Insulinoma or non-insulinoma pancreatogenous hypoglycemia? A diagnostic dilemma
نویسندگان
چکیده
Insulinoma is the most common cause of endogenous hyperinsulinemic hypoglycemia in adults. An alternate etiology, non-insulinoma pancreatogenous hypoglycemia (NIPH), is rare. Clinically, NIPH is characterized by postprandial hyperinsulinemic hypoglycemia, negative 72-h fasts, negative preoperative localization studies for insulinoma and positive selective arterial calcium infusion tests. Histologically, diffuse islet hyperplasia with increased number and size of islet cells is present and confirms the diagnosis. Differentiating NIPH from occult insulinoma preoperatively is challenging. Partial pancreatectomy is the procedure of choice; however, recurrence of symptoms, although less debilitating, occurs commonly. Medical management with diazoxide, verapamil and octreotide can be used for persistent symptoms. Ultimately, near-total or total pancreatectomy may be necessary. We report a case of a 67-year-old male with hypoglycemia in whom preoperative workup, including computerized tomography abdomen, suggested insulinoma, but whose final diagnosis on pathology was NIPH instead.
منابع مشابه
Refractory idiopathic non-insulinoma pancreatogenous hypoglycemia in an adult: case report and review of the literature.
CONTEXT Non-insulinoma pancreatogenous hypoglycemia is a rare cause of spontaneous hypoglycemia in adults. The ideal diagnostic and therapeutic approach is still controversial, not least because most reported cases lack long-term follow-up. CASE REPORT We describe the case of a 58-year-old woman, who was diagnosed with idiopathic non-insulinoma pancreatogenous hypoglycemia in 2001. After rese...
متن کاملReversal of impaired counterregulatory cortisol response following diazoxide treatment in a patient with non insulinoma pancreatogenous hypoglycemia syndrome: Case report and overview of pathogenetic mechanisms.
OBJECTIVE Non-insulinoma pancreatogenous hypoglycemia syndrome (NIPHS) is one of the rare causes of endogenous hyperinsulinism. Its diagnosis is challenging and may require selective intraarterial calcium stimulation and concomitant hepatic vein sampling (SACVS). Impaired counterregulatory hormones' production in response to hypoglycemia has been previously described in patients with diabetes, ...
متن کاملElectrical short-circuit in β-cells from a patient with non-insulinoma pancreatogenous hypoglycemic syndrome (NIPHS): a case report
INTRODUCTION Non-insulinoma pancreatogenous hypoglycemic syndrome is a rare disorder among adults, and, to our knowledge, only about 40 cases have been reported in the literature. CASE PRESENTATION The patient is a previously healthy 35-year-old Caucasian man. His symptoms began four years ago when he suddenly felt weakness in his legs and started sweating for unknown reasons. The symptoms wo...
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We present the case of a 55 yr female who had recurrent severe hypoglycemic attacks with neuroglycopenic symptoms and altered sensorium including coma. The hypoglycemic episodes were not related to fasting. The hypoglycemia was hyperinsulinemic but all imaging modalities for insulinoma were negative. Selective arterial calcium stimulation test localized the lesion to splenic artery territory an...
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Nesidioblastosis is focal or diffuse islet hyperplasia leading to hyperinsulinism with subsequent hypoglycemia in the absence of insulinoma, usually described in neonates and infancy. We described the first adult case of nesidioblastosis in Saudi Arabia and the Gulf region. The diagnosis and treatment of the condition can be very difficult and challenging. Despite the fact that our patient resp...
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عنوان ژورنال:
دوره 2016 شماره
صفحات -
تاریخ انتشار 2016