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Biomedical subjects

C L Witzleben

Publications and source records attributed to C L Witzleben.

At least 37 records · Page 2Linked to original sources
PubMed1983

Antenatal diagnosis and early surgery for choledochal cyst.

The pathogenesis and optimal treatment of choledochal cyst have long remained questions of considerable speculation and dispute. Because the pregnancy of a 37-year-old woman was felt to be at risk, five antenatal ultrasound examinations were made. The fourth examination at 31.5 weeks of gestation demonstrated a choledochal cyst. Following birth, the child was studied with repeat ultrasound examinations and scintigraphy. The results of these studies plus the findings at surgery contributed new evidence regarding the possible pathogenesis and optimal treatment of choledochal cysts. Although the time at which the cyst originated is compatible with the concept of reflux of pancreatic juice into the common duct, no abnormal junction of the pancreatic and common duct was identified. No evidence of obstruction as part of the pathogenesis could be demonstrated. Progressive changes in the choledochal cyst in the first ten days of life suggested that delay in diagnosis and treatment of a choledochal cyst may contribute to early complications such as cholangitis. Early excision of the cyst in the newborn is considered to be the optimal treatment and may pose less risk to the patient than delayed surgical exploration.

Common Bile Duct Diseases↗
PubMed1982

"Nephronophthisis-congenital hepatic fibrosis": an additional hepatorenal disorder.

This report describes two siblings, male and female, with a distinctive hereditary hepatorenal disorder. Review of the literature indicates that 11 cases have been reported outside the United States. The condition may first develop from early infancy to adolescence, and it is characterized in the great majority of instances by clinically severe renal disease. At some time in the course of the disease, hepatomegaly is present in all patients, and splenomegaly is common. Additional lesions and other abnormalities, including retinal lesions, mental retardation, cerebellar and osseous abnormalities, have been reported in some patients. Histologically, the renal lesion resembles nephronophthisis, and the hepatic lesion resembles congenital hepatic fibrosis. Mortality (due to the renal disease) is very high, and the longest-surviving patient was 14 years old at the time of death.

Adolescent↗
PubMed1982

Bile duct paucity ("intrahepatic atresia").

Reduction of the number of interlobular bile ducts (BDP) has potentially significant physiologic consequences. Most of the disorders currently recognized as BDP involve an easily discernible reduction in duct numbers, but actual duct quantitation needs to be done routinely in the examination of liver biopsies in order to recognize and assay the importance of less marked reduction. Ducts are seldom if ever completely absent, and may not be uniformly reduced throughout the liver, so a wedge biopsy is the only completely satisfactory sample for the morphologic evaluation of duct numbers. In some conditions the number of ducts varies greatly with time. BDP may be only one aspect or manifestation of a disease primarily characterized by other features, or it may be the principal cause of distress and a major feature in defining a disorder. In a few conditions, the mechanism of the paucity has been quite well established as the destruction of previously existing ducts, but at present the basis for duct paucity in most conditions remain obscure. The treatment of BDP is similar in all conditions. Because of the wide range of circumstances in which BDP occurs, the prognosis is highly variable and depends on the underlying condition. In some patients BDP is associated with a long life of relatively good quality, in others it is associated with rapidly progressive liver disease, and in others with an intermediate course. In children particularly, conditions with BDP may be familial. In recent years there has been some advance in recognizing individual conditions associated with BDP, but many cases are still unclassifiable. Evaluation of a patient with BDP should be directed not only to diagnosing known conditions, but also to defining new ones, and should include family history, alpha 1-antitrypsin determinations, bile acid analyses, chromosome analysis, and consideration of virus infection. Additional efforts should be made to determine the pathogenesis of the duct paucity in individual conditions in order to prevent, arrest, or reverse the processes involved.

Bile Ducts↗
PubMed1978

Foamy myocardial transformation of infancy: 'lipid' or 'histiocytoid' myocardiopathy.

