Lymph node enlargement as a sign of acute hepatitis A in children

Abdominal ultrasound was performed in 58 children presenting with proven acute hepatitis A and in 63 controls of the same age. There are well-known echographic signs of hepatitis (liver enlargement, gallbladder wall thickening, periportal hyperechogenicity) but they were not constantly found. We describe in all the hepatitis cases an enlargement of lymph nodes located in the hepatic hilum, pancreatic area and small omentum: they appeared hyperechogenic at the centre with hypoechogenic outer layer. Such enlarged lymph nodes were not observed in the controls.Presented at the ESPR meeting in Dublin 1989. Selected for publication by an International Group of the ESPR     

不同年龄胆总管囊肿的临床与肝纤维化特点探讨

目的 探讨不同年龄胆总管囊肿的临床与肝脏纤维化特点与相互关系。方法 分析12例婴儿及36例幼儿、儿童胆总管囊肿的临床资料;肝组织HE染色观察纤维化程度和炎性细胞浸润情况,免疫组化染色观察细胞角化蛋白(AE1/AE3)、人类组织相容性抗原DR(HLA—DR)表达,并与18例胆道闭锁作对照。结果 婴儿胆总管囊肿黄疸出现率12/12,显著高于幼儿儿童组8/36(P<0.01);肝纤维化程度、小叶周边AE1/AE3阳性细胞和HLA—DR在肝脏表达显著高于非婴儿组(P<0.01),低于胆道闭锁组(P<0.01)。肝纤维化程度与黄疸及肝小叶周边AE1/AE3表达分级呈正相关(P<0.05)。结论 婴儿胆总管囊肿以黄疸为主要临床表现,其肝纤维化重于幼儿及儿童组,胆道梗阻和胆小管增生可能是其发生肝纤维化的原因。     

Significance of periportal low-attenuation zones following blunt trauma in children

The CT scans of 400 consecutive children evaluated with CT following blunt abdominal trauma were evaluated to determine the frequency of periportal lowattenuation zones, assess patterns of associated intraabdominal injury, and examine clinical outcome. Periportal low-attenuation zones were noted in 60 children (15%). The presence of these zones was associated with a significantly higher incidence of intraabdominal injury (60% versus 11%,p=0.0001). Injuries most frequently associated with zones of periportal low-attenuation included hepatic (n=23,38%), and adrenal (n=14,23%). Children who had periportal low-attenuation zones tended to be more physiologically unstable as evidenced by a lower Trauma Score (diffuse, 11.9; focal, 13.4) than children without the zones (15.1,p=0.0001). The presence of these zones was also associated with a significantly higher mortality rate (13% versus 1%,p=0.0001). Ten children who had periportal low-attenuation zones and no hepatic injury on CT had a normal appearing liver on gross inspection at surgery or autopsy. In conclusion, periportal lowattenuation zones are common in children who have hepatic injury. These zones may be seen in conjunction with non-hepatic visceral injury or in the absence of intraabdominal injury. The presence of zones of periportal lowattenuation is associated with a higher index of physiologic instability, and higher mortality.     

Ultrasonography of the pancreas,liver, and biliary system in cystic fibrosis.

Abdominal ultrasound imaging was performed in 50 children. Thirty nine were proved to have cystic fibrosis while 11 had respiratory infections and thus acted as controls. The pancreas was abnormal in 75% of cystic fibrosis patients aged under 5 years and in 95% over 5 years. In cystic fibrosis patients over 5 years old who had malabsorption, the pancreas was abnormal in 100%. Abnormalities of the liver parenchyma were found in 23%, of the gallbladder in 24%, and splenomegaly in 8% of the cystic fibrosis patients.     

Liver abnormalities and post‐transplant survival in pediatric Fontan patients

The impact of liver parenchymal abnormalities on survival post‐heart transplant remains unknown in pediatric Fontan patients. We assessed pediatric Fontan patients who underwent heart transplant and had documented pretransplant hepatic ultrasound (U/S) studies. Liver U/S findings were classified as normal (Group 1), mildly abnormal (Group 2, hepatomegaly/vascular congestion), or severely abnormal (Group 3, heterogeneous echotexture/nodularity). Among 30 study patients, 8 were classified as Group 1, 14 as Group 2, while 8 met Group 3 criteria. Pretransplant liver biochemistry and synthetic function were similar in all groups. Six Group 3 patients underwent liver biopsy; 4 demonstrated perisinusoidal or centrilobular fibrosis, and 2 had cirrhosis. Overall mortality was 30% (n = 9). Median follow‐up was 5 years (range, 0.25‐13 years). One‐year survival was similar among all 3 groups (P = .37), with a trend toward higher cumulative 5‐year survival in Group 1 (100%). The majority of pediatric Fontan patients who underwent heart transplant demonstrated abnormal preoperative liver ultrasound findings. Heterogeneous echotexture or nodularity detected on U/S frequently indicates underlying liver parenchymal abnormalities. The presence of severe liver abnormalities was not associated with higher early mortality post‐heart transplant in pediatric Fontan patients; however, late outcomes must be further elucidated.     

