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Objective:To study the risk factors, diagnosis and treatment of transient hyperinsulinemic hypoglycemia (HH) in newborns.Methods:From January 2016 to December 2020, newborns with transient HH (HH group) admitted to our hospital were enrolled in this retrospective nested case-control study. Newborns with similar gestational age (GA) (differences of GA<2 w) without HH were matched with 1∶2 ratio as the non-HH group. Clinical characteristics of the two groups were compared and the risk factors and therapeutic results of HH in newborns were analyzed using SPSS 20.0 statistical software.Results:A total of 39 cases were included in the HH group and 75 cases were matched in the non-HH group. The proportion of small for gestational age (SGA) [51.3%(20/39) vs. 32.0%(24/75)], the amount of amino acids (AA) in intravenous infusion[1.0(0.0,1.0) g/(kg·d) vs. 0.0(0.0,1.0) g/(kg·d)], the incidence of hypoglycemia [(4.3±1.9) times vs. (3.6±1.3) times] and the maximum glucose infusion rate (GIR)[(8.3±2.5) mg/(kg·min) vs. (7.4±1.1) mg/(kg·min)] in the HH group were higher than the non-HH group (all P<0.05). The platelet count in the HH group were lower than the non-HH group [(186.9±60.9)×10 9/L vs. (215.3±61.7)×10 9/L, P<0.05]. SGA ( OR=2.535, 95% CI 1.077~5.971), the amount of intravenous AA ( OR=2.180, 95% CI 1.029~4.619) and the maximum GIR ( OR=1.405, 95% CI 1.088~1.815) were independent risk factors for transient HH. In the HH group, 28/39 cases were treated with Diazoxide or Octreotide and the therapeutic effects were good with few adverse drug reactions (ADR). 37/39 cases were recovered within 28 d of birth and the other 2 cases were recovered at 2.5 m and 3.5 m, respectively. Conclusions:SGA, the amount of intravenous AA and higher GIR are risk factors for transient HH in newborns. Diazoxide or Octreotide are effective with few ADR in the treatment of transient HH. Most patients will recover from transient HH in 2 w to 3 m.
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Resumen La placenta y el hígado son los encargados del metabolismo de los carbohidratos. La glucosa es fundamental para el metabolismo cerebral. La hipoglucemia se define con valores < 47 mg/dl. La hipoglicemia que persiste más de 7 días se atribuye a problemas metabólicos o endocrinológicos y requiere un flujo de glucosa > 12 mg/kg/min para alcanzar normo-glicemia. La hipoglicemia hiperinsulinémica congénita persistente (HHCP) es poco común (1:50,000 nacidos vivos), es la causa más común de hipoglicemia persistente secundaria a una secreción inadecuada de insulina, que puede afectar el neurodesarrollo. Hay una forma difusa y una focal, con manifestaciones clínicas idénticas, pero con mecanismos patológicos diferentes. El tratamiento médico es a base de diazóxido y ocreótide. En el 95% de los casos no hay respuesta al tratamiento médico, requiriendo pancreatectomía subtotal. Se utilizó ocreótide y nifedipino. La tomografía computada con emisión de positrones (PET/TC 18F-DOPA) encontró incremento en la capación pancreática de insulina, se realizó pancreactectomía. Se egresó sin complicaciones y en seguimiento pediátrico sin alteraciones neurológicas.
Abstract The placenta and liver are responsible for the metabolism of carbohydrates. The glucose is fundamental for brain metabolism. Hypoglycaemia is defined as values < 47 mg/dl. Hypoglycaemia that persists for more than 7 days is attributed to metabolic or endocrine problems and requires glucose flow > 12 GKM to reach normoglycemia. Persistent congenital hyperinsulinemic hypoglycemia (PCHH) is uncommon (1:50,000 live births) is the most common cause of persistent hypoglycemia secondary to inadequate insulin secretion, can significantly affect neurodevelopment. There is a diffuse and a focal form, with identical clinical manifestations, but with different pathological mechanisms. The medical treatment is diazoxide and ocreotide. In 95% of cases there is no response to medical treatment, requiring subtotal pancreatectomy. Ocreotide and nifedipine were used. Positron emission computed tomography (PET/CT 18F-DOPA) found an increase in pancreatic insulin capacity, a pancreactectomy was performed. He was discharged without complications and in pediatric follow-up without neurological alterations.
