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{{WBRQuestion
{{WBRQuestion
|QuestionAuthor={{AO}}
|QuestionAuthor= {{SSK}},  {{AO}} (Reviewed by Serge Korjian)
|ExamType=USMLE Step 1
|ExamType=USMLE Step 1
|MainCategory=Behavioral Science/Psychiatry
|MainCategory=Behavioral Science/Psychiatry
|SubCategory=Endocrine
|MainCategory=Behavioral Science/Psychiatry
|MainCategory=Behavioral Science/Psychiatry
|SubCategory=Endocrine
|MainCategory=Behavioral Science/Psychiatry
|MainCategory=Behavioral Science/Psychiatry
|SubCategory=Endocrine
|MainCategory=Behavioral Science/Psychiatry
|MainCategory=Behavioral Science/Psychiatry
|MainCategory=Behavioral Science/Psychiatry
|MainCategory=Behavioral Science/Psychiatry
|MainCategory=Behavioral Science/Psychiatry
|MainCategory=Behavioral Science/Psychiatry
|SubCategory=Endocrine
|MainCategory=Behavioral Science/Psychiatry
|MainCategory=Behavioral Science/Psychiatry
|SubCategory=Endocrine
|MainCategory=Behavioral Science/Psychiatry
|MainCategory=Behavioral Science/Psychiatry
|SubCategory=Endocrine
|MainCategory=Behavioral Science/Psychiatry
|MainCategory=Behavioral Science/Psychiatry
|SubCategory=Endocrine
|MainCategory=Behavioral Science/Psychiatry
|MainCategory=Behavioral Science/Psychiatry
|MainCategory=Behavioral Science/Psychiatry
|MainCategory=Behavioral Science/Psychiatry
|Prompt=A 7-year-old boy is brought to the pediatrics clinic by his mother for fever of 39 °C (102.2 °F), cough and wheezing for the past week. Over the last 24 hours, the child has developed nausea, vomiting, and watery diarrhea.  The child's past medical history is significant for neurofibromatosis, asthma, and seizure disorder.  He is currently taking daily albuterol and valproic acid. His mother explains that she was diagnosed with insulin-dependent diabetes mellitus at his age and fears he may have developed it too.  On physical examination, you note a lethargic and ill-appearing boy.  His heart rate is 96/min, blood pressure is 124/84 mm Hg, temperature is 36.8°C (98.2°F), and respiratory rate is 28/min. You decide to admit the child for evaluation and monitoring. Over the next two days, the patient has recurrent episodes of hypoglycemia, with fingerstick glucose levels as low as 33 mg/dL. Both episodes occur at night, with the mother alerting the nurses of each time. Futher investigation reveals a serum insulin level of 2344 µU/mL (5–25 µU/mL) and a C-peptide level of 0.9 ng/mL (0.8–4.0 ng/mL). Which of the following explains the cause of the boy’s hypoglycemia?
|SubCategory=Endocrine
|Explanation=[[Munchausen syndrome by proxy]] is a pattern of behavior in which a caregiver deliberately exaggerates, fabricates, and/or induces physical, psychological, behavioral, and/or mental health problems in those who are in their care. In this vignette, the child was been injected insulin as demonstrated by the markedly elevated plasma insulin level despite a normal serum C-peptide level, and the mother was most likely responsible.
|Prompt=A 7-year-old boy is brought to the pediatrics clinic for fever of 39 °C (102.2 °F), cough, and wheezing for the past week. Over the last 24 hours, the child has developed nausea, vomiting, and watery diarrhea.  The child's past medical history is significant for neurofibromatosis, asthma, and seizure disorder.  He is currently taking daily albuterol and valproic acid. His mother explains that she was diagnosed with insulin-dependent diabetes mellitus at his age and fears he may have developed it too.  On physical examination, you note a lethargic and ill-appearing boy.  His heart rate is 96/min, blood pressure is 124/84 mm Hg, temperature is 36.8°C (98.2°F), and respiratory rate is 28/min. You decide to admit the child for evaluation and monitoring. Over the next two days, the patient has recurrent episodes of hypoglycemia, with fingerstick glucose levels as low as 33 mg/dL. Both episodes occur at night, with the mother alerting the nurses each time. Further investigation reveals a serum insulin level of 2344 µU/mL (5–25 µU/mL) and a C-peptide level of 0.7 ng/mL (0.8–4.0 ng/mL). What is the most likely diagnosis in this patient?
 
