*Please comment on any abnormalities, prognosis, and treatment.
Image #1 Transverse Right Liver
Image #2 Transverse Left Liver
Image #3 Linear Liver
Image #4 Linear Liver
Image #5 Sagittal Spleen with Kidney
Image #6 Sagittal Left Kidney with measurement
Image #7 Linear Left Kidney
Image #8 Sagittal Right Kidney
Image #9 Sagittal Right Kidney with measurement
Image #10 Linear Right Kidney
11 comments:
Leah,
Her spleen and kidneys are enlarged according to the measurements. The kidneys appear to have alot of anechoic areas in it so i will think she has polycystic kidney disease. She also have a fatty liver looking at the liver images.
Irene,
Yes! You are on the right track. The spleen and kidneys are enlarged. What type of kidney disease do you think she has? Are the findings related? What else is seen?
Leah
I believe this child has Autosomal Recessive Polycystic Kidney Disease (ARPKD) and Congenital Hepatic Fibrosis (CHF). "ARPKD is a chronic progressive disease that results in kidney failure and liver abnormalities and affects those from birth to adolescence." "Hypertension may occur in up to 80% of children and can be life threatening without agreesive treatment. 60-100% of patients have palpably enlarged kidneys." "CHF is associated with biliary ectasia and scaring of the liver in the portal tracts and always accompanies ARPKD." Portal hypertension and varices often result and corresponding splenomegaly.
source:www.arpkdchf.org/documents/What%20is%20ARPKD-CHF.pdf
"Congenital Hepatic Fibrosis (CHF) is a rare disease that affects both the liver and kidneys. The patient is born with this disorder (congenital), and it is inherited as an autosomal recessive trait. The typical liver abnormalities are an enlarged liver (hepatomegaly), increased pressure in the venous system that carries blood from different organs to the liver (portal hypertension), and fiber-like connective tissue that spreads over and through the liver (hepatic fibrosis). Affected individuals also have impaired renal function, usually caused, in children and teenagers, by an autosomal recessive polycystic kidney disease (ARPKD). Impaired renal function associated with CHF in adults is caused by an autosomal dominant polycystic kidney disease (ADPKD)".http://www.cigna.com/healthinfo/nord860.html
I was wondering why liver texture is like the one who had fibrosis of breast tissue
The spleen is also enlarged due to portal hypertension
It's interesting case leah,,
Alexia, Yes, that is the specific type of kidney disease and you have correctly related it to hepatic fibrosis. Grace, you also provided important information and are correct about hepatomegaly and splenomegaly being associated findings. Thanks for sharing info about breast fibrosis!
Leah
An interesting case, it is sad this patient is so young. A variety of symptoms occur as a consquence of ARPKD, including high blood pressure, as was mentioned. Treatment for this condition "may delay the progression of the disease and slow further kidney damage. Combining a low-sodium, low-fat diet that's moderate in protein and calorie content with eliminating smoking, increasing exercise and reducing stress may help control high blood pressure." Even with these preventive measures, "medications are usually needed to control high blood pressure. Medications called angiotensin-converting enzyme (ACE) inhibitors may be used to control high blood pressure...though more than one drug may be necessary for good blood pressure control."
Other symptoms and complications of ARPKD include chronic pain that ranges from mild to severe, cyst complications (such as pain, obstruction of vessels/other organs), bladder/kidney infections, hematuria (can form obstructive blood clots), kidney failure, liver cysts, and intracranial aneurysms (secondary to elevated blood pressure).
http://www.mayoclinic.com/health/polycystic-kidney-disease/DS00245/DSECTION=treatments-and-drugs
Hi, Leah
When I first saw your pictures, I could not recognized either right or left kidney at all. The liver texture looked a little coarse, but anyway I could recognize it right away. However, it took me a while to notice that both kidneys had lots of small cysts all over them. It is a real case of polycystic kidney disease. The size of the kidneys are very large! Thank you for sharing your great case study with us.
In infants with ARPKD who survive mechanical venitlation have a good chance of survival. The 5 year survival rate is 80-95% for those who survive the neonatal period (pertaining to first 4 weeks of life). Because of continued improvements in mechanical ventilation, neonatal support, control of both systemic and portal hypertension, management of End Stage Renal Disease treatment and transplantation, the ARPKD population is now living well into adulthood.
- arpkd.org/documents/What%20is%20ARPKD-CHF.pdf
Good job, everyone! This girl has ARPKD. It's associated with portal hypertension, enlarged kidneys, and enlarged spleen. Renal function is often impaired and liver function, as well. It is a chornic and progressive disease that causes eventual kidney failure and liver abnormalities, hence Congenital Hepatic Fibrosis. This girl is predicted to need a new liver within the next 3 years. She just had a splenorenal shunt placed. The vein from the spleen was disconnected from the portal vein and reconnected to the top of the left renal vein. The left gastric vein is disconnected from the portal vein and tied off. The blood flows from the varices through the splenic vein, to the left renal vein, and empties into the IVC. The blood flow to the liver is through the portal vein.
Great job!
Zouliath said
Autosomal Recessive Polycystic Kidney Disease (ARPKD) is a rare genetic disorder that affects approximately 1:6,000 to 1:40,000 persons in the general population, depending on the source of reference. (There is a higher incidence in certain populations, i.e. Finnish and Afrikaaner.) It is a chronic and progressive disease that causes eventual kidney failure and liver abnormalities, hence Congenital Hepatic Fibrosis (CHF). It is neither contagious, nor affects intelligence. CHF has the potential to cause severe clinical liver complications. The age spectrum for onset of symptoms is from birth to adolescence, seldom adulthood. ARPKD is commonly diagnosed early in life; approximately 50% are diagnosed prenatally.
There is no cure and early newborn death occurs up to 50% of the time, most often not from kidney failure, but from pulmonary hypoplasia.
Patients With polycystic kidneys can also have kidney tumors and cysts in the liver, spleen pancreas, and virtually anywhere.
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