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¹⁸F-DOPA PET/CT is commonly done in patients with persistent hyperinsulinemic hypoglycemia of infancy (PHHI) to look for any focal lesion in the pancreas.We present the findings in a 20-day-old neonate with PHHI who underwent ¹⁸F-DOPA PET/CT. The scan showed diffuse uptake in the pancreas with no focal lesion, physiologic excretion into the genito-urinary system, and interestingly tracer accumulation was seen in the inferior vena cava and ilio-femoral veins which is a non-physiological site for tracer accumulation. The uptake corresponded to a large venous thrombus which was confirmed by a venous Doppler.
Subject(s)
Humans , Infant, Newborn , Congenital Hyperinsulinism , Pancreas , Positron Emission Tomography Computed Tomography , Thrombosis , Veins , Vena Cava, InferiorABSTRACT
Background : Hyperinsulinemic hypoglicemia with severe neuroglycopenic symptoms has been identified as a late and rare complication in patients submitted to Roux-en-Y gastric bypass. However, the potential gravity of its manifestations requires effective treatment of this condition. The absence of treatment makes it necessary to develop more effective clinical or surgical methods. Aim : To present one surgical option to revisional surgery in the treatment of hyperinsulinemic hypoglicemia Methods : The procedure consists in reconstituting alimentary transit through the duodenum and proximal jejunum, while keeping the restrictive part of the gastric bypass. As an additional strategy to maintain weight loss, is realized gastric fundus resection, aiming to suppress ghrelin production more effectively. Results : It was used in three patients with successful results in one year of follow-up. Conclusion : The procedure to reconstruct the food transit through the duodenum and proximal jejunum, keeping the restrictive component of gastric bypass in the treatment of hyperinsulinemic hypoglycemia showed good initial results and validated its application in other cases with this indication.
Racional : Hipoglicemia hiperinsulinêmica com sintomas neuroglicopênicos severos tem sido identificada como complicação tardia e rara em pacientes submetidos à gastroplastia com bypass em Y-de-Roux. Porém, a gravidade potencial de suas manifestações exige tratamento definitivo desta condição. A falta de tratamento efetivo gera a necessidade de desenvolver métodos clínicos ou cirúrgicos mais eficazes. Objetivo: Apresentar proposta de operação revisional para o tratamento da síndrome de hipoglicemia hiperinsulinêmica. Métodos : O procedimento consiste em reconstituir o trânsito alimentar pelo duodeno e jejuno proximal, mantendo o componente restritivo do by-pass gástrico. Como estratégia adicional de manutenção ponderal, é realizada ressecção do fundo gástrico, visando intensificar a supressão da grelina. Resultado: O procedimento foi realizado em três pacientes com bom resultado em seguimento de um ano. Conclusão : O procedimento de reconstituir o trânsito alimentar pelo duodeno e jejuno proximal, mantendo o componente restritivo do by-pass gástrico no tratamento da hipoglicemia hiperinsulinêmica apresentou bons resultados iniciais podendo validar sua indicação para outros casos.
Subject(s)
Adult , Female , Humans , Gastric Bypass/adverse effects , Hyperinsulinism/etiology , Hyperinsulinism/surgery , Hypoglycemia/etiology , Hypoglycemia/surgery , ReoperationABSTRACT
Se presentan 2 casos clínicos de recién nacidas que, entre otros síntomas, presentaron cifras bajas de glucemia mantenidas (menores 2,2 mmol/L), por lo que fueron evaluadas en el Servicio de Endocrinología del Hospital Provincial Pediátrico Docente "Eduardo Agramonte Piña" de Camagüey, donde se les diagnosticó nesidioblastosis -- hipoglucemia neonatal persistente --. En una de las pacientes el tratamiento médico resultó favorable, pero en la otra fue necesario realizar la pancreatectomía subtotal. Actualmente ambas afectadas reciben esquemas terapéuticos adecuados a sus necesidades clínicas.
Two case reports of female newborns are presented who, among other symptoms, presented with low concentrations of maintained glucemia (lower than 2,2 mmol/L), so they were evaluated in the Endocrinology Service of "Eduardo Agramonte Piña" Teaching Pediatric Provincial Hospital in Camagüey, where they were diagnosed nesidioblastosis -- persistent neonatal hypoglycemia --. In one of the patients the medical treatment was favorable, but in the other it was necessary to carry out a subtotal pancreatectomy. At present both patients receive therapeutic schedules adjusted to their clinical needs.
