Congenital dilatation of the large and segmental intrahepatic bile ducts (Caroli's disease) in two Golden retriever littermates

Two, sibling, male Golden retriever puppies, 13 weeks of age, were presented with congenital biliary cysts of the liver involving both hepatic and segmental bile ducts, as well as bilateral polycystic kidney disease. Ultrasonography of the livers of both pups demonstrated segmental cystic lesions that were contiguous with the bile ducts. Histopathology revealed cystic ectatic bile duct hyperplasia and dysplasia with variable portal fibrosis in the liver, while in the kidneys there were radially arranged, cylindrically dilated cysts of the collecting ducts, which extended through the medulla and cortex. This pathology was compatible with that of congenital dilatation of the large and segmental bile ducts (Caroli's disease) described in humans, dogs and rats. In humans Caroli's disease has an autosomal recessive inheritance pattern, while in rats activation of the MEK5/ERK cascade initiates the biliary dysgenesis of Caroli's disease in this species. However, the exact mode of inheritance and pathogenesis of Caroli's disease in dogs is as yet unknown. Previous reports on congenital hepatic cystic diseases of the dog have described Caroli's disease like lesions in various breeds, but these are believed to be the 1st reported cases in the Golden retriever breed.     

Congenital polycystic kidney disease in lambs

AIM: To describe the pathology and inheritance of a congenital polycystic kidney disease (PKD) of sheep.

METHODS: Mode of inheritance of PKD was investigated by evaluation of results of the disorder from planned matings in two consecutive years within subsets of a flock that had a high prevalence of PKD in lambs. Gross pathological and histopath- ological studies were based on tissues derived from this study. Haematoxylin and eosin (H&E)-stained paraffin sections of kidney, liver, extrahepatic biliary and pancreatic ducts, pancreas and epididymis were used to describe the lesions.

RESULTS: Twenty-five lambs affected by PKD, of both sexes, were born, numbers in accord with those expected for an autosomal recessive disorder in the population studied. In all cases for which tissues were available, the renal, bile ductal (intrahepatic and extrahepatic), pancreatic and epididymal tissues had widespread dysplastic changes and associated cyst formation.

CONCLUSIONS: The findings of renal cysts in conjunction with cysts in other organs are unifying features in many of the human and animal forms of PKD and suggest a related pathogenic and genetic base consistent with an autosomal recessive disorder.     

On the ultrastructure of epithelial cells in bile ducts of cattle chronically infected with fasciola hepatica

This paper describes briefly the normal ultrastructure of bile-duct epithelial cells in cattle and the main ultrastructural alterations produced in them by chronic infection with Fasciola hepatica. The studies showed only inconsiderable abnormalities in the ultrastructure of the epithelium in infections of a low degree as also during the recovery phase of the disease. On the other hand, in heavily infected livers pronounced ultrastructural alterations were seen in the surface epithelium of the main intrahepatic bile ducts invaded by the flukes. In such areas the intercellular spaces were filled with dense homogeneous material, and intact desmosomes and junctional complexes were not regularly seen, which indicates that foreign material may leak into the bile. The extracellular material was probably composed of albumin, and the significance of the present results regarding the etiology of hypoalbuminaemia during chronic fascioliasis is discussed.     

慢性肝病患者血清前白蛋白和总胆汁酸检测的临床价值

目的探讨慢性肝病患者血清前白蛋白(PAB)和总胆汁酸(TBA)检测的临床价值。方法分别检测慢性乙肝(轻度、中度、重度)、肝硬化(代偿期和失代偿期)患者及正常对照组的血清PAB、TBA及白蛋白(A)、总胆红素(TB IL)水平,并进行分析。结果各型慢性肝病患者血清PAB均显著低于对照组(P<0.05<或P<0.01),而血清TBA则明显高于对照组(P<0.05<或P<0.01)。轻、中度慢性乙型肝炎患者的血清PAB、TBA异常率均高于同组A和TB IL的异常率(P<0.05)。结论联合检测血清PAB和TBA有助于评估肝脏合成及代谢功能的早期损害。     

