scholarly journals Sirolimus therapy and follow-up in a patient with severe congenital hyperinsulinism following subtotal pancreatectomy

2020 ◽  
Vol 0 (0) ◽  
pp. 0-0
Author(s):  
Qiong Chen ◽  
Yongxing Chen ◽  
Xiaohong Wang ◽  
Haihua Yang ◽  
Yingxian Zhang ◽  
...  
Keyword(s):  
Congenital Hyperinsulinism ◽  
Subtotal Pancreatectomy

Genetic characteristics and long-term follow-up of 11 patients with congenital hyperinsulinism followed in a single center

2016 ◽  
Vol 29 (10) ◽  
Author(s):  
Katharina Warncke ◽  
Franziska Falco ◽  
Wolfgang Rabl ◽  
Ilse Engelsberger ◽  
Julia Saier ◽  
...  
Keyword(s):  
Genetic Testing ◽  
Medical Therapy ◽  
Congenital Hyperinsulinism ◽  
Early Puberty ◽  
Genetic Characteristics ◽  
Subtotal Pancreatectomy ◽  
Laboratory Results ◽  
Long Term Follow Up

AbstractBackground:Congenital hyperinsulinism (CHI) is a rare disease with an estimated incidence of 1:40,000 live births. Here, we characterize 11 patients treated at Munich Children’s Hospital Schwabing.Methods:We analyzed data on birth, treatment and laboratory results including genetic testing and evaluated the long-term course with a follow-up visit.Results:All patients had severe, diazoxide-(DZX)-resistant hypoglycemia, beginning immediately after birth. Two patients were treated by medical therapy, eight underwent subtotal pancreatectomy and one had a partial resection. Both patients who had medical therapy still suffer from occasional hypoglycemia. Six patients with subtotal pancreatectomy were affected by mild hypoglycemia. Seventy-five percent of patients who had surgical treatment developed diabetes mellitus (DM) at a median age of 10.5 (8–13) years. In 89% of patients with available genetic testing, mutations of theConclusions:The majority of CHI-patients not responding to DZX underwent surgery. After subtotal pancreatectomy, patients typically developed diabetes around early puberty.

Congenital hyperinsulinism due to SUR1 (ABCC8) mutation in newborn twins: improvement of clinical outcome after eight years follow-up

2015 ◽  
Author(s):  
Betul Ersoy ◽  
Nermin Tansug ◽  
Abdulkadir Genc ◽  
Deniz Kizilay ◽  
Semiha Kiremitci ◽  
...  
Keyword(s):  
Clinical Outcome ◽  
Congenital Hyperinsulinism

scholarly journals Subtotal Pancreatectomy for Cancer: Closure of the Pancreatic Remnant With Staplers

HPB Surgery ◽  
1990 ◽  
Vol 2 (1) ◽  
pp. 29-39 ◽  
Author(s):  
Bo Ahrén ◽  
Karl-G. Tranberg ◽  
Åke Andrén-Sandberg ◽  
Stig Bengmark
Keyword(s):  
Fatal Outcome ◽  
Endocrine Function ◽  
Periampullary Cancer ◽  
Gastric Stasis ◽  
Subtotal Pancreatectomy ◽  
Pancreatic Remnant ◽  
Number Of Patients ◽  
Long Term Follow Up ◽  
Pancreatic Endocrine Function

This paper presents a 2-year series of 26 consecutive pancreatectomies for periampullary cancer where the pancreatic tail was closed with a stapler in order to avoid complications related to a pancreatico-digestive anastomosis. The follow-up period was 14 months or more. Seven patients developed operative complications. Pancreatic fistulas developed in 3 patients. The fistulas closed spontaneously in 2 of the patients after 2-4 months, lntraabdominal abscesses developed in 4 patients and required surgical drainage. In 1 of these patients, the abscess eroded a large vessel with a fatal outcome resulting in an operative mortality rate of 3.8%. A transient postoperative gastric stasis was observed in seven patients. Postoperative hospital median stay was 27 days (range 10–83 days). Eighteeen patients have died after 4–30 months in recurrent disease and seven patients are alive after a follow-up period of 15–29 months. Pancreatic endocrine function seemed well preserved; diabetes mellitus has developed in only one patient. In conclusion, it appears that subtotal pancreatectomy with closure of the pancreatic remnant with staples gives a low morbidity and mortality. Although the conclusion should be tempered by the small number of patients, the results justify continued evaluation of this technique with long-term follow-up.

scholarly journals Long-term follow-up of 114 patients with congenital hyperinsulinism