Biliary atresia: making the diagnosis by the gallbladder ghost triad

OBJECTIVE: To describe the gallbladder ghost triad and evaluate its usefulness in the sonographic diagnosis of extrahepatic biliary atresia (BA). METHODS: From October 1997 to February 2002, 217 fasted infants with cholestatic jaundice aged 2-12 weeks were examined sonographically. We defined the gallbladder ghost triad as gallbladder length <1.9 cm, lack of smooth/complete echogenic mucosal lining with an indistinct wall and irregular/lobular contour, and used it as a criteria for BA. Gallbladder wall thickness, triangular cord, diffuse periportal echogenicity and hepatic artery calibre were also recorded. Diagnosis of BA was confirmed surgically and histologically. RESULTS: Thirty of 31 babies with BA demonstrated the gallbladder ghost triad. No false-positives were recorded. The 31st BA baby showed a normal gallbladder at 6 weeks, but developed the ghost triad at 8 weeks. Gallbladder wall thickening was seen in 46/186 non-BA babies, but not in BA. Triangular cord was observed in 24/31 babies. Twenty-two of 186 non-BA babies and 5/31 BA babies showed diffuse periportal echogenicity. The hepatic artery appeared more prominent in BA. All 31 babies diagnosed sonographically as BA had surgery. Three non-BA babies had "negative" laparotomies showing hypoplastic bile ducts. CONCLUSIONS: The gallbladder ghost triad is a very accurate sign of BA. Indeterminate cases require close follow-up.     

Gastrointestinal and other clinical manifestations in 17 children with congenital disorders of glycosylation type Ia, Ib, and Ic

OBJECTIVES: The typical signs and symptoms of congenital disorders of glycosylation (CDG) include dysmorphy, failure to thrive, and neurologic abnormalities. However, more and more children diagnosed at a young age are not dysmorphic and do not have neurologic involvement. The authors studied the gastrointestinal and other clinical manifestations of CDG type Ia, Ib, and Ic. METHODS: As of January 2003, 17 children were identified with CDG at the authors' institution. The medical records of the patients were reviewed. RESULTS: Five children had CDG Ia, three children CDG Ib, and nine children CDG Ic. Age at diagnosis ranged from 2 months to 15 years. Failure to thrive was present in 80% of patients with CDG Ia, in 66% of those with CDG Ib, and in 11% of those with CDG Ic. Five children had protein-losing enteropathy (two CDG Ia, two CDG Ib, and one CDG Ic). Hepatomegaly was present in 40% of patients with CDG Ia, in 66% of those with CDG Ib, and in 11% of those with CDG Ic. In CDG Ic, hepatomegaly was transient. In CDG Ia, histologic analysis of the liver showed swollen hepatocytes, steatosis, and fibrosis. In CDG Ib, hamartomatous collections of bile ducts were seen. In one patient with CDG Ib, the clinical picture was restricted to congenital hepatic fibrosis for more than a decade. CONCLUSIONS: The study confirms the heterogeneity of the clinical picture in children with CDG type Ia, Ib, and Ic. Children with protein-losing enteropathy should be tested for CDG. Protein-losing enteropathy can be caused, not only by CDG Ia and Ib, but also by type Ic. Children with congenital hepatic fibrosis should be tested for CDG, even in the absence of other symptoms. In CDG Ib, histologic analysis of the liver showed hamartomatous collections of bile ducts (Meyenburg complex).     

13C and H2 breath tests to study extent and site of starch digestion in children with cystic fibrosis.