Proventricular dilatation disease: an emerging exotic disease of parrots in Australia

Proventricular dilatation disease is a viral disease seen as a segmental neuropathy in parrots. It has always been believed to be a disease exotic to Australia, with the only reported case being a legally imported Green Wing Macaw ( Ara chloroptera ) in 1993. This paper reports a cluster of cases seen in south-east Queensland in 2005 to 2006. Clinical signs, autopsy findings and histopathological findings are described. No pattern or common source for these cases could be identified. The implications for Australian aviculture and avifauna are discussed.     

兔肝内管道研究

兔肝内门管鞘系统所包含的门静脉分为左、右２支：左支供应左内叶、左外叶、右内叶及尾状叶，左外叶静脉可分出背、腹侧静脉，左内叶静脉及右内叶静脉各分出内、外侧静脉，尾状叶的静脉也分出左、右侧静脉；右支供应右外叶，分出右外叶静脉和右叶间静脉，右外叶静脉分出背、腹侧静脉。与人肝相似，兔肝内部也分为左、右２叶及左外段（叶）、左内段（叶）、右外段（叶）、右内段（叶）４段，尾状叶的左、右侧部分别隶属于左、右叶。兔肝的外形可分为左外叶、左内叶、右外叶、右内叶、尾状叶及位于胆囊左侧、门静脉左支横部腹侧及方叶支分布区的方叶。肝动脉和胆管的分支与门静脉的相应支伴行，但其分支形式较复杂。兔的肝静脉系统，除肝中静脉外，汇集各叶血液的肝大静脉还有左外叶肝静脉、左内叶肝静脉、右内叶肝静脉、右外叶肝静脉及尾状叶肝静脉，肝小静脉很少。     

Screening of bacterial DNA in bile sampled from healthy dogs and dogs suffering from liver- or gallbladder-associated disease

Although the biliary system is generally aseptic, gallbladder microbiota has been reported in humans and some animals apart from dogs. We screened and analyzed the bacterial deoxyribonucleic acid in canine gallbladders using bile sampled from 7 healthy dogs and 52 dogs with liver- or gallbladder-associated disease. PCR screening detected bacteria in 17.3% of diseased dogs (9/52) and none in healthy dogs. Microbiota analysis of PCR-positive samples showed that the microbial diversity differed between liver- and gallbladder-associated disease groups. Thus, a specific bacterial community appears to occur at a certain frequency in the bile of diseased dogs.     

Expression of neuronal nitric oxide synthase and renin in dysplastic kidneys of young dogs

Renin and neuronal nitric oxide synthase in the kidney control the renin-angiotensin and tubuloglomerular feedback systems. The present study investigated the expression of renin and neuronal nitric oxide synthase in the dysplastic kidneys of three young dogs. Renin-immunoreactivity, which occurs in the juxtaglomerular and tubular cells of dysplastic kidneys, did not differ from that in the normal kidneys of young dogs. Macula densa cells in the normal kidneys showed neuronal nitric oxide synthase -immunoreactivity, but those in the dysplastic kidneys showed no apparent signals. This observation may be correlated with the pathological mechanisms of renal failure in young dogs.     

