this study to evaluate clinical manifestations of RAP according to its subclassification. METHODS: Patients who visited Samsung Medical Center from January to July 1998 due to recurrent abdominal pain were included. According to questionaire and history taking, RAP was classified into NUD, its subtypes, and IB. RESULTS: Among 118 children, 93 children were included. Fifteen patients were excluded because we knew the causes of RAP and 10 patients were excluded because they couldn't understand the questionaire. Thirty-five patients(37.6%) had symptoms of NUD, 11(11.8%) had symptoms of IB, and 47(50.5%) had both symptoms. Among the patients with NUD, 16(19.5%) were classified into dysmotility-like dyspepsia(NUDD), 18 patients(22%) into ulcer-like dyspepsia(NUDU) and 1 patient(1.2%) into reflux-like dyspepsia(NUDR). Twenty eight patients(34%) had both symptoms of NUDD and NUDU, 8 patients(9.8%) had both NUDD and NUDR, 3 patients(3.7%) had both NUDR and NUDU and 8 patients(9.8%) had all symptoms. Among patients with IB and NUD, symptom of NUDD(63.2%) was most prevailing. NUDR significantly correlated with autonomic nervous symptoms(P=0.006). However, the prevalence rate of H. pylori IgG antibody were not significantly different among subgroups. CONCLUSION: NUDD is the most common symptom that is manifestated in children and it is the same in adults. This study showed that the subclassification of RAP in children is feasible.
Abdominal Pain* ; Adult ; Child ; Humans ; Immunoglobulin G ; Irritable Bowel Syndrome ; Prevalence

PURPOSE: Many diagnostic modalities for neonatal cholestasis have shown features that are helpful, however until recently none of them are not pathognomonic of biliary atresia (BA). We carried out a prospective study of infants with cholestatic jaundice with the aim of establishing an efficient method of diagnosing BA. METHODS: Twenty-seven consecutive infants with cholestatic jaundice were enrolled in this study and ranged from 7 to 152 days in age (mean; 51.2+/-34.2 days). Gastroduodenoscopy was carried out using a fiberscope (Olympus N30). All the babies were fasted for at least 4 hours before the procedure and 20 ml of 10% dextrose solution was given at the time of endoscopy. The endoscopic examination focused on the 5 minutes observation of the evidence of biliary secretion. If there was lack of the evidence of the biliary secretion, endoscopy was removed and repeated the examination with some pause. RESULTS: There are lack of the evidence of biliary secretion in all infants with BA. In non-BA group, 8 out of the 10 infants showed biliary secretion on the first trial, however one (Alagille syndrome) of the two infants without evidence of biliary secretion, finally exhibited biliary secretion on the second trial. The above observations resulted in the diagnostic accuracy of 96.3% with 100.0% sensitivity and 90.0% specificity. CONCLUSION: In light of the results from our relatively small study, endoscopy is a convenient, and relative inexpensive procedure. we strongly support the use of endoscopy for the diagnosis of BA in the screening and evaluation of infantile cholestasis.
Biliary Atresia* ; Cholestasis ; Diagnosis ; Diagnosis, Differential* ; Endoscopy* ; Glucose ; Humans ; Infant ; Jaundice, Obstructive ; Mass Screening ; Prospective Studies ; Sensitivity and Specificity

