Liver transplantation in children with chronic end stage liver disease: factors influencing survival after transplantation.

To identify pretransplant factors that are influencing survival after orthotopic liver transplantation a Cox proportional hazards regression model was applied to 118 children with chronic terminal liver failure transplanted at Medical School Hannover during the period of 1978 to 1994. The response variable was survival, as covariates a total of 19 pretransplant variables were entered--i.e. age, diagnosis (biliary cirrhosis, metabolic cirrhosis, postnecrotic cirrhosis, cryptogenetic cirrhosis) sex, laparotomy prior to OLT, height, weight, standard deviation scores for height and weight, date of first OLT, serum alanine aminotransferase, asparagine aminotransferase, albumin, total bilirubin, cholinesterase activity, glomerular filtration rate, and prothrombin time. Significant independent predictors of survival after OLT were bilirubin (P=0.0024), SDS for weight (P=0.034), and albumin (P=0.039). In a subsequent discriminant analysis cut off points for these variables could be identified--i.e., bilirubin >340 micromol/L, SDS for weight <-2.2 and albumin < 33 g/L. Patients with one or more of these risk factors were grouped as urgent indication group (n=76) and those with no risk factor as elective indication group (n=42). Comparing the posttransplantation survival in these groups there is a statistically significant difference at 1 year (57% vs. 90.5%) and 4 years (49% vs. 90.5%) after OLT (P=0.0001, log rank test). It is concluded that the risk of OLT is much higher if liver function is very poor. Optimal nutritional support prior to transplantation is mandatory to optimise the clinical status of the children and to improve the results of OLT.

Lack of ultrasonographic evidence for severe hepatosplenic morbidity in schistosomiasis mansoni in Mali.

The inhabitants of four villages endemic for Schistosoma mansoni in central Mali (n = 1,106 of both sexes, age range 2-80 years) and of two nonendemic villages in another part of the country were examined parasitologically and ultrasonographically to establish the prevalence of periportal liver fibrosis (PF) and other features of hepatosplenic schistosomiasis. The prevalence of S. mansoni infection ranged from 36% to 93% in the endemic villages. A severe infection (> 400 eggs/g of stool) was found in 16% of the infected individuals. No case of grade III PF (echogenic bands usually > 10 mm in diameter around the central part and major branches of the portal vein and streak-like fibrous bands that extended into the periphery of the liver) and only eight cases of grade II PF (echogenic bands usually > 10 mm in diameter around the central part and major branches of the portal vein) were found; no other signs of severe hepatosplenic disease were encountered. However, grade I PF (echogenic bands usually > 4 mm in diameter that were best visible in the area of the portal vein bifurcation and gall bladder neck) was detected in 21% of all individuals, mainly in adults. In the nonendemic villages, the prevalence of grade I PF in adults was 9%. Generally, there was no significant correlation between the grade of PF and S. mansoni egg output. In one village with a high endemicity level, however, the prevalence of grade PF I increased with the intensity of infection. Morphometric data revealed no significant influence of S. mansoni infection on portal vein stem diameter and spleen size.(ABSTRACT TRUNCATED AT 250 WORDS)

Ultrasonographical investigation of periportal fibrosis in children with Schistosoma mansoni infection: reversibility of morbidity seven months after treatment with praziquantel.

Five hundred thirty six Sudanese schoolchildren with Schistosoma mansoni infection were treated at random with either 20 mg or 40 mg/kg praziquantel. Seven months later 420 children could be reinvestigated by ultrasonography. Reduction of egg excretion and reversibility of sonographically-proven periportal fibrosis (PF) was not significantly different in the two groups. Schistosoma mansoni-induced PF grade II decreased from 22.9% to 6.7% and grade III from 5.2% to 1.6%. An increased prevalence of PF grade I, from 10% to 29.8% of the investigated patients, was observed. This increase was caused partly by a downshifting of patients who had PF II (n = 45) and PF III (n = 8) before therapy, but also by patients who developed PF I in the seven months after therapy (n = 56). The overall percentage of patients with PF before and after treatment was 38.1%. Of 420 children, 17.4% increased in their PF grade, 55% remained at the same level and 27.6% improved. Children younger than 11 years of age had a higher rate of complete reversibility than older ones. The percentage of patients with hepatomegaly decreased significantly (11.6% to 6.9%; p = 0.001). The rate of splenomegaly remained unchanged. It was concluded that within seven months therapy with praziquantel resulted in a considerable qualitative improvement of PF in Sudanese schoolchildren with S. mansoni infection.