Comparative study of chronic kidney disease in dogs and cats: Induction of myofibroblasts

We investigated the kidneys of dogs and cats to clarify whether renal myofibroblasts induction is associated with the severity of chronic kidney disease (CKD). Immunohistochemical expression of myofibroblast markers, α-smooth muscle actin (SMA) and vimentin, were evaluated quantitatively. The degrees of glomerulosclerosis, glomerular hypertrophy, interstitial cell infiltration, and interstitial fibrosis were also evaluated quantitatively. The plasma creatinine (pCre) concentrations correlated with glomerulosclerosis, cell infiltration, and fibrosis in dogs, and only with fibrosis in cats. The α-SMA expression correlated with pCre, glomerulosclerosis, cell infiltration, and fibrosis in dogs, and with pCre and fibrosis in cats. Tubular vimentin expression correlated with fibrosis in cats, but not in dogs. Interstitial vimentin expression correlated with pCre, glomerulosclerosis, cell infiltration, and fibrosis in dogs, but only with pCre in cats. In conclusion, it was suggested that the severity of CKD in dogs and cats was mediated by different pathways associated with myofibroblasts expression.     

Diagnostic value of fasting plasma ammonia and bile acid concentrations in the identification of portosystemic shunting in dogs

Portosystemic shunting occurs frequently either as congenital anomalies of the portal vein (PVA) or as acquired shunting (AS) due to portal hypertension secondary to parenchymal liver disease or portal vein thrombosis. The 2 most commonly used screening tests for portosystemic shunting are bile acid and plasma ammonia concentrations. The purpose of this study was to compare the 12-hour fasting plasma ammonia (AMM) and bile acid concentration (BA) as tests for diagnosing portosystemic shunting. Medical records of 337 dogs were used in which AMM and BA were measured simultaneously and in which portosystemic shunting was confirmed or excluded. These dogs were divided into 2 groups (group 1: portosystemic shunting present, n = 153, and group 2: portosystemic shunting absent, n = 184). Group 1 was subdivided into 2 subgroups (group 1a: PVA, n = 132 and group 1b: AS, n = 21). The sensitivity of AMM in detecting PVA was 100% and of BA was 92.2%. For portosystemic shunting in general (PVA or AS), the sensitivity of AMM was 98% and that of BA was 88.9%. The specificity in the total population of AMM was 89.1% and that of BA was 67.9%. If only dogs with liver diseases were included with (n = 153) or without (n = 28) shunting, the specificity of AMM to detect shunting was 89.3% and that of BA was 17.9%. In conclusion, AMM is a highly sensitive and specific parameter to detect PVA and portosystemic shunting in a general population and in dogs with liver disease, whereas BA is somewhat less sensitive and considerably less specific.     

Hemorrhagic bile pleuritis and peritonitis secondary to traumatic common bile duct rupture,diaphragmatic tear,and rupture of the spleen in a dog

Objective– To describe the diagnosis and successful treatment of bile pleuritis and peritonitis secondary to traumatic rupture of the common bile duct and a diaphragmatic tear in a young dog. Case Summary– A 1‐year‐old German Shepherd dog was referred for evaluation of vomiting and icterus 4 days after being hit by a car. Thoracic radiographs, thoracic and abdominal ultrasonographic examinations, thoraco‐ and abdominocentesis, and positive contrast celiogram indicated hemorrhagic pleuritis and peritonitis, left dorsal diaphragmatic tear, and rupture and infarct of the spleen. Surgical exploration of the abdomen confirmed these findings in addition to a circumferential tear of the common bile duct, leading to a diagnosis of hemorrhagic bile pleuritis and peritonitis. Aerobic and anaerobic bacterial culture of the abdominal fluid yielded no growth. Surgical correction of the traumatic injuries was achieved via common bile duct anastomosis, cholecystojejunostomy, repair of the diaphragm, and splenectomy. The dog developed postoperative signs consistent with aspiration pneumonia but was successfully treated and discharged from the hospital. Clinical signs and laboratory abnormalities resolved and the dog was alive and healthy 8 months after discharge. New or Unique Information Provided– Bile pleuritis is rare in dogs and cats and is usually associated with penetrating, not blunt, abdominal trauma. Multiple organ injury in cases of traumatic bile duct rupture is uncommon; in this dog, rupture of the common bile duct was accompanied by rupture of the diaphragm and spleen.     

