Treatments for SCID
Treatments for SCID
The list of treatments mentioned in various sources
for SCID
includes the following list.
Always seek professional medical advice about any treatment
or change in treatment plans.
SCID: Is the Diagnosis Correct?
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Differential diagnosis list for SCID may include:
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SCID: Research Doctors & Specialists
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Drugs and Medications used to treat SCID:
Note:You must always seek professional medical advice about any prescription drug, OTC drug, medication, treatment
or change in treatment plans.
Some of the different medications used in the treatment of SCID include:
- Immune Globulin (intravenous)
- Carimune
- Carimune NF
- Flebogamma
- Gamimune N
- Gammagard S/D
- Gammar-P
- Gamunex
- Iveegam EN
- Octagam
- Panglobulin
- Panglobulin NF
- Polygam S/D
- Iveegam Immuno
- Cilax
- Intacglobin
- Sandoblobulina
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Discussion of treatments for SCID:
Genes and Disease by the National Center for Biotechnology (Excerpt)
Though invasive, new treatments such as bone marrow and stem-cell transplantation save as many as 80% of SCID patients.
(Source: Genes and Disease by the National Center for Biotechnology)
Primary Immune Deficiency, NIAID Fact Sheet: NIAID (Excerpt)
Transplanting bone marrow from a
healthy sibling whose tissue type closely matches the patient’s is
the most effective treatment. If a matched sibling is not available,
a donor as closely matched as possible is used. Until the transplant
takes effect (in one to three years), intravenous immunoglobulin
(IVIG) is given to normalize antibody levels. SCID patients with ADA
deficiency have been treated successfully with enzyme replacement
therapy called PEG-ADA. Gene therapy for correction of both forms of
SCID is under investigation.
(Source: excerpt from Primary Immune Deficiency, NIAID Fact Sheet: NIAID)
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Book Excerpts: Treatment of SCID
Treatments of SCID: Online Medical Books
16 MEDICAL BOOKS ONLINE!
Review excerpts from medical books online, free, without registration,
for more information about the treatments of SCID.
Common variable immunodeficiency:
Treatment
(Professional Guide to Diseases (Eighth Edition))
Treatment and care of patients with common variable immunodeficiency are essentially the same as for those with X-linked hypogammaglobulinemia.
Injection of immune globulin (usually weekly to monthly) helps maintain the immune response. Because these injections are painful, give them deep into a large muscle mass, such as the gluteal or thigh muscles, and massage well. If the dosage is more than 1.5 ml, divide the dose and inject it into more than one site; for frequent injections, rotate the injection sites. Because immune globulin is composed primarily of IgG, the patient may also need fresh frozen plasma infusions to provide IgA and IgM.
Antibiotics are the mainstay for combating infection. Regular X-rays and pulmonary function studies help monitor lung infection; chest physiotherapy may be ordered to forestall or help clear such infection.
» READ BOOK EXCERPT ONLINE »
Source: Professional Guide to Diseases (Eighth Edition), 2005
Severe combined immunodeficiency disease:
Treatment
(Professional Guide to Diseases (Eighth Edition))
Treatment aims to restore the immune response and prevent infection. Histocompatible bone marrow transplantation is the only satisfactory treatment available to correct immunodeficiency. Because bone marrow cells must be human leukocyte antigen and mixed leukocyte culture matched, the most common donors are histocompatible siblings. However, because bone marrow transplant can produce a potentially fatal graft-versus-host (GVH) reaction, newer methods of bone marrow transplant that eliminate GVH reaction (such as lectin separation and the use of monoclonal antibodies) are being evaluated.
Fetal thymus and liver transplants have achieved limited success. Immune globulin administration may also play a role in treatment. Some SCID infants have received long-term protection by being isolated in a completely sterile environment. However, this approach isn’t effective if the infant already has had recurring infections.
Gene therapy is being used to treat ADA deficiency.
» READ BOOK EXCERPT ONLINE »
Source: Professional Guide to Diseases (Eighth Edition), 2005
Common variable immunodeficiency:
Treatment
(Handbook of Diseases)
Patients with common variable immunodeficiency need essentially the same treatment as patients with X-linked hypogammaglobulinemia.
I.V. immune globulin (usually weekly to monthly) helps maintain immune response. Because immune globulin is made up primarily of IgG, the patient may also need fresh frozen plasma infusions to provide IgA and IgM.
Antibiotics are the mainstay for combating infection. Regular X-rays and pulmonary function studies help monitor infection in the lungs; chest physiotherapy may forestall or help clear such infection.
» READ BOOK EXCERPT ONLINE »
Source: Handbook of Diseases, 2003
Severe combined immunodeficiency disease:
Treatment
(Handbook of Diseases)
Restoring immune response and preventing infection are the first goals of treatment. Histocompatible bone marrow transplant is the only satisfactory treatment available to correct immunodeficiency.
