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1.
Diagn Pathol ; 18(1): 115, 2023 Oct 20.
Artigo em Inglês | MEDLINE | ID: mdl-37864201

RESUMO

BACKGROUND: Adult non-neoplastic hyperinsulinemic hypoglycemia (ANHH), also known as adult-onset nesidioblastosis, is a rare cause of endogenous hyperinsulinemic hypoglycemia in adults. This disease is characterized by diffuse hyperplasia of pancreatic endocrine cells and is diagnosed by a pathological examination. While diagnostic criteria for this disease have already been proposed, we established more quantitative criteria for evaluating islet morphology. METHODS: We measured the number, maximum diameter, total area, and circularity (representing how closely islets resemble perfect spheres) of islets contained in representative sections of ANHH (n = 4) and control cases (n = 5) using the NIS-Elements software program. We also measured the average cell size, percentage of cells with enlarged nuclei, and percentage of cells with recognizable nucleoli for each of three representative islets. We also assessed the interobserver diagnostic concordance of ANHH between five experienced and seven less-experienced pathologists. RESULTS: There was no significant difference in the number, maximum diameter, or total area of islets between the two groups, even after correcting for these parameters per unit area. However, the number of islets with low circularity (< 0.71) per total area of the pancreatic parenchyma was significantly larger in ANHH specimens than in controls. We also found that the percentage of cells with recognizable nucleoli was significantly higher in the ANHH group than in the controls. There were no significant differences in the average cell size or the number of cells with enlarged nuclei between the groups. The correct diagnosis rate with the blind test was 47.5% ± 6.12% for experienced pathologists and 50.0% ± 8.63% for less-experienced pathologists, with no significant differences noted. CONCLUSIONS: Low circularity, which indicates an irregular islet shape, referred to as "irregular shape and occasional enlargement of islets" and "lobulated islet structure" in a previous report, is a useful marker for diagnosing ANHH. An increased percentage of recognizable nucleoli, corresponding to "macronucleoli in ß-cells," has potential diagnostic value.


Assuntos
Hiperinsulinismo , Hipoglicemia , Ilhotas Pancreáticas , Nesidioblastose , Adulto , Humanos , Ilhotas Pancreáticas/patologia , Ilhotas Pancreáticas/cirurgia , Hipoglicemia/diagnóstico , Hipoglicemia/etiologia , Hiperinsulinismo/diagnóstico , Hiperinsulinismo/etiologia , Hiperinsulinismo/patologia , Pâncreas/patologia , Nesidioblastose/complicações , Nesidioblastose/patologia , Nesidioblastose/cirurgia
2.
Endocr Relat Cancer ; 30(9)2023 09 01.
Artigo em Inglês | MEDLINE | ID: mdl-37279235

RESUMO

Nesidioblastoma and nesidioblastosis were terms given to neoplastic and non-neoplastic lesions of the pancreas associated with pancreatogenous hyperinsulinaemic hypoglycaemia. While nesidioblastoma was rapidly replaced by islet cell tumour, nesidioblastosis, defined as the proliferation of islet cells budding off from pancreatic ducts, was the diagnostic term associated with congenital hyperinsulinism of infancy (CHI) and adult non-neoplastic hyperinsulinaemic hypoglycaemia (ANHH). When it was shown that nesidioblastosis was not specific for CHI or ANHH, it was no longer applied to CHI but kept for the morphological diagnosis of ANHH. In severe CHI cases, a diffuse form with hypertrophic ß-cells in all islets can be distinguished from a focal form with hyperactive ß-cells changes in a limited adenomatoid hyperplastic area. Genetically, mutations were identified in several ß-cell genes involved in insulin secretion. Most common are mutations in the ABCC8 or KCNJ11 genes, solely affected in the diffuse form and associated with a focal maternal allelic loss on 11p15.5 in the focal form. Focal CHI can be localized by 18F-DOPA-PET and is thus curable by targeted resection. Diffuse CHI that fails medical treatment requires subtotal pancreatectomy. In ANHH, an idiopathic form can be distinguished from a form associated with gastric bypass, in whom GLP1-induced stimulation of the ß-cells is discussed. While the ß-cells in idiopathic ANHH are diffusely affected and are either hypertrophic or show only little changes, it is controversial whether there is a ß-cell increase or ß-cell hyperactivity in patients with gastric bypass. Recognizing morphological signs of ß-cell hyperactivity needs a good knowledge of the non-neoplastic endocrine pancreas across all ages.


