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01628241 CF 2019 (3)

01628241 CF 2019 (3)

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Part 2: Discuss the issues that may make this diagnosis easy or difficult to manage in an 18-

year-old female.

Cystic fibrosis (CF) is a genetic disease that is caused by a mutation in single cystic fibrosis

transmembrane conductance regulator (CFTR) gene (1). CF is a multisystem disease, which

means it causes a number of organs failure or/and dysfunction (1). One of these disorders is

affecting the pancreas (exocrine and endocrine), which leads to cystic fibrosis related

diabetes (CFRD) (2). In this paper, a comparison between CFRD and type 1 diabetes (T1D)

(definition) will be presented in pathophysiology, geographies , onset, and risk factors based

on scientific evidence.

Pathophysiology:

CFRD

In CFRD, an alteration in the CFTR gene causes the imbalanced insulin (beta cells) and

glucagon (alfa cells) secretion . CFTR on a cell wall control the glucagon suppression which

maintain the chloride levels low inside the cell. When CFTR does not function properly, the

process of balancing glucagon in and out the cell will be impaired. CFTR has an essential

function in B cells, which are responsible of producing insulin, as well by managing the

chloride efflux and cell resting potential to control insulin release. Glucose intolerance in this

population is seen over time, which might explain the late onset of the disease.

T1D

Type 1 diabetes is an autoimmune disorder which represent the first and most

important differences from CFRD. The pathophysiology was observed in tow main

stages, the first stage was the gradual destruction of the ß-cells by inflammatory cells

including reactivation of CD8

T lymphocytes which was observed in non-obese

diabetic mice. As a result, the second stage starts by showing low insulin levels. in

this population, ketosis is one of the major features.

Onset:

CFRD

According to (3) the most common age of onset is 18 – 21 y/o Due to the slow

reduction of insulin production, studies have shown 20% if CFRD starts from

adolescence with ~3% rate of incidence and 40% – 50% of adults with CF (4,5) .

T1D

The most common age of onset is <20 y/o (3). A study (6) have investigated the

onset of diabetes in children and found out that among (n = 2841) of children who

are diabetic, 2686 (94.4%) had T1D with the greatest onset among 5 – 9 years old

with female preponderance.

Diagnosis:

CFRD

Because the onset of CFRD is considered late, after gradual insulin insufficiency,

screening is important in CF patients. Screening is recommended by measuring

fasting plasma glucose (FPG) annually on all patients 14 years of age or older (3). The

CFRD diagnosis should be confirmed when repeated tests of FPG show results of

≥ 126 mg/dL (3). Clinical signs of CFRD include polyuria, polydipsia, delayed

progression of puberty, poor weight gain, poor growth velocity, or an unexplained

chronic decline in pulmonary function (3). In addition, one of the main differences is

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Hemoglobin A1c (HbA1c) levels; in CFRD, measuring (HbA1c) levels is not

recommended because it is usually low or not affected by CFRD (3).

T1D

According to the national institute for health and care excellence (NICE) (7),

diagnosis of T1D on adults is based on the presence of hyperglycemia when the

patients is under the age of 50 Y/O, have ketosis and/or rapid weight loss and/or

BMI below 25 kg/m2 and/or there is a history of autoimmune disease. Ketosis is

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Part 2: Discuss the issues that may make this diagnosis easy or difficult to manage in an 18year-old female. Cystic fibrosis (CF) is a genetic disease that is caused by a mutation in single cystic fibrosis transmembrane conductance regulator (CFTR) gene (1). CF is a multisystem disease, which means it causes a number of organs failure or/and dysfunction (1). One of these disorders is affecting the pancreas (exocrine and endocrine), which leads to cystic fibrosis related diabetes (CFRD) (2). In this paper, a comparison between CFRD and type 1 diabetes (T1D) (definition) will be presented in pathophysiology, geographies , onset, and risk factors based on scientific evidence. Pathophysiology: CFRD In CFRD, an alteration in the CFTR gene causes the imbalanced insulin (beta cells) and glucagon (alfa cells) secretion . CFTR on a cell wall control the glucagon suppression which maintain the chloride levels low inside the cell. When CFTR does not function properly, the process of balancing glucagon in and out the cell will be impaired. CFTR has an essential function in B cells, which are responsible of producing insulin, as well by managing the chloride efflux and cell resting potential to control insulin release. Glucose intolerance in this population is seen over time, which might explain the late onset of the disease. T1D Type 1 diabetes is an autoimmune disorder which represent the first and most important differences from CFRD. The pathophysiology was observed in tow main stages ...
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