2003 ◽  
pp. 43-51 ◽  
Author(s):  
T Meissner ◽  
U Wendel ◽  
P Burgard ◽  
S Schaetzle ◽  
E Mayatepek
Keyword(s):  
Diabetes Mellitus ◽  
Pancreatic Surgery ◽  
Congenital Hyperinsulinism ◽  
Childhood Onset ◽  
Neonatal Onset ◽  
Long Term Follow Up ◽  
High Degree ◽  
Onset Group

BACKGROUND: The term congenital hyperinsulinism (CHI) comprises a group of different genetic disorders with the common finding of recurrent episodes of hyperinsulinemic hypoglycemia. OBJECTIVE: To evaluate the clinical presentation, diagnostic criteria, treatment and long-term follow-up in a large cohort of CHI patients. PATIENTS: The data from 114 patients from different hospitals were obtained by a detailed questionnaire. Patients presented neonatally (65%), during infancy (28%) or during childhood (7%). RESULTS: In 20 of 74 (27%) patients with neonatal onset birth weight was greatly increased (group with standard deviation scores (SDS) >2.0) with a mean SDS of 3.2. Twenty-nine percent of neonatal-onset vs 69% of infancy/childhood-onset patients responded to diazoxide and diet or to a carbohydrate-enriched diet alone. Therefore, we observed a high rate of pancreatic surgery performed in the neonatal-onset group (70%) compared with the infancy/childhood-onset group (28%). Partial (3%), subtotal (37%) or near total (15%) pancreatectomy was performed. After pancreatic surgery there appeared a high risk of persistent hypoglycemia (40%). Immediately post-surgery or with a latency of several Years insulin-dependent diabetes mellitus was observed in operated patients (27%). General outcome was poor with a high degree of psychomotor or mental retardation (44%) or epilepsy (25%). An unfavorable outcome correlated with infancy-onset manifestation (chi(2)=6.1, P=0.01). CONCLUSIONS: The high degree of developmental delay, in particular in infancy-onset patients emphasizes the need for a change in treatment strategies to improve the unfavorable outcome. Evaluation of treatment alternatives should take the high risk of developing diabetes mellitus into account.

scholarly journals The contribution of rapid KATP channel gene mutation analysis to the clinical management of children with congenital hyperinsulinism

2011 ◽  
Vol 164 (5) ◽  
pp. 733-740 ◽  
Author(s):  
I Banerjee ◽  
M Skae ◽  
S E Flanagan ◽  
L Rigby ◽  
L Patel ◽  
...  
Keyword(s):  
Medical Treatment ◽  
Clinical Management ◽  
Ct Scanning ◽  
Favourable Outcome ◽  
Compound Heterozygous ◽  
Congenital Hyperinsulinism ◽  
Subtotal Pancreatectomy ◽  
Pathogenic Mutations ◽  
Positron Emission ◽  
Pet Ct

ObjectiveIn children with congenital hyperinsulinism (CHI), KATP channel genes (ABCC8 and KCNJ11) can be screened rapidly for potential pathogenic mutations. We aimed to assess the contribution of rapid genetic testing to the clinical management of CHI.DesignFollow-up observational study at two CHI referral hospitals.MethodsClinical outcomes such as subtotal pancreatectomy, 18F-Dopa positron emission tomography–computed tomography (PET–CT) scanning, stability on medical treatment and remission were assessed in a cohort of 101 children with CHI.ResultsIn total, 32 (32%) children had pathogenic mutations in KATP channel genes (27 in ABCC8 and five in KCNJ11), of which 11 (34%) were novel. In those negative at initial screening, other mutations (GLUD1, GCK, and HNF4A) were identified in three children. Those with homozygous/compound heterozygous ABCC8/KCNJ11 mutations were more likely to require a subtotal pancreatectomy CHI (7/10, 70%). Those with paternal heterozygous mutations were investigated with 18F-Dopa PET–CT scanning and 7/13 (54%) had a focal lesionectomy, whereas four (31%) required subtotal pancreatectomy for diffuse CHI. Those with maternal heterozygous mutations were most likely to achieve remission (5/5, 100%). In 66 with no identified mutation, 43 (65%) achieved remission, 22 (33%) were stable on medical treatment and only one child required a subtotal pancreatectomy.ConclusionsRapid genetic analysis is important in the management pathway of CHI; it provides aetiological confirmation of the diagnosis, indicates the likely need for a subtotal pancreatectomy and identifies those who require 18F-Dopa PET–CT scanning. In the absence of a mutation, reassurance of a favourable outcome can be given early in the course of CHI.

