This is a genetic disorder that does not just affect the respiratory system, it is the most lethal autosomal recessive disorder in the white population. The exocrine glands are affected resulting in the overproduction of thick secretion that block body passages, in particular, the lungs thereby clogging the lungs.
Clogging the lungs causes breathing difficulties as well as mucus trapping bacterial increase the risk of infection. Pancreatic ducts also become blocked meaning that digestive juices cannot reach the small intestine. The result is poor absorption.
The sweat glands are also implicated with affected individual sweating profusely and losing electrolytes particularly sodium. The end result is an imbalance in the electrolyte levels and an increase in the risk of heat exhaustion. The primary cause of death is respiratory failure.
There is no cure for CF, so the removal of mucus, improving the lifestyle through diet, daily physiotherapy helps to remove the mucus from the lungs. Management is in combination with bronchodilators and antibiotic therapy and expectorant to reduce the mucus, oxygen treatments when needed.
Sudden Infant Death Syndrome (SIDS)
- Is the sudden death of an infant under the age of 1 with no apparent cause, theories have been proposed as to the cause, overlain by the parents to the formalin in the mattress. It is also suggested that there may also be congenital abnormalities of the respiratory system. It is recommended that infants be put to sleep on there back. Children that are most at risk are the children that are born prematurely
Disorders of the heart
- Congenital defects of this organ are the most common, can be asymptomatic through to life-threatening. Risk factors involved include, mother, contracting rubella during pregnancy, alcoholism and poor nutrition
Arterial Septic Defect
- The foramen ovule allows oxygenated blood to flow from the right atrium to the left before birth but closing at birth when the first breath is taken. Sometimes it does not close, this allows the oxygenated blood to flow from the left to right atrium staying in the pulmonary circuit. The end result is that the oxygenated blood is lost in the way and never circulates through the body. This causes the right side of the heart to work harder. The condition affects females more than males and can be corrected by surgery.
Ventricular Septal Defects
- This is the most common congenital heart defect with a hole between the left and right ventricle. Similar to arterial septal defect blood moves from higher pressure left ventricle to the right ventricle. Once again resulting in the right side of the heart being overworked.
Patent Ductus Arteriosus
- The ductus arteriosus remains open after birth allowing blood in higher pressure aorta shunt back to pulmonary artery overloading the heart and pulmonary circuit
Narrowing of the Aorta
- Happens in the descending aorta causing BP above the constriction and lower BP below it causing increased workload for the heart
Four Heart Defects
- Stenosis of the pulmonary valve (reducing flow in the lungs)
- Hypertrophy of the right ventricular wall due to heart working harder to pump blood to the lungs
- Abnormal position of the aorta just above the ventricular defect, so both oxygenated and deoxygenated blood pass through the aorta
- The end result is cyanosis of the tissue or a blue baby
- Surgically treated
Neurological Congenital Disorders
- Reduced oxygen supply to the brain during the development of the foetus (rubella infection of the mother, toxaemia of pregnancy
- During birth, prolonged; labour, insufficient oxygen to the embryo due to prolapsed cord asphyxiation from the cords wrapped around the neck
- In infancy( head trauma, meningitis with the cause often unknown
- It mostly affects males and premature babies affecting motor coordination and muscle action and may be accompanied by seizures hearing and visual deficiencies and mental retardation
- There is no cue with
- This is a disorder where one or more somites do not fuse over the spinal cord, leaving an opening through which the cord can prolapse, Somits become vertebrae. Lack of folate during pregnancy is a major contributor, it also a genetic basis to it. Also viral infection and radiation to the mother during pregnancy. Spinal Bifida can be accompanied by other disorders such as hydrocephalus ( water in the brain cleft lip and palate and clubfoot. The most common form is spina bifida Occulta which shows no symptoms. Meningocele: meninges prolapse forming a fluid-filled sac at surface but nor nerves involved so can be surgically corrected.
- Meaningless and some of the spinal cord protrude meaning neurological involvement. Death may be the ultimate result, but symptoms include paralysis of the lower limbs, skeletal and joint malformation, and incontinence.
Whooping Cough (Pertussis)
In the respiratory system, bacterial diseases that predominantly affect children is Whooping cough which is an acute infection of the respiratory tract caused by a bacteria Bordetella pertussis. The incubation period is generally 6-10 days but can be up to three weeks
Three stages characterise the disorder
- The catarrhal stage, where the mucous membranes become inflamed with a cough, runny Norse and low fever
- The proximal stage is where whooping-cough develops, resulting in cyanosis, distension of the veins of the neck and sometimes vomiting.
- The convalescent stage where the symptoms go down, but there may be more whooping attacks
- overproduction of the leukocytes in leukaemia results in the overcrowding of the brain marrow and decreased function and production of hematopoietic cells
- Can be chronic or acute
- Myeloid or lymphoid
- Each leukaemia differs according to the type of cell
- Acute – blast cells – rapid onset
- Chronic – mature non-functional cells – gradual onset and extended survival
- The leukaemia is thought to be clonal disorders in which a single cell is transformed and proliferates more slowly than the healthy cells. The problem is that these cells block normal cell production
- Familial tendencies have been shown in leukaemia along with an association with chromosomal abnormalities
- Acute lymphoblastic leukaemia is the most common leukaemia in children and as with all leukaemia
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Stoltz, D. A., Meyerholz, D. K., & Welsh, M. J. (2015). Origins of cystic fibrosis lung disease. New England Journal of Medicine, 372(4), 351-362.
Nguyen, A. W., Wagner, E. K., Laber, J. R., Goodfield, L. L., Smallridge, W. E., Harvill, E. T., … & Kaleko, M. (2015). A cocktail of humanized anti–pertussis toxin antibodies limits disease in murine and baboon models of whooping cough. Science translational medicine, 7(316), 316ra195-316ra195.