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ABSTRACT: To clarify the clinical features and pathogenesis of intussusception in neonates.
Fourteen neonates were diagnosed with intussusception between June 1974 and January 2001. Patients were divided into two groups according to whether or not signs were present on the first day of life. The clinical features were interrelated with the pathological findings.
All six patients in the group whose signs were present on the first day of life also had intestinal atresia or malrotation. Among patients whose signs began less than 24 h after birth, five of eight patients suffered hypoxia. Moreover, it was very difficult to establish the diagnosis of intussusception, particularly in the group of late-onset type intussusception.
Hypoxic events may play a crucial aetiologic role in the pathogenesis of late-onset neonatal intussusception.
Journal of Paediatrics and Child Health 08/2004; 40(7):388-91. · 1.28 Impact Factor
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M Kage,
T Fujisawa,
K Shiraki,
T Tanaka,
A Kimura,
K Shimamatsu, E Nakashima,
M Kojiro,
M Koike,
Y Tazawa, [......],
A Matsui,
T Hayashi,
T Etou,
S Terasawa,
K Sugiyama,
H Tajiri,
A Yoden,
Y Kajiwara,
M Sata,
Y Uchimura
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ABSTRACT: Limited information is available regarding the histology of hepatitis C virus infection in children. The aim of this study was to determine the histological pattern of chronic hepatitis C (CHC) in children, and liver biopsy specimens from 109 pediatric patients with CHC were examined. Each biopsy specimen was evaluated based on a numerical scoring system for the stage of fibrosis (1-4), the grade of portal/periportal necroinflammation (0-4), the grade of lobular necroinflammation (0-4), and their sum (final grade). The histological lesions considered to be characteristic of chronic hepatitis were also evaluated. None of the children had liver cirrhosis, and 105 cases (97%) were stage 1 or 2. Only 4 children were stage 3. Two of these 4 cases showed hemosiderosis. A significant correlation was observed between the staging score and the final grade in the pediatric patients (r = .59; P < .0001). The histological characteristics of adult CHC, such as lymphoid aggregate, bile duct injury, and fatty changes, were also observed in the children. In conclusion, the majority of children with CHC presented with mild fibrosis, but a few showed CHC with lobular distortion and hemosiderosis. Frequent blood transfusion may aggravate hepatic lesions in pediatric CHC.
Hepatology 12/2003; 26(3):771 - 775. · 11.66 Impact Factor
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Y Yamato,
A Kimura,
T Murai,
T Yoshimura,
T Kurosawa,
S Terazawa,
A Takao,
K Maeda, E Nakashima,
Y Yamashita,
H Kato
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ABSTRACT: The aim of this study was to evaluate the effects of bile acid treatment and to obtain further information about the pathway of bile acid biosynthesis in a patient with 3beta-hydroxy-delta5-C27-steroid dehydrogenase/isomerase (3beta-HSD) deficiency by gas chromatography-mass spectrometry. Results showed that at 2 months of age, 3beta-hydroxy-5-cholen-24-oic acid (3.0 micromol/mmol Cr, 7.9%) was detected in the urine in essentially the same relative amount as 3beta,7alpha-dihydroxy- and 3beta,7alpha,12alpha-trihydroxy-5-cholen-24-oic acids (3.7 micromol/mmol Cr, 9.8%) during ursodeoxycholic acid treatment combined with prednisolone. As a result, diagnosis was delayed until 18 months of age. One month later with substitution of chenodeoxycholic acid treatment, urinary 3beta,7alpha-dihydroxy- and 3beta,7alpha,12alpha-trihydroxy-5-cholen-24-oic acids decreased significantly, and subsequent improvement of liver dysfunction was accelerated. Chenodeoxycholic acid treatment is useful in 3beta-HSD deficiency. However, in the diagnosis of this disease in early life, it should be noted that the acidic pathway may be the major route for bile acid biosynthesis in the neonatal period. Diagnosis of 3beta-HSD deficiency may have been delayed by administration of ursodeoxycholic acid, resulting in prolonged diagnostic investigation in this child with cholestasis. Further, use of prednisolone may have been contraindicated.
