Hypoglycemia Survey

SURVEY ON HYPOGLYCEMIA PRACTICES IN PEDIATRICS

Background: Current guidelines and clinical practices on the diagnosis and management of hypoglycemia with unknown etiology in children vary across centres and specialties, particularly regarding diagnostic work-up, treatment strategies, and the classification of hypoglycemia. Through this survey, we aim to map current practices and identify areas of consensus and variation.

Objective: To collect data from healthcare professionals on management (i.e. diagnostic and treatment) practices for hypoglycemia with unknown etiology in children beyond the neonatal period.

This project is initiated as a MetabERN and Endo-ERN collaboration.

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I am a:

General pediatrician

Pediatric hepatologist

Pediatrician metabolist

Pediatric endocrinologist

Pediatric resident

Clinical geneticist

Dietitian

Nurse

Other

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Other (specify)

I work in the following country (please indicate the country in which most of your activity takes place):

* must provide value

Other (specify)

Please provide your full name and surname:

* must provide value

Please fill in your e-mail address

* must provide value

If you want to be included in the group co-author list, please provide your full affiliation:

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I work in the following Institution:

Academic Hospital/University Hospital

Non-academic Hospital

Other

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Other (specify)

Have you personally managed an infant or child with hypoglycemia?

* must provide value

Yes

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How long have you been managing children with hypoglycemia?

< 5 years

5 - 10 years

11 - 20 years

> 20 years

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How many children with hypoglycemia have you managed (diagnosed, treated and/or followed up) over the last 5 years?

1-10

11-50

> 50

Unknown

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How many children with ketotic hypoglycemia of unknown etiology have you seen the last year?

1-5

6-15

> 15

Unknown

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Are you a member of any international organization?

SSIEM

ESPE

ISPAD

ASPED

ISPAE

Other

Unknown

None

Other (specify)

In which ERN are you active?

Metab-ERN

Endo-ERN

ERN EURO-NMD

ERN RARE-LIVER

ERN-ITHACA

Other

Unknown

None

Other (specify)

Where are patients referred to your unit from?

Emergency Department

PICU

General practitioner

(Private) practice pediatrician

Other local hospital

Other academic hospital

Other

Unknown

Other (specify)

Which groups of diseases associated with hypoglycemia are followed in your Institution?

Congenital hyperinsulinism

Transient neonatal hyperinsulinism

Insulinoma

Inherited metabolic diseases

Idiopathic ketotic hypoglycemia

Adrenal disorders

Growth hormone deficiency

Congenital portosystemic shunt

Other

Unknown

Other (specify)

What type of follow up appointments are arranged at your Institution for children with hypoglycemia?

Outpatient visits without lab assessment

Outpatient visits including lab assessment

In-patient reassessment

Video consultation

Phone consultation

Other

Unknown

Other (specify)

Up to what age do you routinely follow patients with hypoglycemia?

14 years

16 years

18 years

I also follow up adult patients

Other

Unknown

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Would you like to elaborate on your answer regarding the age up to which you provide follow-up?

Yes

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Elaborate on your answer here:

Other age limit for follow-up (specify)

What evaluation method do you routinely use to detect hypoglycemia?

Symptoms only

Serum/plasma glucose analysed in central laboratory

Point-of-care glucose (glucometer or blood gas analysis)

Continuous glucose monitoring (CGM)

Morning capillary blood glucose and ketone measurements before breakfast

Other

Unknown

Other (specify)

What blood glucose threshold do you use to define hypoglycemia after the neonatal period?

< 70 mg/dL (< 3.9 mmol/L)

< 60 mg/dL (< 3.4 mmol/L)

< 58 mg/dl (< 3.2 mmol/L)

< 50 mg/dL (< 2.8 mmol/L)

Other

Unknown

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Other (specify)

What blood glucose threshold do you use to define severe hypoglycemia after the neonatal period?

< 60 mg/dL (< 3.4 mmol/L)

< 58 mg/dl (< 3.2 mmol/L)

< 50 mg/dL (< 2.8 mmol/L)

< 40 mg/dL (< 2.3 mmol/L)

Other

Unknown

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Other (specify)

Besides glucose, do you routinely assess additional circulating point-of-care parameters during hypoglycemia in the diagnostic phase?

Lactate

Blood Ketones

Urine Ketones

Ammonia

Other

None

Unknown

Other (specify)

Do you (advise to) collect and analyse a critical sample in the diagnostic phase?

Always

Often

Sometimes

Rarely

Never

Unknown

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Why don't you ROUTINELY collect and analyse the critical sample?

I don't think it is necessary in all patients

The tests are not available

Patients often receive treatment before we can order the tests

Other

Unknown

Other (specify)

Besides glucose, what additional parameters do you routinely measure on the critical sample?