Sonomorphological abnormalities in Sudanese children with Schistosoma mansoni infection: a proposed staging-system for field diagnosis of periportal fibrosis.

For the purpose of staging sonographical abnormalities induced by Schistosoma mansoni infection in childhood, 536 patients aged 6-15 years underwent extensive sonographical examination. Specific findings of periportal fibrosis were classified in 3 grades and occurred in study patients but not in controls (n = 60). Grade I consisted of echogenic bands usually with a diameter greater than 4 mm that were best visible in the area of the portal vein bifurcation and gallbladder neck. Frequently a continuous U-shaped echogenic structure extended from the left portal branch to the gallbladder bed. Grade II was characterized by echogenic bands usually greater than 10 mm in diameter around the central part and major branches of the portal vein. In addition to features common to grade II, grade III included streak-like fibrous bands that were not confined to portal vein lumina but extended into the periphery of the liver. Sonographical abnormalities encountered in children with S. mansoni infection differed significantly from those in adults.

Ultrasonographical investigation of periportal fibrosis in children with Schistosoma mansoni infection: reversibility of morbidity twenty-three months after treatment with praziquantel.

In February 1987, 322 Sudanese school children were diagnosed for Schistosoma mansoni infection and treated randomly with praziquantel (either 20 mg/kg or 40 mg/kg body weight). A followup of these subjects was carried out in January 1989. This treatment resulted in a substantial reduction of egg output. Patients underwent complete abdominal ultrasonography and periportal fibrosis of the liver was graded into three degrees of severity. The proportion of patients with periportal fibrosis decreased from 36.6% in February 1987 to 21.7% in January 1989. At the time of followup, higher grades of periportal fibrosis (grades II and III) were encountered in only 4.3% and 0.3% of these patients, respectively, compared with 21.1% and 5.9%, respectively, before therapy. This was paralleled by a significant decrease in hepatomegaly from 10.9% to 7% of the patients. In contrast, the rate of splenomegaly showed a slight increase during the period of observation. The different dosage regimens of praziquantel did not result in a significantly different reversibility of periportal fibrosis or a decrease in egg excretion. The reversibility of specific liver lesions 23 months after antischistosomal therapy with praziquantel was substantial. The improvement was greater at 23 months than that obtained seven months after treatment.

Ultrasonography as a diagnostic aid for a district hospital in the tropics.

To improve diagnostic capabilities, an ultrasound unit was installed at a major hospital in Wad Medani, Sudan. During the implementation period (October 1986 to March 1987) of ultrasound service, 863 patients were examined cooperatively by Sudanese and German physicians. The service covered internal medicine (47.5%), obstetrics and gynecology (31.6%), surgery (12.5%), and pediatrics (8.4%). Pathologic findings were seen in 75% of the patients. In obstetrics, the rate of pathologic findings was 40%. Five hundred eighty-three pathologic findings were detected in organ systems that constituted the primary indication for ultrasonography (of a total of 1,009 indications). The clinical benefit of ultrasonography was evaluated in 289 randomly selected patients in a standardized questionnaire completed by the attending physicians. The final clinical diagnosis was established or substantially revised in 21.5% of the patients, based on the ultrasonographic report. The previously reported diagnosis was supported in 69.5% of the patients. Medical management was directly influenced in 26% of the patients. We conclude that the clinical benefit of ultrasonography at a district hospital in the tropics is substantial, providing cost-effective, immediate therapeutic benefits in 25% of the patients examined. The technique thereby contributes to better and more rational patient management in institutions with limited resources.

Hepatosplenic morbidity in schistosomiasis japonica: evaluation with Doppler sonography.

In Southeast Asia, schistosomiasis japonica is an important cause of hepatic fibrosis and gastrointestinal hemorrhage. Reliable methods to investigate portal hypertension (PHT) clinically and epidemiologically on community level are lacking. Doppler sonography is an established tool for investigating PHT in hospital settings. In Leyte, The Philippines, 137 individuals underwent color Doppler sonography, stool examination, and serology for hepatitis B and C, liver cell injury and cholestasis. A total of 85% of the study population had been infected with Schistosoma japonicum. Sonographically, periportal liver fibrosis was seen in 25% and reticular echogenicities (network pattern) in 44%. Portal blood flow was decreased or portosystemic collaterals were present in 10% (adults throughout) and correlated with periportal fibrosis, but not with network lesions. Chronic viral hepatitis was rare. Thus, hepatic lesions are frequent in adults but not in children in areas endemic for S. japonicum. Periportal liver fibrosis indicates a risk of PHT, and network pattern fibrosis apparently does not. Doppler sonography is suitable for research under tropical field conditions.