Journal of Paediatrics and Child Health 11/2001; 37(5):516-9. · 1.28 Impact Factor
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ABSTRACT: To investigate the role of placental transport of bile acids in fetal bile acid metabolism, such as with regard to synthesis of the unusual bile acids (1 beta- and 6 alpha-hydroxylated and unsaturated bile acids), we measured the concentrations of bile acids in umbilical cord blood, amniotic fluid, maternal serum and maternal urine at delivery by means of gas chromatography-mass spectrometry. Serum and urine from healthy nonpregnant women were used as controls. We detected large amounts of unusual bile acids, especially hyocholic acid and 3 beta-hydroxy-delta 5 bile acids, in amniotic fluid and umbilical cord blood. The concentration of total bile acids in maternal serum was less than that of control serum and umbilical cord blood, and the concentration of total bile acids in maternal urine was higher than that of control urine and amniotic fluid. In conclusion, the fetus synthesized large amounts of unusual bile acids, and these compounds were transported from fetus to mother by placental transfer. We suggest that pregnant women may excrete large amounts of bile acids into the urine to control serum concentration of bile acids in fetus.
The Kurume Medical Journal 02/2001; 48(2):87-91.
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ABSTRACT: Ursodeoxycholic acid absorption in the proximal intestine may be impaired in patients with inflammatory bowel disease.
We examined the intestinal absorption of ursodeoxycholic acid by the oral ursodeoxycholic acid tolerance test in 19 children and adolescents with inflammatory bowel disease at various stages, including 8 patients with unoperated Crohn's disease, 3 patients with ileal-resected Crohn's disease, 8 with ulcerative colitis, and 8 healthy control subjects.
Ursodeoxycholic acid malabsorption was present in all patients with unoperated Crohn's disease in the first diagnosed active stage, in 3 of 5 patients in a relapsing active stage, and in 2 of 8 patients in remission. Ursodeoxycholic acid absorption was significantly lower in patients in the first diagnosed active stage than in the healthy controls (p < 0.01) or in patients in remission (p < 0.01). There was no significant difference between healthy controls and the patients in a relapsing active stage or in remission. Ursodeoxycholic acid absorption was abnormal during the first postoperative month in patients with ileal-resected Crohn's disease, but normalized over time. Malabsorption of ursodeoxycholic acid was not observed in any patients with ulcerative colitis.
These findings suggest that absorption of ursodeoxycholic acid in the proximal intestine is impaired in patients with Crohn's disease and that the oral ursodeoxycholic acid tolerance test is a convenient and useful means of evaluating the absorption of bile acid in the proximal intestine in pediatric patients with ileal or ileocolic Crohn's disease.
Journal of Pediatric Gastroenterology and Nutrition 03/1998; 26(3):279-85. · 2.30 Impact Factor
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A Kimura,
M Suzuki,
T Murai,
T Kurosawa,
M Tohma,
M Sata,
T Inoue,
A Hoshiyama, E Nakashima,
Y Yamashita,
T Fujisawa,
H Kato
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ABSTRACT: Urinary 3-oxo-delta4 bile acids have been detected in infants who ultimately died of liver disease. We used qualitative and quantitative methods to compare urinary 3-oxo-delta4 bile acids in liver disease, determining their composition and evaluating the prognostic implication in patients of various ages with various liver diseases.
Gas chromatography-mass spectrometry was used to measure 3-oxo-delta4 bile acids in the urine of patients and healthy controls.
Patients with a deficiency of 3-oxo-delta4-steroid 5beta-reductase and acute hepatic failure exhibited a significantly higher percentage of 3-oxo-delta4 bile acids in total bile acids in urine than the healthy controls or other patient groups, including those with neonatal cholestasis or biliary atresia (p<0.0001). The urinary 3-oxo-delta4 bile acids in patients with 3-oxo-delta4-steroid 5beta-reductase deficiency who had a poor prognosis were mainly 7alpha-hydroxy-3-oxochol-4-en-24-oic acid and 3-oxochola-4,6-dien-24-oic acid.
Our results indicate that an increase in the 7alpha-hydroxy-3-oxochol-4-en-24-oic acid and 3-oxochola-4,6-dien-24-oic acid in the urine of patients with hepatobiliary disease indicates a poor prognosis.
