Two newborn infants, male (A) and female (B), with lethal hyperammonaemia are described in the same family. In both, symptoms started on the second day of life. Lethargy and hypotonia were the most prominent initial findings and were followed by convulsions and coma. In both, blood ammonia levels rose to ...

The mesocaval graft for portal decompression is applicable in infants and children with portal hypertension secondary to extrahepatic or intrahepatic causes. It is recommended in patients in whom extensive previous surgery in the portahepatis (Kasai procedure) would make dissection of the portal vein difficult and endanger the integrity of the ...

Hepatic fibrosis with obliterative lesions of the small hepatic veins occurred in a three month old infant with fatal congenital leukaemia treated with cytostatic drugs. The vascular changes were characterized by an unusual, hitherto unreported angiomatoid, proliferation of the endothelium. The process is compared with the more common subendothelial-fibrous type ...

A 61-year-old female presented with a right transudative pleural effusion in the absence of ascites and other stigmata of chronic liver disease. A diagnosis of cirrhosis with a secondary hydrothorax was eventually established; however, ascites could never be demonstrated clinically or radiographically until just before her death, when hypertonic saline ...

Preliminary observations of the effect of surgical excision of the endolymphatic duct and sac anlage in normal mouse embryo inner ear embryogenesis "in vitro" are reported. Otocysts of twelfth and thirteenth gestation day mouse embryos were grown in organ culture for 9 and 8 days respectively. The organ culture specimens ...

It has been suggested that the quantitative estimation of serum alpha-1-fetoprotein may help in distinguishing the neonatal hepatitis syndrome from biliary atresia. We measured the serum AFP concentration in 52 neonates and infants with various hepatobiliary disorders, including neonatal hepatitis syndrome (group I), biliary atresia (group II), and other hepatopathies ...

Scintiscans of liver and spleen using technetium 99m sulphur colloid in 15 infants with extrahepatic biliary atresia and 11 infants with severe obstructive jaundice (7 with genetic deficiency of alpha 1-antitrypsin) showed similar hepatic size, pattern of isotope uptake, and splenic abnormality with no distinguishing features. In 37 older children ...

Electon microscopic observations were made on liver biopsy specimens from nine infants and children diagnosed as having Reye's syndrome by clinical, laboratory, and light microscopic criteria. In addition to excessive fat content, mitochondrial abnormalities were the most frequent abnormal finding in the liver. However, no correlation could be established between ...

Serum bile acid patterns were determined in 31 infants ranging in age from 4 days to 24 weeks. Fifteen infants with extrahepatic biliary atresia had a mean concentration of serum bile acids of 90 microng/ml +/- 47 SD;; 16 infants with neonatal hepatitis had a mean concentration of 60 +/- ...

Aetiological factors were sought prospectively in 55 babies with extrahepatic biliary atresia, in 105 with neonatal hepatitis, and in 11 with intrahepatic biliary atresia, seen as a result of nearly complete ascertainment of these conditions in the State of Victoria between 1963 and 1974. In neonatal hepatitis infective causes were ...

Liver biopsy was performed to exclude anatomic obstruction of the biliary tract in five prematurely born infants who had developed conjugated hyperbilirubinemia during intravenous alimentation with a protein hydrolysate. Each was being treated after having undergone a segmental intestinal resection for necrotizing enterocolitis. Bacterial and viral infections, metabolic disorders, and ...

A 3-month-old infant presented with multiple cutaneous haemangiomata, and was found to have a cardiac murmur and hepatomegaly. The latter increased strikingly over the next 3 months, and was accompanied by cardiomegaly. Other usual signs of congestive cardiac failure were not present, however, and an arteriogram showed the presence of ...

The clinical features of spontaneous perforation of the bile ducts in infancy differ sufficiently from those of other causes of neonatal jaundice to allow early preoperative diagnosis as demonstrated by a report of this condition in a 6-week-old infant. Despite its rarity, the clinician should be aware of this entity ...

Cholestasis has been previously described after long-term hyperalimentation in infants. The present case documents the development of cholestasis in an adult after a relatively short period of total parenteral nutrition (TPN), i.e., hyperalimentation. Other causes for cholestasis, such as exogenous or endogenous hepatotoxic agents or allergic type hepatitis, do not ...

Four infants are described who presented with rapid enlargement of the liver. This was found to be due to neuroblastoma which had metastasized to the liver; the condition was associated with high levels of urinary vanillylmandelic acid (VMA). In 3 infants the primary tumour was in the adrenal gland and ...

In a prospective regional survey of neonatal hepatitis syndrome 32 infants had extrahepatic biliary atresia (EHBA) and 103 had hepatitis. No cause for the lesion was found in infants with extrahepatic biliary atresia, but in 32 with hepatitis a specific cause was identified, 24 having genetic deficiency of the serum ...

Since 1974, 16 consecutive infants with biliary atresia have been treated by hepatic portoenterostomy employing an exteriorized Roux-en-Y intestinal segment (Miluliez). Simultaneous, sequential analyses of bile pigments and lipids in serum and biliary drainage were performed. In the 11 patients with sustained bile drainage, progressive increases in bile volume, bilirubin ...

