Clinical Care Considerations

The focus is to keep healthy and growing, treating manifestations as they occur. The accumulation of information here does NOT include all dimensions of clinical healthcare, does NOT provide medical advice, but is a resource and a continuous “work in progress”, as we learn more about comprehensive, multi- disciplinary clinical care options.


Offer mechanical ventilation and support at birth to distinguish those with true pulmonary hypoplasia.


High blood pressure is often present during the first year of life. Poorly controlled hypertension is a major factor in premature renal function deterioration and if untreated may also lead to heart failure.

Drugs available:

• Angiotensin Converting Enzyme Inhibitors or ACE Inhibitors

• Calcium Channel Blockers

• Diuretics


For unknown reasons, approximately 25% of the children will experience FTT.

• Utilize anti-reflux measures

• In carefully evaluated and selected children, enteral or parenteral supplementation may be useful

• Growth hormone does work


Because of the potential for blunt trauma or serious injury to enlarged organs, contact sports should be avoided, including football, wrestling, hockey, kick boxing, boxing, karate, and lacrosse. Sports like youth soccer, baseball, volleyball, and basketball are generally safe (although elbowing can be dangerous).

A spleen protector is not scientifically proven, and doesn’t provide zero risk for activities such as bicycle riding (potential for handle bar injury), soccer and skiing, but if fitted and worn properly, provides a significant barrier to protect organs.

UTI (Urinary Tract Infection):

Abnormal urinalysis does not always indicate infection.

• Culture and treat infections promptly, utilizing antibiotic regimens

• Aggressive treatment may prevent additional scarring

• Avoid instrumentation of GU tract


Ascending cholangitis can be a serious life-threatening complication.

Children with fever for 3 days without an identifiable source should be worked up for cholangitis. The evaluation includes blood cultures and one may consider a liver biopsy or aspiration for culture.

• Requires aggressive IV antibiotic therapy.

• Ursodeoxycholic acid (ursodiol) may assist in moving bile and may prevent bile stasis

HYPERSPLENISM: An exaggeration of the hemolytic (blood destruction) function of the spleen, resulting in deficiency of blood elements. Potential treatment may be needed for:

• Thrombocytopenia -low blood platelet count, the “sticky factors” of the blood utilized for blood clotting.

• Anema – low red blood count, oxygen carrying component of blood. Low results may be to due to hypersplenism or chronic renal failure. (Epogen replacement therapy is available).

• Leukopenia -low blood leukocyte level, function is complicated but chiefly fights bacteria and microorganisms.

• Coagulopathy-such as high prothrombin or PT level (vitamin-K supplements available).


(The following information for Portal Hypertension and Care of Bleeding Esophageal Varices is by Dr. David Piccoli, Division Chief, Pediatric Gastroenterology and Nutrition, The Children’s Hospital of Philadelphia.)

• Medical Therapy: Designed to decrease the pressure and volume in the portal system.

• Sclerotherapy: Injection of the esophageal varices to clot them and thicken the wall, so that flow will go in a different direction.

• Variceal Ligation (Banding): Placing rubber band ligatures around the dilated veins to help them clot and block flow. There is some evidence that variceal ligation has advantages over sclerotherapy in certain situations.

• Surgical Shunts: Taking the high pressure portal or splenic blood flow and plugging it into low pressure vessels.

• Radiologic Shunts: TIPS- making a connection between the high pressure portal vein and the low pressure hepatic vein.

• Liver Transplantation

This article was written by Colleen B. Zak, RN with Dr. Kevin E. Meyers, M.D., Assistant Divisional Chief of Nephrology at The Children’s Hospital of Philadelphia and is copyrighted. Reprint is permitted if kept intact and authors and the ARPKD/CHF Alliance are given credit.
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