Peds test
Perry: Maternal Child Nursing Care, 7th Edition
Chapter 47: The Child With Endocrine Dysfunction
Key Points
The endocrine system has three components: the cells, which send chemicals message via hormones; target cells or organs, which receive the message; and the environment through which the chemical is transported from the site of synthesis to the sites of cellular action.
Hypopituitarism is diminished or deficient secretion of one or more anterior pituitary hormones. The consequences can lead to the following: gonadotropin deficiency with absence or regression of secondary sex characteristics; growth hormone (GH) deficiency, in which children display stunted somatic growth; thyroid-stimulating hormone deficiency; and adrenocorticotropin deficiency.
The most common organic cause of pituitary undersecretion is tumors in the pituitary or hypothalamic region
Pituitary hyperfunction can cause excess GH before closure of the epiphyseal shafts. This results in proportional overgrowth of the long bones. Vertical growth is accompanied by rapid and increased development of muscles and viscera. If oversecretion of GH occurs after epiphyseal closure, growth is in the transverse direction, producing a condition known as acromegaly.
Manifestations of sexual development before age 9 years in boys or age 8 years in girls have traditionally been considered precocious development, and these children were recommended for further evaluation.
The principal disorder of posterior pituitary hypofunction is diabetes insipidus (DI), also known as neurogenic DI or central DI, resulting from undersecretion of antidiuretic hormone (ADH), or vasopressin, and producing a state of uncontrolled diuresis.
The disorder that results from hypersecretion of ADH from the posterior pituitary hormone is known as syndrome of inappropriate ADH secretion (SIADH). It is observed with increased frequency in a variety of conditions (those involving infections, tumors, or other central nervous system (CNS) disease or trauma), and is one of the most common causes of hyponatremia in the pediatric population.
The main physiologic action of thyroid (TH) is to regulate the basal metabolic rate and control the processes of growth and tissue differentiation.
Disorders of thyroid function include hypothyroidism, lymphocytic thyroiditis, goiter, and hyperthyroidism.
After diagnosis and implementation of thyroxine therapy for hypothyroidism, the importance of compliance and periodic monitoring of response to therapy should be stressed to parents.
Therapy for hyperthyroidism is directed at slowing the rate of hormone secretion and may include antithyroid drugs, subtotal thyroidectomy, or ablation with radioiodine.
Hypoparathyroidism can be inherited or acquired and is rare in children. Impaired secretion of parathyroid hormone (PTH) results in hypocalcemia and hyperphosphatemia.
The adrenal cortex secretes three important groups of hormones: glucocorticoids, mineralocorticoids, and sex steroids.
Disorders of adrenal function include acute adrenocortical insufficiency, primary adrenal insufficiency (Addison disease), Cushing syndrome, and CAH.
Five categories of Cushing syndrome are pituitary, adrenal, ectopic, iatrogenic, and food dependent.
Management of CAH includes assignment of a sex according to genotype; administration of cortisone; and, possibly, reconstructive surgery.
Diabetes mellitus (DM) is categorized as type 1 diabetes and type 2.
Type 1 DM is characterized by destruction of the pancreatic β cells, which produce insulin; this usually leads to absolute insulin deficiency. Type 1 DM has two forms. Immune-mediated DM results from an autoimmune destruction of the β cells. The focus of type 1 DM is insulin replacement, diet, and exercise.
Type 2 DM usually arises because of insulin resistance in which the body fails to use insulin properly combined with relative (rather than absolute) insulin deficiency. People with type 2 DM can range from predominantly insulin resistant with relative insulin deficiency to predominantly deficient in insulin secretion with some insulin resistance.
Education of families includes explanation of DM, meal planning, administering insulin injections, monitoring general hygienic practices, promoting exercise, record keeping, and observing for complications.
Management of DKA includes fluid and electrolyte therapy.
Few situations in nursing exceed the challenges of caring for a child with cancer. Nurses should base support of patients and their families on the premise that with clear communication and compassionate care, fear diminishes, hope emerges, and the cancer journey feels less overwhelming.
The incidence of specific types of childhood cancer can vary according to age, sex, race, or ethnicity. Approximately 1700 children younger than 20 years old die from their disease each year, making cancer the leading cause of death from disease in this age-group.
