Osgood-Schlatter Disease

This is ostreochondritis at the point of insertion of the patellar tendon of the upper tibia. It commonly affects boys and results in pain below the knee. The pain is worse with movement and improves with rest. There is usually swelling and tenderness. The X-ray shows fragmentation of the tibial tubercle because of partial avascular necrosis. The disease is self-limiting so that it improves when bone growth stops. Treatments is symptomatic with avoidance of maneuvers that bring of the pain. This includes cycling and basketball where quadriceps contraction stresses the tendon. Activity can be resumed when the pain resumed.

Legg-Calves-Perthes

This is a rare condition (1:18.000 live births) and occurs mostly in Caucasian children aged 4-9 years with a sex predominance of boys (5:1). It is avascular necrosis of the femoral head of unknown cause. It is thought to be related to repeated trauma to the femoral head, which already has poor vascularization. It is more common in children with delayed growth and is bilateral in 10-18 percent of cases. Examination shows limitation of hip movement and X-rays of the hips show the abnormal femoral head. It is important to consinder the diagnosis in a child who has a limp. Treatment involves physical therapy and reduction of weight bearing.

Orthopedic Problems

Orthopedic Problems in children are common and are present in many chronic illnesses. A common problem in pediatrics is gait disturbance. Difficulty walking may be caused by various disorders. Some of the conditions are more common at specific ages. Legg-Calves-Perthes disease occurs from 4-9 years of age; slipped capital femoral epiphysis from 10-17 years of age. Congenital dislocation of the tip is more common in females (6:1).

Bone Marrow Transplantation

Bone Marrow Transplantation is increasingly being performed for several hematologic, oncologic, metabolic and genetic disorders. The healthy bone marrow may be taken from patient (autograft) or it may be taken from a donor (homograft). Donated bone marrow must match the patient's tissue type. The donor may be a living realtive (usually a brother or a sister-allogeneic) or from an unrelated donor fund through the national marrow donor program which lists more than 700.000 potential donors. Donors are matched through HLA tissue typing.

Indication for bone marrow transplant include blood cell disorders, especially aplastic anemia, leukemia or lymphoma, as a part of aggressive cancer treatments (chemotherapy, radiation therapy), and many immunodeficiency syndromes. It may be useful in managing hereditary hemoglobinopathies and metaboliv storage diseases.

Liver Transplantation

Various surgical techniques have been developed to address the problem of the lack of cadaver livers. Reduced-size or cutdown-and-split liver transplants, in which only a portion of a liver is utilized for the transplant, have been successful. Biliary atresia accounts for at least 50 percent of pediatric patients undergoing liver transplantation. The second most common reason for transplantation is liver disease due to inborn errors of metabolism, with alpha1-antitrypsin deficiency being the most common, and others including tyrosinemia and Wilson’s disease.

Lung Transplantation

Pediatric lung tranplants are typicall performed on cystic fibrosis (CF) patients who make up about 70 percent of pediatric lung tranplant patients. Primary pulmonary hypertension, interstitial lung disease, and bronchopulmonary  dysplasia make up the rest of pediatric lung transplant population.

Dobule-lung tranplants is bilateral, sequential, single-lung tranplant wich means that one lung is removed and then replaced and then second lung is removed and replaced. Both does not develop in the transplanted lung. One-year survival approaches 90 percent and 5-year survival is about 65 pecent. Living-lobar tranplantation involves replacing the diseased lungs with lobes from a living donor.

Heart Transplantation

About 75 percent of children requiring heart transplant under 1 year have serious congenital heart defects that cannot be repaired by corrective surgery. The remaining 25 percent have cardiomyopathy. Of children aged 1 to 5 years, half have inoperable congenital heart disease and half have myocarditis of cardiomypathy. Of patients over 5 years , more than 60 oercent require transplant for cardiomyophaty and less than 40 percent for congenital heart disease.

Kidney Transplantation

There is an almost 40-years history of renal transplantation. Kidney transplants are the second most common organ transplant (corneal transplant is number 1) in the United States with over 9.000 cases (adult and pediatric) per year. Indications for pediatric renal transplantation include symptoms of uremia not responsive to standard therapy, failure to thrive, delayed psychomotor development, fluid overload, and metabolic bone disease due to renal osteodystrophy. There are many kidney disorders that result in end-stage renal disease but the major indication in chronic glomerulonephritis.

Between 80-90 percent of transplantation kidneys are functioning 2 years after operation. The main problem is graft  rejection. Living-related 1 year graft survival is 90 percent, with 85 percent 2-year and 75 percent 5-year survival. The corresponding figures for cadaver transplants are 76, 71 and 62 (1992). The half-life of cadaveric kidney is about 8 years. This compares poorly with the average of 12 and 26 years for living-donor kidneys matched for one and two haplotypes (tissue-typing factors) respectively.

The longer a patient with kidney disease remains on dialysis while waiting for a kidney transplant, the more likely the patient is to die prematurely even after receiving a new kidney.

Organ Transplantation

Children with chronic medical problems may have an increased need for surgery. Surgery may be needed to improve the underlying chronic condition or may be for causes independent of the chronic condition. For example , appendicitis may affect a child with asthma or diabetes.

The most common surgical procedures for children are repair of inguinal hernia, orchiopexy, and placements of ear tubes. Most of these procedures are performed under general anesthesia on an out-patient bases. Children with chronic disorders may have anesthetic risks beyond those of normal children so it may be prudent in some cases to perform the procedures as in-patients.

End-stage failure of some organ systems may be amenable to replacement. Kidney, heart, lung, and liver are the predominant organs that have been transplanted in children. Additional transplants include pancreas, small intestine, and increasingly multiple organ transplants are being performed. Patients accepted onto a hospital's tranplant waiting list are registered with the United Network for Organ Sharing, Organ Center where  a centralized computer network links all organ procurement organizations and transplants centers. Various factors are involved for donor selection including blood type, tissue type, and size of the organ, and each organ has specific criteria.

In 1997, the UNOS reported 3.565 children and 76.526 adults were waiting for organ transplants. According to the International Pediatric Transplant Association, children in the USA receive 11 percent of total heart transplants, 7 percent of lung transplants, 13 percent of liver transplants, and about 5 percent of kidney transplants. A higher percentage of children than adults die waiting for transplant.

The importance of immunosuppression regimens in the improved survival of transplants cannot be overstressed. Regimens usualy involve prednisone, cyclosporine or tacrolimus, and mycophenolate mofetil of azathioprine. The major complications include infection, rejection, and malignancy, in addition to surgical problems.