Subject(s)
Pancreatectomy , Nesidioblastosis , Hypoglycemia , Hospitals, PediatricABSTRACT
OBJETIVO: Rever a apresentação dos casos de hipoglicemia hiperinsulinêmica da infância (HHI), tratamento e histologia nos serviços de endocrinologia pediátrica no Brasil. MATERIAIS E MÉTODO: Os serviços receberam protocolo para resgatar dados de nascimento, resultados laboratoriais, tipo de tratamento instituído, necessidade de pancreatectomia e histologia. RESULTADOS: Vinte e cinco casos de HHI de seis centros foram resgatados, 15 do sexo masculino, 3/25 nascidos de parto normal. A mediana de idade do diagnóstico foi 10,3 dias. As dosagens de glicose e insulina na amostra sérica crítica apresentaram mediana de 24,7 mg/dL e 26,3 UI/dL. A velocidade de infusão de glicose endovenosa foi superior a 10 mg/kg/min em todos os casos (M:19,1). Diazóxido foi utilizado em 15/25, octreotide em 10, corticoide em 8, hormônio de crescimento em 3, nifedipina em 2 e glucagon em 1. Quarenta por cento (10/25) foram pancreatectomizados, nos quais a análise histológica revelou a forma difusa da patologia. CONCLUSÃO: Primeira análise crítica de uma amostra brasileira de portadores de HHI congênita. Arq Bras Endocrinol Metab. 2012;56(9):666-71.
OBJECTIVE: To review the presentation of hyperinsulinemic hypoglycemia of the infancy (HHI), its treatment and histology in Brazilian pediatric endocrinology sections. MATERIALS AND METHOD: The protocol analyzed data of birth, laboratory results, treatment, surgery, and pancreas histology. RESULTS: Twenty-five cases of HHI from six centers were analyzed: 15 male, 3/25 born by vaginal delivery. The average age at diagnosis was 10.3 days. Glucose and insulin levels in the critical sample showed an average of 24.7 mg/dL and 26.3 UI/dL. Intravenous infusion of the glucose was greater than 10 mg/kg/min in all cases (M:19,1). Diazoxide was used in 15/25 of the cases, octreotide in 10, glucocorticoid in 8, growth hormone in 3, nifedipine in 2 and glucagon in 1. Ten of the cases underwent pancreatectomy and histology results showed the diffuse form of disease. CONCLUSION: This is the first critic review of a Brazilian sample with congenital HHI. Arq Bras Endocrinol Metab. 2012;56(9):666-71.
Subject(s)
Female , Humans , Infant , Infant, Newborn , Male , Congenital Hyperinsulinism/diagnosis , Congenital Hyperinsulinism/therapy , Brazil , Blood Glucose/analysis , Congenital Hyperinsulinism/etiology , Insulin/blood , Medical Records , PancreatectomyABSTRACT
Objective To describe the clinical presentation, localization techniques, surgical procedures and outcome in patients with insulinoma. Methods Retrospective analysis of case records of patients diagnosed with insulinoma between January 1993 and June 2009 at a tertiary-care hospital was done. Seventeen patients underwent diagnostic 72-h fast. The sensitivity of computed tomography (CT) of pancreas was judged using intraoperative findings as the gold standard. Results Twenty-six patients (19 women) with mean age 42.2 years (median age 39.5 years) with biochemical/ imaging evidence of insulinoma were included. All patients who underwent 72-h fast developed symptomatic hypoglycemia within 48 h. The sensitivity and positive predictive value (PPV) of CT of pancreas was 68.4% and 92.8%, respectively. Twenty patients underwent surgery, of whom four were operated on without preoperative localization. Four patients had recurrence of symptoms. Of the six patients who were not operated upon, the lesion was localized in four. Conclusion This audit shows that all patients with insulinoma develop hypoglycemia within 48 h of fasting. CT of pancreas localizes the lesion in two thirds of cases and would be a useful initial investigation in patients with suspected insulinoma.
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Hyperinsulinemic hypoglycemia is the most common cause of persistent hypoglycemia in infancy. While most of the cases are sporadic more than 100 mutations have been reported in the familial type. The authors report a case of familial hyperinsulinemic hypoglycemia with homozygous T294M mutation of the KCNJ11 gene, which responded to diazoxide therapy.