scholarly journals Subtotal Pancreatectomy for Congenital Hyperinsulinism: Our Experience and Review of Literature

2013 ◽  
Vol 77 (S3) ◽  
pp. 778-782 ◽  
Author(s):  
Rajeev Redkar ◽  
Parag J. Karkera ◽  
Janani Krishnan ◽  
Varun Hathiramani
Keyword(s):  
Congenital Hyperinsulinism ◽  
Review Of Literature ◽  
Subtotal Pancreatectomy

scholarly journals Continuous Intragastric Dextrose: A Therapeutic Option for Refractory Hypoglycemia in Congenital Hyperinsulinism

2018 ◽  
Vol 91 (1) ◽  
pp. 62-68 ◽  
Author(s):  
Mary Ellen Vajravelu ◽  
Morgan Congdon ◽  
Lauren Mitteer ◽  
Jamie Koh ◽  
Stephanie Givler ◽  
...  
Keyword(s):  
Adverse Events ◽  
Therapeutic Option ◽  
Congenital Hyperinsulinism ◽  
Feeding Problems ◽  
Conventional Medical Therapy ◽  
Z Scores ◽  
Multivariable Regression ◽  
Secondary Outcomes ◽  
Over Time

Feeding problems are frequent in infants with congenital hyperinsulinism (HI) and may be exacerbated by continuous enteral nutrition (EN) used to maintain euglycemia. Our center’s HI team uses dextrose solution given continuously via gastric tube (intrasgastric dextrose, IGD) for infants not fully responsive to conventional medical therapy or pancreatectomy. Here, we describe our practice as well as growth, feeding, and adverse events in infants with HI exposed to IGD. Methods: This was a retrospective cohort of infants with HI treated with IGD from 2009–2017. Primary outcomes were weight-for-length and body mass index Z-scores (WFL-Z and BMI-Z) in the year following IGD initiation. Secondary outcomes included EN use and adverse events. We used multivariable regression to assess covariates of interest. Results: We studied 32 subjects (13 female) with a median age at IGD initiation of 73 days (range 17–367); median follow-up was 11.2 months (range 5.0–14.2). WFL-Z did not change significantly over time (p > 0.05). EN use decreased significantly over time, i.e., at 0 months: 72% (95% CI 53–85) vs. at 12 months 39% (95% CI 22—59). No potential adverse events led to discontinuation of IGD. Conclusions: Over a median follow-up of nearly 1 year, IGD was well-tolerated, with no change in WFL-Z or BMI-Z from baseline.

Octreotide-induced severe paradoxical hyperglycemia and bradycardia during subtotal pancreatectomy for congenital hyperinsulinism in an infant

2007 ◽  
Vol 17 (11) ◽  
pp. 1117-1119 ◽  
Author(s):  
Yatindra Kumar Batra ◽  
Subramanyam Rajeev ◽  
Tanvir Samra ◽  
Katragadda LN Rao
Keyword(s):  
Congenital Hyperinsulinism ◽  
Subtotal Pancreatectomy

Long-term Follow-up of Patients with Congenital Hyperinsulinism in Austria

2008 ◽  
Vol 21 (6) ◽  
pp. 523-532
Author(s):  
Birgit Rami ◽  
Saadet Mercimek-Mahmutoglu ◽  
Martha Feucht ◽  
Marion Herle ◽  
Olaf Rittinger ◽  
...  
Keyword(s):  
Mental Retardation ◽  
Pancreatic Insufficiency ◽  
Congenital Hyperinsulinism ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Follow Up Study ◽  
Long Term Follow Up ◽  
Prevalence Of Obesity

Abstract Aim: To assess the neurological and clinical long-term outcome of patients diagnosed with congenital hyperinsulinism (CHI) in Austria. Patients and Methods: Fourteen patients diagnosed with CHI (1978-2000) were investigated retrospectively by reviewing hospital records. Thirteen of them were evaluated with either a questionnaire or clinical, neurological and biochemical investigations (age at evaluation 4.2- 25.5 years) in a follow-up study in the year 2004. Results: Fifty percent of the patients needed a pancreatectomy. The prevalence of mental retardation was 31%, of epilepsy 15% and of pancreatic insufficiency 14%. None of our patients had developed diabetes mellitus. Additionally the prevalence of obesity was 43% in patients after pancreatectomy. Sixty-nine percent of the patients had no further treatment at the time of follow-up. Conclusion: Despite early diagnosis and intensive treatment, 31% of the patients presented with mental retardation.

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