Characteristics such as birth weight, advanced parental age, and congenital anomalies (chromosome abnormalities) have been found to be risk factors for childhood cancer. A small percentage of cancer occurs in children with cancer predisposition syndromes. Germline mutations are passed from parents to children and point to genetic mechanisms in childhood cancer etiology.
Known risk factors for childhood cancers include exposure to ionizing radiation, carcinogens (cigarette smoking, ultraviolet radiation, etc.), immunosuppressive therapy, infections (such as, Epstein Barr virus), race, and genetic conditions.
Diagnostic evaluation of a child suspected of having cancer may include laboratory tests, diagnostic procedures, diagnostic imaging, and pathologic and molecular evaluation.
The use of multimodal therapy consisting of surgery, chemotherapy, biotherapy, HSCT, and radiotherapy; enrollment of large numbers of children in cooperative group clinical trials or protocols; and improvements in supportive care have greatly increased the survival of children with cancer. Three year survival rates now exceed 80%.
Treatment modalities for childhood cancers include surgery, chemotherapy, radiation therapy, biologic therapy, and blood or bone marrow transplantation.
Tumor lysis syndrome is a pediatric oncologic emergency that occurs in malignancies that have high WBC count at diagnosis, a large tumor burden, are very sensitive to chemotherapy, or have a rapid proliferative rate. Other oncologic emergencies include hyperleukocytosis, superior vena cava syndrome, spinal cord compression, and disseminated intravascular coagulation.
The nursing care management of the child with cancer involves educating the child and family on disease and treatment; child and family safely perform self-care as appropriate; administering treatment on schedule with appropriate drug doses; managing the side effects of treatment; preventing treatment complications; supporting child and family coping skills; maintaining quality of life during treatment; ensuring the child and family adjust to chronic illness; and maintaining growth and development during treatment.
Possible signs and symptoms of childhood cancer are unusual mass or swelling, unexplained paleness and loss of energy, easy bruising, persistent/localizing pain or limping, prolonged/unexplained fever or illness, frequent headaches often with vomiting, sudden eye or vision changes, and unexplained rapid weight loss.
A major concern for the child receiving treatment for cancer is the risk for the development of complications secondary to the treatment. Infection, hemorrhage, anemia, nausea and vomiting, altered nutrition, neurologic problems, hemorrhagic cystitis, alopecia, and mucosal ulceration are all examples of complications and side effects.
Health promotion of the child with cancer includes dental care, immunizations, isolation to protect against communicable disease exposure, and patient and family education.
Leukemia is a broad term given to a group of malignant diseases of the bone marrow and lymphatic system. The two forms most prevalent in children are acute lymphoblastic leukemia (ALL) and acute myeloid leukemia (AML).
In all types of leukemia, the proliferating cells depress bone marrow production of the formed elements of the blood by competing for and depriving the normal cells of the essential nutrients for metabolism. The three main consequences are (1) anemia from decreased erythrocytes, (2) infection from neutropenia, and (3) bleeding from decreased platelet production.
Four types of standard treatment are used for leukemia: chemotherapy, radiation therapy, chemotherapy with stem cell transplant, and targeted therapy.
The lymphomas, a group of neoplastic diseases that arise from the lymphoid and hematopoietic systems, are divided into Hodgkin disease and NHL. Hodgkin disease is characterized by painless enlargement of lymph nodes. The most common finding is enlarged, firm, nontender, movable nodes in the supraclavicular or cervical area. Primary treatment involves chemotherapy and radiation.
Most children with non-Hodgkin lymphoma (NHL) are treated with combination chemotherapy, and sometimes radiation. Similar to leukemic therapy, the protocols include induction, consolidation, and maintenance phases, some with intrathecal chemotherapy.
Tumors of the CNS are the most common solid tumor in children and account for about 25% of all childhood cancers. The signs and symptoms of brain tumors are directly related to their anatomic location, size, rate of growth, and to some extent the child’s age. Treatment may involve the use of surgery, radiation therapy, and chemotherapy, depending on the type of tumor. The treatment of choice is total removal of the tumor without residual neurologic damage.
Nursing care management of the child with a brain tumor includes assessing for signs and symptoms, preparing the family for diagnostic and operative procedures, preventing postoperative complications, correctly positioning the patient, regulating fluids, providing comfort measures, supporting the family, and helping the child and family return to optimum functioning.