Subject(s)
Antihypertensive Agents/therapeutic use , Congenital Hyperinsulinism/drug therapy , Congenital Hyperinsulinism/genetics , Diazoxide/therapeutic use , Female , Homozygote , Humans , India , Infant, Newborn , Mutation , Potassium Channels, Inwardly Rectifying/geneticsABSTRACT
PURPOSE: Insulinomas and nesidioblastosis are surgically curarable hyperinsulinemic hypoglycemic diseases; however the symptoms, diagnosis, and localization of these diseases are sometimes difficult. METHODS: From April 1990 to December 2008, patients with diagnosis of pancreatic insulinoma and nesidioblastosis were retrospectively reviewed. RESULTS: In the entire cohort, there were 6 patients with multiple endocrine neoplasia type 1 (MEN 1), 7 patients with nesidioblastosis, and 7 patients with multiple lesions. The incidence of neuroglycopenic symptoms (71.5%) was more prevalent than autonomic symptoms (26.5%). The pre-operative localization methods were computed tomography (CT), transabdominal ultrasonography, endoscopic ultrasonography (EUS), magnetic resonance imaging (MRI), angiography, selective arterial calcium stimulation with venous sampling (ASVS), and transhepatic portal venous sampling (THPVS) which had sensitivities of 75%, 40%, 75%, 64%, 48%, 20%, and 87%, respectively. During the second half of the study period, CT had a sensitivity of 83%. THPVS was the most sensitive method of all the diagnostic tools. Intra-operative palpation and ultrasonography were the most powerful modalities for tumor localization. The surgical treatments included enucleation (20%), distal pancreatectomy (51%), central pancreatectomy (12%), pancreaticoduodenectomy (15%), and near-total pancreatectomy (1.5%). From 2006, laparascopic surgery was used as the preferred treatment modality for insulinomas. CONCLUSION: Early diagnosis and precise localization is the most important aspect of treating insulinomas. For better localization, dual phase spiral CT and intra-operative ultrasonography play an important role in tumor localization.
Subject(s)
Humans , Angiography , Calcium , Cohort Studies , Early Diagnosis , Endosonography , Incidence , Insulinoma , Magnetic Resonance Imaging , Multiple Endocrine Neoplasia Type 1 , Nesidioblastosis , Palpation , Pancreatectomy , Pancreaticoduodenectomy , Retrospective Studies , Tomography, Spiral ComputedABSTRACT
PURPOSE: Insulinomas and nesidioblastosis are surgically curarable hyperinsulinemic hypoglycemic diseases; however the symptoms, diagnosis, and localization of these diseases are sometimes difficult. METHODS: From April 1990 to December 2008, patients with diagnosis of pancreatic insulinoma and nesidioblastosis were retrospectively reviewed. RESULTS: In the entire cohort, there were 6 patients with multiple endocrine neoplasia type 1 (MEN 1), 7 patients with nesidioblastosis, and 7 patients with multiple lesions. The incidence of neuroglycopenic symptoms (71.5%) was more prevalent than autonomic symptoms (26.5%). The pre-operative localization methods were computed tomography (CT), transabdominal ultrasonography, endoscopic ultrasonography (EUS), magnetic resonance imaging (MRI), angiography, selective arterial calcium stimulation with venous sampling (ASVS), and transhepatic portal venous sampling (THPVS) which had sensitivities of 75%, 40%, 75%, 64%, 48%, 20%, and 87%, respectively. During the second half of the study period, CT had a sensitivity of 83%. THPVS was the most sensitive method of all the diagnostic tools. Intra-operative palpation and ultrasonography were the most powerful modalities for tumor localization. The surgical treatments included enucleation (20%), distal pancreatectomy (51%), central pancreatectomy (12%), pancreaticoduodenectomy (15%), and near-total pancreatectomy (1.5%). From 2006, laparascopic surgery was used as the preferred treatment modality for insulinomas. CONCLUSION: Early diagnosis and precise localization is the most important aspect of treating insulinomas. For better localization, dual phase spiral CT and intra-operative ultrasonography play an important role in tumor localization.