Assuntos
Adenoma de Células das Ilhotas Pancreáticas , Hiperinsulinismo Congênito , Hiperinsulinismo , Nesidioblastose , Neoplasias Pancreáticas , Humanos , Adulto , Hiperinsulinismo Congênito/genética , Hiperinsulinismo Congênito/patologia , Nesidioblastose/diagnóstico , Nesidioblastose/patologia , Nesidioblastose/cirurgia , Hiperinsulinismo/genética , Pâncreas/patologia
3.
Int J Surg Pathol ; 27(8): 912-918, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31244364

RESUMO

Nesidioblastosis is an uncommon cause of organic persistent hyperinsulinemic hypoglycemia in adults. We report a case of adult-onset diffuse ß-cell nesidioblastosis in a 49-year-old woman who was status-post Roux-en-Y gastric bypass and distal pancreatectomy for a well-differentiated pancreatic neuroendocrine tumor. While the neuroendocrine tumor was suspected to be an insulinoma, persistent hypoglycemia postoperatively suggested either incomplete resection or a second pancreatic neoplasm. Completion pancreatectomy revealed islet ß-cell hyperplasia and nuclear pleomorphism consistent with ß-cell nesidioblastosis. The patient's blood glucose levels normalized after completion pancreatectomy. While ß-cell nesidioblastosis and insulinomas can coexist in the same patient, pathologists should be aware of ß-cell nesidioblastosis as a potential cause for hyperinsulinemic hypoglycemia and should exclude it in patients who have not shown definitive clinical response after surgical excision of a pancreatic neuroendocrine tumor.


Assuntos
Células Secretoras de Insulina/patologia , Nesidioblastose/diagnóstico , Tumores Neuroendócrinos/diagnóstico , Neoplasias Pancreáticas/diagnóstico , Idade de Início , Diagnóstico Diferencial , Feminino , Humanos , Insulinoma/diagnóstico , Insulinoma/patologia , Pessoa de Meia-Idade , Nesidioblastose/complicações , Nesidioblastose/patologia , Nesidioblastose/cirurgia , Tumores Neuroendócrinos/complicações , Tumores Neuroendócrinos/patologia , Tumores Neuroendócrinos/cirurgia , Pancreatectomia , Neoplasias Pancreáticas/complicações , Neoplasias Pancreáticas/patologia , Neoplasias Pancreáticas/cirurgia
5.
Endocr Pathol ; 27(1): 21-4, 2016 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-26318442

RESUMO

Mediastinal teratomas with elements of mature pancreatic tissue are rare. Only a very few cases of pancreatic tissue with nesidioblastosis in teratoma have been reported. Here, we report a case of a 12-year-old male who presented with pleural effusion and was revealed to have a large anterior mediastinal mass. Biopsy of the mass revealed benign mature teratoma. After biopsy, the teratoma ruptured into the right thoracic cavity. It was then excised and sent to pathology for further evaluation. Preoperatively, there was no evidence of hyperinsulinemia or hypoglycemia. Postoperatively, there was no change in blood glucose levels. Histologically, the mass showed large areas of mature pancreatic tissue flanking a small intestine-like structure. Numerous endocrine cell islets, poorly defined groups of neuroendocrine cells and ductular-insular complexes characteristic of nesidioblastosis were dispersed in the exocrine pancreatic parenchyma. In addition, other parts of the tumor containing keratinizing squamous epithelium with cutaneous adnexal glands, small intestine, and bronchus including cartilage and respiratory epithelium were observed. Some islets contained two or more cell types while others were monophenotypic. Immunohistochemical staining showed pronounced expression of pancreatic polypeptide, moderate expression of somatostatin and insulin and nearly complete absence of glucagon-containing cells. The selective deletion of glucagon might hold clues to an important regulatory mechanism in pancreatic development.