Beta-hydroxybutyrate

Lactate

Free fatty acids

Insulin

C-peptide

Growth hormone

Cortisol

Acylcarnitines

Amino acids

Ammonia

Urine organic acids

Oral hypoglycemic agents

Toxicology screening (blood/urine)

Other

Unknown

Other (specify)

Which analytes can be measured IN-HOUSE (in your hospital) on the critical sample?

Beta-hydroxybutyrate

Lactate

Free fatty acids

Insulin

C-peptide

Growth hormone

Cortisol

Acylcarnitines

Amino acids

Ammonia

Urine organic acids

Oral hypoglycemic agents

Toxicology screening (blood/urine)

Other

Unknown

Other (specify)

Do you routinely perform a controlled fasting challenge for the etiological diagnosis of unexplained hypoglycemia?

Always

Often

Sometimes

Rarely

Never

Unknown

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Why do you routinely perform a controlled fasting challenge for the etiological diagnosis of unexplained hypoglycemia?

I think it is necessary in the majority of patients

I want to know for how long the patient can fast until hypoglycemia

Critical sample is not routinely available

Other

Unknown

Other (specify)

Do you routinely perform an i.m. glucagon test for the etiological diagnosis of hypoglycemia?

Always

Often

Sometimes

Rarely

Never

Unknown

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Why do you routinely perform an i.m. glucagon test for the etiological diagnosis of unexplained hypoglycemia?

I think it is necessary in the majority of patients

Critical sample has not been available in the patient

I do it to document hyperinsulinism

I do it to document GSD subtypes

I do it to document rebound hypoglycemia

Other

Unknown

Other (specify)

Do you routinely perform an oral glucose tolerance test for the etiological diagnosis of hypoglycemia?

Always

Often

Sometimes

Rarely

Never

Unknown

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Why do you routinely perform an oral glucose tolerance test for the etiological diagnosis of hypoglycemia?

I think it is necessary in the majority of patients

Critical sample has not been available in the patient

I do it to document rebound hypoglycemia

I do it to document possible diabetic response or glucose intolerance

Other

Unknown

Other (specify)

Do you routinely perform genetic analysis for etiological diagnosis of hypoglycemia?

Always

Often

Sometimes

Rarely

Never

Unknown

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Why don't you routinely perform genetic analysis for etiological diagnosis of hypoglycemia?

Most of the cases are suspected to be physiological ketotic hypoglycemia

Most of the cases only display a single episode of hypoglycemia

I don't need it to establish the hypoglycemia subtype diagnosis

It is not available

It is too expensive

Other

Unknown

Other (specify)

What types of in‑house (in your hospital) genetic analysis are available for genetic disorders associated with hypoglycemia?

Targeted single-gene analysis

Hypoglycemia gene panel analysis

Whole exome sequencing

Whole genome sequencing

Microarray analysis

Karyotyping

Copy number variants analysis

Long read sequencing

Trio exome sequencing

Trio genome sequencing

None

Other

Unknown

Other (specify)

Which of the above mentioned genetic analysis do you routinely use for genetic diagnostic confirmation (think of the majority of your patients with hypoglycemia)?

Targeted single-gene analysis

Hypoglycemia gene panel analysis

Whole exome sequencing

Whole genome sequencing

Microarray analysis

Karyotyping

Copy number variants analysis

Long read sequencing

Trio exome sequencing

Trio genome sequencing

None

Other

Unknown

Other (specify)

Do you routinely perform abdominal ultrasound for the etiological diagnosis of hypoglycemia?

Always

Often

Sometimes

Rarely

Never

Unknown

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Why do you routinely perform abdominal ultrasound for the etiological diagnosis of hypoglycemia?

I think it is necessary in the majority of patients

It provides clues to the diagnosis

Other

Unknown

Other (specify)

Do you routinely use continuous glucose monitoring (CGM) devices in the etiological/diagnostic process of a child with unexplained hypoglycemia?

Always

Often

Sometimes

Rarely

Never

Unknown

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Why don't you routinely continuous glucose monitoring (CGM) devices in the etiological/diagnostic process of a child with unexplained hypoglycemia?

CGM is not reimbursed

CGM is not available

CGM is unreliable in the low range

Quality of life burden

Lack of expertise

It has no additional value in the diagnostic phase

Other

Unknown

Other (specify)

Are there any additional test(s) or procedure(s) you routinely use in patients with unexplained hypoglycemia (think of the majority of the patients with hypoglycemia you see)?

Yes

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If yes:

Growth hormone stimulation test

Synacthen test

Skin biopsy

Muscle biopsy

Liver biopsy

Lumbal puncture for spinal fluid glucose concentration

Other

Other (specify)

Regarding your previous answer on additional tests/procedures, please specify the reason for the test(s) and/or procedure(s)

In case no i.v. line is available, how do you routinely manage acute hypoglycemia in conscious children with no etiological diagnosis?