BACKGROUND: Starch is an important source of energy for children with cystic fibrosis, but little is known about their capacity to digest it. METHODS: A 13C breath test was used to measure starch digestion and oxidation in 16 children with cystic fibrosis (median [range] age, 7.9 [4-15] years; 7 girls, 9 boys) and 5 normal healthy control subjects (median age, 8.3 [7-13] years; 3 girls, 2 boys). A test meal of 13C flour and lactulose was consumed and breath samples were obtained half-hourly thereafter for 6 hours to measure 13C enrichment by isotope ratio mass spectrometry and H2 by electrochemistry. The test was repeated on 10 children with cystic fibrosis when they were taking pancreatic supplements. RESULTS: The median (range) cumulative percentage 13C dose recovery (cPDR), was 35% (18-52%) in control subjects, 18% (9-33%) in children with cystic fibrosis without enzymes, and 29% (22-51%) in those with pancreatic supplements. cPDR differed significantly between healthy control subjects and children with cystic fibrosis without enzymes (p = 0.01) and between children with cystic fibrosis with and without enzymes (p < 0.0001), but there was no difference between control subjects and children with cystic fibrosis taking enzymes (p = 0.5). Eight children with cystic fibrosis had a cPDR within control range, and in six there was a second peak in 13CO2 enrichment coincident with an increase in H2. CONCLUSIONS: Starch digestion and oxidation are diminished in children with cystic fibrosis, but pancreatic enzymes restored them to near normal levels. A second peak in 13CO2 enrichment, suggestive of colonic starch fermentation was absent in healthy children, but present in some children with cystic fibrosis and abolished by pancreatic enzymes.     

Portal vein measurement by ultrasonography in patients with long-standing cystic fibrosis: preliminary observations

Adult cystic fibrosis (CF) patients are increasingly diagnosed with hepatic cirrhosis, cholecystitis, and cholelithiasis. A continuing diagnostic problem is the early detection of cirrhosis prior to diffuse liver involvement. Sonography has been used in evaluating the pancreas, gallbladder, liver, and spleen in cystic fibrosis patients. We used a real-time mechanical sector scanner to study the portal veins in adult CF patients randomly selected from our CF population and correlated the portal vein diameters with liver function studies. A measurement greater than 12 mm was interpreted as a probable sign of portal hypertension. Of 21 patients studied, 14 had portal veins that measured greater than 12 mm, and 12 patients had (although not necessarily at the time of the exam) elevated serum alkaline phosphatase levels. Three patients had associated splenomegaly and thrombocytopenia, and 10 patients also exhibited cholelithiasis and abnormal gallbladders on sonography. Initial results indicate that portal vein measurement may be a sensitive indicator of early portal hypertension. Sonography may, thus, isolate patients at high risk for possible future complications. Serial sonograms can be performed easily in evaluating the progress of liver disease.     

Low levels of pyridoxal 5'-phosphate in patients with cystic fibrosis

To determine the effect of cystic fibrosis on the regulation of plasma pyridoxal 5'-phosphate (PLP), the biologically active form of vitamin B6, we measured this compound in plasma from 56 patients with cystic fibrosis. The concentration of PLP in plasma was assayed by a radioenzymatic technique. The results of this study showed that PLP concentration was decreased significantly (6.44 +/- 5.20 ng/mL, mean +/- SD; median 4.45 ng/mL) in patients with cystic fibrosis as compared with a group of hospitalized children with neither cystic fibrosis nor hepatic disease serving as a control group (13.2 +/- 5.04 ng/mL, mean +/- SD; median 12.5 ng/mL). Additionally, 25% of the population with cystic fibrosis exhibited exceedingly low plasma PLP level (less than 2.75 ng/mL). In patients with cystic fibrosis, significant inverse linear associations were found between plasma PLP and serum levels of SGOT and SGPT (PLP v SGOT: r = -.60, P less than .03; PLP v SGPT: r = -.50, P less than .03). This study demonstrated that a deficiency of plasma PLP is a common abnormality in cystic fibrosis and that the low PLP level may be a reflection of impaired vitamin B6 metabolism associated with this disorder.     

Aetiology of chronic suppurative lung disease

Forty one (1%) of 4000 children referred for respiratory disease had chronic suppurative lung disease not due to cystic fibrosis. Further investigations showed congenital malformations in six (15%), primary ciliary dyskinesia syndrome in seven (17%), 11 had immunological abnormalities (27%), and two bronchiectasis due to aspiration (5%). Therefore the underlying cause for the disease was found in 63%. Identification of predisposing causes may facilitate prevention of further bronchial damage.     

Aetiology of chronic suppurative lung disease.

Forty one (1%) of 4000 children referred for respiratory disease had chronic suppurative lung disease not due to cystic fibrosis. Further investigations showed congenital malformations in six (15%), primary ciliary dyskinesia syndrome in seven (17%), 11 had immunological abnormalities (27%), and two bronchiectasis due to aspiration (5%). Therefore the underlying cause for the disease was found in 63%. Identification of predisposing causes may facilitate prevention of further bronchial damage.     

