Podcast
Questions and Answers
What is the primary treatment focus for managing GERD?
What is the primary treatment focus for managing GERD?
- Medications and lifestyle changes (correct)
- Surgery to correct the esophagus
- High calorie and protein diet
- Mechanical interventions for the intestines
Which of the following is among the CFTR modulators approved for therapeutic use?
Which of the following is among the CFTR modulators approved for therapeutic use?
- Kalydeco (correct)
- Trikafta (correct)
- Lumacaftor
- All of the above
What is the recommended approach for couples seeking prenatal care when one partner is Caucasian?
What is the recommended approach for couples seeking prenatal care when one partner is Caucasian?
- Biopsy of the placenta
- No special testing needed
- Prenatal genetic screening only
- CF carrier testing is advised (correct)
What treatments are suggested for a female infertility rate affecting 20-35%?
What treatments are suggested for a female infertility rate affecting 20-35%?
Which of the following options can be used for the management of the vas deferens being absent?
Which of the following options can be used for the management of the vas deferens being absent?
What is the recommended antidote for acetaminophen overdose?
What is the recommended antidote for acetaminophen overdose?
Which of the following is NOT a risk factor for developing conjunctivitis?
Which of the following is NOT a risk factor for developing conjunctivitis?
Which type of hemoglobin is most commonly associated with sickle cell anemia?
Which type of hemoglobin is most commonly associated with sickle cell anemia?
What is the purpose of hemoglobin electrophoresis?
What is the purpose of hemoglobin electrophoresis?
Which treatment option is NOT typically used for conjunctivitis?
Which treatment option is NOT typically used for conjunctivitis?
What is a significant drawback of intensive therapy for diabetes management?
What is a significant drawback of intensive therapy for diabetes management?
What is one of the primary benefits of using an insulin pump?
What is one of the primary benefits of using an insulin pump?
What is the ideal carbohydrate-to-insulin ratio for an infant using an insulin pump?
What is the ideal carbohydrate-to-insulin ratio for an infant using an insulin pump?
Which of the following is a complication of diabetic ketoacidosis (DKA)?
Which of the following is a complication of diabetic ketoacidosis (DKA)?
What is a common sign of cerebral edema in a patient?
What is a common sign of cerebral edema in a patient?
What treatment is recommended to manage cerebral edema?
What treatment is recommended to manage cerebral edema?
What routine is involved in intensive therapy for diabetes?
What routine is involved in intensive therapy for diabetes?
At what time should blood glucose be monitored at least once a week under intensive therapy?
At what time should blood glucose be monitored at least once a week under intensive therapy?
What is a common sign of insulin resistance that can be observed during a physical assessment?
What is a common sign of insulin resistance that can be observed during a physical assessment?
What outcome is associated with diabetes that has the lowest reported occurrence?
What outcome is associated with diabetes that has the lowest reported occurrence?
Which statement best describes the cognitive capabilities of adolescents regarding diabetes self-care?
Which statement best describes the cognitive capabilities of adolescents regarding diabetes self-care?
What is the recommended treatment for growth hormone deficiency in children?
What is the recommended treatment for growth hormone deficiency in children?
What should be done if a toddler shows signs of extreme sleepiness or irritability?
What should be done if a toddler shows signs of extreme sleepiness or irritability?
How does regular physical activity affect insulin sensitivity?
How does regular physical activity affect insulin sensitivity?
What is the role of medical alert identification for diabetics?
What is the role of medical alert identification for diabetics?
What is a potential psychological impact of precocious puberty in children?
What is a potential psychological impact of precocious puberty in children?
What condition is characterized by the excretion of undigested food in stool that appears increased, frothy, and foul?
What condition is characterized by the excretion of undigested food in stool that appears increased, frothy, and foul?
What percentage of individuals with cystic fibrosis are likely to develop liver cirrhosis?
What percentage of individuals with cystic fibrosis are likely to develop liver cirrhosis?
Which of the following is a significant metabolic disorder resulting from excessive water loss in cystic fibrosis patients?