Administer complex carbohydrates

Administer oral/buccal/G-tube glucose/glucose gel

Administer fruit juice

Administer another sugary drink

Other

Unknown

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Other (specify)

What kind of oral glucose bolus do you routinely (suggest to) administer?

10% dextrose solution

33% dextrose solution

Sugar sachet

Glucose tablets

Glucose oral solution/gel

Other

Unknown

Other (specify)

What amount of oral glucose bolus do you routinely (suggest to) administer?

100 mg/kg (e.g. 1 ml/kg 10% dextrose/glucose)

200 mg/kg (e.g. 2 ml/kg 10% dextrose/glucose)

300 mg/kg (e.g. 3 ml/kg 10% dextrose/glucose)

> 300 mg/kg (e.g. >3 ml/kg 10% dextrose/glucose)

Sugar sachets depending on body weight

Other

Unknown

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Other (specify)

How much time (approximately) after oral management do you (suggest to) first recheck blood glucose?

5 minutes

15 minutes

30 minutes

60 minutes

No recheck

Other

Unknown

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Other duration before first recheck (specify):

In case an i.v. line is available, how do you most often manage acute hypoglycemia as a first line treatment in children with no etiological diagnosis?

5% dextrose continuous maintenance

10% dextrose bolus

10% dextrose continuous maintenance

10% dextrose bolus followed by maintenance

20% dextrose bolus followed by maintenance

Other

Unknown

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Other (specify)

In case dextrose solution i.v. bolus is used, what amount of glucose do you routinely (suggest to) administer?

100 mg/kg (e.g. 1 ml/kg 10% dextrose/glucose)

200 mg/kg (e.g. 2 ml/kg 10% dextrose/glucose)

300 mg/kg (e.g. 3 ml/kg 10% dextrose/glucose)

> 300 mg/kg (e.g. >3 ml/kg 10% dextrose/glucose)

Other

Unknown

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Other (specify)

In children with recurrent or severe hypoglycemia with no etiological diagnosis, which additional drugs do you eventually use to treat acute hypoglycemia?

Glucagon

Corticosteroids

Both glucagon and corticosteroids

None

Other

Unknown

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Other (specify)

What recommendations do you routinely provide to prevent day-to-day recurrence of hypoglycemia in children with no etiological diagnosis?

No recommendation

Use of oral glucose solution

Bed time snack

Increased complex carbohydrate intake

Maltodextrin supplementation

Corn starch supplementation

Other

Unknown

Other (specify)

What recommendations do you routinely provide in case of decreased food intake and/or infectious episodes in children with no etiological diagnosis of hypoglycemia?

No recommendation

Use of oral rehydration solutions

Use of oral glucose solution

Increased complex carbohydrate intake

Maltodextrin supplementation

Corn starch supplementation or an equivalent

Bed time snack

Sick-day regimen

Emergency letter

Paracetamol in case of fever

Shorten the intervals between meals

Other

Unknown

Other (specify)

Do you use continuous glucose monitoring devices in the follow up/monitoring a child with unexplained hypoglycemia?

Always

Often

Sometimes

Rarely

Never

Unknown

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Why don't you do so?

CGM is not reimbursed

Quality of life burden

Lack of expertise

It has no additional value in the follow-up phase

Other

Unknown

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Other (specify)

After completion of hypoglycemia investigations without identifying a diagnosis, do you routinely refer the patient for follow-up?

Yes, to their local/regional hospital

Yes, to an academic/University hospital centre close to them

No, we perform follow-up ourselves

Other

Unknown

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Other (specify)

What are the main barriers to transferring medical records/results to specialized centers within your country for children with unexplained hypoglycemia?

No significant barriers

Medical records are not available electronically

Electronic systems are not interoperable between institutions

No secure electronic method is available for transferring health data

Transfer relies on manual, time-consuming processes, for example email, post, or physical media

Technical limitations prevent the transfer of large files or specific data formats, for example imaging, genetic data, or continuous glucose monitoring data

Data protection, patient consent, or other legal requirements restrict data sharing

Restrictions on sharing health data across regions

Other

Unknown

Other (specify)

Which of the following best describes access to specialist advice from another institution within your country for a child with unexplained hypoglycemia before or during referral?

Specialist advice is always readily available

Specialist advice is possible but not readily available

Specialist advice is readily available only for selected or urgent cases

Specialist advice is generally not available before referral

Other

Unknown

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Other (specify)

Which of the following best describes access to specialist expertise in another country when the required expertise is not available within your country?

Access to specialist expertise in another country is readily available

Access is available, but it is often difficult or time-consuming to organize

Access is available only through formal international networks or established agreements

Access is generally not available

The required expertise is available within my country

Other

Unknown

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Other (specify)

Do you routinely provide emergency letters to your patients with unexplained hypoglycemia?

Yes, using a regional/national standard letter

Yes, using emergencyprotocol.net

Yes, using customized letters

Yes, for traveling only

Other

Unknown

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Other (specify)

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