Because bone marrow cells must be matched according to human leukocyte antigen and mixed leukocyte culture, the most common donors are histocompatible siblings. But bone marrow transplant can produce a potentially fatal graft-versus-host (GVH) reaction, so newer methods of bone marrow transplant that eliminate GVH reaction (such as lectin separation and the use of monoclonal antibodies) are being evaluated.
Fetal thymus and liver transplants have achieved limited success. Administration of immune globulin may also play a role in treatment. Some SCID infants have received long-term protection by being isolated in a completely sterile environment. However, this approach isn’t effective if the infant already has had recurring infections.
Gene therapy is being used for ADA deficiency.
» READ BOOK EXCERPT ONLINE »
Source: Handbook of Diseases, 2003
Cough - Case 4-2: 7-Week-Old Boy:
VI. Treatment
(Pediatric Complaints and Diagnostic Dilemmas)
As mentioned, infants with small VSDs usually do not require any intervention.
They do require careful surveillance during the first 6 months of life, to
assess growth and respiratory status. Many of these small VSDs close
spontaneously. Importantly, these patients still require endocarditis
prophylaxis.
Usually, those infants with moderate or large VSDs develop some degree of
congestive heart failure. Medical management is often the initial therapy and
may include furosemide, chlorothiazide, spironolactone, and digoxin. On
occasion, afterload reduction with captopril is also required. In those
patients with persistent failure to thrive, caloric augmentation may be
required. If the patient
's congestive heart failure and growth failure are not controlled with medical
management, surgical intervention is required.
» READ BOOK EXCERPT ONLINE »
Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
Cough - Case 4-6: 4-Month-Old Boy:
VI. Treatment
(Pediatric Complaints and Diagnostic Dilemmas)
Because young infants with pertussis have a high risk for complications, there
should be a low threshold for admitting these patients. Many of these infants
require admission to the intensive care unit to monitor for apneic episodes and
neurologic sequelae.
Infants should be treated with a macrolide antibiotic, and erythromycin is the
most common choice. The length of therapy is generally recommended to be 14
days. Azithromycin and clarithromycin appear to be effective as well. There is
some controversy as to whether antibiotics given during the catarrhal stage
decrease disease severity. However, antibiotics should still be given, even in
the paroxysmal stage, because they limit the spread of the disease to others.
Studies are underway to assess the efficacy of pertussis immune globulin as an
adjunctive therapy in extremely ill infants.
Antibiotic prophylaxis is recommended for all household members and close
contacts and usually consists of 10 to 14 days of erythromycin. Prevention is
essential to limit the morbidity and mortality from pertussis, and the
acellular pertussis vaccine is currently the recommended form. It is given in
combination with diphtheria and tetanus toxoids (DTaP). It is recommended that
children receive five doses before school entry.
» READ BOOK EXCERPT ONLINE »
Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
Diarrhea - Case 17-1: 2-Month-Old Boy:
VI. Treatment
(Pediatric Complaints and Diagnostic Dilemmas)
In cases of incarcerated hernia, time is of the essence. Compromised blood flow
to the affected loop of bowel can result in strangulation and bowel necrosis
within 2 hours, so medical intervention is necessary. Reduction of the
incarcerated hernia by experienced pediatric surgical staff is optimal. A
gentle attempt at reduction using pressure on the scrotum with simultaneous
counterpressure above the external inguinal ring is indicated but should never
be forcefully done. Intravenous hydration and nasogastric tube decompression,
in anticipation of definitive surgical management, are also indicated.
Emergency surgery is sometimes required if the incarcerated hernia is not
reducible. If the incarcerated loop of bowel is reduced, surgery may be
postponed 12 to 36 hours so that the bowel edema can resolve.
Elective repair of an asymptomatic inguinal hernia should be performed as soon
as possible after diagnosis, to avoid complications such as incarceration. All
inguinal hernias require surgical intervention, because they do not resolve
spontaneously. In boys, undescended testes may be associated with inguinal
hernia, requiring orchiopexy. There is still some debate as to the importance
of surgical exploration of the contralateral side in search of an occult
inguinal hernia not detected by physical examination, as was the case in this
patient. This decision is left to the individual surgeon, but contralateral
exploration is commonly performed.
» READ BOOK EXCERPT ONLINE »
Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
Diarrhea - Case 17-4: 15-Month-Old Boy:
VI. Treatment
(Pediatric Complaints and Diagnostic Dilemmas)
Surgical resection is usually performed. Low-risk patients may not need any
additional therapy. Radiotherapy and chemotherapy are used, depending on the
stage of the disease. Patients with high-risk disease may have some improvement
in short-term survival with autologous bone marrow transplantation, but
longer-term outcome is still poor. Surgical removal of the tumor usually cures
the secretory diarrhea. The use of somatostatin analogues also has a
therapeutic effect on the secretory diarrhea, but the definitive therapy for
the diarrhea remains surgical.