Which of the following is a significant metabolic disorder resulting from excessive water loss in cystic fibrosis patients?
What is the most useful diagnostic test for cystic fibrosis?
What is the most useful diagnostic test for cystic fibrosis?
What must be ensured for accurate results in the Sweat Chloride Test?
What must be ensured for accurate results in the Sweat Chloride Test?
Which of the following treatments is NOT commonly included for respiratory management in cystic fibrosis patients?
Which of the following treatments is NOT commonly included for respiratory management in cystic fibrosis patients?
What complication can arise in cystic fibrosis patients due to lack of hydration leading to severe constipation?
What complication can arise in cystic fibrosis patients due to lack of hydration leading to severe constipation?
Which of the following vitamins is not typically given in its water-soluble form to cystic fibrosis patients?
Which of the following vitamins is not typically given in its water-soluble form to cystic fibrosis patients?
The treatment of cystic fibrosis often includes which of the following to improve nutritional intake?
The treatment of cystic fibrosis often includes which of the following to improve nutritional intake?
Delayed puberty in females can be an associated presentation of which health condition?
Delayed puberty in females can be an associated presentation of which health condition?
What is essential to obtain prior to administering immunizations?
What is essential to obtain prior to administering immunizations?
Which of the following symptoms is NOT commonly associated with erythema infectiosum?
Which of the following symptoms is NOT commonly associated with erythema infectiosum?
What is a key characteristic of the measles prodrome?
What is a key characteristic of the measles prodrome?
Which statement regarding meningococcal disease is true?
Which statement regarding meningococcal disease is true?
Which of these options is NOT a recommended practice for vaccine storage?
Which of these options is NOT a recommended practice for vaccine storage?
What is a key way that molluscum contagiosum spreads?
What is a key way that molluscum contagiosum spreads?
What is the appearance of the rash in measles typically characterized by?
What is the appearance of the rash in measles typically characterized by?
Which complication is most likely to occur with untreated meningococcal disease?
Which complication is most likely to occur with untreated meningococcal disease?
Flashcards
Meconium ileus
Meconium ileus
A condition where thickened mucus blocks the intestines, causing blockage and difficulty passing stool. Common in newborns with cystic fibrosis.
Distal intestinal obstruction syndrome
Distal intestinal obstruction syndrome
Difficulty digesting and absorbing fats, leading to bulky, foul-smelling stools. Caused by decreased pancreatic enzyme production in cystic fibrosis.
Rectal prolapse
Rectal prolapse
The process of the rectum protruding outside the anus, often caused by chronic constipation. This can occur in CF due to thick mucus blocking stool passage.
Hyponatremic/Hypochloremic alkalosis
Hyponatremic/Hypochloremic alkalosis
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Hypoalbuminemia
Hypoalbuminemia
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Sweat Chloride Test
Sweat Chloride Test
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Pulmonary Function Test (PFT)
Pulmonary Function Test (PFT)
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Chest Physiotherapy (CPT)
Chest Physiotherapy (CPT)
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Pancreatic enzymes
Pancreatic enzymes
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Vitamin Supplementation
Vitamin Supplementation
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High Calorie & Protein Diet
High Calorie & Protein Diet
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CFTR Modulators
CFTR Modulators
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CF Carrier Testing
CF Carrier Testing
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Newborn Screening for CF
Newborn Screening for CF
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Pulmonologist
Pulmonologist
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Sickle Cell Anemia
Sickle Cell Anemia
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Hemoglobin Electrophoresis
Hemoglobin Electrophoresis
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Sickle Cell Trait
Sickle Cell Trait
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Sickle Turbidity Quick Screen Test
Sickle Turbidity Quick Screen Test
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Naloxone (Narcan)
Naloxone (Narcan)
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Intensive Therapy for Diabetes
Intensive Therapy for Diabetes
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Insulin Pump
Insulin Pump
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Diabetic Ketoacidosis (DKA)
Diabetic Ketoacidosis (DKA)
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Cerebral Edema in DKA
Cerebral Edema in DKA
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Hyperventilation for Cerebral Edema
Hyperventilation for Cerebral Edema
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Mannitol for Cerebral Edema
Mannitol for Cerebral Edema
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Decreased Fluid Therapy for Cerebral Edema
Decreased Fluid Therapy for Cerebral Edema
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Head of Bed Elevation for Cerebral Edema
Head of Bed Elevation for Cerebral Edema
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Acanthosis nigricans
Acanthosis nigricans
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Growth Hormone Deficiency in Children
Growth Hormone Deficiency in Children
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Precocious Puberty
Precocious Puberty
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Insulin Sensitivity
Insulin Sensitivity
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Insulin Absorption Rate
Insulin Absorption Rate
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Toddler Sleepiness or Irritability
Toddler Sleepiness or Irritability
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Delayed Puberty in Diabetes
Delayed Puberty in Diabetes
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Medical Alert Identification for Diabetics
Medical Alert Identification for Diabetics
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What is Molluscum Contagiosum?