Sonographic prediction of chronic lung disease in the premature undergoing mechanical ventilation

The aims of the study are to investigate the possible role of ultrasound (US) of the chest in predicting the development of chronic lung disease (CLD) in patients with hyaline membrane disease (HMD) and to determine the optimal age for the sonographic examination. One hundred and five consecutive prematures undergoing mechanical ventilation were prospectively studied by US of the chest. The US examinations were performed at birth and at least once a week until discharge from the neonatal unit. The sonographic patterns observed behind the diaphragm and their evolutions were recorded and correlated with the clinical and radiological data at day 28, which corresponds to the currently accepted limit for determining the presence of CLD. CLD is currently defined as oxygen dependency on day 28 with radiographic abnormalities. A diffuse retrodiaphragmatic hyperechogenicity was observed in all the patients with HMD. The hyperechogenicity resolved completely in patients with an uncomplicated clinical evolution. In contrast, in patients with CLD the hyperechogenicity resolved only partially, resulting in less diffuse and less extensive hyperechogenicity. Day 18 was the earliest day where the persistence of the abnormal retrodiaphragmatic hyperechogenicity was observed in 100 % of the patients presenting CLD at day 28. At that time, 95.2 % of the patients without abnormal hyperechogenicity showed uncomplicated evolution and no CLD. US can be a useful diagnostic tool to determine the occurrence of CLD and to predict as early as day 18 the prematures at risk for the disease.     

Weight and menstrual function in patients with eating disorders and cystic fibrosis

Many patients with eating disorders have menstrual dysfunction. In patients with anorexia nervosa, amenorrhea has been linked to weight loss. However, many patients with bulimia nervosa, even those of average or greater than average weight, also experience menstrual abnormalities, including amenorrhea. It was hypothesized that low weight per se is not responsible for menstrual dysfunction in patients with eating disorders. First, 16 patients with bulimia nervosa of average weight and 29 patients with cystic fibrosis of below average weight of similar menstrual age were compared. Of the patients with bulimia nervosa, 11 (73%) had had secondary amenorrhea at some time compared with 8 (28%) of the patients with cystic fibrosis (P less than .01). At the time of study, 6 (40%) of the patients with bulimia nervosa and 21 (78%) of the patients with cystic fibrosis were having regular cycles (P less than .01). Next 18 patients with anorexia nervosa were compared with 18 patients with cystic fibrosis matched for weight and menstrual age. Although 18 (100%) of the patients with anorexia nervosa had had secondary amenorrhea, only 6 (33%) of the patients with cystic fibrosis had amenorrhea. Although only 1 (6%) of the anorexia nervosa group was currently having regular cycles, 14 (78%) of the patients with cystic fibrosis were (P less than .0001). Closer approximation to ideal body weight was associated with regular menses only in the cystic fibrosis group. Exercise did not appear related to regularity of menstruation in any group. Body weight is not the major factor responsible for menstrual abnormalities in patients with eating disorders.     

朗格汉斯组织细胞增生症儿童肝脏受累的CT表现

目的：探讨朗格汉斯组织细胞增生症(LCH)儿童肝脏受累的CT表现。方法：回顾性分析9例经临床、实验室、病理检查确诊为LCH肝脏受累患儿的CT表现,其中男6例,女3例,全部病例均行肝脏CT平扫及动脉期、门脉期双期增强CT扫描。结果：9例患儿中, 主要CT表现为肝大（8例）;门静脉周围树枝状低密度灶及“门脉晕征”（7例）,增强后动脉期呈轻度至中度强化;肝内胆管扩张（5例）;肝门区及腹膜后淋巴结肿大（4例）;弥漫性低密度小结节灶（3例）,增强后呈环状强化。结论：LCH肝脏受累患儿CT表现具有一定的特点,了解这些特点有助于该病的早期诊断和治疗。     

Energy content of stools in normal healthy controls and patients with cystic fibrosis.

Stool energy losses and the sources of energy within the stool were determined in 20 healthy controls and 20 patients with cystic fibrosis while on their habitual pancreatic enzyme replacement treatment. Stool energy losses were equivalent to 3.5% of gross energy intake in healthy children (range 1.3-5.8%). Despite a comparable gross energy intake, stool energy losses were three times greater in patients with cystic fibrosis than controls averaging 10.6% of gross energy intake (range 4.9-19.7%). Stool lipid could account for only 29% and 41% of the energy within the stool in controls and patients with cystic fibrosis respectively and was poorly related to stool energy. Approximately 30% of the energy within the stool could be attributable to colonic bacteria in both the healthy children and patients with cystic fibrosis. These results suggest that stool energy losses in healthy children are relatively modest but that even when patients with cystic fibrosis are symptomatically well controlled on pancreatic enzyme replacement, raised stool energy losses may continue to contribute towards an energy deficit sufficient to limit growth in cystic fibrosis. As the energy content per gram wet weight remains relatively constant (8 kJ/g), stool energy losses may be estimated from simple measurements of stool wet weight.     

