Monitoring recommendations for patients with PKD according to age group
| Study . | Children (<18 y) . | Adults (≥18 y) . |
|---|---|---|
| Complete blood counts, reticulocyte count, and bilirubin | At least annually, more often depending on hemolytic episodes and transfusion needs | At least annually, more often depending on hemolytic episodes and transfusion needs |
| Serum ferritin and TS* | Every 3-6 mo in RT | Every 3-6 mo in RT |
| Annually in NRT | Annually in NRT | |
| Every 1-3 mo while on chelation | Every 1-3 mo while on chelation | |
| Liver iron concentration† | In RT, first MRI after 10-14 transfusions and then annually | Annually in RT |
| In NRT, first MRI, if available, when patient can have an unsedated study, particularly if ferritin >500 μg/L. Follow-up MRI studies: annually if >5 mg/g, every 5 y if <5 mg/g. | In NRT, MRI frequency, if available, based on the following: annually if >5 mg/g, every 5 y if <5 mg/g. | |
| Abdominal US | Consider first right upper quadrant US after age 2 y, then every 2-3 y in childhood or until cholecystectomy. After cholecystectomy, consider every 2-3 y if evidence of intrahepatic cholestasis. | Right upper quadrant US every 2-3 y or until cholecystectomy. After cholecystectomy, every 2-3 y if evidence of intrahepatic cholestasis. |
| US should be obtained prior to splenectomy. If undergoing splenectomy, cholecystectomy should be considered, even in the absence of gallstones. | US should be obtained prior to splenectomy. If undergoing splenectomy, cholecystectomy should be considered, even in the absence of gallstones. | |
| DEXA scan | Consider first DEXA scan between ages 16 and 18 y, then annually if low bone density. Evaluate 25-hydroxyvitamin D levels. | Annually if osteopenic. Evaluate 25-hydroxyvitamin D levels. In nonosteopenic patients, bone mineral density can be assessed at different intervals according to age and sex. |
| Viral hepatitis serology | Annually in RT | Annually in RT |
| Endocrinopathy panel (thyroid hormone, sex hormones, fructosamine) | Annually if RT or if significant iron overload | |
| Echocardiogram | Consider if age >30 y, prior to pregnancy, and at any age if concern for cardiac dysfunction and/or pulmonary hypertension |
| Study . | Children (<18 y) . | Adults (≥18 y) . |
|---|---|---|
| Complete blood counts, reticulocyte count, and bilirubin | At least annually, more often depending on hemolytic episodes and transfusion needs | At least annually, more often depending on hemolytic episodes and transfusion needs |
| Serum ferritin and TS* | Every 3-6 mo in RT | Every 3-6 mo in RT |
| Annually in NRT | Annually in NRT | |
| Every 1-3 mo while on chelation | Every 1-3 mo while on chelation | |
| Liver iron concentration† | In RT, first MRI after 10-14 transfusions and then annually | Annually in RT |
| In NRT, first MRI, if available, when patient can have an unsedated study, particularly if ferritin >500 μg/L. Follow-up MRI studies: annually if >5 mg/g, every 5 y if <5 mg/g. | In NRT, MRI frequency, if available, based on the following: annually if >5 mg/g, every 5 y if <5 mg/g. | |
| Abdominal US | Consider first right upper quadrant US after age 2 y, then every 2-3 y in childhood or until cholecystectomy. After cholecystectomy, consider every 2-3 y if evidence of intrahepatic cholestasis. | Right upper quadrant US every 2-3 y or until cholecystectomy. After cholecystectomy, every 2-3 y if evidence of intrahepatic cholestasis. |
| US should be obtained prior to splenectomy. If undergoing splenectomy, cholecystectomy should be considered, even in the absence of gallstones. | US should be obtained prior to splenectomy. If undergoing splenectomy, cholecystectomy should be considered, even in the absence of gallstones. | |
| DEXA scan | Consider first DEXA scan between ages 16 and 18 y, then annually if low bone density. Evaluate 25-hydroxyvitamin D levels. | Annually if osteopenic. Evaluate 25-hydroxyvitamin D levels. In nonosteopenic patients, bone mineral density can be assessed at different intervals according to age and sex. |
| Viral hepatitis serology | Annually in RT | Annually in RT |
| Endocrinopathy panel (thyroid hormone, sex hormones, fructosamine) | Annually if RT or if significant iron overload | |
| Echocardiogram | Consider if age >30 y, prior to pregnancy, and at any age if concern for cardiac dysfunction and/or pulmonary hypertension |
DEXA, dual-energy x-ray absorptiometry; NRT, nonregularly transfused patients (<6 transfusions per year); RT, regularly transfused patients (≥6 transfusions per year); TS, transferrin saturation; US, ultrasound.
Iron overload can be defined as liver iron concentration > 5 mg/g or serum ferritin > 800 μg/L and TS > 60% (if T2* MRI not available)
Determined by T2* MRI.