Assuntos
Neoplasias do Mediastino/patologia , Pâncreas/patologia , Teratoma/patologia , Biomarcadores Tumorais/análise , Diferenciação Celular , Criança , Glucagon/metabolismo , Humanos , Imuno-Histoquímica , Ilhotas Pancreáticas/patologia , Masculino , Nesidioblastose/patologia
6.
J Clin Endocrinol Metab ; 100(11): 4189-97, 2015 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-26312578

RESUMO

CONTEXT: In adult patients with endogenous hyperinsulinemic hypoglycemia and negative or inconclusive noninvasive imaging, insulinoma and non-insulinoma pancreatogenous hypoglycemic syndrome (NIPHS) resulting from diffuse nesidioblastosis must be considered in the differential diagnosis. It is not known whether the biochemical results of selective arterial calcium stimulation (SACST) with hepatic venous sampling can differentiate insulinoma from diffuse nesidioblastosis. OBJECTIVE: To determine the specificity of SACST with hepatic venous sampling in differentiating insulinoma from diffuse nesidioblastosis. DESIGN: Retrospective review (January 1996 to March 2014). SETTING: Tertiary referral center. PATIENTS OR OTHER PARTICIPANTS: A total of 116 patients with biochemical evidence of endogenous hyperinsulinemic hypoglycemia and negative or inconclusive noninvasive imaging who were subsequently shown at surgery to have insulinoma (n = 42) or nesidioblastosis (n = 74) after undergoing SACST with hepatic venous sampling. INTERVENTION(S): SACST with hepatic venous sampling before pancreatic exploration. MAIN OUTCOME MEASURE(S): Receiver operating characteristic curves were generated from the biochemical results of SACST to determine the specificity of the maximum hepatic venous insulin concentration (mHVI) and the relative-fold increase in hepatic venous insulin concentration (rHVI) over baseline after calcium injection from the dominant artery in differentiating insulinoma from nesidioblastosis. RESULTS: The mHVI (21.5-fold; P < .001) and rHVI (3.9-fold; P < .001) were significantly higher in the insulinoma group compared to the nesidioblastosis group. The areas under the receiver operating characteristic curve for mHVI and rHVI were excellent (0.94; P < .0001) and good (0.83; P < .0001), respectively, for differentiating insulinoma from nesidioblastosis. mHVI cutoffs of > 91.5 and > 263.5 µIU/mL were 95 and 100% specific for insulinoma, respectively. A 19-fold increase in rHVI over baseline was 99% specific for insulinoma. CONCLUSIONS: These data suggest that the mHVI and rHVI at SACST may be useful in differentiating insulinoma from nesidioblastosis with high specificity in patients with hyperinsulinemic hypoglycemia and negative or inconclusive noninvasive imaging.


Assuntos
Cálcio/farmacologia , Insulinoma/diagnóstico , Nesidioblastose/diagnóstico , Neoplasias Pancreáticas/diagnóstico , Adulto , Idoso , Índice de Massa Corporal , Diagnóstico Diferencial , Feminino , Veias Hepáticas , Humanos , Hiperinsulinismo/diagnóstico , Hiperinsulinismo/etiologia , Hipoglicemia/diagnóstico , Hipoglicemia/etiologia , Insulinoma/patologia , Insulinoma/cirurgia , Masculino , Pessoa de Meia-Idade , Nesidioblastose/patologia , Nesidioblastose/cirurgia , Neoplasias Pancreáticas/patologia , Neoplasias Pancreáticas/cirurgia , Curva ROC , Estudos Retrospectivos
8.
BMJ Case Rep ; 20152015 Jan 06.
Artigo em Inglês | MEDLINE | ID: mdl-25564641

RESUMO

A 68-year-old male with a longstanding history of severe gastric outlet obstruction secondary to peptic stricture was found unconscious at home with profound hypoglycaemia. He denied history of fasting, diabetes mellitus or use of hypoglycaemic agents. Systemic examination and metabolic profile were unremarkable. Hypoglycaemic episodes persisted during hospitalisation, requiring continuous intravenous dextrose and eventually diazoxide. Further investigative work up, including 72 h fasting study, revealed a hyperinsulinemic state. MRI and endoscopic ultrasound were unremarkable but mesenteric angiography with hepatic venous sampling revealed a subtle area of hypervascularity in the head of pancreas. An exploratory laparotomy was then performed and resulted in resection of a nodular lesion in the head of pancreas with retrocolic gastrojejunostomy for repair of pyloric stenosis. Histopathology of pancreatic nodule confirmed nesidioblastosis. Postoperatively the patient was weaned of parenteral dextrose and did not have any further episodes of hypoglycaemia.