Subject(s)
Humans , Angiography , Calcium , Cohort Studies , Early Diagnosis , Endosonography , Incidence , Insulinoma , Magnetic Resonance Imaging , Multiple Endocrine Neoplasia Type 1 , Nesidioblastosis , Palpation , Pancreatectomy , Pancreaticoduodenectomy , Retrospective Studies , Tomography, Spiral ComputedABSTRACT
Nesidioblastosis is a term that describes the prolonged differentiation of islets of Langerhan's cells from the epithelium of the pancreatic duct. Nesidioblastosis is a primarily childhood disease, which is often associated with hyperinsulinemic hypoglycemia, but rarely found in adults. A 62-year-old man with hyperinsulinemic hypoglycemia complained of general weakness and mental change while fasting. Abdominopelvic CT, celiac angiography, intra-arterial calcium stimulation test and endoscopic ultrasonography did not reveal any tumor in the pancreas. A subtotal pancreatectomy (nearly 80% of pancreas was removed) was then carried out. He was diagnosed with nesidioblastosis inform the pathology after the operation. Herein, our experience of a 62-year-old Nesidioblastosis patient is reported.
Subject(s)
Adult , Humans , Middle Aged , Angiography , Calcium , Endosonography , Epithelium , Fasting , Hypoglycemia , Nesidioblastosis , Pancreas , Pancreatectomy , Pancreatic Ducts , PathologyABSTRACT
Nesidiodysplasia encompasses increased, maldistributed, and/or, improperly regulated or programmed endocrine cells associated with a clinical endocrine dysfunction. It is a frequent cause of hyperinsulinemic hypoglycemia in neonates and infants, but rare in adults. A 63 year-old woman was admitted due to the loss of consciousness and intense sweating. Hyperinsulinemic hypogiycemia was diagnosed. Under the presumptive diagnosis of insulinoma, abdominal ultrasonography and CT scan were done, but no definite tumor was found. Only a suspicious gradient in insulin concentration was found around the body of the pancreas by percutaneous portal venous sampling. While an exploratory laparotomy was performed, an intraoperative sonography was done. As a result, cystic mass like lesion was found. Then mass enucleation was done. Pathologic examination of the enucleated pancreas revealed no mass and the characteristic nesidiodysplasia. However, after the operation, hypoglycemic symptoms were repeated. So we are instructing her to eat meals frequently.
Subject(s)
Adult , Female , Humans , Infant , Infant, Newborn , Middle Aged , Diagnosis , Endocrine Cells , Hypoglycemia , Insulin , Insulinoma , Laparotomy , Meals , Pancreas , Sweat , Sweating , Tomography, X-Ray Computed , Ultrasonography , UnconsciousnessABSTRACT
A 72-year-old man who had suffered several episodes of syncope was diagnosed as having hyperinsulinemic hypoglycemia. Although imaging studies and percutaneous transhepatic portal venous sampling did not reveal the existence of any tumors in the pancreas, distal pancreatectomy was performed because the possibility of a small pancreatic endocrine tumor could not be completely rejected. External examination of the surgically removed pancreas did not reveal any tumors. Microscopically, the pancreas exhibited diffuse islet cell hyperplasia without nesidioblastosis. The patient remains euglycemic and has tolerated 24-hour fasting without any medication for a period of 10 months after the operation.
Subject(s)
Aged , Humans , Male , Hyperinsulinism/etiology , Hyperplasia , Hypoglycemia/etiology , Islets of Langerhans/surgery , Islets of Langerhans/pathology , Pancreatectomy/methods , Treatment OutcomeABSTRACT
PURPOSE: Persistent hyperinsulinemic hypoglycemia of infancy(PHHI), which is characterised by inappropriate insulin secretion in spite of hypoglycemia, needs urgent treatment to prevent cerebral hypoglycemic damage. Although pancreatectomy is the treatment of choice for PHHI, there are several complications which follow treatment. We suggest that aggressive medical therapy, when effective, is preferable to partial pancreatectomy. METHODS: We evaluated 8 patients with PHHI admitted to the Department of Pediatrics, Samsung Medical Center from November 1996 to January 1999. Children with hypoglycemia in the range of 3-50mg/dl were included. Octreotide was administered at dosage of 100-150 microgram/day. When the patients did not respond to octreotide, diazoxide and nifedipine were given in addition. RESULTS: In four of eight patients, octreotide was discontinued after 15 to 165 days. One patient was given diazoxide instead. The remaining 3 patients are still being treated with octreotide. CONCLUSION: We believe that maximum effort should be made to attain euglycemia with medication, and pancreatectomy should be reserved for patients in whom these measures fail to restore normoglycemia.
