ISSN:0277-2116    

出版商:OVID    

年代:1988

数据来源: OVID

ISSN:0277-2116    

出版商:OVID    

年代:1988

数据来源: OVID

摘要:

A total of 87 cases of chronic intestinal pseudo-obstruction were identified as fitting the criteria for the syndrome. Five families, three with autosomal dominant inheritance, accounted for 15 cases. The remainder were single cases in families, indicating either autosomal recessive inheritance, spontaneous mutation, or acquired disease. Of the 87 patients, 47 were male; 19 patients were symptomatic at birth and 37 (43%) within the first month of life; 64% were diagnosed by the first year of age and the remainder were diagnosed by 18 years of age. Patients currently range in age from 3 months to 24 years. Abdominal distension in 70, vomiting in 50, and constipation in 50 of the 87 cases were the three commonest presenting symptoms. Diarrhea and failure to thrive were present in 20 cases. Urinary tract infection and failure to void were present in 10 cases. Diagnosis was established by clinical presentation, radiographic studies. and at exploratory laparotomy. Esophageal motility was abnormal in 14 patients. Anal manometric studies were done in 16 and snowed normal recto-anal inhibitory reflex in all but one. Only 34 patients had biopsy studies as part of their evaluation and, of these, only 12 were full-thickness. Abnormal plexuses were found in eight and degeneration of smooth muscles in four. There was little or no benefit from the use of any medication to promote motility. One patient with intractable pseudo-obstruction benefited from a subtotal enterectomy. Of those patients not lost to follow-up, 31.4% died; 43% of these deaths occurred within the first 6 months from complications of total parenteral nutrition. One infant had a complete spontaneous remission by 1 year of age, with normalization of radiographic findings; another had partial remission by 6 months of age.

ISSN:0277-2116    

出版商:OVID    

年代:1988

数据来源: OVID

摘要:

A simple, noninvasive test is proposed to measure the activity of the hepatocellular monooxygenase system in vivo. Elimination rates of the stable isotope15N are measured in urine after an oral dose of the15N-labeled phenacetin homologue [15N]methacetin. Different forms of obtaining and expressing results are investigated in order to determine optimum methodology and differentiation for clinieal application in pediatrics. The best dis criminating power and reliability of results are seen with the measurement of15N elimination half-life. For follow-up, a more easily measured parameter eliminated dose over 9 h is sufficient. In the latter case, only total nitrogen and15N contents of a collective urine sample are measured.

ISSN:0277-2116    

出版商:OVID    

年代:1988

数据来源: OVID

摘要:

In children with biliary atrcsia, defective intestinal absorption of vitamin D and impaired hepatic uptake and 25-hydroxylation of vitamin D lead to a deficiency of vitamin D and rickets. We recently observed severe rickets in a 3-year-old boy with corrected biliary atrcsia resulting in jaundice, despite oral treatment with 1α-hydroxyvitamin D, (1α-OHD,) or 1,25-dihydroxyvitamin D, [1,25-(OH)2D3]. He had low 25-hydroxyvitamin D (25-OHD) and high 1.25-(OH)2D serum levels. Intramuscular vitamin D2administration produced radiological and biochemical evidence of recovery. Oral 1.25-(OH)2D3(O.1 μg/kg) and 25-OHD3(10 μg/kg) tolerance tests were done to assess the ability to absorb vitamin D and the effectiveness of using these drugs orally. Eleven children with corrected biliary atresia, aged 9 months to 7 years, were studied. In oral 1.25-(OH)2D3tolerance tests, the increments above the baseline serum levels of 1.25-(OH)2D were 140.7 ± 27.4 pg/ml in non-jaundiced patients (n = 5). In jaundiced patients (n - 3). 1,25-(OH)2D3absorption in two patients with high basal 1.25–(OH)2D values was lower than that of nonjaundiced patients: however, the absorption in the third patient with a low basal value was similar to that of nonjaundiced patients. In oral 25-OHD3tolerance tests, the mean increase of serum 25-OHD was 48.9 ± 30.6 ng/ml in non-jaundiced patients (n = 5) and 23.7 ± 9.5 ng/ml in jaundiced patients (n - 4), the peak serum 25-OHD levels being reached 6–12 h after 25-OHD3loading. The increase of serum 25-OHD in jaundiced patients tended to be less than that in nonjaundiced patients at 4 hours after 25-OHD3administration (15.1 ± 12.6 vs 48.9 ± 30.6 ng/ml), but the difference was not significant. In addition, oral treatment with 25-OHD, (4 μg/kg/day) for 3 months resulted in marked increases in the serum 25-OHD level and hone density in the jaundiced patients (n = 3).

