scholarly journals Hypoglycemic Syndrome in a Patient with Proinsulin-Only Secreting Pancreatic Adenoma (Proinsulinoma)

2011 ◽  
Vol 2011 ◽  
pp. 1-5 ◽  
Author(s):  
Gian Paolo Fadini ◽  
Alberto Maran ◽  
Anna Valerio ◽  
Francesco Meduri ◽  
Mariarosa Pelizzo ◽  
...  

We describe an unusual case of hypoglycemic syndrome in a 69-year old woman with a proinsulin-only secreting pancreatic endocrine adenoma. The clinical history was highly suggestive of an organic hypoglycemia, with normal or relatively low insulin concentrations and elevated proinsulin levels. Magnetic resonance and computed tomography of the abdomen showed a 1 cm pancreatic nodule and multiple accessory spleens. The diagnosis was confirmed by selective angiography, showing location and vascularization of the nodule, despite no response to intra-arterial calcium. After resection, the hypoglycemic syndrome resolved. The surgical specimen was comprised of a neuroendocrine adenomatous tissue with high proinsulin immunoreactivity. Study of this unusual case of proinsulinoma underlines (i) the need to assay proinsulin in patients with hypoglycemia and normal immunoreactive insulin, (ii) the differential diagnosis in the presence of accessory spleens, (iii) the unresponsiveness to intra-arterial calcium stimulation, and (iv) the extensive evaluation needed to reach a final diagnosis.

2016 ◽  
Vol 19 (1) ◽  
pp. 028
Author(s):  
Shengjun Wu ◽  
Peng Teng ◽  
Yiming Ni ◽  
Renyuan Li

Coronary sinus aneurysm (CSA) is an extremely rare entity. Herein, we present an unusual case of an 18-year-old symptomatic female patient with a giant CSA. Secondary vena cava aneurysms were also manifested. The final diagnosis was confirmed by enhanced computed tomography (CT) and cardiac catheterization. As far as we know, it is the first case that such a giant CSA coexists with secondary vena cava aneurysms. Considering the complexity of postoperative reconstruction, we believe that heart transplantation may be the optimal way for treatment. The patient received anticoagulant due to the superior vena cava (SVC) thrombosis while waiting for a donor.


2019 ◽  
Vol 2019 ◽  
pp. 1-3
Author(s):  
Mohammad Tinawi

The patient is a 75-year-old man who presented with right arm pain, edema, and erythema. The same manifestations appeared in the other arm 3 weeks later. He also developed fever, acute kidney injury, anemia, and truncal edema. Initial extensive evaluation was unrevealing. He was noted to have elevated creatine kinase, and a diagnostic muscle biopsy lead to diagnosis of inflammatory myositis. He improved with corticosteroids.


2021 ◽  
Vol 1 (2) ◽  
pp. 65-67
Author(s):  
AC Diallo ◽  
A Ndong ◽  
I Niang ◽  
MB Ba ◽  
JA Thiam ◽  
...  

OBJECTIVE: We report the case of a patient presenting an abdominal mass whose final diagnosis is a gastrointestinal stromal tumor (GIST). CLINICAL OBSERVATION: It was a 61-year-old patient with no pathological history received for a progressive increase in the volume of the abdomen evolving for one year and painless. On examination, the patient was in good general condition, with normal patterns. There was an abdomino-pelvic mass of about 20 cm long axis, mobile. The rest of the exam was unremarkable. The biological assessment was normal. Abdominopelvic computed tomography revealed a tissue mass with necrosis. During the surgical exploration, a mass developed on the mesenteric border 45 cm from the Treitz angle was noted. It is not associated with ascites or peritoneal carcinoma. Bowel resection removing the mass was performed followed by end-to-end anastomosis. Pathological examination of the surgical specimen diagnosed GIST. The postoperative course at three months was excellent both clinically and radiologically. CONCLUSION: The case that we report underlines the possible jejunal localization of GIST and the clinical presentation may be usual. It also shows the difficulty of obtaining a preoperative diagnosis and the central role of surgery in management.


Author(s):  
Rosa Lillo ◽  
Angelica Bibiana Delogu ◽  
Gessica Ingrasciotta ◽  
Gianluigi Perri ◽  
Maria Grandinetti ◽  
...  

A woman complaining of dyspnea and chest pain since childhood, was referred to our hospital with an initial diagnosis of biventricular hypertrophic cardiomyopathy. Multimodality imaging evaluation revealed massive right ventricular (RV) hypertrophy and severe RV outflow tract obstruction, with a final diagnosis of double chambered RV associated with small ventricular septal defect with right-to-left shunt and right partial anomalous pulmonary vein return. This represents an uncommon combination of congenital abnormalities, extremely rarely diagnosed in adulthood.