PURPOSE: The role of H. pylori in the pathogenesis of recurrent abdominal pain in children is uncertain. In order to find a correlation between H. pylori infection and recurrent abdominal pain in children, the present study was designed to compare the clinical manifestations between recurrent abdominal pain children with and without H. pylori infection and to investigate the effects of H. pylori eradication on abdominal pain. METHODS: Among 148 children with recurrent abdominal pain, a total of 129 children with recurrent abdominal pain were the subjects of this study. The remaining 19 patients were excluded due to intra-abdominal lesions causing abdominal pain (18 cases), and a lost case during follow-up (1 case). The subjects were classified into 2 groups : the case group consisted of the 22 patients who had evidence of H. pylori infection. The rest, 107 patients who were H. pylori negative, became the control group. Clinical features of recurrent abdominal pain by questionnaire on the location and characteristics of the pain, family history of non ulcer dyspepsia, irritable bowel and passive smoking were investigated by comparison of these two group. The effect of H. pylori suppression by antibiotics (amoxicillin, for 2 weeks and bismuth, for 4 weeks) on abdominal pain in the case group was also evaluated. RESULTS: The questionnaire revealed that pain localized more in the epigastric or supraumbilical area in the case group (8 out of 21 vs. 14 out of 88: P=0.023) and the family histories of irritable bowel were more common in control group (74 out of 100 vs.. 11 out of 22; P=0.013). The case group, treated with antibiotics, had significantly greater improvement in subjective and objective symptoms compared with control group (p=0.040 and p=0.037 respectively). CONCLUSION: The above results suggest that H. pylori infection may induce chronic abdominal pain in children.
Abdominal Pain* ; Anti-Bacterial Agents ; Bismuth ; Child* ; Dyspepsia ; Follow-Up Studies ; Helicobacter pylori* ; Helicobacter* ; Humans ; Surveys and Questionnaires ; Tobacco Smoke Pollution ; Ulcer

Globally, acute malnutrition triggers more than 50% of childhood mortality in children under 5 years old, which implies that about 3.5 million children die of malnutrition each year. Prior to the advent of ready-to-use therapeutic food (RUTF), the management of acute malnutrition was limited to hospitals, resulting in low coverage rates with high mortality, as malnourished cases were indentified at later stages often plagued with complications. However, current availability of RUTF has enabled malnourished children to be treated at communities. Further, because RUTF is dehydrated and sealed, it has the added advantage of a lower risk of bacterial contamination, thereby prolonging its storage life at room temperature. Recent data indicate that Community Management of Acute Malnutrition (CMAM) is as cost effective as other high-impact public health measures such as oral rehydration therapy for acute diarrheal diseases, vitamin A supplementation, and antibiotic treatment for acute respiratory infections. Despite the high efficacy of CMAM programs, CMAM still draws insufficient attention for global implementation, suggesting that CMAM programs should be integrated into local or regional routine health systems. Knowledge gaps requiring further research include: the definition of practical screening criteria for malnourished children at communities, the need for systematic antibiotic therapy during malnutrition treatment, and the dietary management of severe malnutrition in children below 6 months of age.
Child ; Child Nutrition Disorders ; Developing Countries ; Fluid Therapy ; Humans ; Imidazoles ; Malnutrition ; Mass Screening ; Nitro Compounds ; Public Health ; Respiratory Tract Infections ; Vitamin A

Steroid 21 hydroxylase deficiency is a major cause of congenital adrenal hyperplasia(CAH) and is caused by genetic impairment (CYP21B) of this enzyme. In the human genome, CYP21B is located within MHC class III region on the short arm of chromosome 6. Most of the genes in this region are highly polymorphic and crowded. Also the CYP21B gene is accompanied by its pseudogene (CYP21A) and tandemly arranged with two genes of fourth component of complement. This highly complex gene arrangement in this area may predispose genetic unstability of CYP21 genes,i.e. mutations. In the current study, we tried to investigate the frequency of duplication and deletion of CYP21 genes and pattern of the genetic alteration of these genes by RFLPs. We also compared the genetic alteration of CYP21 in normal subjects with those of the CAH patients. According to our study, 15% of the normal Korean population have duplication or deletion of CYP21. There was one normal subject with heterozygous deletion of CYP21B gene. Of the 5 CAH patients examined, we found abnormal patterns in 2 patients. One was a large scale gene conversion and the other was a deletion of CYP21B and C4 locus II genes with gene conversion. These results suggest that high frequency of duplication and deletion of CYP21 and C4 in the general population may provide the genetic pool of instable CYP21 genes and these duplicated or deleted genes may result in gene conversions between CYP21A(pseudogene) and CYP21B(true gene) by preventing the normal recombination event.
Adrenogenital Syndrome* ; Arm ; Chromosomes, Human, Pair 6 ; Complement System Proteins ; Gene Conversion ; Gene Order ; Genome, Human ; Humans ; Polymorphism, Restriction Fragment Length ; Pseudogenes ; Recombination, Genetic ; Steroid 21-Hydroxylase*