Publication Details > Case Report Type > Unique/unexpected symptoms or presentations of a disease

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Mohammad Alali Kuwait Board of Internal Medicine, Kuwait City, Safat, Kuwait

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Sulaiman Hajji Department of Internal Medicine, Adan Hospital, Kuwait City, Kuwait

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Khalid Aljenaee Department of Internal Medicine, Adan Hospital, Kuwait City, Kuwait

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Summary

Endometrioid carcinomas of the ovary are a subtype of epithelial ovarian tumors, with sertoliform endometrioid carcinomas being a rare variant. We report a case of a previously healthy premenopausal woman presenting with androgenic symptoms in the form of hirsutism and male pattern alopecia. On further testing, she was found to have high levels of luteinizing hormone and total testosterone levels, and imaging revealed a large pelvic abdominal mass in the right ovary. She underwent total hysterectomy with bilateral salpingo-oophorectomy. Microscopy and histopathology confirmed the diagnosis of sertoliform endometrioid carcinoma. Her symptoms improved significantly on follow-up. Androgenic tumors might not be common in premenopausal women; however, it is important to maintain a high level of suspicion in patients presenting with virilizing symptoms especially of rapid progression.

Learning points

  • Our 47-year-old patient presented with virilizing symptoms that were rapidly progressing, which raises the suspicion of an underlying androgen secreting neoplasm.

  • Sertoliform endometrioid carcinoma (SEC) is an extremely rare variant of endometrioid carcinomas and tend to present at an earlier stage as compared to most endometrioid carcinomas of the ovary.

  • Recognition of SEC in virilizing patients is important as it is a well-differentiated, low-grade malignancy with a good prognosis when confined to the ovary.

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Takuya Kumagai Postgraduate Clinical Training Center, Akita Red Cross Hospital, Akita, Japan

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Syohei Koyama Department of Metabolism, Akita Red Cross Hospital, Akita, Japan

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Haruka Yorozu Department of Gastroenterology, Akita Red Cross Hospital, Akita, Japan

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Ayaka Kokita Department of Metabolism, Akita Red Cross Hospital, Akita, Japan

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Naoko Shimizu Department of Metabolism, Akita Red Cross Hospital, Akita, Japan

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Yumi Suganuma Department of Metabolism, Akita Red Cross Hospital, Akita, Japan

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Takashi Goto Department of Metabolism, Akita Red Cross Hospital, Akita, Japan

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Summary

There are very few reports of syndrome of inappropriate antidiuresis hormone secretion (SIADH) after receiving severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) mRNA vaccine. Herein, we present the case of an 84-year-old woman who developed severe hyponatremia following the second administration of the SARS-CoV-2 mRNA vaccine. The patient presented with nausea, vomiting, and headache. Laboratory tests showed a plasma sodium level of 119 mmol/L. After receiving 500 mL of intravenous saline over a 2-h period, her plasma sodium level raised to 121 mmol/L, but her symptoms persisted. Considering that rapid plasma sodium correction was necessary, we started 3% saline solution overnight. Her plasma sodium level raised to 132 mmol/L and her symptoms completely resolved. Clinical and laboratory findings were consistent with a diagnosis of SIADH. In the absence of any other triggering factors, we concluded that the condition was likely associated with the vaccination. Clinicians should be aware of the potential for hyponatremia, particularly SIADH, associated with SARS-CoV-2 mRNA vaccination.

Learning points

  • Reports of syndrome of inappropriate antidiuresis hormone secretion after receiving severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccination are limited.

  • If nausea, headache, and confusion are observed immediately after SARS-CoV-2 vaccination, clinicians should consider the presence of hyponatremia.

  • As similar case reports to date have presented with severe hyponatremia, prompt treatment may be required.

