Rett syndrome is characterised by:

Correct Answer: D

Rationale: Rett syndrome is a rare genetic disorder that primarily affects girls and leads to severe impairments in motor, cognitive, and social functioning. The correct answer is D) Wringing hand movements. This is a classic feature of Rett syndrome, where affected individuals show repetitive, stereotyped hand movements such as wringing, clapping, or tapping. Option A) Social withdrawal is not typically a defining feature of Rett syndrome. While individuals with Rett syndrome may experience social difficulties, the hallmark symptoms are more related to motor function. Option B) Self-mutilation can occur in some cases of Rett syndrome but is not as characteristic as the hand-wringing movements seen in these individuals. Option C) Macrocephaly, or abnormally large head size, is not typically associated with Rett syndrome. In fact, individuals with Rett syndrome often have normal head size or even microcephaly. In an educational context, understanding the specific clinical manifestations of Rett syndrome is crucial for healthcare professionals, especially nurses caring for pediatric patients. By recognizing the key features of the syndrome, healthcare providers can facilitate early diagnosis, appropriate interventions, and supportive care for individuals with Rett syndrome and their families. This knowledge enhances the quality of care provided and contributes to improved outcomes for these vulnerable patients.

Correct Answer: B

Rationale: In pediatric cardiovascular disorders nursing, understanding the clinical manifestations of hemophilia is crucial for providing safe and effective care to pediatric patients. The correct answer is option B) Intracranial bleeding. Hemophilia is a genetic disorder characterized by a deficiency in clotting factors, leading to prolonged and excessive bleeding. Intracranial bleeding is a severe complication of hemophilia and can be life-threatening, especially in pediatric patients. Option A) Bleeding following neonatal intramuscular injection is not a common clinical manifestation of hemophilia. While individuals with hemophilia may experience bleeding after invasive procedures, it is not specific to neonatal injections. Option C) Gingival bleeding is more commonly associated with conditions like gingivitis or blood clotting disorders like thrombocytopenia, rather than hemophilia specifically. Option D) Menorrhagia, or excessive menstrual bleeding, is not a typical clinical manifestation of hemophilia in pediatric patients. While females with hemophilia may experience menorrhagia, it is not a primary clinical manifestation seen in pediatric patients with hemophilia. Educationally, identifying the clinical manifestations of hemophilia, such as intracranial bleeding, helps nurses and healthcare providers promptly recognize and address complications in pediatric patients with this condition. Understanding these manifestations is essential for early intervention and preventing severe outcomes in pediatric patients with hemophilia.

Correct Answer: B

Rationale: In the context of pediatric cardiovascular disorders nursing, understanding distal renal tubular acidosis (dRTA) is crucial. The correct answer is B) May be primary or secondary. Explanation of why B is correct: Distal renal tubular acidosis can be primary, due to a genetic defect affecting the kidneys' ability to regulate acid-base balance, or secondary, caused by conditions like autoimmune diseases or medications. Recognizing this distinction is essential for appropriate management and treatment. Explanation of why others are wrong: A) Is characterised by hypokalaemia - While hypokalemia can occur in some types of renal tubular acidosis, it is more commonly associated with Type 1 renal tubular acidosis, not specifically distal RTA. C) Presents with growth failure in infancy - Growth failure is more commonly seen in proximal renal tubular acidosis, not distal RTA. D) Urinary pH is usually < 5 - In distal RTA, the urinary pH is typically higher (>5.5) due to impaired acidification of urine in the distal tubules. Educational context: Understanding the nuances of different types of renal tubular acidosis is vital for nursing care, especially in pediatric patients with cardiovascular disorders. Recognizing the specific characteristics of distal RTA can guide appropriate interventions and prevent complications related to acid-base imbalances. Nurses must be able to differentiate between types of RTA to provide safe and effective care for these patients.

Correct Answer: B

Rationale: The correct answer is B) In a twice daily regimen involving Mixtard 30 or Humulin M3, the bedtime reading is an effect of the long-acting component. Rationale: In a twice daily regimen with Mixtard 30 or Humulin M3, the bedtime reading is predominantly influenced by the long-acting component of the insulin mixture. This is because the long-acting insulin component works over an extended period, including during the night, helping to maintain stable blood sugar levels. The short-acting component in these mixtures primarily covers mealtime insulin requirements. Explanation of Incorrect Options: A) Short-acting insulin typically reaches peak action within 1-3 hours, not 4-6 hours. This option is incorrect as it provides inaccurate information about the peak action time of short-acting insulin. C) A bedtime blood sugar level of 10 mmol/l may be acceptable depending on the individual's specific diabetes management plan and target range. It is not universally unacceptable and can vary based on the patient's condition and treatment goals. D) While rotating injection sites is recommended to prevent lipodystrophy and ensure consistent insulin absorption, using the same site for injections does not directly lead to increasing insulin dosage. It may impact insulin absorption but is not a common cause of increasing dosage. Educational Context: Understanding the pharmacokinetics and pharmacodynamics of different insulin types is crucial for nurses caring for pediatric patients with diabetes. By knowing how each component of a mixed insulin regimen works, nurses can effectively manage blood glucose levels and prevent complications. Educating patients and families on insulin administration techniques, regimen specifics, and monitoring parameters is essential for successful diabetes management in pediatric patients.

Correct Answer: A

Rationale: In the context of pediatric cardiovascular disorders nursing, understanding diseases that primarily involve the motor unit is crucial for providing comprehensive care. The correct answer is A) Werdnig-Hoffman disease, also known as spinal muscular atrophy type 1. This disease specifically affects the motor neurons in the spinal cord, leading to progressive muscle weakness and atrophy. Guillain-Barre syndrome (option B) is a disorder that affects the peripheral nervous system, causing ascending paralysis but does not primarily involve the motor unit. Facioscapulohumeral dystrophy (option C) is a genetic muscle disorder that affects the face, shoulders, and upper arms primarily, not just the motor unit. Charcot-Marie-Tooth disease (option D) is a hereditary neuropathy that affects the peripheral nerves, leading to muscle weakness and sensory loss, but it does not primarily involve the motor unit. Educationally, differentiating between these diseases is essential as it aids in accurate diagnosis, treatment, and management strategies. Understanding the specific pathophysiology of each condition helps nurses and healthcare providers deliver individualized care to pediatric patients with cardiovascular disorders and motor unit involvement. By grasping these distinctions, caregivers can offer targeted interventions to improve outcomes and quality of life for children affected by these conditions.