Journal of Hepatology 02/1998; 28(2):270-9. · 9.26 Impact Factor
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M Kage,
T Fujisawa,
K Shiraki,
T Tanaka,
A Kimura,
K Shimamatsu, E Nakashima,
M Kojiro,
M Koike,
Y Tazawa, [......],
A Matsui,
T Hayashi,
T Etou,
S Terasawa,
K Sugiyama,
H Tajiri,
A Yoden,
Y Kajiwara,
M Sata,
Y Uchimura
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[hide abstract]
ABSTRACT: Limited information is available regarding the histology of hepatitis C virus infection in children. The aim of this study was to determine the histological pattern of chronic hepatitis C (CHC) in children, and liver biopsy specimens from 109 pediatric patients with CHC were examined. Each biopsy specimen was evaluated based on a numerical scoring system for the stage of fibrosis (1-4), the grade of portal/periportal necroinflammation (0-4), the grade of lobular necroinflammation (0-4), and their sum (final grade). The histological lesions considered to be characteristic of chronic hepatitis were also evaluated. None of the children had liver cirrhosis, and 105 cases (97%) were stage 1 or 2. Only 4 children were stage 3. Two of these 4 cases showed hemosiderosis. A significant correlation was observed between the staging score and the final grade in the pediatric patients (r = .59; P < .0001). The histological characteristics of adult CHC, such as lymphoid aggregate, bile duct injury, and fatty changes, were also observed in the children. In conclusion, the majority of children with CHC presented with mild fibrosis, but a few showed CHC with lobular distortion and hemosiderosis. Frequent blood transfusion may aggravate hepatic lesions in pediatric CHC.
Hepatology 09/1997; 26(3):771-5. · 11.66 Impact Factor
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M Kage,
S Ogasawara,
K Kosai, E Nakashima,
K Shimamatsu,
M Kojiro,
A Kimura,
T Fujisawa,
Y Matsukuma,
Y Ito,
S Kondo,
K Kawano,
M Sata
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ABSTRACT: In order to examine whether saliva and breast-milk are mediators of the vertical transmission of hepatitis C virus (HCV) from an HCV carrier mother, serum, saliva, and breast-milk samples from 11 HCV carrier mothers were collected at the time of delivery, and at approximately 1- to 3-months intervals for as long as 30 months postpartum. Serum was also sampled from their children. All samples were analysed for the presence of HCV RNA, using the nested polymerase chain reaction method. No HCV RNA was detected in any breast-milk samples. In saliva, HCV RNA was detected in four of the 11 mothers (36%). These four mothers also had liver function abnormalities. Hepatitis C virus RNA was not detected in any serum samples of the children, and all children had normal liver function. The children were monitored for periods from 2 to 44 months. During this period, there was no evidence of virus transmission. Breast-milk is not likely to be a source of mother-to-child transmission of HCV. Maternal saliva may harbour HCV, but it may not result in infant infection.
Journal of Gastroenterology and Hepatology 08/1997; 12(7):518-21. · 2.87 Impact Factor
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ABSTRACT: To clarify the characteristics of fibrosis developed in the process from chronic hepatitis C to cirrhosis, a morphometric analysis of liver biopsy samples was conducted on 25 chronic hepatitis C patients and 20 chronic hepatitis B patients (controls). Hepatitis C patients were followed up for 3 to 23 years. The mean number of liver biopsies performed on these patients was 3.8. Each biopsy was evaluated for the degree of fibrosis by using two methods: a semiquantitative method with a staging scoring system, and morphometry using a computed image analysis system. A significant correlation was observed between the Stage and the area of fibrosis (AF = the ratio of the area of fibrosis to that of the entire tissue specimen). The AF in cirrhosis was significantly higher in hepatitis C patients than in hepatitis B patients. The ratio of AF in the last biopsy sample to AF in the initial biopsy sample was significantly higher in hepatitis C patients than in hepatitis B patients. Evolution from chronic hepatitis C to cirrhosis occurred more frequently in patients aged > or = 50 years, and this time period was 1.8 times shorter than that in patients aged < 50 years. AF in the initial biopsy related significantly to the period of evolution from chronic hepatitis C to cirrhosis. AF in the initial biopsy might be a predictive factor for prognosis.
Hepatology 05/1997; 25(4):1028-31. · 11.66 Impact Factor
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ABSTRACT: Two Japanese brothers with neonatal cholestasis associated with pigment granules in the hepatocytes and hepatosteatosis were evaluated for the possible role of hepatosteatosis in the Dubin-Johnson syndrome.
The morphology of pigment accumulation and the laboratory data in these cases were examined. The elevation of urinary coproporphrin isomer I to more than 90% and the presence cholestasis resembled that in the Dubin-Johnson syndrome, but the hypertriglyceridaemia ( > 1.13 mmol/L as triolein) and the hepatosteatosis differed. Both infants were thought to have familial hypertriglyceridaemia. However, this diagnosis was difficult to confirm in the absence of data on the normal values of apolipoprotein and lipoprotein isomer for infants.
A neonatal variant of the Dubin-Johnson syndrome may account for the unusual findings in these infants.
Journal of Paediatrics and Child Health 01/1996; 31(6):557-60. · 1.28 Impact Factor