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Erica A Steen University of California, San Diego, San Diego, California, USA

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Mary E Patterson University of California, San Diego, San Diego, California, USA
Rady Children’s Hospital, Department of Pediatrics, University of California, San Diego, California, USA

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Michelle Rivera-Vega Rady Children’s Hospital, Department of Pediatrics, University of California, San Diego, California, USA

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Susan A Phillips University of California, San Diego, San Diego, California, USA
Rady Children’s Hospital, Department of Pediatrics, University of California, San Diego, California, USA

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Summary

An 11-year-old girl with past medical history of septic shock and multi-organ failure at age 5 presented to her primary care doctor with concern for pallor of the lips. Laboratory studies demonstrated low free thyroxine (T4) and normal thyroid-stimulating hormone (TSH). A referral to endocrinology was made where the patient was evaluated, and laboratory evaluation was repeated. The patient was asymptomatic and clinically euthyroid with a height consistent with her mid-parental height and was in mid- to late-puberty. The repeated laboratory evaluation demonstrated a pattern suggestive of primary hypothyroidism with low free T4 and an elevated TSH. However, the magnitude of elevation of TSH was less than expected, given the degree of lowering of free T4; therefore, central hypothyroidism was considered. Workup was initiated, and laboratory studies and MRI imaging confirmed an underlying diagnosis of panhypopituitarism in the setting of pituitary stalk interruption syndrome.

Learning points

  • Pituitary stalk interruption syndrome is a rare but important cause of panhypopituitarism.

  • Central hypothyroidism should be suspected in patients with low free thyroxine with an inappropriate degree of elevation of thyroid-stimulating hormone.

  • Workup of central hypothyroidism should include multi-pituitary hormone assessment, and, if evident, MRI imaging should be done.

  • Adrenal insufficiency should be suspected in a hypotensive, critically ill patient who is failing to improve on standard-of-care therapy.

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Nam Quang Tran Department of Endocrinology, University of Medicine and Pharmacy at Ho Chi Minh City, Ho Chi Minh City, Vietnam
Department of Endocrinology, University Medical Center at Ho Chi Minh City, Ho Chi Minh City, Vietnam

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Chien Cong Phan Department of Imaging, University Medical Center at Ho Chi Minh City, Ho Chi Minh City, Vietnam

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Tran Bao Vuong Department of Endocrinology, University of Medicine and Pharmacy at Ho Chi Minh City, Ho Chi Minh City, Vietnam

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Thang Viet Tran Department of Endocrinology, University of Medicine and Pharmacy at Ho Chi Minh City, Ho Chi Minh City, Vietnam
Department of Endocrinology, University Medical Center at Ho Chi Minh City, Ho Chi Minh City, Vietnam

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Phat Tung Ma Department of Endocrinology, University of Medicine and Pharmacy at Ho Chi Minh City, Ho Chi Minh City, Vietnam
Department of Endocrinology, University Medical Center at Ho Chi Minh City, Ho Chi Minh City, Vietnam

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Summary

Mitochondrial diseases are a group of rare diseases presenting with heterogeneous clinical, biochemical, and genetic disorders caused by mutations in the mitochondrial or nuclear genome. Multiple organs can be affected, particularly those with high energy demand. Diabetes is a common endocrine manifestation of mitochondrial diseases. The onset of mitochondrial diabetes can be latent or acute, and the presenting phenotype can be type 1- or type 2-like. Studies show that diabetes ais associated with latent progression of cognitive decline in patients with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome. Herein, we report a case of rapid cognitive decline after the acute onset of diabetes in a patient with MELAS syndrome. The patient was a 36-year-old woman who was hospitalized due to hyperglycemic crisis and seizures. She was diagnosed with MELAS syndrome two years previously, and had gradually progressing dementia and hearing loss. However, following the acute onset of diabetes, she developed rapid cognitive decline and loss of ability to perform daily activities. In conclusion, the acute onset of diabetes could be an associated risk factor for rapid cognitive decline in patients with MELAS syndrome. Thus, these patients as well as healthy carriers with related genetic mutations should undergo diabetes education and screening tests. Moreover, clinicians should be aware of the possibility for acute onset of hyperglycemic crisis, particularly in the presence of triggering factors.

Learning points

  • Diabetes is a common endocrine manifestation of mitochondrial diseases, presenting with a type 1- or type 2-like phenotype depending on the level of insulinopenia.

  • Metformin should be avoided in patients with mitochondrial diseases to prevent metformin-induced lactic acidosis.

  • Mitochondrial diabetes can manifest before or after the onset of MELAS syndrome.

  • In patients with MELAS syndrome, diabetes can initially manifest with a life-threatening severe hyperglycemic crisis and can cause rapid cognitive decline.

  • Diabetes screening tests (e.g. hemoglobin A1c, oral glucose tolerance test, or random blood glucose level measurement) should be performed either systematically or in the presence of symptoms, particularly after triggering events.

  • Genetic testing and counseling should be provided to patients and their families for the purpose of better understanding the inheritance, progression, and possible outcomes of the disease.

