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Endocrinology, Diabetes & Metabolism Mar 2024Insulin-like growth factor-2 (IGF-2)-mediated hypoglycemia is a rare yet clinically significant entity with considerable morbidity and mortality. Existing literature is... (Review)
Review
INTRODUCTION
Insulin-like growth factor-2 (IGF-2)-mediated hypoglycemia is a rare yet clinically significant entity with considerable morbidity and mortality. Existing literature is limited and fails to offer a comprehensive understanding of its clinical trajectory, management and prognostication.
METHODS
Systematic review of English-language articles reporting primary patient data on IMH was searched using electronic databases (PubMed, Scopus and Embase) from any date up to 21 December 2022. Data were analysed in STATA-16.
RESULTS
The systematic review contains 172 studies, including 1 Randomised controlled trial, 1 prospective observational study, 5 retrospective observational studies, 150 case reports, 11 case series and 4 conference abstracts. A total of 233 patients were analysed, averaging 60.6 ± 17.1 years in age, with comparable proportions of males and females. The commonest tumours associated with Insulin-like Growth Factor-2-mediated hypoglycaemia were fibrous tumours (N = 124, 53.2%), followed by non-fibrous tumours originating from the liver (N = 21, 9%), hemangiopericytomas (N = 20, 8.5%) and mesotheliomas (N = 11, 4.7%). Hypoglycaemia was the presenting feature of NICT in 42% of cases. Predominant clinical features included loss of consciousness (26.7%) and confusion (21%). The mean IGF-2 and IGF-1 levels were 882.3 ± 630.6 ng/dL and 41.8 ± 47.8, respectively, with no significant correlation between these levels and patient outcomes. Surgical removal was the most employed treatment modality (47.2%), followed by medication therapy. The recovery rate was 77%, with chronic liver disease (CLD) significantly associated with a poor outcome (OR: 7.23, P: 0.03). Tumours originating from fibrous tissues were significantly associated with recovery (p < .001). In the logistic regression model, CLD remained a significant predictor of poor outcomes.
CONCLUSION
This systematic review highlights that most non-islet-cell tumour-hypoglycaemia (NICTH) is due to fibrous tumours. NICTs demonstrate a variable prognosis, which is fair if originating from fibrous tissue. Management such as octreotide, corticosteroids, diazoxide, embolization, radiotherapy and surgical resection have disparate success rates.
Topics: Male; Female; Humans; Insulin-Like Growth Factor II; Insulin-Like Peptides; Retrospective Studies; Hypoglycemia; Observational Studies as Topic
PubMed: 38411039
DOI: 10.1002/edm2.471 -
Journal of Pediatric Surgery Feb 2024Chyle leakage/ascites after surgical resection of neuroblastic tumors may delay the start of chemotherapy and worsen prognosis. Previous studies have reported a highly... (Review)
Review
BACKGROUND
Chyle leakage/ascites after surgical resection of neuroblastic tumors may delay the start of chemotherapy and worsen prognosis. Previous studies have reported a highly variable incidence and risk factors remain largely unknown. This study aims to analyze the true incidence of chyle leaks and ascites and seeks to identify risk factors and optimal treatment strategies.
METHODS
Medline/Embase databases were searched according to PRISMA guidelines. Literature reviews, case reports, and non-English papers were excluded. Data were extracted independently following paper selection by 2 authors.
RESULTS
The final analysis yielded 15 studies with N = 1468 patients. Chylous ascites was recorded postoperatively in 171 patients (12%). Most patients experiencing chyle leaks were successfully treated conservatively with drainage, bowel rest, parenteral nutrition and octreotide with variable combinations of these treatment options. 7/171 (4%) patients required operative exploration to control troublesome persistent chyle leaks. In risk factor analysis, higher tumor stage was significantly associated with the risk of chyle leak (P < 0.0001) whereas no correlation was observed with adrenal vs non-adrenal tumor location, INRG risk groups and tumor laterality.
CONCLUSION
Chyle leakage after surgery for neuroblastic tumors is a common morbid complication occurring in some 12% of patients. Higher INSS tumor stage portends greater risk(s). Conservative therapy strategies appear successful in the majority of cases. To avert this complication meticulous mesenteric lymphatic ligation is recommended especially for those patients with higher tumor stage(s) requiring extensive radical surgery including retroperitoneal lymph node resection.
LEVEL OF EVIDENCE
III.
TYPE OF STUDY
Systematic review.
PubMed: 38490882
DOI: 10.1016/j.jpedsurg.2024.02.018 -
Heart (British Cardiac Society) Aug 2023Chylopericardium (CPE) is a rare condition associated with accumulation of triglyceride-rich chylous fluid in the pericardial cavity. Due to minimal information on CPE...