Neuroblastoma is the most common extracranial solid tumor of childhood and the most common cancer diagnosed in infancy. Neuroblastoma is a “silent” tumor. In more than 70% of cases, diagnosis is made after metastasis occurs, with the first signs caused by involvement in a nonprimary site, usually the lymph nodes, bone marrow, skeletal system, or liver.
Bone tumors consist of osteosarcoma and Ewing sarcoma are the primary bone tumors that occur most often in young people. Most malignant bone tumors produce localized pain in the affected site, which may be severe or dull and may be attributed to trauma or the vague complaint of “growing pains.”
Osteosarcoma (osteogenic sarcoma) presumably arises from bone-forming mesenchyme, which gives rise to malignant osteoid tissue. Most primary tumor sites are in the diametaphyseal region (wider part of the shaft, adjacent to the epiphyseal growth plate) of long bones, especially in the lower extremities.
Ewing sarcomas, which includes primitive neuroectodermal tumor of the bone, arise in the marrow spaces of the bone rather than from osseous tissue. Limb salvage procedures might be feasible in extremity lesions, and amputation may be considered if the results of radiation therapy would render the extremity useless or deformed (e.g., from retarded growth in young children). The treatment of choice for the majority of lesions is involved-field radiation therapy and chemotherapy.
Other solid tumors include Wilms’ tumor, rhabdomyosarcoma, and retinoblastoma.
In general, most children with teratomas and localized gonadal tumors that are surgically resected can be observed without the need for further therapy.
The most common type of liver tumor is hepatoblastoma. Surgical resection is the treatment of choice for these tumors but is usually performed after the administration of chemotherapy to make the tumor resection more successful.
Survival for children with cancer has greatly improved over the past decades, so that long-term survival is expected for more than 80% of children with access to contemporary therapy for cancer.
Radiation therapy is frequently used in the treatment of childhood cancer, usually in conjunction with chemotherapy or surgery. It can be used for curative purposes and for palliation to relieve symptoms by shrinking the size of the tumor. Recent advances in radiation therapy that allow the beam to be aimed precisely have optimized its beneficial effects and minimized many of the undesirable side effects by sparing normal tissue.
The long-term consequences of radiation therapy include tissue necrosis, subsequent malignancies, endocrine dysfunction, and behavioral or intellectual deficits. For these reasons, the use of irradiation is deferred for as long as possible in young children. Chemotherapy may allow a delay or reduction in radiation therapy.
• Several characteristics influence skin responses in infants and young children. Their skin is far more susceptible to superficial bacterial infection compared with older children and adults. They are more likely to have associated systemic symptoms with some infections and are more apt to react to a primary irritant than to a sensitizing allergen. Infants and young children are more frequently affected by chronic atopic dermatitis (eczema). Infants’ skin is much more prone to develop a toxic erythema as a result of skin eruptions or drug reactions and is subject to maceration, infection, and the moisture retention associated with diaper rash.
• Lesions of the skin can result from a variety of etiologic factors. In general, skin lesions originate from (1) contact with injurious agents such as infectious organisms, toxic chemicals, and physical trauma, (2) hereditary factors, (3) an external factor that produces a reaction in the skin (e.g., allergens), or (4) a systemic disease in which the lesions are a cutaneous manifestation.
• More than half of dermatologic problems are various forms of dermatitis. This implies a sequence of inflammatory changes in the skin that are grossly and microscopically similar but diverse in course and causation.
• Dermatitis skin lesions’ distribution, size, and morphologic characteristics provide significant information. The usual extrinsic causes are physical, chemical, or allergic irritants or infectious agents such as bacteria, fungi, viruses, or animal parasites. Intrinsic causes such as a specific infection (e.g., measles or chickenpox), drug sensitization, or other allergic phenomena can produce skin manifestations.
• Wounds are structural or physiologic disruptions of the integument that call for normal or abnormal tissue repair responses. During the past two decades, understanding of wound healing has revolutionized the interventions used to promote healing. Emphasis has shifted from interventions directed at maintaining a dry environment that promotes eschar formation to those that promote a moist, crust-free environment that enhances the migration of epithelial cells across the wound and facilitates resurfacing.
• The human body tends to heal; therefore, direct treatment is directed toward eliminating or ameliorating factors that interfere with normal healing processes. Some disorders may demand aggressive therapy, but by and large, the major aims of any treatment are to prevent further damage, eliminate the cause, prevent complications, and provide relief from discomfort while tissues undergo healing.