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Luca Foppiani Internal Medicine, Galliera Hospital, Genoa, Italy

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Maria Gabriella Poeta Neurology, Galliera Hospital, Genoa, Italy

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Mariangela Rutigliani Department of Pathology, Galliera Hospital, Genoa, Italy

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Simona Parodi Neuroradiology, Galliera Hospital, Genoa, Italy

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Ugo Catrambone Department of Surgery, Galliera Hospital, Genoa, Italy

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Lorenzo Cavalleri Anesthesia and Intensive Care Unit, Galliera Hospital, Genoa, Italy

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Giancarlo Antonucci Internal Medicine, Galliera Hospital, Genoa, Italy

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Patrizia Del Monte Endocrinology, Galliera Hospital, Genoa, Italy

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Arnoldo Piccardo Nuclear Medicine, Galliera Hospital, Genoa, Italy

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Summary

Cushing’s syndrome due to ectopic adrenocorticotropic hormone (ACTH) secretion (EAS) by a pheochromocytoma is a challenging condition. A woman with hypertension and an anamnestic report of a ‘non-secreting’ left adrenal mass developed uncontrolled blood pressure (BP), hyperglycaemia and severe hypokalaemia. ACTH-dependent severe hypercortisolism was ascertained in the absence of Cushingoid features, and a psycho-organic syndrome developed. Brain imaging revealed a splenial lesion of the corpus callosum and a pituitary microadenoma. The adrenal mass displayed high uptake on both 18F-FDG PET/CT and 68Ga-DOTATOC PET/CT; urinary metanephrine levels were greatly increased. The combination of antihypertensive drugs, high-dose potassium infusion, insulin and steroidogenesis inhibitor normalized BP, metabolic parameters and cortisol levels; laparoscopic left adrenalectomy under intravenous hydrocortisone infusion was performed. On combined histology and immunohistochemistry, an ACTH-secreting pheochromocytoma was diagnosed. The patient's clinical condition improved and remission of both hypercortisolism and catecholamine hypersecretion ensued. Brain magnetic resonance imaging showed a reduction of the splenial lesion. Off-therapy BP and metabolic parameters remained normal. The patient was discharged on cortisone replacement therapy for post-surgical hypocortisolism. EAS due to pheochromocytoma displays multifaceted clinical features and requires prompt diagnosis and multidisciplinary management in order to overcome the related severe clinical derangements.

Learning points

  • A small but significant number of cases of adrenocorticotropic hormone (ACTH)-dependent Cushing’s syndrome are caused by ectopic ACTH secretion by neuroendocrine tumours, which is usually associated with severe hypercortisolism causing severe clinical and metabolic derangements.

  • Ectopic ACTH secretion by a pheochromocytoma is exceedingly rare but can be life-threatening, owing to the simultaneous excess of both cortisol and catecholamines.

  • The combination of biochemical and hormonal testing and imaging procedures is mandatory for the diagnosis of ectopic ACTH secretion, and in the presence of an adrenal mass, the possibility of an ACTH-secreting pheochromocytoma should be taken into account.

  • Immediate-acting steroidogenesis inhibitors are required for the treatment of hypercortisolism, and catecholamine excess should also be appropriately managed before surgical removal of the tumour.

  • A multidisciplinary approach is required for the treatment of this challenging entity.

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Foram Patel Department of Pediatrics, Southern Illinois University, Springfield, Illinois, USA

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Ginger Darling Department of Pediatrics, Division of Neonatology, Southern Illinois University, Springfield, Illinois, USA

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Ahmed Torky Department of Pediatrics, Division of Pediatric Endocrinology and Diabetes, Southern Illinois University, Springfield, Illinois, USA