OBJECTIVE
Chylopericardium (CPE) is a rare condition associated with accumulation of triglyceride-rich chylous fluid in the pericardial cavity. Due to minimal information on CPE within the literature, we conducted a systematic review of all published CPE cases to understand its clinical characteristics, management and outcomes.
METHODS
We performed a literature search and identified cases of patients with CPE from 1946 until May 2021 in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. We identified relevant articles for pooled analyses of clinical, diagnostic and outcome data.
RESULTS
A total of 95 articles with 98 patients were identified. Patient demographics demonstrated male predominance (55%), with a mean age of 37±15 years. Time from symptom onset to diagnosis was 5 (Q1 4.5, Q3 14) days, with 74% of patients symptomatic on presentation. Idiopathic CPE (60%) was the most common aetiology. Cardiac tamponade secondary to CPE was seen in 38% of cases. Pericardial fluid analysis was required in 94% of cases. Lymphangiography identified the leakage site in 59% of patients. Medical therapy (total parenteral nutrition, medium-chain triglycerides or octreotide) was undertaken in 63% of cases. In our cohort, 32% progressed towards surgical intervention. During a median follow-up of 180 (Q1 180, Q3 377) days, CPE recurred in 16% of cases. Of the patients with recurrence, 10% were rehospitalised.
CONCLUSION
CPE tends to develop in younger patients and may cause serious complications. Many patients fail medical therapy, thereby requiring surgical intervention. Although overall mortality is low, associated morbidities warrant close follow-up and possible reintervention and hospitalisations.
Topics: Humans; Male; Young Adult; Adult; Middle Aged; Female; Pericardial Effusion; Cardiac Tamponade; Triglycerides
PubMed: 36702544
DOI: 10.1136/heartjnl-2022-321798 -
JMA Journal Jul 2023Somatostatin analogs are expected to reduce lymphatic leakage. However, whether they can be used after axillary lymphadenectomy is unclear. This study aimed to assess... (Review)
Review
BACKGROUND
Somatostatin analogs are expected to reduce lymphatic leakage. However, whether they can be used after axillary lymphadenectomy is unclear. This study aimed to assess the efficacy and safety of somatostatin analogs in axillary lymphadenectomy for breast cancer patients.
METHODS
We performed a random-effects meta-analysis by searching electronic databases for randomized trials and trial registries until June 2022. The primary outcomes were the volume of drained fluid, the duration of drainage, and seroma incidence. Bias was assessed using the Cochrane Collaboration's tool and the Grading of Recommendations, Assessment, Development, and Evaluations approach.
RESULTS
Six trials (738 participants) and one protocol without results were included. Somatostatin analogs may reduce the volume of drained fluid (mean difference = -22.07 mL, 95% confidence interval [CI] = -42.09 to -2.05; I = 56%) while resulting in a slight-to-no difference in the duration of drainage (mean difference = -0.48 days, 95% CI = -1.43 to 0.46; I = 87%) and seroma incidence (risk ratio = 0.91, 95% CI = 0.61-1.34; I = 55%). The certainty of the evidence was low.
CONCLUSIONS
There was limited evidence supporting somatostatin analogs for lymphorrhea after axillary lymphadenectomy. Multicenter randomized controlled trials are needed to confirm the efficacy and safety of somatostatin analogs after axillary lymphadenectomy.
PubMed: 37560373
DOI: 10.31662/jmaj.2022-0219 -
Thyroid Research Feb 2024Coexistence of TSH-secreting pituitary adenoma (TSHoma) and Graves' disease (GD) is rare and complicates the management decision.
BACKGROUND
Coexistence of TSH-secreting pituitary adenoma (TSHoma) and Graves' disease (GD) is rare and complicates the management decision.
METHODS
We present a case of the co-existence of TSHoma and GD. In addition, we systematically searched articles describing TSHoma and GD in the same patient published until 20th March 2023, using Pubmed, Scopus and Embase.
CASE PRESENTATION
A 46-year-old man presented with symptoms of thyrotoxicosis. His thyroid function tests showed serum TSH 3.35 (reference range 0.3-4.2) mIU/L, FT3 19.7 (3.7-6.4) pmol/L, and FT4 68.9 (11-23.3) pmol/L. The serum TSH receptor antibody was 11.5 mIU/L (positive at ≥ 1.75 mIU/L). Pituitary magnetic resonance imaging showed macroadenoma compressing the optic chiasm. The patient underwent trans-sphenoidal resection of pituitary adenoma. Postoperatively, he remained on maintenance carbimazole and octreotide.