Subject(s)
Child , Humans , Congenital Hyperinsulinism , Diazoxide , Hypoglycemia , Insulin , Nifedipine , Octreotide , Pancreatectomy , PediatricsABSTRACT
PURPOSE: The purpose of this study was to describe the clinical outcome after pancreatcetmy and its relationship with pathological appearances and clinical features in patients with persistent hyperinsulinemic hypoglycemia of infancy(PHHI). METHODS: Medical records of 10 patients(9 males and 1 female, mean age:40.4+/-1.5 months) who were diagnosed as PHHI and underwent pancreatectomy from 1988 to 2000 were reviewed. Clincal and biochemical data were recorded. Subjects were classified arbitrarily into early-onset or late-onset group according to age of onset. Pathologic appearance of pancreas was divided into 2 forms:diffuse or focal. The former had a focal pancreatic adenomatous hyperplasia and the latter was characterized by increased number of betacells with similar distribution seen in normal neonates. RESULTS: One patient had focal, and nine had diffuse lesions. After near-total pancreatectomy, 4 patients(40.0%) showed complete response, 4(40.0%) had persistent hypoglycemia, and 2(20.0%) developed diabetes mellitus. As neurological sequelae, 6 patients(60.0%) had persistent seizures, and 6(60.0%) had delayed motor and speech development. No clinical or biochemical factors related to postoperative outcome were found. CONCLUSION: This data indicate that early diagnosis of patients who present with hypoglycemic symptoms in infancy, especially early in life, and development of more effective therapy are warranted, because there is no clinical or biochemical factor predicting final outcome after near-total pancreatectomy and only 40% of patients with PHHI remained euglycemic after surgery with possible severe neurological sequelae.
Subject(s)
Female , Humans , Infant, Newborn , Male , Age of Onset , Congenital Hyperinsulinism , Diabetes Mellitus , Early Diagnosis , Hyperplasia , Hypoglycemia , Medical Records , Pancreas , Pancreatectomy , SeizuresABSTRACT
Nesidioblastosis, also known as persistent hyperinsulinemic hypoglycemia of infancy(PHHI) or familial hyperinsulinsm, is the most common cause of recurrent severe hypoglycemia in infancy. It is an autosomal recessive disorder characterized by irregular insulin secretion leading to inappropriately raised plasma insulin concentration compared to blood glucose levels. Recently, mutations in the sulfonylurea receptor(SUR) have been described in association with PHHI. The mainstay of medical treatment is glucose infusion and diazoxide or long acting somatostatin. If medical treatment fails in preventing hypoglycemia, near total pancreatectomy is recommended. We report one case of nesidioblastosis cured by near total pancreatectomy with brief review of literatures.
Subject(s)
Humans , Infant, Newborn , Blood Glucose , Congenital Hyperinsulinism , Diazoxide , Glucose , Hypoglycemia , Insulin , Nesidioblastosis , Pancreatectomy , Plasma , SomatostatinABSTRACT
BACKGROUNDS: Hyperinsulinemic hypoglycemia is caused by insulinoma mostly and by nesidioblastosis. While an insulinoma is the most common functional endocrine tumor of pancreas, nesidioblastosis primarily is a childhood disease and is rarely reported in adults. Nesidioblastosis has been defined as a diffuse islet cell hyperplasia accompanied by a differentiation of the islets arising from the pancreatic ductal epithelium. Preoperative localization and proper surgical treatment are crucial because these disases can induce critical and permanent neurologic sequela from the hypoglycemia. However, nesidioblastosis has to be considered differently from the insulinoma in terms of diagnosis and therapeutic aspects in adults. METHODS: We retrospectively analyzed 13 and 3 patients who had been diagnosed as having an insulinoma and nesidioblastosis and who had undergone operations during the 8-year period from 1990 to 1998 at Asan Medical Center. We compared the 2 diseases with respect to diagnosis and therapy. RESULTS: There were 3 men and 10 women with an insulinoma and their mean age was 45 (17~64 years). There were 2 men and 1 woman with nesidioblastosis and their mean age was 45 (22~58 years). The most common clinical manifestation was loss of consciousness, and all the patients had findings compatible with Whipple's triad. The median duration of symptoms before diagnosis was 28 months (6~120 months) in insulinoma and 1.1 months (7 days~2 months) in nesidioblastosis (p=0.009). Hyperinsulinemic hypoglycemia was confirmed during prolonged fasting and the concomitant insulin level was 3~130 U/ml (median=25) in the insulinoma patients and 37~202 U/ml (median=67) in the nesidioblastosis patients (p=0.03). Insulinoma can be localized in 12 patients (93%) preoperatively. The combination of negative angiography and a lack of difference in the insulin concentration gradientin THPVC (transhepatic portal vein catheterization) suggested preoperatively a nesidioblastosis in only one patient (33.3%). All the patients with nesidioblastosis was confirmed intraoperatively by a frozen biopsy. In terms of treating the insulinoma, an enucleation was performed in 5, and pancreatic resection in 8. In nesidioblastosis, subtotal pancreatectomy was done on 2 and pybrus preserving pancreaticoduodenectomy (70%) on one patient. Following the operation, the symtoms of hypoglycemia and the laboratory values were normal in all the patients. CONCLUSION: We observed 13 cases of insulinoma (81%) and 3 of nesidioblastosis (19%). Preoperative suspicion, proper utilization of diagnostic tools, and prudent intraoperative diagnostic procedures enhanced the diagnostic accuracy for hyperinsulinemic hypoglycemia and led to better treatment strategies.