The public health significance of urinary schistosomiasis as a cause of morbidity in two districts in Mali.

Schistosoma haematobium-related morbidity was studied in the perennial irrigation area of Office du Niger and the small reservoirs area of Plateau Dogon in Mali. Questionnaire, clinical, parasitologic, and ultrasound examination data were collected from 1,041 individuals at the baseline survey in 1991; 705 were re-examined one year after treatment. At baseline, the overall prevalence of S. haematobium infection was 55.2%; half of those infected had no clinical symptoms and 30% had pathologic lesions. Both infection and morbidity were more frequent in children than in adults, with a peak prevalence at 7-14 years of age. The rates of lesions were more than twice as high in those heavily infected as in lightly infected individuals. Reagent strip testing for microhematuria was more sensitive in detecting individuals with pathologic lesions than in detecting individuals with infection. One year after treatment with praziquantel, more than 80% of the urinary tract lesions had cleared. It is concluded that S. haematobium-related morbidity is frequent in Mali, but passive case detection for treatment would not cover a great deal of early stages of the disease; active intervention using reagent strip testing for microhematuria at the most peripheral levels would be an efficient system for morbidity control and monitoring of control operations.

Ultrasonographical investigation of periportal fibrosis in children with Schistosoma mansoni infection: evaluation of morbidity.

Morbidity of Schistosoma mansoni infection was assessed in 536 infected Sudanese schoolchildren using an ultrasonographical staging system for periportal fibrosis of the liver. S. mansoni ova excretion in stools was mild in 28%, moderate in 58%, and severe in 14% of the patients. Grade 1 periportal fibrosis was found in 10.3% grade 2 in 23.1%, and grade 3 in 4.7% of S. mansoni-infected children. Girls and boys were equally affected. The distribution of periportal fibrosis compared to the age of patients was homogenous. There was no significant difference of the rate of periportal fibrosis in the 3 groups of intensity of infection. However, in the 204 patients who showed periportal fibrosis, there was a strong relation between severity of ultrasonographically detectable fibrosis with intensity of egg excretion. In 332 children, of which 73 had a heavy infection, no signs of periportal fibrosis were detected. Sudanese children show a variable susceptibility towards the development of S. mansoni-induced periportal fibrosis. High risk patients can be detected at a childhood stage by measuring the intensity of infection and investigating the liver by ultrasound.

Organometric investigations of the spleen and liver by ultrasound in Schistosoma mansoni endemic and nonendemic villages in Senegal.

With the intention of ultrasonographically assessing hepatosplenic morbidity in Schistosoma mansoni infection and of validating the grading system applied (Cairo classification), 191 subjects in a schistosomiasis endemic village and 247 controls from a nonendemic village in northern Senegal underwent sonographic examination of the liver and spleen. Measurements of the diameters of the peripheral periportal vein branches, the main portal vein stem, liver size (left lobe and right lobe), and spleen length in the endemic village were compared with those in the nonendemic village to evaluate the much discussed influence of S. mansoni infection on those variables. To subtract this presumed influence from reference values for the named variables, they are given as measured in the nonendemic village, stratified by body weight, enabling future investigators on schistosomiasis-induced morbidity to refer to these reference values. The 95th percentile regarding peripheral periportal vein branch diameter in the control groups was exceeded in 24% of the subjects in the endemic group. It was exceeded by 6% for the main portal vein stem diameter, 13% for the left liver lobe, 12% for the right liver lobe, and 14% for the spleen length. According to the Cairo classification, 97% of the endemic population and 81% of the controls had periportal thickening of the liver, mostly grade I. We conclude that 1) hepatic morbidity in the S. mansoni endemic area was low, despite strikingly high intensities of infection; 2) the Cairo classification in its present form overestimates periportal thickening, especially in the case of mild morbidity; and 3) body height-dependent reference values, obtained from endemic controls, must be applied for organometric parameters.

Ultrasonography in a Senegalese community recently exposed to Schistosoma mansoni infection.