What is Molluscum Contagiosum?
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What is Meningococcal disease?
What is Meningococcal disease?
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What is Erythema Infectiosum (Fifth Disease)?
What is Erythema Infectiosum (Fifth Disease)?
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What is Measles?
What is Measles?
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What is Mononucleosis?
What is Mononucleosis?
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What is the Measles prodrome?
What is the Measles prodrome?
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What are Koplik spots?
What are Koplik spots?
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What is photophobia?
What is photophobia?
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Study Notes
HIRSCHSPRUNG DISEASE
- Also called congenital aganglionic megacolon
- Mechanical obstruction from inadequate motility of the intestine
- Incidence: 1 in 5,000 live births; more common in males and Down syndrome
- Birth defect; nerve cells missing in intestines, lacking ganglion cells
- Typically affects the rectum, preventing relaxation and defecation
- Gold standard: Rectal biopsy showing absence of ganglion cells in the colon
- Due to the inability to pass stool, the sphincter doesn't relax, causing stool to back up
- Results in a megacolon (distended sigmoid colon)
PATHOPHYSIOLOGY
- Aganglionic segment usually includes rectum and proximal colon
- Accumulation of stool with distention
- Failure of internal anal sphincter to relax
- Enterocolitis may occur
- Ribbon-like stools common
CHILD WITH A COLOSTOMY
- When lacking ganglion cells, portions of the intestine are removed
- May be temporary or permanent
- Temporary: bowel rest for 6 months
- Permanent: removal of more than 1 foot of intestine
- Megacolon is treated with resection/colostomy surgery.
- May need resection repeatedly to treat affected portions
INTUSSUSCEPTION
- Telescoping or invagination of one part of the intestine into another
- Usually involves the ileum
- Cutting off blood supply
- Occasionally tied to intestinal lesions, sometimes unknown cause
- Abdominal pain, abdominal mass and blood stools are classic triad
- Ileum telescopes into itself
MANIFESTATIONS
- Acute abdominal pain alternating with intervals of no pain
- Empty RLQ, but all issues within RUQ causing intervals.
- Passage of red currant jelly stools
- Sausage-shaped mass in RUQ
CYSTIC FIBROSIS
- Exocrine gland dysfunction leading to multisystem involvement
- Most common lethal genetic illness among white children
- Approximately 1,000 new diagnoses per year in the US
PATHOPHYSIOLOGY
- In normal cells, chloride channels actively transport chloride to the cell surface, hydrating secretions (e.g., mucus in the lungs).
- In CF, chloride channels are abnormal in all exocrine glands.
- Water doesn't follow chloride, making secretions thick and viscous.
- Increased viscosity of mucus-producing glands
- Mechanical obstruction, thick secretions, and concretions in glands and ducts lead to problems
- Primarily affects the respiratory tract and pancreas.
RESPIRATORY MANIFESTATIONS
- Mucus does not carry inhaled materials out of the lungs.
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