• When possible, eliminate factors that contribute to the dermatitis and prolong the course of the disease. The most common offenders in pediatrics are environmental factors (e.g., soaps, bubble baths, shampoos, rough or tight clothing, wet diapers, blankets, and toys) and natural elements (e.g., dirt, sand, heat, cold, moisture, and wind).
• Parents can generally manage small wounds to the skin at home. Instruct parents to wash their hands and then wash the wound gently with mild soap and water or with normal saline. Caution them to avoid povidone–iodine, alcohol, and hydrogen peroxide because these products are toxic to wounds.
• Dressings help wound heal by providing a moist healing environment; protecting the wound from infection and trauma; providing compression in the event of anticipated bleeding or swelling; applying medication; absorbing drainage; debriding necrotic tissue; reducing pain; and controlling odor.
• Staphylococcal infections occur most often in children in the younger age-groups; the incidence decreases with advancing age. All of these factors point to the importance of careful hand washing and cleanliness when caring for infected children and their lesions to prevent spread of the infection and as an essential prophylactic measure when caring for infants and small children.
• The dermatophytoses (ringworm) are infections caused by a group of closely related filamentous fungi that invade primarily the stratum corneum, hair, and nails. These are superficial infections by organisms that live on, not in, the skin. When teaching families about the care of children with ringworm, emphasize good health and hygiene. Both 2% ketoconazole and 1% selenium sulfide shampoos may reduce colony counts of dermatophytes. These shampoos can be used in combination with oral therapy to reduce the transmission of the disease to others.
• Scabies is an endemic infestation caused by the scabies mite, Sarcoptes scabiei. Lesions are created as the impregnated female scabies mite burrows into the stratum corneum of the epidermis (never into living tissue), where she deposits her eggs and feces. The treatment of scabies is the application of a scabicide. The drug of choice in children and infants older than 2 months is permethrin 5% cream (Elimite).
• Pediculosis capitis (head lice) is an infestation of the scalp by Pediculus humanus capitis, a common parasite in school-age children. Treatment consists of the application of pediculicides and manual removal of nit cases. Because of its efficacy and lack of toxicity, the drug of choice for infants and children is permethrin 1% cream rinse (Nix), which kills adult lice and nits.
• Lyme disease is the most common tickborne disorder in the United States. It is caused by the spirochete Borrelia burgdorferi, which enters the skin and bloodstream through the saliva and feces of ticks, especially the deer tick. The diagnosis is based primarily on the history, observation of the lesion, and clinical manifestations. At the time the rash appears or shortly thereafter, children older than 8 years of age are treated with oral doxycycline or amoxicillin, and children younger than 8 years of age are given amoxicillin.
• Contact dermatitis is an inflammatory reaction of the skin to chemical substances, natural or synthetic, that evoke a hypersensitivity response or to those agents that cause direct irritation. The initial reaction occurs in an exposed region, commonly the face and neck, backs of the hands, forearms, male genitalia, and lower legs. The major goal in treatment is to prevent further exposure of the skin to the offending substance. Provided there is no further irritation, the skin’s normal recuperative powers produce satisfactory results without treatment.
• Contact with the dry or succulent portions of any of three poisonous plants (ivy, oak, or sumac) produces localized, streaked or spotty, oozing, and painful impetiginous lesions. Urushiol has an effect as soon as it touches the skin. It penetrates through the epidermis as a mixture of compound molecules called catechols. These catechols bond skin proteins, where they initiate an immune response. Treatment of the lesions includes application of calamine lotion, soothing Burow solution compresses, or Aveeno baths to relieve discomfort. Topical corticosteroid gel is effective for prevention or relief of inflammation, especially when applied before blisters form. Oral corticosteroids may be needed for severe reactions, and a sedative such as diphenhydramine may be ordered.
• Nonhuman mammal bites are common injuries and are inflicted by both wild and domestic animals. Approximately half the victims of dog bites are younger than 5 years of age; boys are bitten more frequently than girls. General wound care consists of rinsing the wound with copious amounts of saline or Ringer lactate delivered under pressure via a large syringe and washing the surrounding skin with mild soap. A clean pressure dressing is applied, and the extremity is elevated if the wound is bleeding. Medical evaluation is advised because of the danger of tetanus and rabies, although dogs in most urban areas are required to be immunized against rabies.
• Diaper dermatitis is common in infants and is caused by wearing diapers. Cornstarch is preferred over talcum powder for the treatment of diaper dermatitis since talcum powder can cause inhalation injury.