Assuntos
Hipoglicemia/diagnóstico , Nesidioblastose/diagnóstico , Pâncreas/patologia , Pancreatopatias/diagnóstico , Estenose Pilórica/cirurgia , Idoso , Angiografia , Diazóxido/uso terapêutico , Endossonografia , Derivação Gástrica , Obstrução da Saída Gástrica/cirurgia , Glucose/uso terapêutico , Humanos , Hiperinsulinismo/diagnóstico , Hiperinsulinismo/etiologia , Hiperinsulinismo/cirurgia , Hipoglicemia/tratamento farmacológico , Hipoglicemia/etiologia , Masculino , Nesidioblastose/complicações , Nesidioblastose/patologia , Nesidioblastose/cirurgia , Pâncreas/cirurgia , Pancreatopatias/complicações , Pancreatopatias/patologia , Pancreatopatias/cirurgia
9.
Int J Surg Pathol ; 23(1): 68-70, 2015 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-25326474

RESUMO

Here, we present the case of a 31-year-old woman patient who underwent distal pancreatectomy with the history of gastric bypass surgery for obesity. The final histopathological diagnosis of the lesion was nesidioblastosis. Nesidioblastosis is the most common cause of organic persistent hyperinsulinemic hypoglycemia in newborns; however, it is rare in adults. In adults, it is difficult to diagnose nesidioblastosis with only clinical findings. The definitive diagnosis of the disease depends on the histopathological examination of pancreatic tissue and the exclusion of insulinoma.


Assuntos
Derivação Gástrica/efeitos adversos , Nesidioblastose/etiologia , Obesidade/cirurgia , Pancreatectomia , Adulto , Feminino , Humanos , Nesidioblastose/patologia , Nesidioblastose/cirurgia , Resultado do Tratamento
10.
Surg Obes Relat Dis ; 10(6): 1220-5, 2014.
Artigo em Inglês | MEDLINE | ID: mdl-25002326

RESUMO

An association between post-Roux-en-Y gastric bypass (RYGB) hypoglycemia and nesidioblastosis was reported in 2005 and may cause serious neuroglycopenic symptoms. Most patients with postprandial hypoglycemia after RYGB respond to nutritional and medical treatment. A subset of patients, however, may not respond adequately and surgery may be considered. This review describes the current experience with surgical intervention for severe post-RYGB hypoglycemia. PubMed and MEDLINE searches were made for reports describing clinical outcome after such surgery. Fourteen papers including 75 patients were identified. Different surgical interventions were applied including gastric tube placement, reversal of the bypass with and without concomitant sleeve resection, gastric pouch restriction, and pancreatic resection and reresection. Pancreatic resection was performed in 51 (68%) patients, 17 (23%) had RYGB reversal and eleven (15%) had gastric pouch restriction alone. Eight (11%) patients received 2 or more consecutive procedures for hypoglycemia and combined interventions were made in several patients. Resolution of the symptoms occurred in 34/51 (67%) patients after pancreatic resection, 13/17 (76%) after reversal, and 9/11 (82%) after pouch restriction. Mean follow up, however, was short for most series and the methods applied for evaluation of hypoglycemia varied. Weight regain, diabetes and recurrent symptoms were late complications. The optimal therapy for hypoglycemia after RYGB is not defined. Long-term evaluations and knowledge about the physiology of post-RYGB hypoglycemia, may enable therapy with improved control of the glucose excursions.


