Skip to main content

SCID- SEVERE COMBINED IMMUNODEFICIENCES - Cuddles Child Clinic

Combined immunodeficiency involves malfunctioning of multiple components of the immune system, including both humoral immunity and cell mediated immunity.

•    It is also called as “bubble boy disease”
•    It is X-linked genetic disorder
•    It characteristically lead to death from overwhelming infection in the first year of life unless they have undergone successful bone marrow transplantation.
•    In it neither T cells nor the B cells work properly


Types :
•    Typical SCID
•    Leaky SCID
•    Variant SCID

Clinical presentation of SCID:
•    Children with SCID may develop infections caused by organisms or vaccines
•    Organisms which are most dangerous to cause fatal diseases are: pneumonia, fungal infection, viral infections like chickenpox, CMV, influenza, measles etc.
•    Patients with SCID having rash are mistakenly diagnosed as eczema, but is actually caused by reaction of the mother’s T-cells.

Diagnosis
:
•    Low lymphocyte count <1500 per cubic millimeter
•    Complement level in blood
•    Culture method
•    Perinatal amniocentesis and chorionic villous sampling
•    Immunoglobulin levels
•    Protein electrophoresis
•    WBC count
•    Bead microarray for detection of SCID

Treatment:
•    Bone marrow transplant may be used to treat certain immunodeficiency conditions
•    Passive immunity from certain bacteria and germs when exposed to
•    Immunoglobulin replacement therapy
•    Cord blood transplants may be an alternative
•    Gene therapy

Prevention:
•    Genetic counseling
•    Way to avoid serious infections like HIV
•    Good nutrition
•    Perinatal diagnosis

Child named David Vetter in 1970 also named bubble boy who lived for 12 years in a plastic, germ free bubble. Early diagnoses and detection helps in treating children with SCID.

Comments

Popular posts from this blog

Stridor in Children - कडल्स चाइल्ड क्लिनिक

 Noisy high pitched sound while breathing:- it is a sound which involves upper airway tract like nose, mouth, sinuses, larynx and wind pipe. Stridor occurs while breathing in i.e Inspiration. Causes can be:- 1) Congenital anomaly of upper airway tract 2) Infections like croup, tonsillitis, adenoiditis 3) Ingestion of substances like food/small object in upper airway path 4) Injury in upper airway tract 5) Allergic reaction in upper airway tract 6) Tumour of same tract. Symptoms :- main symptom involves harsh noisy sound while breathing in. Children are more at trouble due to narrow and shorter upper air way system Diagnosis :- ENT specialist doctor are the doctor who treats this condition like stridor. Tests:- 1) Chest and neck Xray 2) CT scan/MRI 3) Laryngoscopy:- to check throat and larynx 4) Bronchoscopy:- to check trachea and tubes in addition to above ones 5) Spirometery:- to know how much air is breathed in and out 6) Pulse oximetry:- to measure amount of

Diaper and Clothing in Babies | शिशुओं में डायपर और वस्त्र

Diaper and clothing in newborns is something which comes in mind before delivery for first time parents. Usually parents keep their decision before baby is born and are ready with stuff. Diapers have been in use since long back time, but they keep on launching every time with some betterment. Earlier reusable cloth diapers were used but nowadays people prefer Use and throw diapers. A child may need 10 diapers a day.  Cloth diapers: many parents still prefer these reusable cloth diapers because these are economical, environment friendly, easy to wash and can be used multiple times. These diapers come in Velcro end or snap end to close them properly. These are of square shape; you have to fold it into triangle form and place buttocks of baby with long side of triangle behind upper waist and third corner facing down towards feet. Bring all corners of triangle in front near umbilicus and secure them with knot or some pin. These should be washed in soft detergent which is hypoallergic to ba

How do you choose a Child Care Centre? शिशु देखभाल केंद्र

Child clinic is one place where most of child fears to go, but this is the place where mental and physical growth is assessed and appropriate guidance and treatment is offered. ROUTINE PATIENT VISITS : Routine or regular checkup of children should be done by giving well spaced time slots so as to avoid crowding in clinics. This helps in avoiding transmission of infection. Receptionist should be well versed and knowledgeable. Basic parameter assessment can be done at reception area also. There should not be delay in meeting parents to the doctor. AFTER HOURS HELP Sometimes any medical emergency can be there like deterioration of child health and any new symptoms arises for which you cannot wait until next day, so for that there should be provision of After Hours Help. A dedicated help line number should be there on which parents can have their queries cleared. On receiving end any trained staffed in pediatric field can be there who can give you right advice and support to manage child’s