Author(s):  
Thulasi Raman Ramalingam

AbstractInborn errors of immunity (IEI) are a group of inherited heterogeneous disorders affecting the immune system characterized by increased susceptibility to infections, immune dysregulation, and lymphoproliferation. Flow cytometry (FCM) is a rapid and reliable technique for evaluation and enumeration of immune cells. It also helps in understanding the functional and signaling pathways of the immune system. Lymphocyte subset analysis is a simple and effective screening tool in suspected combined and humoral immunodeficiency patients. Qualitative phagocytic defects such as chronic granulomatous disease and leucocyte adhesion defect are easily diagnosed by FCM. Study of intracellular proteins (e.g., BTK, WASP, DOCK8), cytokine production, and signaling molecules (e.g., STAT3) by FCM is very useful but also quite challenging to establish. T and B lymphocyte interaction for normal class switching of B cells can be assessed and can help in diagnosis of combined variable immunodeficiency and hyperimmunoglobulin M syndrome. FCM is also used in posttransplant monitoring of IEI patients and also in prenatal diagnosis in suspected cases. It is also useful in validation of variants of uncertain significance obtained in exome sequencing. FCM results should always be interpreted with clinical history and, if needed, should be confirmed with molecular genetic studies before establishing the final diagnosis. Ensuring good sample quality and running parallel controls with patient samples will avoid the preanalytical and analytical errors. This review describes the applications of FCM in the diagnosis of various IEI.


2019 ◽  
Vol 27 (5) ◽  
pp. 568-573
Author(s):  
Carolina Martinez-Ciarpaglini ◽  
Isidro Machado ◽  
Akihiko Yoshida ◽  
Gema Nieto ◽  
Ana P. Berbegall ◽  
...  

Adult neuroblastoma is an extremely infrequent neoplasm, usually occurring in the adrenal medulla or in the paraspinal sympathetic ganglia, as its childhood counterpart. We report a very unusual case of a Schwannian stroma-poor adult neuroblastoma of inguinal location, showing aberrant expression of germ cell markers: SALL4 and OCT4. This aberrant marker expression, the unusual positivity for NKX2.2 and the very scattered (instead of diffuse strong) PHOX2B expression, complicated the initial diagnosis. In this case, the posttreatment histological evaluation revealed the neuroblastic nature of the lesion. Neuroblastoma maturation after treatment is an unusual finding in adults, and in this case, added an important clue for the final diagnosis. Germs cells markers expression in neuroblastoma is an interesting feature to explore and may define a subset of neuroblastomas with a different biological nature.


Author(s):  
Ronald B. Postuma

A diagnosis of REM sleep behavior disorder (RBD), a disorder characterized by “acting out” of dreams during REM sleep, has critical implications for a patient’s future. Aside from being a treatable parasomnia, usually managed with melatonin or clonazepam, RBD is the most powerful risk factor for Parkinson disease and dementia with Lewy bodies yet discovered. Over 70% of patients with idiopathic RBD will develop a neurodegenerative synucleinopathy. Moreover, the disease course is more severe in patients with RBD than those without. Numerous screens have been developed to aid detection, and clinical history can help distinguish RBD from NREM parasomnia. However, final diagnosis relies on polysomnographic documentation of REM atonia loss. Given the profound implications of idiopathic RBD, patients need careful counseling and the offer of neurological follow-up to detect and treat prodromal disease symptoms. Recognition of RBD is also a means to discover and test protective therapies against neurodegenerative disease.


2021 ◽  
Author(s):  
Ruri Ogawa ◽  
Yoshiyuki Minami ◽  
Junya Ono ◽  
Yoriaki Kanri ◽  
Eizaburo Kobayashi ◽  
...  

1982 ◽  
Vol 56 (5) ◽  
pp. 732-733 ◽  
Author(s):  
Francesco Tognetti ◽  
Massimo Poppi ◽  
Giulio Gaist ◽  
Franco Servadei

✓ An unusual case of pudendal neuralgia due to a solitary neurofibroma of the perineal region is described. The authors outline the long clinical history. There was complete pain relief after removal of the lesion.


2011 ◽  
Vol 30 (5) ◽  
pp. 313-319
Author(s):  
Natalie Mercy

Fetus in fetu is an extremely unusual occurrence where a rare congenital tumor containing a vertebral axis, often containing parts of other organs or limbs, is identified. Normally identified in the newborn period, the size and location of the mass may result in significant multiorgan compromise. This article presents the challenges encountered by the neonatal intensive care unit team in providing care for a late preterm female infant presenting with gross abdominal distension. Description of the case includes preoperative management along with management of severe coagulopathy and hemorrhage that arose during the intraoperative and postsurgical course of care. A brief overview of abdominal masses in the newborn is presented. The importance in determining the final diagnosis of fetus in fetu and its differentiation from teratoma is discussed.


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