Inhabitants of Ndombo (n = 614), a village in an area recently infected with Schistosoma mansoni in Northern Senegal, were examined clinically, parasitologically, and ultrasonographically to investigate the presence and degree of S. mansoni-related hepatosplenic morbidity after a few years of exposure to schistosomal infection of regional canals. Despite previous praziquantel treatment of 56% of the inhabitants prior to our investigation, the prevalence of S. mansoni infection in 1993 was 90%, and 42% of the villagers excreted more than 1,000 eggs per gram of stool. Previously untreated individuals were found to have significantly higher egg counts than treated ones. Despite the high intensities of infection, ultrasonographically detected severe periportal thickening of the liver was infrequent. Grading according to body length-dependent normal values of cross-section diameter of peripheral portal vein branches of a European control group correlated with intensities of infection. Of the total group of patients, 30% (n = 182) had more severe thickening of portal vein branch diameters above the 97th percentile and 70% of these had a splenomegaly. The highest egg counts and the most frequent development of periportal thickening were found in 11-20 year-old individuals. Periportal thickening was less frequent in praziquantel-treated adolescents than in untreated ones. This suggests that early antischistosomal medication may be useful to limit schistosomiasis-induced hepatic morbidity especially in children, even though reinfection seems inevitable.

Diagnostic value of connective tissue metabolites in Schistosoma mansoni related liver disease.

Reliable non-invasive markers of hepatosplenic involvement in schistosomiasis are needed for determination of morbidity levels in endemic populations and for diagnosis and follow-up of affected individuals. Serum levels of connective tissue metabolites have been investigated as fibrosis markers in various hepatic disorders, but their accuracy in the detection of hepatosplenic schistosomiasis under endemic conditions has not been fully elucidated. 206 adult inhabitants of a Tanzanian village highly endemic for schistosomiasis mansoni (prevalence 88%) underwent clinical, parasitological and sonographic work-up; sera were tested for aminoterminal procollagen III-peptide (PIIIP), carboxyterminal procollagen IV peptide (NC1) and laminin. Connective tissue marker levels did not correlate with the presence or intensity of infection. NC1 levels were significantly correlated with periportal liver fibrosis (P < 0.001), splenomegaly (P < 0.002), portal vein dilatation (P < 0.004) and the presence of portosystemic collaterals (P < 0.001); for PIIIP and laminin, none of the respective relationships was significant. Due to wide overlap of NC1 levels between individuals with normal sonography findings and those with advanced periportal fibrosis and portal hypertension, the sensitivity and positive predictive value of this markers to detect these individuals were low (< 40%), although specificity and overall accuracy in the given setting were good (80-90%). It is concluded that PIIIP and laminin are not useful as diagnostic serum markers of hepatosplenic schistosomiasis at the community level; NC1 was significantly related to various indices of hepatosplenic involvement, but its low sensitivity precludes its use as a screening tool under endemic conditions.

Inter-observer variance in ultrasonographical assessment of Schistosoma mansoni-related morbidity in young schoolchildren.

49 Sudanese schoolchildren aged 6-9 years with Schistosoma mansoni infection were ultrasonographically examined by two independent observers in a double-blind fashion. The first observer recorded normal appearance of the liver in 23 cases, whereas the second observer recorded the appearance as normal in 33 cases. There were 23 concordant observations. For Grade I periportal fibrosis (PF), 13 observations were concordant. PF Grade II was rarely observed (2 versus 3 cases), and Grade III was not recorded at all. In total, 38 out of 49 observations were concordant (77.5%). These preliminary data from two ultrasound observers, from observations on a limited number of patients, can be seen as an indication of a potential inter-observer variation of around 20% for the distinction between the absence of PF and a low level of PF.

Ultrasound organometry: the importance of body height adjusted normal ranges in assessing liver and spleen parameters among Chinese subjects with Schistosoma japonicum infection.

Hepatosplenic measurements among 550 Chinese subjects, aged 3-59 years from Yueyang city--a nonendemic area for schistosomiasis in Hunan province, China--were performed to define normal ranges of ultrasound organometry for assessing hepatosplenic morbidity in Schistosoma japonicum infection. Measurements included the size of the liver (left lobe and right lobe), the main portal vein stem, the peripheral periportal vein branches, and spleen length and thickness. The results document the significant relationship between body height and organometric parameters. The reference values stratified by body height improve the accuracy of assessment. Thus, height-based normal ranges established in this study can be applied in hospital routine and in field studies of patients infected with S. japonicum in Hunan province and in other endemic areas of China.

Evaluation of ultrasonographic staging systems for the assessment of Schistosoma mansoni induced hepatic involvement.

