Neonatal liver cirrhosis. Sometimes cirrhosis is found similar to that of the adult, but with more widespread fibrosis. Biliary fibrosis resulting from obstruction of bile flow may also be found , but it takes just over a month for the clinical and pathological picture to fully develop.
Summary
[ hide ]
- 1 Frequency
- 2 Etiology
- 3 Diagnosis
- 4 Laboratory data
- 5 Biopsy and autopsy findings
- 6 Treatment
- 7 Internal links
- 8 Sources
Frequency
At Children’s Hospital in Boston , Craig and others were able to record 98 cases over the course of 30 years, yet their rarity is greatest in the neonatal period: Thaler was only able to discover 24 cases in the first few weeks of breastfeeding among children. attended at the Toronto Hospital for Sick Children, in the span of 23 years.
Etiology
Most cirrhosis in children develops after persistent obstruction of bile flow. Many of them derive from congenital atresia, while those subsequent to cystic fibrosis, choledochian cyst or other obstructions are relatively rare. Erythroblastosis and galactosemia explain a few cases. The least, by far, come from giant or viral cell hepatitis. According to Thaler and Gellis, cirrhosis of these children is not apparent before the age of one year, having been observed in approximately 50% of their infants with hepatitis , who continued to have liver disorders throughout this period of time.
On the other hand, a small number of infants who appear ill at birth or from the first weeks of life, with a picture of cirrhosis from the beginning, which progresses rapidly and usually ends fatally in a few months, at autopsy or biopsy of the livers. of these patients show the typical alterations of fully developed cirrhosis.
Most observers rate these cases of rapidly progressive fetal or neonatal hepatitis, but Thaler’s studies cast doubt on this etiology , however, this author has not attempted to offer an alternative pathogenic theory.
Diagnosis
Thaler’s description of 24 children with neonatal cirrhosis is worth summarizing here. Most of these children were underweight at birth and almost all were boys. In 8 the disease was recognizable at birth; the case with the later presentation was at 9 weeks of age.
The initial symptom was for the most part a gastrointestinal disorder, with vomiting and diarrhea . They did not gain weight and even lost it. Jaundice then developed , with light stool and dark urine in most. The body temperature was not elevated. In a few cases, seizures , abdominal distension, or peripheral edema were recorded . In many of them there was hepatomegaly , while a third presented splenomegaly .
The course was rapidly progressive, with the aggregation of anemia , hypoproteinemia, ascites , edema and deeper jaundice. Terminal hemorrhagic manifestations were frequent. None of these children survived more than 6 months from the establishment of the picture.
Laboratory data
Most showed leukocytosis . Half had some form of hemolytic anemia . The serum bilirubin rate was increased, and the direct reaction bilirubin comprised 35 to 40% of the total. The proteins total existing in serum were decreased, but the γ globulins were relatively increased. The urine contained all bile; some contained an excess of urobilinogen and others did not.
Biopsy and autopsy findings
All patients accumulated ascitic fluid. The livers were hyperplastic and with fine or coarse granulation. There was disseminated liver fibrosis, almost always periportal, distortion of the liver plaques, cellular vacuolization, distended central veins and hyperplasia of the ducts. Only rarely were giant cells found. Necrosis and acute inflammation were not found . The difference between these livers and those of giant cell hepatitis is noticeable from the beginning.
Treatment
In the very limited number of operable cases, biliary cirrhosis can be improved or cured with an appropriate shunt. In the other varieties of cirrhosis, the only thing that can be offered to patients is a supportive therapy .