Assuntos
Derivação Gástrica/efeitos adversos , Hiperinsulinismo/etiologia , Hipoglicemia/etiologia , Nesidioblastose/cirurgia , Obesidade Mórbida/cirurgia , Adulto , Idoso , Índice de Massa Corporal , Feminino , Seguimentos , Derivação Gástrica/métodos , Humanos , Hiperinsulinismo/epidemiologia , Hiperinsulinismo/fisiopatologia , Hipoglicemia/epidemiologia , Hipoglicemia/fisiopatologia , Incidência , Masculino , Pessoa de Meia-Idade , Nesidioblastose/epidemiologia , Nesidioblastose/patologia , Obesidade Mórbida/diagnóstico , Pancreatectomia/métodos , Reoperação/métodos , Medição de Risco
11.
Neoplasma ; 61(3): 252-6, 2014.
Artigo em Inglês | MEDLINE | ID: mdl-24645840

RESUMO

The persistent hyperinsulinemic hypoglycemia may be caused either by a solitary tumor of the pancreas secreting excessive amount of insulin, known as insulinoma or, rarely, by nesidioblastosis. Nesidioblastosis is a rare cause of persistent hyperinsulinemic hypoglycemia in adults. The incidence of nesidioblastosis in adults is unknown, but it is generally thought to be very low. The ß cell changes in adult nesidioblastosis suggest a dysregulation of the function of the cell. The cause of the functional dysregulation in adults is unknown. The pathogenesis of adult nesidioblastosis may be different from infantile congenital hyperinsulinism caused by a genetic effect. Histologically nesidioblastosis is almost always characterized by a proliferation of abnormal ß cells throughout the entire pancreas. Clinically and biochemically , it is not possible to distinguish between diffuse nesidioblastosis and insulinoma. If all highly selective noninvasive imaging techiques fail to identify a tumor, selective arterial calcium stimulation testing should be performed. The final diagnosis relies on the histopathologic evaluation. The treatment of adult nesidioblastosis is surgical resection of the pancreas.


Assuntos
Nesidioblastose/etiologia , Adulto , Humanos , Incidência , Células Secretoras de Insulina/patologia , Nesidioblastose/diagnóstico , Nesidioblastose/patologia , Nesidioblastose/terapia
12.
Pancreatology ; 13(5): 544-8, 2013.
Artigo em Inglês | MEDLINE | ID: mdl-24075522

RESUMO

INTRODUCTION: Nesidioblastosis is a rare disease caused by hyperplasia of pancreatic islets, developing a state of hypoglycemia due to an increase in the insulin production. It is the leading cause of hyperinsulinic hypoglycemia in childhood, whereas in adults it only represents the 0.5-5% of cases. The pathogenesis is still unknown. We have studied several genetic mutations associated with dependent potassium channel of ATP present in the beta cells of the pancreas, as well as in patients underwent bariatric surgery because of the metabolic changes involved. REPORT: Woman (38 years old) attends consultation of General Surgery derived from Endocrinology before symptoms of persistent hypoglycemia. Factitious hypoglycemia and syndromes of neuroendocrine origin were ruled out. Imaging tests failed to identify space-occupying lesions. The medical treatment failed, persisting hypoglycemia symptoms. Before the given analytical and radiological findings obtained, and the persistence of symptoms affecting the quality of life of the patient, we opted for surgical treatment performing a pancreatectomy of the 80% of the gland. The final pathologic diagnosis was nesidioblastosis. DISCUSSION: Nesidioblastosis is a rare pathology, but it must be present in the differential diagnosis of hypoglycemia symptoms with endogenous hyperinsulinism in adults, once the intake of sulfonylureas and possible pancreatic neoformations have been ruled out.


Assuntos
Ilhotas Pancreáticas/patologia , Nesidioblastose/patologia , Adulto , Diagnóstico Diferencial , Feminino , Humanos , Hiperplasia/complicações , Hipoglicemia/diagnóstico , Nesidioblastose/diagnóstico , Nesidioblastose/cirurgia , Pancreatectomia , Neoplasias Pancreáticas/diagnóstico
13.
Przegl Lek ; 69(1): 9-14, 2012.
Artigo em Polonês | MEDLINE | ID: mdl-22764512