» READ BOOK EXCERPT ONLINE »
Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
Jaundice - Case 15-3: 2-Month-Old Boy:
VI. Treatment
(Pediatric Complaints and Diagnostic Dilemmas)
Replacement of the hormones produced by the pituitary's target organs is the cornerstone of hypopituitarism therapy. Thyroid hormone
replacement should begin as soon as confirmatory testing is completed; delays
in therapy can result in increased risk of cognitive impairment. Jaundice
improves as the underlying endocrine disorder is treated.
» READ BOOK EXCERPT ONLINE »
Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
Pallor - Case 10-1: 3-Week-Old Boy:
VI. Treatment
(Pediatric Complaints and Diagnostic Dilemmas)
The treatment includes use of corticosteroids. The currently recommended dose is
2 mg/kg per day of prednisone, given in three or four divided doses.
Reticulocytes appear within 1 to 2 weeks, but the rise in hemoglobin is delayed
for several more weeks. Once the hemoglobin level reaches 10 g/dL, the steroid
dose is gradually tapered until the patient is receiving a single daily dose
that adequately maintains appropriate hemoglobin levels. Response followed by
steroid dependence is seen in 60% of patients. Approximately one fifth of the
steroid-responsive patients may ultimately be maintained without steroids.
Approximately 30% to 40% of patients have poor or no response to steroids and
require chronic transfusion therapy to maintain normal hemoglobin. These
children require leukocyte-depleted packed RBC transfusions every 3 to 6 weeks,
with the goal of keeping the hemoglobin level higher than 6 g/dL. Concurrent
chelation of iron with subcutaneously administered desferrioxamine may decrease
some chronic transfusion-related complications. Complications of chronic
transfusion therapy are similar to those seen with other conditions that employ
this modality (e.g., thalassemia). Bone marrow transplantation has been
successful for some patients.
Median survival time is 43 years, but approximately 13% of patients die within
the first 6 years of life. Deaths occur from complications of iron overload,
pneumonia, sepsis, and occasionally transplant-related complications, leukemia,
and pulmonary emboli.
» READ BOOK EXCERPT ONLINE »
Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
Pallor - Case 10-3: 5-Month-Old Boy:
VI. Treatment
(Pediatric Complaints and Diagnostic Dilemmas)
The mainstay of treatment is corticosteroids, given at a dosage of 2 to 10 mg/kg
per day. In severely ill patients such as this child, the steroids should be
given intravenously. Otherwise, the care is supportive and includes replacement
therapy. Compatible cross-matching may not be possible, but if the patient is
in extremis blood type group O, Rh-negative blood should be used in aliquots
that are given slowly and in amounts sufficient to stabilize the cardiovascular
system. The patient should be adequately hydrated to avoid renal involvement.
Splenectomy may be necessary if steroid therapy fails.
» READ BOOK EXCERPT ONLINE »
Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
Seizures - Case 19-3: 8-Month-Old Boy:
VI. Treatment
(Pediatric Complaints and Diagnostic Dilemmas)
Treatment consists of providing a continuous dietary source of glucose to
prevent hypoglycemia. When hypoglycemia is prevented, the biochemical
abnormalities and growth improve and liver size decreases. Infants require
frequent feedings, approximately every 2 to 3 hours during the day and every 3
hours at night. A variety of methods can be used to provide a continuous source
of glucose at night in older children, including intravenous dextrose infusion,
continuous intragastric feeding via a nasogastric or gastrostomy tube, and the
use of low glycemic index foods such as cornstarch. Orally administered
uncooked cornstarch seems to act as an intestinal reservoir of glucose that is
slowly absorbed into circulation. It has been used successfully in infants as
young as 8 months of age and may obviate the need for continuous intragastric
infusion of formula overnight. It can be mixed with water, formula, or
artificially sweetened fluids in 4- to 6-hour intervals overnight. The optimal
schedule requires validation by serial glucose monitoring. Allopurinol and
lipid-lowering agents are used for severe uric acid and lipid abnormalities.
Hepatocyte infusion and liver transplantation may be curative, but the
long-term complications in children with GSD are not yet known.
» READ BOOK EXCERPT ONLINE »
Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
Vomiting - Case 3-1: 7-Week-Old Boy:
VI. Treatment
(Pediatric Complaints and Diagnostic Dilemmas)
Treatment regimens for methemoglobinemia depend on the level and the patient's symptoms. In general, only symptomatic patients with methemoglobin levels
greater than 20% or asymptomatic patients with methemoglobin levels greater
than 30% require specific therapy. Patients with concurrent problems that
impair oxygen delivery, such as anemia, cardiac disease, or pulmonary disease,
should be treated even if they have low methemoglobin levels. Symptomatic
patients must receive proper airway management and supplemental oxygen as
needed. Intravenous methylene blue, after reduction to leukomethylene blue by
NADPH-methemoglobin reductase, aids in the reduction of methemoglobin back to
hemoglobin. It is the treatment of choice and should reduce methemoglobin
levels significantly within 1 hour after administration. Exchange transfusions
are necessary for those patients who have extremely high levels of
methemoglobin that do not respond to methylene blue therapy.
