A 14-year-old girl presented with progressive swelling of the jaw. Six weeks before this admission, a nonpainful lump in the left lower jaw developed. The mass increased in size, and swelling of the ...left side of the face and neck developed. A biopsy specimen from the mass showed abundant giant cells. The calcium level was 11.8 mg per deciliter. A diagnosis was made.
The clinical utility and implications of continuous glucose monitoring (CGM) in cystic fibrosis (CF) are unclear.
We examined the correlation between CGM measures and clinical outcomes in adults with ...CF, investigated the relationship between hemoglobin A1c (HbA1c) and CGM-derived average glucose (AG), and explored CGM measures that distinguish cystic fibrosis-related diabetes (CFRD) from normal and abnormal glucose tolerance.
This prospective observational study included 77 adults with CF who had CGM and HbA1c measured at 2 to 3 time points 3 months apart.
Thirty-one of the 77 participants met American Diabetes Association-recommended diagnostic criteria for CFRD by oral glucose tolerance testing and/or HbA1c. In all participants, CGM measures of hyperglycemia and glycemic variability correlated with nutritional status and pulmonary function. HbA1c was correlated with AG (R2 = 0.71, P < 0.001), with no significant difference between this regression line and that previously established in type 1 and type 2 diabetes and healthy volunteers. Cutoffs of 17.5% time > 140 mg/dL and 3.4% time > 180 mg/dL had sensitivities of 87% and 90%, respectively, and specificities of 95%, for identifying CFRD. Area under the curve and percent of participants correctly classified with CFRD were higher for AG, SD, % time > 140, > 180, and > 250 mg/dL than for HbA1c.
CGM measures of hyperglycemia and glycemic variability are superior to HbA1c in distinguishing those with and without CFRD. CGM-derived AG is strongly correlated with HbA1c in adults with CF, with a similar relationship to other diabetes populations. Future studies are needed to investigate CGM as a diagnostic and screening tool for CFRD.
Case 30-2022: A Newborn Girl with Hypoglycemia Sen, Sarbattama; Westra, Sjirk J.; Matute, Juan D. ...
The New England journal of medicine,
09/2022, Letnik:
387, Številka:
13
Journal Article
Recenzirano
A newborn girl was admitted to the neonatal ICU with prolonged hypoglycemia. At 34 weeks of gestation, an ultrasonographic survey of the fetal anatomy had revealed an intraabdominal cystic mass. A ...diagnostic test was performed.
•Development of CFLD adds significant morbidity and mortality to those with CF.•CFLD is associated with an increased prevalence of endocrinopathies.•Current treatment options for CFLD remain ...limited.•Early recognition and treatment of CFLD associated conditions is critical.
Cystic fibrosis-liver disease (CFLD) is one of the most common non-pulmonary complications in the CF population, is associated with significant morbidity and represents the third leading cause of mortality in those with CF. CFLD encompasses a broad spectrum of hepatobiliary manifestations ranging from mild transaminitis, biliary disease, hepatic steatosis, focal biliary cirrhosis and multilobular biliary cirrhosis. The diagnosis of CFLD and prediction of disease progression remains a clinical challenge. The identification of novel CFLD biomarkers as well as the role of newer imaging techniques such as elastography to allow for early detection and intervention are active areas of research focus. Biliary cirrhosis with portal hypertension represents the most severe spectrum of CFLD, almost exclusively develops in the pediatric population, and is associated with a decline in pulmonary function, poor nutritional status, and greater risk of hospitalization. Furthermore, those with CFLD are at increased risk for vitamin deficiencies and endocrinopathies including CF-related diabetes, CF-related bone disease and hypogonadism, which can have further implications on disease outcomes and management. Effective treatment for CFLD remains limited and current interventions focus on optimization of nutritional status, identification and treatment of comorbid conditions, as well as early detection and management of CFLD specific sequelae such as portal hypertension or variceal bleeding. The extent to which highly effective modulator therapies may prevent the development or modify the progression of CFLD remains an active area of research. In this review, we discuss the challenges with defining and evaluating CFLD and the endocrine considerations and current management of CFLD.
Cystic fibrosis-related diabetes (CFRD) affects up to 50% of adults with cystic fibrosis and adds significant morbidity and treatment burden. We evaluated the safety and efficacy of automated insulin ...delivery with the iLet bionic pancreas (BP) in adults with CFRD in a single-center, open-label, random-order, crossover trial.