ISSN:0277-2116    

出版商:OVID    

年代:1988

数据来源: OVID

摘要:

Direct visualization of the extrahepatie biliary tree is difficult in infants and young children without resorting to an intraoperative cholangiogrum. Small size and lack of dilated intrahepatic ducts especially in infants with cholestatic jaundice often preclude using the techniques of percutaneous transhcpatic cholangiography or endoscopic retrograde cholangiopancreatography. We studied the feasibility of ultrasound-guided percutaneous Cholecystocholangiography (US-guided PCC) in differentiating extrahepatie from intrahepatic causes of cholestatic jaundice in four infants. Clinical, laboratory, radiographic, and histologic criteria had not conclusively excluded extrahepatie obstruction prior to the use of this technique. Four infants, between 4 and 10 weeks of age. were studied without complications. In one patient, biliary atresia was diagnosed at 4 weeks of age; in another, aspirated bile was cultured leading to a specific diagnosis of bacterial cholangitis; and, in two others, intrahepatic cholestasis was confirmed by the demonstrated patency of the extrahepatie biliary tree. In infants and young children in whom a gallbladder lumen can be seen, US-guided PCC offers a potential rapid, nonoperative means of differentiating intrahepatic and extrahepatie causes of cholestatic jaundice, defining the anatomy of the extrahepatie biliary tree, and directly sampling gallbladder bile.

ISSN:0277-2116    

出版商:OVID    

年代:1988

数据来源: OVID

摘要:

The histopathologic abnormalities in endoscopic biopsies from the stomach and duodenal bulb were correlated with the visual findings of upper gastrointestinal endoscopy in 94 children (mean age, 8.4 years; 51 boys and 43 girls) with a variety of complaints. Histology was graded by observers blinded to the endoscopic findings, and both endoscopy and histology were graded using scales reflecting increasing severity with increasing grade. In all three locations studied (gastric body, antrum, and duodenal bulb), endoscopic grade was significantly higher than the histologic grade. Correlation was especially poor with mild endoscopic findings such as erythema and granularity/nodularity, which had little predictive value for histologic inflammation. Few patients had severe disease with eight of 94 having ulcer by endoscopy and seven of 94 having greater than grade 2 histologic disease in any location. We conclude that endoscopy without biopsy should not be used to diagnose gastroduodenal inflammation in pediatric patients.

ISSN:0277-2116    

出版商:OVID    

年代:1988

数据来源: OVID

摘要:

In order to compare three premeditation regimens, 58 children and adolescents were randomized to three groups: Group I, meperidine intramuscular; Group II, atropine intramuscular and diazepam intravenous; and Group III, meperidine, promethazine, and chlorpromazine intramuscular. Almost all patients required supplemental intravenous diazepam or meperidine to obtain adequate sedation. An endoscopist without knowledge of the medication group scored each patient for adequacy of sedation. Sedation was more effective in Group III than in Groups I and II (p < 0.005); however. Group III patients were less arousable at completion of the procedure (p < 0.0005) and had a greater duration of sedation (220 min compared with 56 and 88 min for Groups I and II, respectively, p < 0.001). Complications were minor and not associated with any specific treatment. The evidence from this study supports the following conclusions: (a) adequate sedation for esophagogastroduodenoscopy in pediatric patients requires sedation with more than one of the drugs used in this study; and (b) the regimen meperidine, promethazine, and chlorpromazine, with diazepam supplement, was superior in providing sedation, but patients required a longer period of recovery than did patients sedated with one of the other regimens.

ISSN:0277-2116    

出版商:OVID    

年代:1988

数据来源: OVID

摘要:

The existence of a significant linear relationship between the concentration of chlorides and the activity of pepsinogen (PG) in the urine was ascertained in the case of full-term infants 3 days to 6 weeks of age. At the age of 4–6 weeks, a significant relationship was found between the urinary pepsinogen activity and the urinary creatinine concentration, and between the urinary pepsinogen activity in the urine and the urine osmolality. Immediately after birth, the Pg7 fraction of PG II in the urine was found in all cases and, at the age of 4–6 weeks, in 11% of cases. In regard to the time factor, the conspicuous drop in the occurrence of the Pg7 fraction corresponds to the new qualitative relationship between the pepsinogen activity in the urine and the creatinine concentration in the urine and between the former and the urine osmolality. In premature infants, the Pg7 fraction disappears more slowly. The spectrum of pepsinogens in the urine was examined in children suffering from various diseases. In a girl with lymphoma, we found the Pg7 fraction, but this finding was temporary only.

ISSN:0277-2116    

出版商:OVID    

年代:1988

数据来源: OVID

摘要:

Endoscopic laser therapy may be useful in controlling upper gastrointestinal bleeding due to vascular abnormalities. This report presents the case of an 11-year-old boy with an unusual vasculocutaneous disorder complicated by chronic recurrent bleeding that necessitated frequent blood transfusions. Of a number of investigative tests, only endoscopy was diagnostic in determining that the bleeding was secondary to multiple gastric vascular lesions. Moreover, laser photocoagulation of the identified lesions resulted in a dramatic decrease in both transfusion requirements and hospitalization.

ISSN:0277-2116    

出版商:OVID    

年代:1988

数据来源: OVID