Two cases of a distinctive infantile myocardiopathy were studied by light and electron microscopy. They represent the 15th and 16th reported examples of this condition, and include the first associated with morphologically classical endocardial fibroelastosis. The condition is characterized by the presence of clusters of foamy or granular cells scattered throughout the myocardium. Ultrastructural studies indicate (1) that lipid storage is not a feature of the condition, and (2) that the abnormal fibers are characterized by a reduced number of fibrils and an increased number of mitochondria. It is proposed that the lesion should be called "foamy myocardial transformation of infancy," and it is speculated that the condition is an acquired metabolic disorder, perhaps occurring in patients abnormally predisposed to such an acquisition.

Cardiomyopathies↗
PubMed1977

Acute renal failure secondary to acute pyelonephritis.

A six year-old girl presented with acute oliguric renal failure, secondary to acute, non-obstructive pyelonephritis. Evidence for pyelonephritis as the cause of renal failure included: the evolution of typical changes on serial intravenous pyelograms, an acute interstitial inflammatory exudate on percutaneous renal biopsy, and gram-positive cocci on gram stain of the biopsy tissue. Although a specific causative organism was not conclusively identified, enterococcus was isolated from the initial catheterized urine specimen. The patient recovered from the acute illness but was left with impaired renal function, hypertension, and cortical scarring. Acute, non-obstructive pyelonephritis can produce acute renal failure in children and must be considered in the differential diagnosis of this syndrome.

Acute Disease↗
PubMed1975

Medullary cystic disease vs nephronophthisis. A valid distinction?

It has been proposed that medullary cystic disease and nephronophthisis are distinguishable by the mode of inheritance and the age of onset. The former is inherited as an autosomal dominant, the latter as an autosomal recessive, with the onset at the average age of 26.7 years and 10.5 years, respectively. The present kindred would appear to have a dominant inheritance and onset at 10.1 years of age. This emphasizes the importance of examining those families in whom medullary cystic disease is suspected, prior to genetic consultation.

Adolescent↗
PubMed1975

Bilirubin as a cholestatic agent. III. Prevention of bilirubin-related cholestasis by sulfobromophthalein.

The administration of sulfobromophthalein sodium (BSP) to manganese-loaded animals had been found to prevent effectively the cholestasis normally created by bilirubin infusion following manganese loading. Measurement of bilirubin levels in blood, liver, and bile in manganese-bilirubin animals with and without BSP fails to provide convincing evidence for a BSP-induced shift in site or magnitude of total bilirubin concentration as an explanation for this anticholestatic action of BSP. Nevertheless, increasing the dose of bilirubin partially reinstitutes the cholestasis. The results are important in that they demonstrate (1) that under certain circumstances intrahepatic cholestasis can be immediately and directly prevented by a pharmacologic agent (by a means not as yet defined) and (2) that the cholestatic activity of bilirubin is, to some extent or under some circumstances, separable from its concentration in blood, liver, and bile.

Animals↗
PubMed1975

Bilirubin as a cholestatic agent. IV. Effect of bilirubin and sulfobromophthalein (BSP) on biliary manganese excretion.

These experiments demonstrate that the adminstration of bilirubin does not significantly alter biliary manganese excretion in manganese-loaded animals. Sulfobromophthalein sodium (BSP), which has been shown to afford prophylaxis against manganese-bilirubin cholestasis, significantly increases the biliary excretion of manganese. These results are meaningful in that they indicate that the amount of manganese per se in the bile is not critical in this cholestasis, and suggest that intracanalicular reactions are probably not etiologically critical in this model. They point to the conclusion that the critical cholestatic events are occurring within the hepatocyte. The results also indicate that the biliary excretion of manganese is obligate, and to some extent independent of bile flow. The critical cholestatic events are presumably mediated through the biochemical effects of either a manganese-bilirubin complex or of manganese and bilirubin acting separately but synergistically.

Animals↗
PubMed1973

Bilirubin as a cholestatic agent. II. Effect of variable doses of bilirubin on the severity of manganese-bilirubin cholestasis.

These experiments demonstrate that the cholestasis following manganese-bilirubin administration varies directly in severity in relation to the amount of bilirubin administered. This suggests that the cholestasis is related to critical bilirubin accumulation in some compartment of the biliary excretory pathway. However, in the immediate precholestatic period, the levels of bilirubin in all three compartments of blood, liver and bile also vary in direct relation to the amount of bilirubin administered. The critical site of bilirubin accumulation or concentration is not, therefore, defined by these experiments.

Animals↗