RESUMO

Nesidioplastosis in adults is one of a rare causes of hyperinsulinemic hypoglycemia. Symptoms include chronic or recurrent hypoglycemias, often with neurological signs. Due to the looses of consciousness with coexisting seizures, in many cases patients are treated on epilepsy. Right diagnosis is usually late established, when the damages in the central nervous system (CNS) are irreversible. Early diagnosis of the disease and appropriate treatment might help to avoid serious disability in these patients. The aim of the study was to asses modern diagnostics of the nesidioblastosis with an emphasis on the biochemical and hormonal tests and imaging modalities. Patients enrolled to the study were aged between 18 and 72 years of age, and had chronic or recurrent hypoglycemia caused by hyperinsulinemia. In all patients fasting glucose and fasting insulinemia tests were performed, as well as the fasting blood test or in the 24-hour profile tests. Several techniques were used including ultrasound (US), abdominal computer tomography (CT), in two patients magnetic resonance imaging, scintigraphy of somatostatin receptors in seven patients, and in two patients scintigraphy with glucagone-like peptide-1 (GLP-1) analogue-labeled marker was done. In the performed tests low values of the blood glucose were found, whereas insulin levels, however not adequate to the blood glucose, were nearly always within the normal range. In the standard imaging only in one patient tumor lesion in the pancreatic tail was revealed, though not confirmed in the intraoperative histology. In the scintigraphy examination with the somatostatin analogue in one patient slightly increased collection of the marker in whole pancreas was reported and in the other patient focal collection in the pancreatic tail was observed. Scintigraphy with GLP-1 analogue revealed focal collection of the marker in one case. Five patients were underwent surgical treatment. In the histopathology in all operated patients hyperplasia of the endocrine pancreatic cells with positive immuno. histochemic reaction on the insulin was found. In the three cases despite hyperplasia of pancreatic islets, small sizes insulinomas were detected as well. 1. The diagnosis of nesidioblastosis should be taken into consideration in all patients with unclear-cause hypoglycemias, in whom simultaneously insulin blood level is inadequate to the level of glucose. 2. Widely available imaging examinations: US, CT, MRI are useless in the diagnosis of nesidioblastosis. 3. Among the imaging methods in preoperative diagnostics of hypoglycemia with concomitant hyperinsulinemia somatostatin receptor scintigraphy seems to have specific, though limited role - it is valuable only in the severe, diffused lesions. 4. Recurrent hypoglycemias after 70% excision of the pancreas may indicate the possibility of coexistence of pancreatic islets hyperplasia and insulin secreting insulinoma.


Assuntos
Nesidioblastose/diagnóstico , Adulto , Idoso , Diagnóstico Diferencial , Diagnóstico por Imagem/métodos , Diagnóstico Precoce , Feminino , Humanos , Hipoglicemia/etiologia , Insulinoma/diagnóstico , Masculino , Pessoa de Meia-Idade , Nesidioblastose/complicações , Nesidioblastose/patologia , Nesidioblastose/cirurgia , Neoplasias Pancreáticas/diagnóstico , Recidiva , Adulto Jovem
14.
Semin Pediatr Surg ; 20(1): 3-12, 2011 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-21185997

RESUMO

Congenital hyperinsulinism is clinically characterized by an inappropriate insulin secretion resulting in recurrent severe hypoglycemia. Nesidioblastosis, the proliferation of islet cells budding off from ducts, has been considered for years as the histologic lesion responsible for the syndrome. In our morphologic studies, we demonstrate that nesidioblastosis is not specific of the disease, which is actually not a single entity. Indeed, we recognize the existence of 2 different forms-a diffuse form and a focal form-and demonstrate that they can be differentiated by morphologic criteria, even on frozen sections during surgery. This histologic distinction directs the therapeutic approach because the patients experiencing the focal form of the syndrome can be completely cured by a very limited pancreatectomy. Molecular findings confirmed the reliability of this histologic distinction, showing a specific background for each form.


Assuntos
Hiperinsulinismo Congênito/patologia , Ilhotas Pancreáticas/patologia , Nesidioblastose/patologia , Hiperinsulinismo Congênito/etiologia , Hiperinsulinismo Congênito/cirurgia , Humanos , Imuno-Histoquímica , Lactente , Recém-Nascido , Células Secretoras de Insulina/metabolismo , Pancreatectomia
16.
Ethiop Med J ; 48(1): 73-9, 2010 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-20608002

RESUMO

We present history, physical examination, diagnosis and histological findings of one case of insulinoma and another case of nesidioblastosis. Insulinoma is a rare endocrine tumor, which is usually benign, characterized by hypoglycemic symptoms. The first case presented with seizure, which made diagnosis difficult. The second case presented with typical hypoglycemic symptoms. Both cases underwent partial pancrectomy and splenectomy after workup. The histology of the first case was consistent with insulinoma, while the second case was that of nesidioblastosis.