• Atopic dermatitis, or eczema, refers to a category of dermatologic disorders characterized by chronic relapsing inflammatory lesions that itch. Itching is the common symptom, and can be mild, moderate, or severe. Scratching can exacerbate the itching, and the lesions disappear once scratching stops. Nursing management includes hydrating the skin, relieving pruritus, reducing inflammation, and preventing infection.
• Seborrheic dermatitis is a chronic, recurrent, inflammatory reaction of the skin. It occurs most commonly on the scalp (cradle cap) but may involve the eyelids (blepharitis), external ear canal (otitis externa), nasolabial folds, and inguinal region. Shampooing and cleaning the area daily with a mild shampoo or soap help alleviate skin flaking.
• Acne vulgaris is the most common skin condition of adolescence. Acne is caused by excessive sebum production; alterations in follicular growth; differentiation with colonization of Propionibacterium acnes; and immune response and inflammation. Gentle cleansing with a mild soap and topical and system medications are common treatments.
• Chilblain and frostbite are the skin’s reactions to cold. Rewarming the skin may produce pain, which is managed with analgesics and sedatives.
The nurse assesses the following in children with suspected or diagnosed cardiac conditions: nutritional state, color, chest deformities, unusual pulsations, respiratory excursion, clubbing of fingers, chest and abdomen inspection, peripheral pulses, heart rate and rhythm, and character of heart sounds.
The most common tests used in assessing cardiac function are MRI, ECG, echocardiography, and cardiac catheterization.
Cardiac catheterization procedures can be divided into three groups: (1) diagnostic catheterizations used to diagnose congenital heart defects; (2) interventional catheterizations, in which catheters or balloon devices are used to alter cardiac anatomy; and (3) electrophysiology studies, in which catheters with electrodes are used to evaluate dysrhythmias.
Several prenatal factors may predispose children to CHD; these include maternal chronic illness, alcohol consumption, and exposure to environmental toxins and infections.
Congenital heart defects can be divided into four main groups, as determined by hemodynamic patterns: (1) defects that result in increased pulmonary blood flow, (2) decreased pulmonary blood flow, (3) obstruction of blood flow out of the heart, and (4) mixed blood flow.
Clinical manifestations of CHF are impaired myocardial function, pulmonary congestion, and systemic venous congestion.
Nursing measures in the care of a child with CHF are to assist in improving cardiac function, removing accumulated fluid and sodium, decreasing cardiac demands, reducing afterload, improving tissue oxygenation and reducing respiratory distress, maintaining nutritional status, and supporting the child and family.
Clinical manifestations of hypoxemia are cyanosis, polycythemia, clubbing, and delayed growth and development. The child is at increased risk for CVAs, brain abscess, and developmental delays.
Caring for the child with CHD and the family requires helping them to adjust to the disorder and to cope with the effects of the defect and fostering growth-promoting family relationships.
Preoperative care of the child with a congenital heart defect involves introducing the child and family to the hospital and preparing them for preoperative and postoperative procedures.
Providing postoperative care includes observing vital signs and arterial and venous pressures, maintaining respiratory status, allowing maximum rest, providing comfort, monitoring fluids, planning for progressive activities, giving emotional support, observing for and treating complications of surgery, and planning for discharge and home care.
Acquired cardiovascular disorders include infective endocarditis (IE), acute rheumatic fever (ARF), rheumatic heart disease (RHD), hyperlipidemia (hypercholesterolemia), and cardiac dysrhythmias.
Acute RF is a systemic inflammatory disease that can damage the cardiac valves and is associated with previous group A strep (GAS) infection.
Children who have had ARF are susceptible to recurrent infections that are likely to result in rheumatic heart disease and further damage to the heart valves. Prophylactic treatment against recurrence of ARF is started after acute therapy.
The objectives of nursing care for the child with ARF are to (1) encourage compliance with drug regimens, (2) facilitate recovery from the illness, and (3) provide emotional support.
Common cardiac dysrhythmias in children include bradycardias, tachydysrhythmias, and conduction disturbances.
Heart transplantation has become a treatment option for infants and children with worsening HF and limited life expectancy. Indications for heart transplantation in children are cardiomyopathy, end-stage CHD, and with some forms of complex congenital cardiac defects, such as hypoplastic left heart syndrome.
Education of the child with hypertension and the family focuses on drug therapy, diet control, weight loss, and appropriate exercise.