Serum alpha-fetoprotein levels were measured using a sensitive radioimmunoassay in 77 infants presenting with persistent conjugated hyperbilirubinaemia. A breed range of alpha-fetoprotein concentrations occurred in both the 23 infants with extrahepatic biliary atresia and the 35 with idiopathic neonatal hepatitis but the 13 with alpha-1-antitrypsin deficiency had uniformly low levels. ...

An infant explored for obstructive jaundice died 6 days postoperatively and was found to have extensive infarction of the common duct. This would have gone undetected had the patient survived and without careful sampling at autopsy. The incidence of this unsuspected complication is unknown. Surgical infarction may be one cause ...

Hepatic fatty cirrhosis (HFC) has been known to occur in certain domestic livestock species since 1931. Early studies in Texas indicated that HFC was restricted to five western counties. Recently HFC was identified in white-tailed deer (Odocoileus virginianus texanus) in South Texas for the first time. Of the deer examined, ...

A series of 32 infants with persistant jaundice in whom an unequivocal differentiation between intrahepatic cholestasis and biliary atresia could not be made is reviewed. A protocol including Lipoprotein-X (LP-X) determinations before and after a short course of cholestyramine (CSM) was carried out in all. A fall in serum LP-X ...

We prosepctively studied 200,000 newborns to determine the frequency and clinical characteristics of alpha1-antitrypsin deficiency. One hundred and twenty Pi Z, 48 Pi SZ, two PI Z-and one Pi S-infants were identified and followed to the age of six months. Fourteen of 120 Pi Z infants had prolonged obstructive jaundice, ...

This report describes the clinical, biochemical, and hepatic morphologic findings in ten children with severe serum alpha1-antitrypsin deficiency. Genetic protease inhibitor (Pi) phenotyping, using acid-starch gel and crossed antigen-antibody electrophoresis, demonstrated Pi phenotype ZZ in all our cases. In eight patients, manifestations of liver disease appeared during the first year ...

A 16-month-old female infant with severe mental and motor retardation, clinically diagnosed as Leigh's encephalomyelopathy, forms the basis of this study. This infant was found to have lactic acidosis, low cerebrospinal fluid glucose, hyperalaninemia, and increased levels of urine lactate, pyruvate and alanine. These laboratory studies suggested an inborn error ...

The infant born with a posterolateral defect in the right side of the diaphragm may appear normal at birth. His abdominal viscera may be normally located; the right side of the diaphragm may seem to be intact. Later, part of the liver may herniate. As it does, it displaces the ...

A clinical, enzymatic, and pathological study of an infant with the acute form of hereditary tyrosinaemia is presented. Treatment with a diet low in methionine, tyrosine, and phenylalanine was unsuccessful. A selection of specific and nonspecific hepatic enzymes, obtained at necropsy within one hour of the infant's death at 9 ...

From July 1971 to March 1975, elevan infants receiving total or partial parenteral alimentation at the University of Florida showed histologic evidence of intrahepatic cholestasis. The clinical records of these patients have been examined. These infants were critically ill and had protracted hospital courses with only two survivors. Liver biopsies ...

Infants of very low birth weight who require prolonged assisted ventilation are unable to tolerate oral feedings. To provide and maintain nutrition in these infants, a modified form of parenteral nutrition (dextrose 10% and crystalline L-amino acids, 1.7-3.4 g%) is used in our nursery. Intrahepatic cholestasis was diagnosed at autopsy ...

Dynamics of the lymph node modifications as well as hepatic and immunoserological in various cirrhogenic hepatopathies have been studied on human and experimental material. Lymph nodes showed early and progressively immunomorphological reactions with maximum intensity in evolutive stages of certain cirrhoses. Such reactions as well as in other areas of ...

In a group of 15 marasmic infants, the activity of the liver enzyme "succinic dehydrogenase" (SDH) was studied. This was compared with the activity of that enzyme in the livers of 10 normal infants, taken as controls. Histologic changes were found in only one third of the marasmic cases studied ...

A total of 45 patients with severe psoriasis vulgaris treated with Methylaminopterin NFN (Methotrexate Lederle) were HL-A typed in order to seek a possible correlation between HL-A antigens and the histological abnormalities in post-Methotrexate (MTX) liver biopsies. Highly significantly increased frequencies of w17 and TY (P less than 0.0001) were ...

A female baby 7 weeks of age developed a fatal tuberculous disease following BCG vaccination. The infant had Swiss type agammaglobulinaemia, associated with oral thrush, pneumonitis, umbilical infection and chronic enteritis not responding to any treatment. Post mortem revealed an aplasia of the thymus, hypoplasia of the lymph system, miliary ...

Metabolism of calcium and magnesium may be disturbed in hepatobiliary disease because of deficient or absent bile flow into the gut, since bile is important for the intestinal absorption of these elements. In the present paper the tubular reabsorption of phosphate (TRP), calcium (TRCa), and magnesium (TRMg) were determined in ...