Subject(s)
Adult , Female , Humans , Male , Angiography , Biopsy , Diagnosis , Epithelium , Fasting , Hyperplasia , Hypoglycemia , Insulin , Insulinoma , Islets of Langerhans , Nesidioblastosis , Pancreas , Pancreatectomy , Pancreatic Ducts , Pancreaticoduodenectomy , Portal Vein , Retrospective Studies , UnconsciousnessABSTRACT
Nesidioblastosis is a term that describes the prolonged differentiation of the Langerhan's islet cells from the epithelium of the pancreatic duct. Islet cell lesions accompanied with hyperinsulinemic, 80% are found to be benign islet cell tumors. In additional 10% are malignant and the remaining are nesidioblastosis. Children are usually affected and develop hyperinsulinemic hypoglycemia, but it is rare in adults. Only 20 cases has been reported worldwide and 6 cases in Korea. A 41-year old man & 42-year old woman was admitted due to intermittent hypoglycemic symptoms, that had been relieved by carbohydrate ingestion. Hyperinsulinemic hypoglycemia was documented during prolonged fasting. Under the presumptive diagnosis of insulinoma, Abdominal CT, Celiac angiogram and percutaneous transhepatic portal venous sampling were done, but we could not find any definitive mass. 85% of the pancreas & 88% of the pancreas was removed respectively. Pathologic examination of the resected pancreas revealed irregularly sized islets and scattering of small endocrine cell clusters throughout the acinar tissue and ductuloinsular complex.
Subject(s)
Adult , Child , Female , Humans , Adenoma, Islet Cell , Diagnosis , Eating , Endocrine Cells , Epithelium , Fasting , Hypoglycemia , Insulinoma , Islets of Langerhans , Korea , Nesidioblastosis , Pancreas , Pancreatic Ducts , Tomography, X-Ray ComputedABSTRACT
Hyperinsulinemic hypoglycemia is a relatively rare disease in childhood period except neonate, but hypoglycemia due to delicate imbalance between glucose production & consumption is evoked easily and left permanent damage to brain at these period. The definition is that serum insulin level is above 10microU/ml when blood sugar level is below the 40mg/dl and so I/G ratio is higher than 0.4. The clinical manifestations are irrtability, frequent feeding and seizures etc. and there is no specific pancreatic pathology in most cases. We experienced 2 cases of hyperinsulinemic hypoglycemia with pancreatic hyperplasia and pancreatic adenoma each other. The diagnosis was made on clinical manifestations, laboratory results, radiologic and pathologic findings. We reported these cases with brief review of literature.
Subject(s)
Humans , Infant, Newborn , Adenoma , Blood Glucose , Brain , Diagnosis , Glucose , Hyperplasia , Hypoglycemia , Insulin , Pathology , Rare Diseases , SeizuresABSTRACT
Nesidioblastosis is characterized by a diffuse proliferation of islet cells arising from pancreatic ducts and is the most common cause of hyperinsulinemic hypoglycemia in newborns and infantile. It is exceedingly rare in adults and no concensus regarding its diagnosis and management is available. We herein describe an elderly man with fasting hypoglycemia, inappropriate insulin hypersecretion. And pathologic examination of his pancreas revealed the characteristic finding of nesidioblastosis confirmed by immunohistochemical stain.