|Explanation=The patient in this scenario is presenting for recurrent hypoglycemic episodes that are most likely related to exogenous administration of insulin. The classical triad in of patients with true hypoglycemia is known as the Whipple's triad. It consists of (1) symptoms known or likely to be caused by hypoglycemia, (2) a low plasma glucose measured at the time of the symptoms, and (3) a relief of symptoms when glucose level is restored to normal. In patients who are not diabetic, a thorough work-up to uncover the etiology of hypoglycemia is indicated. Insulin levels followed by C-peptide (with or without proinsulin) measurement in patients with elevated insulin levels are required as a primary assessment. In patients with elevated C-peptide, further evaluation for sulfonylurea abuse, insulinoma, or autoimmune hypoglycemia. Autoimmune syndromes are a rare cause of hypoglycemia and can be diagnosed by screening for specific antibodies. Sulfonylureas are insulin secretagogues that increase endogenous insulin thus increasing C-peptide concentrations. In patients with low C-peptide i.e. low endogenous production of insulin, and high insulin levels, the cause is exogenous insulin administration and is almost always factitious. This patient is a victim of Munchausen's syndrome by proxy, where the mother induced a health condition in the child while under her care by injecting him with her own insulin.
Munchausen syndrome by proxy should be suspected in the following scenarios:
|AnswerA=Insulinoma
* Persistent or recurrent illness that cannot be explained
|AnswerAExp=Insulinomas are an endogenous source of insulin that release C-peptide as part of the production process of insulin. C-peptide would be elevated along with insulin levels in patients with insulinoma.
* Discrepancies between clinical findings and history
|AnswerB=Sulfonylurea intake
* Symptoms that occur only when the mother (or suspected perpetrator) is present
|AnswerBExp=Sulfonylureas are insulin secretagogues that increase endogenous insulin thus increasing C-peptide concentrations.
* Symptoms or treatment course that is not clinically consistent
|AnswerC=Exogenous insulin administration
* A working diagnosis that is less plausible than Munchausen syndrome by proxy
|AnswerCExp=Given this patient's high insulin levels and low C-peptide, exogenous administration of insulin is the most likely etiology.
* A mother who welcomes even painful medical tests for her child, is constantly at the bedside, and has previous medical experience, yet seems less concerned than the medical staff about the health of her child
|AnswerD=Type 1 diabetes mellitus
* Family history of sudden or unexplained infant death
|AnswerDExp=Patients with documented type 1 diabetes mellitus who are on insulin therapy can commonly have episodes of hypoglycemia that often only require dose adjustment of their insulin. However, this patient has never been diagnosed with diabetes mellitus, and should not be on therapy. Diabetes mellitus type 1 as a sole etiology would likely cause hyperglycemia.
|AnswerA=Malingering
|AnswerE=Adrenal insufficiency
|AnswerAExp=Incorrect.  This is fabricating or exaggerating the symptoms of mental or physical disorders in order to attain a "secondary gain" which may include financial compensation, avoiding school, work or military service, obtaining drugs, getting lighter criminal sentences, or simply to attract attention or sympathy.  These complaints cease with attainment of the secondary gain.
|AnswerEExp=Adrenal insufficiency is an important cause of hypoglycemia, but given the documented elevation in insulin levels, it is low on the list of differentials.
|AnswerB=Munchausen’s syndrome by proxy
|EducationalObjectives=Exogenous insulin abuse is a common phenomenon that presents with true hypoglycemia (Whipple's triad) with elevated insulin levels and low C-peptide and proinsulin.
|AnswerBExp=Correct.  This happens when illness in a child is caused by a caregiver.
|References=Scarlett JA, Mako ME, Rubenstein AH, et al. Factitious hypoglycemia. Diagnosis by measurement of serum C-peptide immunoreactivity and insulin-binding antibodies. N Engl J Med. 1977;297(19):1029-32.
|AnswerC=Adjustment disorder
|RightAnswer=C
|AnswerCExp=Incorrect.  This is a condition where an individual is unable to adjust to or cope with a particular stressor, like a major life event.  Symptoms may include anxiety or depression, and which lasts for less than 6 months.
|WBRKeyword=Insulin, Insulinoma, Munchausen's syndrome by proxy, Hypoglycemia, C-peptide
|AnswerD=Munchausen syndrome
|Approved=Yes
|AnswerDExp=Incorrect. [[Munchausen syndrome]] is a psychiatric factitious disorder wherein those affected exaggerate diseases, illnesses, or psychological trauma to draw attention, sympathy, or reassurance to themselves. It is also sometimes known as hospital addiction syndrome, thick chart syndrome, or hospital hopper syndrome.  There is a history of multiple hospital admissions and willingness to receive invasive procedures.
|AnswerE=Somatization disorder
|AnswerEExp=Incorrect.  [[Somatization disorder]], a.k.a Briquet's syndrome or [[hysteria]], is characterized by recurring, multiple, clinically significant complaints about pain, gastrointestinal, sexual and pseudoneurological symptoms. Individuals with somatization disorder typically visit many doctors in pursuit of effective treatment
|RightAnswer=B
|WBRKeyword=Munchausen syndrome by proxy
|Approved=No
}}
}}