Immune responses to liver membrane antigens in patients with cystic fibrosis and liver disease

Biliary obstruction by viscid mucus, although important, may not be the only factor for the development of liver disease in some patients with cystic fibrosis. In the present study the relationship between immune responses to liver antigens and the presence of liver damage was investigated using the leucocyte migration test and lymphocyte cytotoxicity to isolated rabbit hepatocytes. Inhibition of leucocyte migration by purified liver-specific lipoprotein, derived from hepatocyte plasma membrane, was found in 9 of 11 children with liver disease, but in only 5 of 14 with cystic fibrosis and no overt liver disease (P < 0.025). Lymphocyte toxicity to isolated rabbit hepatocytes was significantly increased in 10 of 13 children with liver disease, but in only 6 of 29 children without liver disease (P < 0.001). Experiments using lymphocyte subpopulations showed that the cytotoxicity was mediated by a non-T-cell population and could be blocked with liver-specific lipoprotein in 7 out of 10 cases, suggesting that the reaction in these patients was specifically directed against liver-specific lipoprotein. The study suggests that sensitisation against liver membrane antigens, whether arising primarily or secondarily in some way to other hepatic lesions, may contribute to the progression of liver damage in cystic fibrosis.     

Intestinal permeability to 51Cr-EDTA and orocecal transit time in cystic fibrosis.

Intestinal permeability was investigated in 16 children with cystic fibrosis and 14 age-matched controls, making use of 51Cr-EDTA as probe molecule. Orocecal transit time was also evaluated using the lactulose/hydrogen breath test. There was an increase in permeability to 51Cr-EDTA in patients with cystic fibrosis (mean 13.62%) compared with the control group (mean 1.08%). The orocecal transit time was prolonged in patients with cystic fibrosis compared with controls. Mean fasting breath hydrogen concentration was significantly greater in cystic fibrosis patients (13 ppm) compared with control patients (3 ppm). There was no correlation between the percentage of 51Cr-EDTA excreted and the orocecal transit time in either control or cystic fibrosis patients.     

Liver disease does not affect lipolysis as measured with the 13C-mixed triacylglycerol breath test in children with cystic fibrosis

BACKGROUND: Liver disease associated with cystic fibrosis may not only limit the solubilisation and absorption of the products of fat digestion, but also may depress the activity of pancreatic lipase. The purpose of this study was to measure the effect of liver disease on triacylglycerol lipolysis using the 13C-mixed triacylglycerol breath test. METHODS: Forty children with cystic fibrosis took 13C-mixed triacylglycerol with a standard breakfast and the child's normal pancreatic enzyme replacement therapy. Breath samples were collected before and every 30 minutes after ingestion for 6 hours. The cumulative percentage dose of 13C recovered at 6 hours was calculated from sequential measurements of 13C enrichment of breath CO2, measured by isotope ratio mass spectrometry. Liver abnormalities and portal hypertension were defined by ultrasound scan and clinical examination. RESULTS: Twenty-four children had liver abnormalities, including 5 with portal hypertension. No difference was found between cumulative percentage dose of 13C recovered at 6 hours in 16 children with no liver abnormality (mean, 21.4%+/-11.1%), 19 children with liver abnormalities (22.2%+/-10.0%) and 5 children with portal hypertension (20.9%+/-7.1%). CONCLUSION: Intestinal lipolysis is not reduced in cystic fibrosis liver disease when measured using the 13C mixed triacylglycerol breath test. These findings affirm the test's use as an indirect measure of fat digestion that is not affected by inadequate intraluminal bile salts or liver disease.     

Ultrasound in the Management of Hepatic Veno-Occlusive Disease in Three Children Treated with Dactinomycin and Vincristine

Three children, treated with dactinomycin and vincristine without radiotherapy showed hepatic toxicity consistent with diagnostic criteria for hepatic veno-occlusive disease (VOD). Two patients were affected with Wilms' tumor and the third with malignant fibromatosis. The clinical manifestations of VOD were mild and regressed in all patients after supportive therapy. Serial ultrasonography (US) was performed in all cases and was useful in confirming the diagnosis and in evaluating the severity of the disease. US features of VOD were hepatomegaly, gallbladder wall thickening, ill-defined borders of the hepatic vessels, and ascites. Parenchymal heterogeneity was still present after several months as a result of the hepatic injury. The hazy appearance of the portal vessels and the gallbladder wall thickening seemed directly correlated with the degree of hepatic involvement in the early phase of VOD.