Glucose-6-phosphate dehydrogenase (G6PD) is the first enzyme in the hexose
monophosphate shunt, which is the sole source of NADPH in the red blood cell.
Patients with G6PD deficiency may not produce sufficient NADPH to reduce
methylene blue to leukomethylene blue. Therefore, methylene blue therapy may
not be effective in patients with G6PD deficiency. In such patients, methylene
blue may also induce hemolysis.
» READ BOOK EXCERPT ONLINE »
Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
Wheezing - Case 1-3: 5-Week-Old Boy:
VI. Treatment
(Pediatric Complaints and Diagnostic Dilemmas)
Symptomatic intralobar and extralobar sequestrations require immediate
resection. Asymptomatic sequestrations also require removal because of the risk
of subsequent serious infection. Extralobar sequestrations, because of their
separate pleural covering, can often be removed without disruption of the
normal lung. Intralobar sequestrations require lobectomy for removal because of
the inability to separate the sequestration from the normal lung. In cases of
acute infection, preoperative antibiotic coverage should be directed against
common respiratory pathogens,
Staphylococcus aureus, and anaerobes. Ampicillin-sulbactam or the combination of clindamycin and
cefotaxime are appropriate empiric antibiotic choices. The resected tissue
should be sent for aerobic and anaerobic bacterial cultures, mycobacterial
cultures, and fungal cultures.
Intraoperative mortality is highest in those with associated congenital
anomalies. Intraoperative complications are usually caused by severance of a
systemic artery. Postoperative complications include emphysema, hemothorax, and
bronchopleural fistulas; the incidence of each is approximately 1%. The
long-term prognosis in those patients without other debilitating congenital
anomalies is excellent.
In this case, MRA was performed and the extralobar sequestration was removed
without complications on the following day. Cultures of the resected
sequestration were sterile. The patient recovered uneventfully.
» READ BOOK EXCERPT ONLINE »
Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
Wheezing - Case 1-5: 5-Week-Old Boy:
VI. Treatment
(Pediatric Complaints and Diagnostic Dilemmas)
Complete surgical repair should be performed as early as possible. A large
side-to-side anastomosis is created between the left atrium and the common
pulmonary vein. Occasionally, the distal portion of the common pulmonary vein
is ligated. The foramen ovale or atrial septal defect is closed. A hypoplastic
left atrium may require surgical enlargement.
Residual stenosis at the left atrial-venous anastomosis created at operative
repair develops in 10% of children. This stenosis requires reintervention and
patch plasty. Postoperative pulmonary venous obstruction occurs 1 to 3 months
after repair in 5% of patients. Late atrial arrhythmias develop in a small
number of patients.
» READ BOOK EXCERPT ONLINE »
Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
Wheezing - Case 1-6: 4-Month-Old Boy:
VI. Treatment
(Pediatric Complaints and Diagnostic Dilemmas)
TMP-SMX remains the treatment of choice for PCP in children. The oral route may
be used in mild cases, if the patient can reliably take and retain oral
medication. Most children require intravenous treatment. The course of
treatment is 14 to 21 days. If no improvement is demonstrated in 5 to 7 days,
treatment should be changed to pentamidine. Pentamidine, atovaquone, or
primaquine plus clindamycin may be used for children who are unable to tolerate
TMP-SMX. Adjunctive therapy with corticosteroids reduces the occurrence of
respiratory failure and improves oxygenation in moderately ill adults with PCP;
limited data in children suggest similar benefits.
Children with an episode of PCP require posttreatment prophylaxis with TMP-SMX
(5 mg/kg per day), either daily or three days per week for the duration of risk
(indefinitely for those with HIV). Because of the high risk of acquiring PCP
during the first year of life, all infants born to HIV-infected mothers require
PCP prophylaxis from 4 weeks to 12 months of age, regardless of their
CD4-positive T-lymphocyte count; prophylaxis may be discontinued sooner if HIV
infection is excluded. After 12 months of age, prophylaxis is required if the
percentage of CD4+ T cells is less than 15% or if the CD4+ count is less than
500 cells/
µL (less than 200 cells/µL in those older than 5 years of age). Patients in other high-risk groups,
including those with severe combined immune deficiency, lymphoproliferative
malignancy, and organ transplantation, also require PCP prophylaxis.
» READ BOOK EXCERPT ONLINE »
Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
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