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Summary

Neonatal hypoglycemia is a serious condition that can have a major impact on the growing neonatal brain. The differential diagnosis of neonatal hypoglycemia is broad and includes hyperinsulinism as well as panhypopituitarism. The FOXA2 gene has been involved in the development of the pancreas as well as the pituitary gland. Six cases have been reported thus far with FOXA2 mutations presenting with variable degrees of hypopituitarism, and only two patients had permanent hyperinsulinism; other cases have been reported with microdeletions in 20p11, the location that encompasses FOXA2, and those patients presented with a wider phenotype. A full-term female infant presented with severe hypoglycemia. Critical sampling showed an insulin of 1 mIU/mL, suppressed beta-hydroxybutyric acids, and suppressed free fatty acids. Blood glucose responded to glucagon administration. Growth hormone (GH) stimulation test later showed undetectable GH in all samples, and cortisol failed to respond appropriately to stimulation. Gonadotropins were undetectable at 1 month of life, and MRI showed ectopic posterior pituitary, interrupted stalk, hypoplastic anterior pituitary, cavum septum pellucidum, and diminutive appearance of optic nerves. Whole-exome sequencing revealed a likely pathogenic de novo c.604 T>C, p.Tyr202His FOXA2 mutation. We expand the known phenotype on FOXA2 mutations and report a likely pathogenic, novel mutation associated with hyperinsulinism and panhypopituitarism.

Learning points

  • FOXA2 has been shown to play an important role in the neuroectodermal and endodermal development.

  • FOXA2 mutation may lead to the rare combination of hyperinsulinism and panhypopituitarism.

  • Patients reported so far all responded well to diazoxide. Dysmorphology may be subtle, and liver functions should be monitored.

Open access
Bruno Bouça Department of Endocrinology, Diabetes and Metabolism - Centro Hospitalar Universitário de Lisboa Central, Lisbon, Portugal
Nova Medical School/ Faculdade de Ciencias Medicas, Universidade Nova de Lisboa, Lisbon, Portugal

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Mariana Cascão Intensive Care Unit - Centro Hospitalar Universitário de Lisboa Central, Lisbon, Portugal

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Pedro Fiúza Department of Internal Medicine, Unit 7.2 - Centro Hospitalar Universitário de Lisboa Central, Lisbon, Portugal

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Sara Amaral Department of Endocrinology, Diabetes and Metabolism - Centro Hospitalar Universitário de Lisboa Central, Lisbon, Portugal
Nova Medical School/ Faculdade de Ciencias Medicas, Universidade Nova de Lisboa, Lisbon, Portugal

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Paula Bogalho Department of Endocrinology, Diabetes and Metabolism - Centro Hospitalar Universitário de Lisboa Central, Lisbon, Portugal
Nova Medical School/ Faculdade de Ciencias Medicas, Universidade Nova de Lisboa, Lisbon, Portugal

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José Silva-Nunes Department of Endocrinology, Diabetes and Metabolism - Centro Hospitalar Universitário de Lisboa Central, Lisbon, Portugal
Nova Medical School/ Faculdade de Ciencias Medicas, Universidade Nova de Lisboa, Lisbon, Portugal
Health and Technology Research Center (H&TRC), Escola Superior de Tecnologia da Saude de Lisboa, Lisbon, Portugal

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Summary

17-Alpha-hydroxylase deficiency (17OHD) is a rare autosomal recessive disease, representing 1% of cases of congenital adrenal hyperplasia. A 44-year-old female presented to the emergency department complaining of generalized asthenia and polyarthralgia for about 2 weeks. On examination, she was hypertensive (174/100 mmHg), and laboratory results revealed hypokalemia and hypocortisolism. She had an uncharacteristic morphotype, BMI of 16.7 kg/m2, cutaneous hyperpigmentation, and Tanner stage M1P1, with normal female external genitalia. She reported to have primary amenorrhea. Further analytical evaluations of her hormone levels were performed CT scan revealed adrenal bilateral hyperplasia and absence of female internal genitalia. A nodular lesion was observed in the left inguinal canal with 25 × 10 mm, compatible with a testicular remnant. Genetic analysis identified the c.3G>A p.(Met1?) variant in homozygosity in the CYP17A1 gene, classified as pathogenic, confirming the diagnosis of 17OHD. Karyotype analysis was compatible with 46,XY. The association of severe hypokalemia, hypertension, hypocortisolism, and oligo/amenorrhea and the absence of secondary sexual characteristics favored the diagnosis of 17OHD, confirmed by genetic testing. As in other published clinical cases, diagnosis outside pediatric age is not rare and should be considered when severe hypokalemia occurs in hypertensive adults with a lack of secondary sexual characteristics.

Learning points

  • The association of severe hypokalemia, hypertension, hypocortisolism, and oligo/amenorrhea and the absence of secondary sexual characteristics favor the diagnosis of 17-alpha-hydroxylase deficiency (17OHD).

  • Diagnosis outside pediatric age is not rare.