Pituitary aplasia or hypoplasia occurs in anencephalic and normocephalic infants. Two infants with hypopituitarism are described; in each the diagnosis was made clinically. The infants had frank clinical and histologic evidence of the neonatal hepatitis syndrome. There may be more than a chance relationship between the clinicopathologic findings of neonatal ...

A total of 240 cases of a bleeding syndrome in infants due to prothrombin complex deficiency of unknown aetiology were reviewed. The majority of patients were breast fed, aged 1-2 months and the syndrome was more prevalent in males. Clinical manifestations consisted of bleeding, pallor and mild hepatomegaly in the ...

Bile salt synthesis and bile salt pool size were determined by isotope dilution in two groups of healthy premature infants, utilizing nonradioactive deuterium-labeled bile salts. All 9 infants were between 32 and 36 weeks of gestation; however, in one group (4 infants), the mothers had received either dexamethasone or phenobarbital ...

Sixty-nine specimens of liver tissue from 53 infants with neonatal hepatitis or its sequelae were examined without knowledge of the alpha1-antitrypsin phenotype. Distinctive, diastase-resistant, PAS-positive, pure magenta-coloured, sharply defined globules, 2-20 microns in diameter were found in periportal and paraseptal hepatocytes in all liver biopsies from eight alpha1-antitrypsin deficient (PiZZ) ...

Poisoning with aflatoxin derived from mouldy bread was confirmed as the cause of death of one dog and was suspected as the cause of death of two other dogs on the same ration. A jaundiced carcass, firm bile-stained liver and haemorrhage into the gastro-intestinal tract were seen at autopsy. Swelling ...

A newborn infant with congenital neuroblastoma complicated by disseminated intravascular coagulation is described. At birth the infant showed liver and spleen enlargement and shortly thereafter malignant cells were found in the bone marrow. On the fifth day of life the infant started to bleed and coabulation analysis indicated disseminated intravascular ...

A series of 12 infants undergoing hepatic portoenterostomy for incorrectable-type biliary atresia is reviewed. There has been no evidence of a sustained postoperative increase in bile excretion or improvement in biliary cirrhosis in any patient. Survival statistics for the group as a whole are poor with a mean postop survival ...

Focal biliary cirrhosis is an uncommon finding in infants with cystic fibrosis, but it is present in more than a fifth of surviving children and adolescents. It was found at postmortem examination in only five of 47 infants with CF younger than 3 months, in five of 32 infants from ...

This report is a response to the suggestion, first raised in The Journal in 1971, 1 that intravenous alimentation may be responsible for intrahepatic cholestasis in premature infants. Nine of 15 premature infants (30 weeks' gestation or less, birth weight up to 1,250 gm) who survived at least five days ...

A case illustration is given of an infant with biliary atresia who had the Kasai operation more than 1 1/2 years ago and is now, by all biochemical and histological criteria, cured. The early results with this operation in 14 patients are similar to those reported by the Japanese, that ...

A specific deficiency of methionine adenosyltransferase has been demonstrated in the liver of an infant with hypermethioninemia. Since the enzymatic activity was below that in fetal liver and the metabolic abnormality has persisted (the infant now being 1 year of age), there is probably a genetic mutation. Mass screening for ...

A solitary hepatic abscess was found at postmortem examination in a neonate who died at 25 days of age. Elevation of the right diaphragm was noted radiologically from the first day of life, suggesting congenital presence of the abscess. Recognition of the previously unsuspected association between hepatic abscess and congenital ...

The activities of phenylalanine p-hydroxylase, tyrosine: alpha-ketoglutarate and phenylalanine: pyruvate aminotransferases, and aromatic alpha-ketoacid reductase have been measured in liver biopsies obtained from children with a series of disorders other than phenylketonuria and from human foetuses aged 8.5 to 24 weeks. The importance of such a study in relation to ...

1. The activities of the mitochondrial and cytosol isoenzyme forms of l-alanine-glyoxylate and l-alanine-2-oxoglutarate aminotransferases were determined in rat liver during foetal and neonatal development. 2. The mitochondrial glyoxylate aminotransferase activity begins to develop in late-foetal liver, increases rapidly at birth to a peak during suckling and then decreases at ...

Infant and weanling mice infected experimentally with non-neuroadapted Western equine encephalitis virus exhibited a differing spectrum of target-organ pathology as well as a different clinical response. The infant mice sickened and died within 48 hr. Pathological changes in this group were largely limited to such mesodermally derived tissues as muscle, ...

Infant and weanling mice infected experimentally with non-neuroadapted Western equine encephalitis virus exhibited a differing spectrum of target-organ pathology as well as a different clinical response. The infant mice sickened and died within 48 hr. Pathological changes in this group were largely limited to such mesodermally derived tissues as muscle, ...

Five out of 28 infants investigated in a regional survey of neonatal hepatitis were found to have genetically-determined deficiency of α1-antitrypsin (ZZ phenotype). The clinical course and pathological changes varied considerably. All five infants had an acute hepatitis-like illness, and although this subsided cirrhosis later developed in three cases. The ...