Congenital nutritional myodegeneration (white muscle disease) in a red deer (Cervus elaphus) calf

CASE HISTORY A 5-day-old red deer calf was submitted with tachypnoea and dyspnoea, and was reluctant to move.

CLINICAL FINDINGS: Muscular damage was established via elevated creatinine phosphokinase (CPK) activities (5,000 U/L), while concentrations of Se in whole blood were low (24.8 nmol/L). The animal died despite treatment with penicillin-streptomycin and 0.1 mg/kg Se/vitamin E administered by S/C injection.

DIAGNOSIS: Necropsy and histological examination of cardiac and skeletal muscle confirmed the presumptive diagnosis of congenital white muscle disease (WMD). Prophylactic administration of a Se/vitamin E commercial preparation (as above) to another calf born in the same herd one month later was associated with good health and apparently normal growth and development.

CLINICAL RELEVANCE: Congenital WMD due to Se deficiency can be fatal in red deer calves. However, prophylactic administration of Se and vitamin E to neonatal calves may be beneficial for neonatal red deer calves.     

Evaluation of albuminuria and its relationship with blood pressure in dogs with chronic kidney disease

Background: Microalbuminuria and hypertension have long been associated with a guarded prognosis in human patients with a variety of diseases. In veterinary medicine, tests for microalbuminuria have been used for detecting early kidney damage, but there is little information regarding its association with high blood pressure in dogs with chronic kidney disease (CKD). Objective: The objective of this study was to evaluate albuminuria and its association with arterial hypertension in dogs with CKD. Methods: Urinary albumin:creatinine (UAC) ratio, urinary protein:creatinine (UPC) ratio, and systolic blood pressure were determined in 39 clinically healthy dogs and 40 dogs with CKD. Results: UAC in dogs with CKD (range, 0.002–7.99; median, 0.38) was statistically different from that of control dogs (range, 0.0005–0.01; median, 0.002). Microalbuminuria (UAC 0.03–0.3) and macroalbuminuria (UAC>0.3) were detected in 32.5% and 50% of dogs with CKD, respectively. Sixty percent (24/40) of dogs with CKD had systolic pressure ≥180 mmHg; in these dogs, UAC ratio (range, 0.006–7.99; median, 1.72) was significantly higher than in dogs with CKD and systolic pressure<180 mmHg (range, 0.002–4.83; median, 0.10). Of hypertensive dogs with CKD, those with UPC>1.0 usually had macroalbuminuria, those with UPC 0.5–1.0 usually had microalbuminuria, and those with UPC<0.5 usually lacked albuminuria. Conclusions: UAC ratio was higher in hypertensive than in normotensive dogs with CKD. Tests designed to detect microalbuminuria may be useful for hypertensive dogs with CKD and a UPC≤1.0 to detect the onset and magnitude of albuminuria. Once macroalbuminuria is overt, the UPC ratio itself can be used for the same purpose.     

Sensitivity and specificity of fasting ammonia and serum bile acids in the diagnosis of portosystemic shunts in dogs and cats

Background: Portosystemic shunt (PSS) is the most common cause of hepatic encephalopathy in dogs and cats. Fasting ammonia and serum bile acids (SBA) are used to diagnose PSS, but their true sensitivity and specificity have not been fully evaluated, especially in cats. Objectives: The purpose of this study was to determine the diagnostic accuracy of fasting ammonia and SBA concentrations in the diagnosis of PSS in dogs and cats and to compare diagnostic accuracy between species. Methods: A retrospective analysis of data from 373 dogs and 85 cats presented to the clinic from 1996 to 2006 was carried out. Based on clinical, laboratory, and imaging findings, animals were grouped as having PSS, parenchymal hepatic disease, or extrahepatic disease. The sensitivity and specificity of ammonia and SBA concentrations for the diagnosis of PSS were calculated and receiver‐operating characteristic analysis was used to optimize cut‐offs. Results: Using the upper limit of laboratory reference intervals (ammonia, 59 μmol/L; SBA, 20 μmol/L), the sensitivity and specificity of ammonia was 85% and 86% in dogs, and 83% and 76% in cats, respectively. The sensitivity and specificity of SBA was 93% and 67% in dogs, and 100% and 71% in cats, respectively. Using optimal cut‐off points for ammonia (dogs, 57 μmol/L; cats, 94 μmol/L) the sensitivity and specificity was 91% and 84% in dogs and 83% and 86% in cats, respectively. Using optimal cut‐off points for SBA (dogs, 58 μmol/L; cats, 34 μmol/L) the sensitivity and specificity was 78% and 87% in dogs and 100% and 84% in cats. Conclusion: Increased fasting ammonia and SBA concentrations are accurate indicators of PSS. An improvement in diagnostic accuracy can be achieved by using defined optimal cut‐off points for the selective diagnosis of PSS.     