For the sonographic assessment and grading of hepatosplenic morbidity induced by Schistosoma mansoni infection, several quantitative and qualitative classification systems have been used. In an attempt to evaluate two staging systems, a study was performed as part of a schistosomiasis research and control programme in Richard Toll, Senegal. A total of 700 residents of the township N'diangué were parasitologically, clinically and sonographically examined in July 1993. Two ultrasound observers (M.D. and E.D.) applied the Cairo and the Managil classification (E.D. only) for the grading of periportal thickening of the liver. In spite of high prevalence and intensity of infection, severe hepatic morbidity was rare. According to the Cairo classification, there was a high percentage of subjects with grade I periportal thickening, with considerable inter-observer variability. In the Cairo classification, which is based on the diameter of peripheral portal vein branches, firm cut-offs are used, independent of body height. We show the relationship between body height and portal vein diameters and recommend the use of body height-dependent reference values to avoid falsely high percentages of periportal thickening, especially in children. To minimize inter-observer variability, a clarification of existing instructions for taking measurements for grading is suggested. These suggestions have been considered during the follow-up expert meeting on the Cairo classification in Niamey under the auspices of the World Health Organization in October 1996.

Cystic echinococcosis with perforation into the biliary tract in an eight-year-old girl.

Cystic echinococcosis (CE) is a zoonotic infection caused by echinococcus granulosus and is still frequent in endemic areas. CE is predominantly localised in the liver. The therapy of choice is the surgical sanitation of the lesions, increasingly through interventional procedures such as percutaneous punctation of the cysts, aspiration of the cystic fluid, instillation of a protoscolicidal solution and reaspiration of this solution (PAIR). However, in some cases, such as perforation into the biliary tract, PAIR is not feasible. We report on an eight-year-old girl who suffered from CE with perforation of one cyst into the biliary tract. The diagnosis was confirmed by ERCP and cholangioscopy and a right hemihepatectomy was performed. The girl received adjuvant chemotherapy with albendazol perioperatively and is still asymptomatic with no indications of disease 10 months after the operation.

Sonographical morphometrical findings of the liver and spleen in Sudanese patients with Schistosoma mansoni induced periportal fibrosis.

Sonographic measurements of the liver, spleen and related blood vessels were performed in 87 healthy controls and in 50 patients with periportal fibrosis (pF) as well as in 52 patients with pF and at least one episode of gastro-oesophageal bleeding due to chronic Schistosoma mansoni infection. There was a progressing shrinkage of the liver paralleled by an increasing rate of patients with a larger left than right liver lobe as involvement of pF due to S. mansoni infection progressed. This was accompanied by an increasing percentage of pathological gallbladder wall enlargement, splenomegaly and an increasing portal vein diameter. It is suggested that organomorphometrical parameters of the liver and spleen can be used for sonographical evaluation of patients with S. mansoni infection as these may be useful for their clinical assessment of the progression of disease.

Cost saving with ultrasonography in a developing country district hospital.

OBJECTIVE: To estimate the potential monetary reduction induced by the introduction of an ultrasound unit in a major district hospital in a developing country. DESIGN: Propective study. SUBJECTS: Patients referred with abnominal diseases and pregnancy. SETTING: Wad Medani Teaching Hospital, Central Sudan. RESULTS: Local specialists referring the patients stated that an estimated 792 radiologic procedures would have been carried out to obtain the same amount of information as achieved by ultrasound. Such procedures budgeted approximately 8100 US dollars, in terms of equipment, material and personnel by 1987 rates. Such savings have benefited all departments of the hospital. CONCLUSION: The authors consider this as evidence for the fact that despite its initial high investment (15,000 US dollars), availability of ultrasound virtually reduced expenditure on other radiological diagnostic procedures. This is of special benefit for the limited budgets of hospitals in non-industrialised countries.

Ultrasonographic evaluation of gallbladder function in patients with Schistosoma mansoni infection.

Gallbladder function was studied in 22 patients with portal hypertension due to advanced Schistosoma mansoni infection and in 17 healthy adults. The fasting volume of the gallbladder was significantly less in patients than in controls. The gallbladder wall was grossly thickened in the study group. There was a significant difference between the patients and controls in the reduction of gallbladder volume following a fatty meal. Although the portal vein diameter and the splenic volume were significantly larger in study patients, no significant correlation could be detected between the reduction in volume or wall thickness of the gallbladder and any of the relevant sonographic features of schistosomiasis-induced portal hypertension.