RESULTS
Fourteen articles comprising 15 patients were identified from the systemic search. A total of 16 patients (including the current case) were included in the systematic review. The mean (± SD) age at diagnosis was 41 ± 13.6 years. The majority were females (75%). The median (IQR) TSH was 1.95 (0.12-5.5) mIU/L, the median (IQR) free T3 was 11.7 (7.6-19.7) pmol/L and the median (IQR) free T4 level was 47.6 (33.3-64.4) pmol/L. Ten (76.9%) patients had positive TSH receptor antibody levels. 84.6% had pituitary macroadenoma. Pituitary surgery was performed in 12 (75%) patients. At the last follow-up, 4 (25%) patients had complete resolution of symptoms after pituitary surgery, 3 (18.7%) were on maintenance treatment with thionamides for GD, 1 (6.25%) on beta-blockers and 1 (6.25%) on somatostatin analog.
CONCLUSION
TSHoma and GD can co-exist, and it is essential to identify this rare association as it can significantly impact treatment strategies.
PubMed: 38311752
DOI: 10.1186/s13044-023-00184-2 -
Cureus Jul 2023Hepatorenal syndrome (HRS), a consequence of liver cirrhosis, is the development of renal failure, which carries a grave prognosis. Reversing acute renal failure with... (Review)
Review
BACKGROUND
Hepatorenal syndrome (HRS), a consequence of liver cirrhosis, is the development of renal failure, which carries a grave prognosis. Reversing acute renal failure with various vasoconstrictor therapies at an appropriate time favors a good prognosis, especially when a liver transplant is not feasible.
OBJECTIVE
This study aims to compare various treatment modalities to deduce an effective way to manage HRS.
METHODS
The authors conducted a literature search in PubMed, Google Scholar, the Cochrane Library, and Science Direct in October 2022, using regular and MeSH keywords. A total of 1072 articles were identified. The PRISMA guidelines were used, the PICO framework was addressed, and the inclusion criteria were set based on studies from the past 10 years. After quality assessment, 14 studies were included for in-depth analysis in this review. Results: A total of 14 studies were included after quality assessment, including randomized controlled trials, systematic reviews, meta-analyses, and observational cohort studies. Nine hundred and forty-one patients represented this review's experimental and observational studies, apart from the other systematic reviews analyzed. Nine studies discovered that Terlipressin, especially when administered with albumin, was more effective than other conventional treatment modalities, including norepinephrine and midodrine, in terms of improving mortality and reversing the HRS. Four studies suggested that terlipressin exhibited similar effectiveness but found no significant difference. In contrast, one study found that norepinephrine was superior to terlipressin when particularly considering the adverse effects.
CONCLUSION
Terlipressin, one of the most widely used vasoconstrictor agents across the world, seems to be effective in reversing renal failure in HRS. Although adverse effects are seen with this agent, it is still beneficial when compared to other medications. Further studies with larger sample sizes may be warranted.
PubMed: 37621788
DOI: 10.7759/cureus.42367 -
Quantitative Imaging in Medicine and... Oct 2023The imaging of somatostatin receptors (SSTRs) plays a significant role in imaging neuroendocrine tumors (NETs). However, there has been no clear definition on whether it...
BACKGROUND
The imaging of somatostatin receptors (SSTRs) plays a significant role in imaging neuroendocrine tumors (NETs). However, there has been no clear definition on whether it is necessary to withdraw somatostatin analogs (SSAs) before SSTRs imaging. We aimed to assess whether nonradioactive SSAs affect the uptake of radiolabeled SSAs on imaging for NETs patients.
METHODS
The databases of PubMed, Embase, and Web of Science (WoS) were searched until March 12, 2022 to identify eligible studies. Maximum standardized uptake values (SUVmax) in tumor and normal tissues were extracted, pooled, and compared before and after SSAs treatment. The change of tumor-to-background/liver ratio was also described. The quality of each study was assessed using the revised Quality Assessment of Diagnostic Accuracy Studies-2 tool.
RESULTS
A total of 9 articles involving 285 patients were included and 5 studies using Gallium-68-labeled [1,4,7,10-tetraazacyclododecane-N,N',N'',N'''-tetraacetic acid]-D-Phe-Tyr-Thr-octreotide (Ga-DOTATATE) were used for pooled evaluation. We found a significantly decreased SUVmax in the liver (9.56±2.47 7.62±2.12, P=0.001) and spleen (25.74±7.14 20.39±6.07, P=0.006) after SSAs treatment whereas no significant differences were observed in the uptake of thyroid, adrenal, and pituitary gland. For either primary tumor sites or metastases, the SUVmax did not change significantly before and after SSAs treatment. The tumor-to-liver/background ratio increased following SSAs therapy. High heterogeneity was observed across the studies, mainly due to inherent diversity of study design, sample size, and scanning technique.
CONCLUSIONS
Based on current evidence, long-acting SSAs therapy before imaging has no effect on the uptake of radiolabeled SSAs at tumor primary sites and metastatic lesions, but results in a significant reduction of uptake in the liver and spleen. These findings may implicate the unnecessary discontinuation of SSAs before radiolabeled SSAs imaging.
PubMed: 37869289
DOI: 10.21037/qims-23-477