Latest revision as of 00:46, 28 October 2020

 
Author [[PageAuthor::Serge Korjian M.D., Ayokunle Olubaniyi, M.B,B.S [1] (Reviewed by Serge Korjian)]]
Exam Type ExamType::USMLE Step 1
Main Category MainCategory::Behavioral Science/Psychiatry
Sub Category SubCategory::Endocrine
Prompt [[Prompt::A 7-year-old boy is brought to the pediatrics clinic for fever of 39 °C (102.2 °F), cough, and wheezing for the past week. Over the last 24 hours, the child has developed nausea, vomiting, and watery diarrhea. The child's past medical history is significant for neurofibromatosis, asthma, and seizure disorder. He is currently taking daily albuterol and valproic acid. His mother explains that she was diagnosed with insulin-dependent diabetes mellitus at his age and fears he may have developed it too. On physical examination, you note a lethargic and ill-appearing boy. His heart rate is 96/min, blood pressure is 124/84 mm Hg, temperature is 36.8°C (98.2°F), and respiratory rate is 28/min. You decide to admit the child for evaluation and monitoring. Over the next two days, the patient has recurrent episodes of hypoglycemia, with fingerstick glucose levels as low as 33 mg/dL. Both episodes occur at night, with the mother alerting the nurses each time. Further investigation reveals a serum insulin level of 2344 µU/mL (5–25 µU/mL) and a C-peptide level of 0.7 ng/mL (0.8–4.0 ng/mL). What is the most likely diagnosis in this patient?]]
Answer A AnswerA::Insulinoma
Answer A Explanation AnswerAExp::Insulinomas are an endogenous source of insulin that release C-peptide as part of the production process of insulin. C-peptide would be elevated along with insulin levels in patients with insulinoma.
Answer B AnswerB::Sulfonylurea intake
Answer B Explanation AnswerBExp::Sulfonylureas are insulin secretagogues that increase endogenous insulin thus increasing C-peptide concentrations.
Answer C AnswerC::Exogenous insulin administration
Answer C Explanation AnswerCExp::Given this patient's high insulin levels and low C-peptide, exogenous administration of insulin is the most likely etiology.
Answer D AnswerD::Type 1 diabetes mellitus
Answer D Explanation [[AnswerDExp::Patients with documented type 1 diabetes mellitus who are on insulin therapy can commonly have episodes of hypoglycemia that often only require dose adjustment of their insulin. However, this patient has never been diagnosed with diabetes mellitus, and should not be on therapy. Diabetes mellitus type 1 as a sole etiology would likely cause hyperglycemia.]]
Answer E AnswerE::Adrenal insufficiency
Answer E Explanation AnswerEExp::Adrenal insufficiency is an important cause of hypoglycemia, but given the documented elevation in insulin levels, it is low on the list of differentials.
Right Answer RightAnswer::C
Explanation [[Explanation::The patient in this scenario is presenting for recurrent hypoglycemic episodes that are most likely related to exogenous administration of insulin. The classical triad in of patients with true hypoglycemia is known as the Whipple's triad. It consists of (1) symptoms known or likely to be caused by hypoglycemia, (2) a low plasma glucose measured at the time of the symptoms, and (3) a relief of symptoms when glucose level is restored to normal. In patients who are not diabetic, a thorough work-up to uncover the etiology of hypoglycemia is indicated. Insulin levels followed by C-peptide (with or without proinsulin) measurement in patients with elevated insulin levels are required as a primary assessment. In patients with elevated C-peptide, further evaluation for sulfonylurea abuse, insulinoma, or autoimmune hypoglycemia. Autoimmune syndromes are a rare cause of hypoglycemia and can be diagnosed by screening for specific antibodies. Sulfonylureas are insulin secretagogues that increase endogenous insulin thus increasing C-peptide concentrations. In patients with low C-peptide i.e. low endogenous production of insulin, and high insulin levels, the cause is exogenous insulin administration and is almost always factitious. This patient is a victim of Munchausen's syndrome by proxy, where the mother induced a health condition in the child while under her care by injecting him with her own insulin.

Educational Objective: Exogenous insulin abuse is a common phenomenon that presents with true hypoglycemia (Whipple's triad) with elevated insulin levels and low C-peptide and proinsulin.
References: Scarlett JA, Mako ME, Rubenstein AH, et al. Factitious hypoglycemia. Diagnosis by measurement of serum C-peptide immunoreactivity and insulin-binding antibodies. N Engl J Med. 1977;297(19):1029-32.]]

Approved Approved::Yes
Keyword WBRKeyword::Insulin, WBRKeyword::Insulinoma, WBRKeyword::Munchausen's syndrome by proxy, WBRKeyword::Hypoglycemia, WBRKeyword::C-peptide
Linked Question Linked::
Order in Linked Questions LinkedOrder::