Kawasaki disease is an extensive inflammation of small vessels and capillaries that may progress to involve the coronary arteries and cause aneurysm formation. The administration of gamma globulin (IVIG) is an important aspect of treatment.
Emergency treatment for shock includes ventilation, fluid administration, cardiovascular support, and general support.
Common allergens associated with anaphylaxis are drugs, latex, foods, venom from bees or snakes, and biologic agents.
Septic shock is defined as sepsis with organ dysfunction and hypotension. Management includes providing hemodynamic stability and adequate oxygenation and using antimicrobials to treat the infectious organism.
Toxic shock syndrome management is directed at prevention. Avoiding the use of tampons offers the most certain preventive measure.
Anemia is defined as reduction of RBCs or Hgb concentration to levels below normal for age; disorders are classified by etiology and physiology or morphology.
The nurse’s role in treatment of anemia is to assist in establishing a diagnosis and a cause, prepare the child for laboratory tests, administer prescribed medications (oral iron) and dietary education, decrease tissue oxygen needs, and prevent complications.
The main nursing goal in prevention of nutritional anemia is parent/family education regarding correct feeding practices.
Sickle cell anemia (SCA) is one of a group of diseases collectively called hemoglobinopathies in which normal adult Hgb (Hgb or HbA) is partly or completely replaced by abnormal sickle Hgb (HgS).
Nursing care of the child with SCA focuses on the following: teaching the family how to prevent and recognize sickle cell problems; managing pain and other symptomatic factors during crises; and helping the child and parents adjust to a lifelong, chronic disease.
In β-thalassemia, there is a partial or complete deficiency in the synthesis of the β-chain of the Hgb molecule. Consequently, there is a compensatory increase in the synthesis of α-chains, and γ-chain production remains activated, resulting in defective Hgb formation.
Nursing care of the child with β-thalassemia includes promoting compliance with transfusion and chelation therapy, observing for complications of multiple blood transfusions, assisting the child in coping with the effects of illness, and fostering parent-child adjustment to long-term illness.
Causes of acquired aplastic anemia (AA) include irradiation, drugs, industrial and household chemicals, infections, and infiltration and replacement of myeloid elements; however, most cases are idiopathic.
AA therapy is focused on restoring function of the marrow by immunosuppressive therapy (IST) or replacement of bone marrow through transplantation.
The term hemophilia refers to a group of bleeding disorders in which there is a deficiency, dysfunction, or absence of one of the blood coagulation factors (proteins). In about 80% of all cases of hemophilia, the inheritance pattern is demonstrated as X-linked recessive.
Nursing care of the child with hemophilia involves preventing bleeding by decreasing the risk of injury, recognizing and managing bleeding with factor replacement, preventing the crippling effects of joint degeneration, and preparing and supporting the child and family for home care.
A number of immunodeficiency disorders can cause profound, often life-threatening alterations within the body’s immune system. The most series of these conditions completely depress immunity.
HIV infection is primarily acquired in infancy from an HIV-infected parent and in adolescence and young adults (13-24 yers old) engaging in high risk behaviors.
The HIV virus primarily infects a specific subset of T lymphocytes, the CD4+ T cells. The virus takes over the machinery of the CD4+ lymphocyte and uses it to replicate itself, thus rendering the CD4+ cell dysfunctional.
The goals of therapy for HIV infection include slowing the growth of the virus, preventing and treating opportunistic infections, and providing nutritional support and symptomatic treatment.
Severe combined immunodeficiency disease (SCID) is a defect characterized by absence of both humoral and cell-mediated immunity.
The definitive treatment for SCID is HSCT from a histocompatible donor, a haploidentical donor (usually a parent), or a matched unrelated donor bone marrow stem cells transplant or umbilical cord donor. IVIG infusions and PCP prophylaxis (maintaining the child in a sterile environment) are used to augment the humoral immunity until the transplant is performed.
Wiskott-Aldrich syndrome is an X-linked recessive disorder characterized by a triad of abnormalities: (1) thrombocytopenia, (2) eczema, and (3) immunodeficiency of selective functions of B lymphocytes and T lymphocytes.
Blood transfusions supply needed blood components and remarkably improve quality of life and survival.
Apheresis is the removal of blood from an individual, separation of the blood into its components, retention of one or more of these components, and reinfusion of the remainder of the blood into the individual.