Survival in cats with naturally occurring chronic kidney disease (2000-2002)

Background: Duration of survival of cats with naturally occurring chronic kidney disease (CKD) is poorly characterized.
Hypothesis: Stage of kidney disease based on serum creatinine concentration (SCr) at the time of diagnosis and after correction of prerenal azotemia is strongly associated with duration of survival in cats.
Animals: Two hundred and eleven client-owned cats with naturally occurring CKD evaluated between April 2000 and January 2002.
Methods: Retrospective case review of 733 cats with SCr > 2.3 mg/dL. Examination of the medical records identified 211 cats that met all other inclusion and exclusion criteria for this study. Clinical characteristics, clinicopathologic data, and survival times were extracted from the medical record. Owners and referring veterinarians were contacted by phone to obtain follow-up if it was not documented in the record. Kaplan-Meier survival curves were performed to determine survival times for International Renal Interest Society (IRIS) stage both at diagnosis and at baseline (ie, after correction of prerenal azotemia).
Results: Median survival for cats in IRIS stage IIb at the time of diagnosis was 1,151 days (range 2–3,107), and was longer than survival in stage III (median 778, range 22–2,100) or stage IV (median 103, range 1–1,920) ( P -value < .0001). P -value for effect of stage at diagnosis was <.0001.
Conclusions and Clinical Importance: IRIS stage of CKD based on serum creatinine at the time of diagnosis is strongly predictive of survival in cats with naturally occurring CKD.     

Copper-associated liver disease in Dalmatians: a review of 10 dogs (1998-2001)

This retrospective study summarizes 10 Dalmatians suspected of having hepatic copper toxicosis. Hepatic copper toxicosis can result from either a primary metabolic defect in hepatic copper metabolism or from altered hepatic biliary excretion of copper. An inherited copper-associated hepatopathy has been documented in Bedlington Terriers, and there is evidence for familial copper-associated liver disease in West Highland White (WHW) Terriers and Skye Terriers. Nine of the 10 Dalmatians in this study presented for gastrointestinal clinical signs, including anorexia and vomiting. All animals had increased alanine aminotransferase (ALT) enzyme activity, and 9 of 10 had increased alkaline phosphatase (ALP) enzyme activity. The relative increase in ALT activity was much greater than the relative increase in ALP activity, suggesting a predominantly hepatocellular rather than cholestatic liver disease. The mean hepatic copper concentration for 9 Dalmatians was 3,197 microg/g dry weight liver (dwl) (normal, <450 microg/g). In 5 of these 9 dogs, hepatic copper concentrations exceeded 2,000 microg/g dwl. Necroinflammatory alterations associated with copper-laden parenchymal cells were the notable histopathologic finding. The inflammatory infiltrate was either primarily lymphocytic or neutrophilic. Morphologic features of cholestasis generally were not prominent except in those dogs with severe pathology. These findings lend support to the hypothesis that a primary metabolic defect in hepatic copper metabolism occurs in the Dalmatian breed. The mechanism and genetic basis of this condition require further study.