  • 17OHD should be considered when severe hypokalemia occurs in hypertensive adults with a lack of secondary sexual characteristics.

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Osamu Horikawa Department of Medicine, Shiga University of Medical Science, Otsu, Shiga, Japan

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Satoshi Ugi Department of Medicine, Shiga University of Medical Science, Otsu, Shiga, Japan
Department of Medicine, Omihachiman Community Medical Center, Omihachiman, Shiga, Japan

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Tomofumi Takayoshi Division of Diabetes and Endocrinology, Kobe University Graduate School of Medicine, Kobe, Japan

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Yasushi Omura Department of Internal Medicine, Kohka Public Hospital, Kohka, Shiga, Japan

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Maya Yonishi Department of Medicine, Shiga University of Medical Science, Otsu, Shiga, Japan

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Daisuke Sato Department of Medicine, Shiga University of Medical Science, Otsu, Shiga, Japan

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Yukihiro Fujita Department of Medicine, Shiga University of Medical Science, Otsu, Shiga, Japan

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Tomoya Fuke Department of Medicine, Saiseikai Shiga Hospital, Ritto, Shiga, Japan

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Yushi Hirota Division of Diabetes and Endocrinology, Kobe University Graduate School of Medicine, Kobe, Japan

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Wataru Ogawa Division of Diabetes and Endocrinology, Kobe University Graduate School of Medicine, Kobe, Japan

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Hiroshi Maegawa Department of Medicine, Shiga University of Medical Science, Otsu, Shiga, Japan

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Summary

A 17-year-old boy was referred to our endocrinology clinic for a clinical investigation of hyperinsulinemia. An oral glucose tolerance test showed plasma glucose concentrations in the normal range. However, insulin concentrations were considerably elevated (0 min: 71 μU/mL; 60 min: 953 μU/mL), suggesting severe insulin resistance. An insulin tolerance test confirmed that he had insulin resistance. There was no apparent hormonal or metabolic cause, including obesity. The patient had no outward features of hyperinsulinemia, including acanthosis nigricans or hirsutism. However, his mother and grandfather also had hyperinsulinemia. Genetic testing showed that the patient (proband), his mother, and his grandfather had a novel p.Val1086del heterozygous mutation in exon 17 of the insulin receptor gene (INSR). Although all three family members have the same mutation, their clinical courses have been different. The onset of the mother’s diabetes was estimated at 50 years, whereas the grandfather developed diabetes at 77 years.

Learning points

  • Type A insulin resistance syndrome is caused by mutations in the insulin receptor (INSR) gene and results in severe insulin resistance.

  • Genetic evaluation should be considered in adolescents or young adults with dysglycemia when an atypical phenotype, such as severe insulin resistance, or a relevant family history is observed.

  • Clinical courses may differ even if the same genetic mutation is found in a family.

Open access
Toshitaka Sawamura Department of Internal Medicine, Asanogawa General Hospital, Kosakamachinaka, Kanazawa, Ishikawa, Japan
Department of Endocrinology and Metabolism, Kanazawa University Graduate School of Medicine, Takaramachi, Kanazawa, Japan
Department of Health Promotion and Medicine of the Future, Kanazawa University, Takaramachi, Kanazawa, Japan

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Shigehiro Karashima Department of Health Promotion and Medicine of the Future, Kanazawa University, Takaramachi, Kanazawa, Japan

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Ai Ohmori Department of Internal Medicine, Asanogawa General Hospital, Kosakamachinaka, Kanazawa, Ishikawa, Japan
Department of Endocrinology and Metabolism, Kanazawa University Graduate School of Medicine, Takaramachi, Kanazawa, Japan

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Kei Sawada Department of Internal Medicine, Asanogawa General Hospital, Kosakamachinaka, Kanazawa, Ishikawa, Japan

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Daisuke Aono Department of Endocrinology and Metabolism, Kanazawa University Graduate School of Medicine, Takaramachi, Kanazawa, Japan
Department of Health Promotion and Medicine of the Future, Kanazawa University, Takaramachi, Kanazawa, Japan

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Mitsuhiro Kometani Department of Endocrinology and Metabolism, Kanazawa University Graduate School of Medicine, Takaramachi, Kanazawa, Japan
Department of Health Promotion and Medicine of the Future, Kanazawa University, Takaramachi, Kanazawa, Japan

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Yoshiyu Takeda Department of Internal Medicine, Asanogawa General Hospital, Kosakamachinaka, Kanazawa, Ishikawa, Japan