Assuntos
Insulinoma/complicações , Nesidioblastose/complicações , Neoplasias Pancreáticas/complicações , Adulto , Feminino , Humanos , Hiperinsulinismo/etiologia , Hiperinsulinismo/fisiopatologia , Hipoglicemia/etiologia , Hipoglicemia/fisiopatologia , Insulinoma/patologia , Insulinoma/cirurgia , Laparoscopia/métodos , Nesidioblastose/patologia , Nesidioblastose/cirurgia , Pancreatectomia , Neoplasias Pancreáticas/patologia , Neoplasias Pancreáticas/cirurgia , Esplenectomia
17.
Surg Endosc ; 24(10): 2547-55, 2010 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-20354884

RESUMO

INTRODUCTION: Postgastric bypass noninsulinoma hyperinsulinemic pancreatogenous hypoglycemia defines a group of patients with postprandial neuroglycopenic symptoms similar to insulinoma but in many cases more severe. There are few reports of patients with this condition. We describe our surgical experience for the management of this rare condition. METHODS: A retrospective study was performed at St. Vincent Hospital, Indianapolis. Fifteen patients were identified with symptomatic postgastric bypass hypoglycemia for the period 2004-2008. All patients were initially treated with medical therapy for hypoglycemia. Nine patients eventually underwent surgical treatment. The preoperative workup included triple-phase contrast CT scan of the abdomen, endoscopic ultrasound of the pancreas, a 72-h fast followed by a mixed meal test, and calcium-stimulated selective arteriography. Intraoperative pancreatic ultrasound also was performed in all patients. Patients then underwent thorough abdominal exploration, exploration of the entire pancreas, and extended distal pancreatectomy. RESULTS: Nine patients underwent surgery. The mean duration of symptoms was 14 months. The 72-h fast was negative in eight patients (as expected). Triple-phase contrast CT scan of the abdomen was negative in eight patients and showed a cyst in the head of pancreas in one patient. Extended distal (80%) pancreatectomy was performed in all nine patients. The procedure was attempted laparoscopically in eight patients but was converted to open in three. One patient had an open procedure from start to finish. Pathology showed changes compatible with nesidioblastosis with varying degrees of hyperplasia of islets and islet cells. Follow-up ranged from 8-54 (median, 22) months. All patients initially reported marked relief of symptoms. Over time, two patients had complete resolution of symptoms; three patients developed occasional symptoms (once or twice per month), which did not require any medication; two patients developed more frequent symptoms (more than twice per month), which were controlled with medications; and two patients had severe symptoms refractory to medical therapy (calcium channel blockers, diazoxide, octreotide). DISCUSSION: Postprandial hypoglycemia after gastric bypass surgery with endogenous hyperinsulinemia is being increasingly recognized and reported in the literature. Our experience with nine patients is one of the largest. The etiology of this condition is not entirely understood. There may be yet unknown factors involved but increased secretion of glucagon-like peptide 1 and decreased grehlin are being implicated in islet cell hypertrophy. There is no "gold standard" treatment-medical or surgical-but distal pancreatectomy to debulk the hypertrophic islets and islet cells is the main surgical modality in patients with severe symptoms refractory to medical management.


Assuntos
Derivação Gástrica/efeitos adversos , Hiperinsulinismo/etiologia , Hipoglicemia/etiologia , Laparoscopia , Nesidioblastose/cirurgia , Pancreatectomia , Adulto , Feminino , Humanos , Hiperinsulinismo/cirurgia , Masculino , Pessoa de Meia-Idade , Nesidioblastose/etiologia , Nesidioblastose/patologia , Pâncreas/patologia , Período Pós-Prandial , Síndrome
18.
Toxicol Lett ; 193(2): 179-82, 2010 Mar 15.
Artigo em Inglês | MEDLINE | ID: mdl-20083172

RESUMO

Pancreas of Beagle dogs deriving from 166 male and 166 female animals were examined microscopically and were found to show nesidioblastosis-like structural alteration in 29 dogs. The lesion was represented by endocrine and ductular epithelial cell proliferation. The incidence profile and the severity of the changes observed were closely associated with the age of the dogs, younger dogs being more often and more seriously affected than older dogs. No link with altered insulin function has been established as serum glucose levels were found to be within the normal range. The pathology of spontaneous extra-islet endocrine cell proliferation in the young Beagle dogs, described in this study, has some similarities to that of nesidioblastosis of human neonates and infants.