Twenty participants with CFRD were assigned in random order to 14 days each on the BP or their usual care (UC). No restrictions were placed on diet or activity. The primary outcome was the percent time sensor-measured glucose was in target range 70-180 mg/dL (time in range TIR) on days 3-14 of each arm, and key secondary outcomes included mean continuous glucose monitoring (CGM) glucose and the percent time sensor-measured glucose was in hypoglycemic range <54 mg/dL.
TIR was significantly higher in the BP arm than the UC arm (75 ± 11% vs. 62 ± 22%, P = 0.001). Mean CGM glucose was lower in the BP arm than in the UC arm (150 ± 19 vs. 171 ± 45 mg/dL, P = 0.007). There was no significant difference in percent time with sensor-measured glucose <54 mg/dL (0.27% vs. 0.36%, P = 1.0), although self-reported symptomatic hypoglycemia episodes were higher during the BP arm than the UC arm (0.7 vs. 0.4 median episodes per day, P = 0.01). No episodes of diabetic ketoacidosis or severe hypoglycemia occurred in either arm.
Adults with CFRD had improved glucose control without an increase in CGM-measured hypoglycemia with the BP compared with their UC, suggesting that this may be an important therapeutic option for this patient population.
•Individuals with CFRD have unique diabetes physiology and comorbidities.•Perceptions of diabetes technology use in the CF community are positive.•Limited evidence supports the clinical effectiveness ...of these technologies in CFRD.•Automated insulin delivery improves A1c and decreases patient burden in T1D.•Proof of benefits to device use in CFRD may improve insurance coverage and increase sustained use.
There have been tremendous advances in diabetes technology in the last decade. Continuous glucose monitors (CGM), insulin pumps, and automated insulin delivery (AID) systems aim to improve glycemic control while simultaneously decreasing the burden of diabetes management. Although diabetes technologies have been shown to decrease both hypoglycemia and hyperglycemia and to improve health-related quality of life in individuals with type 1 diabetes, the impact of these devices in individuals with cystic fibrosis-related diabetes (CFRD) is less clear. There are unique aspects of CFRD, including the different underlying pathophysiology and unique lived health care experience and comorbidities, that likely affect the use, efficacy, and uptake of diabetes technology in this population. Small studies suggest that CGM is accurate and may be helpful in guiding insulin therapy for individuals with CFRD. Insulin pump use has been linked to improvements in lean body mass and hemoglobin A1c among adults with CFRD. A recent pilot study highlighted the promise of AID systems in this population. This article provides an overview of practical aspects of diabetes technology use and device limitations that clinicians must be aware of in caring for individuals with CF and CFRD. Cost and limited insurance coverage remain significant barriers to wider implementation of diabetes technology use among patients with CFRD. Future studies exploring strategies to improve patient and CF provider education about these devices and studies showing the effectiveness of these technologies on health and patient-reported outcomes may lead to improved insurance coverage and increased rates of uptake and sustained use of these technologies in the CFRD community.
Cystic fibrosis-related diabetes (CFRD) is the most common extrapulmonary manifestation of cystic fibrosis. The current standard of care for CFRD involves treatment with insulin, typically via ...multiple daily injections. We conducted a small pilot study comparing usual care with automated glycemic control using the bihormonal (insulin and glucagon) and insulin-only configurations of the bionic pancreas. Both configurations of the bionic pancreas achieved good glycemic control, with mean glucose levels <150 mg/dl and minimal hypoglycemia. Subjects reported improved treatment satisfaction and reduced burden of diabetes management with the bionic pancreas. Further investigation of automated glycemic control in the treatment of CFRD is warranted.
New Zealand, a geographically remote Pacific island with easily sealable borders, implemented a nationwide 'lockdown' of all non-essential services to curb the spread of COVID-19. Here, we generate ...649 SARS-CoV-2 genome sequences from infected patients in New Zealand with samples collected during the 'first wave', representing 56% of all confirmed cases in this time period. Despite its remoteness, the viruses imported into New Zealand represented nearly all of the genomic diversity sequenced from the global virus population. These data helped to quantify the effectiveness of public health interventions. For example, the effective reproductive number, R
of New Zealand's largest cluster decreased from 7 to 0.2 within the first week of lockdown. Similarly, only 19% of virus introductions into New Zealand resulted in ongoing transmission of more than one additional case. Overall, these results demonstrate the utility of genomic pathogen surveillance to inform public health and disease mitigation.
PDF