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Takashi Yoneda Department of Endocrinology and Metabolism, Kanazawa University Graduate School of Medicine, Takaramachi, Kanazawa, Japan
Department of Health Promotion and Medicine of the Future, Kanazawa University, Takaramachi, Kanazawa, Japan

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Summary

Fulminant type 1 diabetes (FT1D) is a subtype of diabetes characterized by rapid progression of β-cell destruction, hyperglycemia, and diabetic ketoacidosis (DKA). The pathogenesis of this disease remains unclear. However, viral infections, HLA genes, and immune checkpoint inhibitor use were reportedly involved in this disease. A 51-year-old Japanese man with no chronic medical condition was admitted to our hospital with complaints of nausea and vomiting. Cough, sore throat, nasal discharge, and diarrhea were not noted. He had a medical history of at least two influenza infections. His vaccination history was notable for receiving an inactive split influenza vaccine 12 days prior to developing these symptoms. He was diagnosed with DKA associated with FT1D. His HLA class II genotypes were nonsusceptible to FT1D, and he had a negative history of immune checkpoint inhibitor use. The destruction of the pancreas by cytotoxic T cells is reported to be involved in FT1D. Inactive split influenza vaccines do not directly activate cytotoxic T cells. However, these could activate the redifferentiation of memory CD8-positive T cells into cytotoxic T cells and induce FT1D, as this patient had a history of influenza infections.

Learning points

  • Influenza split vaccination could cause fulminant type 1 diabetes (FT1D).

  • The mechanism of influenza split vaccine-induced FT1D might be through the redifferentiation of CD8-positive memory T cells into cytotoxic T cells.

Open access
Wafa Belabed Department of Endocrinology and Diabetology, Hedi Chaker University Hospital, Sfax, Tunisia

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Fatma Mnif Department of Endocrinology and Diabetology, Hedi Chaker University Hospital, Sfax, Tunisia

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Abdel Mouhaymen Missaoui Department of Endocrinology and Diabetology, Hedi Chaker University Hospital, Sfax, Tunisia

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Mouna Elleuch Department of Endocrinology and Diabetology, Hedi Chaker University Hospital, Sfax, Tunisia

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Dhoha Ben Salah Department of Endocrinology and Diabetology, Hedi Chaker University Hospital, Sfax, Tunisia

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Nadia Charfi Department of Endocrinology and Diabetology, Hedi Chaker University Hospital, Sfax, Tunisia

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Mouna Mnif Department of Endocrinology and Diabetology, Hedi Chaker University Hospital, Sfax, Tunisia

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Nabila Rekik Department of Endocrinology and Diabetology, Hedi Chaker University Hospital, Sfax, Tunisia

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Faten Hadj Kacem Department of Endocrinology and Diabetology, Hedi Chaker University Hospital, Sfax, Tunisia

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Mohamed Abid Department of Endocrinology and Diabetology, Hedi Chaker University Hospital, Sfax, Tunisia

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Summary

A 55-year-old patient was admitted to our department for the management of a repetitive alteration of consciousness. Biological investigation results were consistent with endogenous hyperinsulinemic hypoglycemia. Insulinoma was therefore suspected. Abdominal computed tomography and endoscopic ultrasound showed no obvious pancreatic mass.Somatostatin receptor scintigraphy showed abnormal radioactive uptake in both the pancreatic tail and the uncinate process. Contrariwise, abdominal magnetic resonance imaging showed a unique lesion in the pancreas tail. The patient was then proposed for pancreatic surgery. Both intraoperative manual palpation and intraoperative ultrasonography of the pancreas showed a single corporal lesion of 1.5 cm. No lesion was found in the uncinate process. After a left pancreatectomy, the lesion was histopathologically confirmed to be a well-differentiated neuroendocrine tumor. The symptoms of the patient resolved almost immediately following the surgery. The follow-up is one and a half years to date.

Learning points

  • The exact preoperative localization of the pancreatic mass remains the most challenging part of insulinoma diagnostic workup.

  • The radiologist’s experience is the best warrantor to a precise localization of the tumor.

  • 111In-DTPA-octreotide uptake in the pancreatic uncinate process may be physiological and its interpretation must, therefore, be vigilant.

  • Manual palpation along with intraoperative ultrasonography is considered as the most effective method for the localization of insulinomas during open surgery.

Open access