Assuntos
Doenças do Cão/patologia , Ilhotas Pancreáticas/patologia , Nesidioblastose/veterinária , Animais , Glicemia , Proliferação de Células , Doenças do Cão/metabolismo , Cães , Feminino , Insulina/metabolismo , Ilhotas Pancreáticas/metabolismo , Masculino , Nesidioblastose/metabolismo , Nesidioblastose/patologia , Pâncreas/metabolismo , Pâncreas/patologia
19.
Pancreas ; 39(1): 57-63, 2010 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-19745778

RESUMO

OBJECTIVES: The probability of insulin independence after intraportal islet autotransplantation (IAT) for chronic pancreatitis (CP) treated by total pancreatectomy (TP) relates to the number of islets isolated from the excised pancreas. Our goal was to correlate the islet yield with the histopathologic findings and the clinical parameters in pediatric (age, <19 years) CP patients undergoing TP-IAT. METHODS: Eighteen pediatric CP patients aged 5 to 18 years (median, 15.6 years) who underwent TP-IAT were studied. Demographics and clinical history came from medical records. Histopathologic specimens from the pancreas were evaluated for presence and severity of fibrosis, acinar cell atrophy, inflammation, and nesidioblastosis by a surgical pathologist blinded to clinical information. RESULTS: Fibrosis and acinar atrophy negatively correlated with islet yield (P = 0.02, r = -0.50), particularly in hereditary CP (P = 0.01). Previous duct drainage surgeries also had a strong negative correlation (P = 0.01). Islet yield was better in younger (preteen) children (P = 0.02, r = -0.61) and in those with pancreatitis of shorter duration (P = 0.04, r = -0.39). CONCLUSIONS: For preserving beta cell mass, it is best to perform TP-IAT early in the course of CP in children, and prior drainage procedures should be avoided to maximize the number of islets available, especially in hereditary disease.


Assuntos
Transplante das Ilhotas Pancreáticas/métodos , Pâncreas/cirurgia , Pancreatectomia/métodos , Pancreatite Crônica/cirurgia , Adolescente , Fatores Etários , Criança , Pré-Escolar , Feminino , Fibrose , Seguimentos , Humanos , Masculino , Nesidioblastose/patologia , Avaliação de Resultados em Cuidados de Saúde , Pâncreas/patologia , Pancreatite Crônica/patologia
20.
Hum Pathol ; 41(3): 447-51, 2010 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-20004945

RESUMO

Adult nesidioblastosis is an uncommon cause of hyperinsulinemic hypoglycemia characterized by diffuse islet hyperplasia with beta-cell hypertrophy and atypia. The cause of nesidioblastosis in adults is unclear but may be different from nesidioblastosis in infants. In contrast to infants, a focal form of adult nesidioblastosis (ie, "nesidioblastoma") has not been documented, although proposed. We report a 44-year-old man with symptomatic hypoglycemia and localized nesidioblastosis treated with surgical enucleation resulting in normalization of blood glucose. Postoperative euglycemia has persisted in this patient to date (4 months at the time of manuscript submission).


Assuntos
Nesidioblastose/patologia , Pâncreas/patologia , Neoplasias Pancreáticas/patologia , Adulto , Glicemia/análise , Humanos , Hipoglicemia/etiologia , Hipoglicemia/cirurgia , Células Secretoras de Insulina/patologia , Masculino , Nesidioblastose/complicações , Nesidioblastose/cirurgia , Pâncreas/cirurgia , Neoplasias Pancreáticas/complicações , Neoplasias Pancreáticas/cirurgia , Resultado do Tratamento
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