Does Glycogen Storage Disorder Cause Large for Gestational Age Babies

Am J Obstet Gynecol. Author manuscript; available in PMC 2013 Oct thirty.

Published in final edited grade as:

PMCID: PMC3812528

NIHMSID: NIHMS54329

Pregnancies in Glycogen Storage Disease Type Ia

Daniëlle HJ Martens, Physician,^one January Peter Rake, MD PhD,^two Martin Schwarz, Medico,ⁱⁱⁱ Kurt Ullrich, MD PhD,⁴ David A Weinstein, Doctor MMSc,^v Martin Merkel, MD PhD,⁶ Pieter JJ Sauer, MD MMSc,^one and Thou Peter A Smit, MD PhD¹

Daniëlle HJ Martens

ⁱBeatrix Children's Hospital, University Medical Center Groningen, holland

January Peter Rake

²Department of Pediatrics, Martini Infirmary, Groningen, kingdom of the netherlands

Martin Schwarz

^threeDepartment of Internal Medicine, University Hospital Düsseldorf, Germany

Kurt Ullrich

⁴Department of Pediatrics, University Hospital Hamburg, Germany

David A Weinstein

⁵Glycogen Storage Disease Program and the Division of Pediatric Endocrinology, University of Florida College of Medicine, Gainesville, FL, United states of america

Martin Merkel

⁶Three^rd Department of Internal Medicine, University Hospital Hamburg, Germany

Pieter JJ Sauer

¹Beatrix Children's Infirmary, Academy Medical Center Groningen, kingdom of the netherlands

G Peter A Smit

^oneBeatrix Children's Hospital, University Medical Center Groningen, the Netherlands

Abstruse

Objective

Reports on pregnancies in women with GSD-Ia are scarce. Due to improved life expectancy, pregnancy is becoming an important upshot. We draw xv pregnancies focusing on dietary treatment, biochemical parameters and GSD-Ia complications.

Study Design

Carbohydrate requirements (mg/kg/min), triglyceride and uric acid levels, liver ultrasonography and creatinine clearance were investigated before, during and later on pregnancy. Information of the newborns were obtained from the records.

Results

In the first trimester, a significant increase in carbohydrate requirements was observed (p=0,007). Most patients had acceptable triglyceride and uric acid levels during pregnancy. No increase in size/number of adenomas was seen. In 3/4 patients, a decrease in GFR was observed afterwards pregnancy. In three pregnancies, lactic acidosis developed during delivery with severe multi-organ failure in one. All simply one of the children are salubrious and show expert psychomotor development.

Conclusion

Successful pregnancies are possible in GSD-Ia patients, although specific GSD-Ia related risks are present.

Keywords: pregnancy, glycogen storage disease type Ia, glucose metabolism, complications

Introduction

Glycogen storage disease type Ia (GSD Ia) is an autosomal recessive inborn mistake of carbohydrate metabolism due to a deficiency of the enzyme glucose-half-dozen-phosphatase. Deficiency of this enzyme leads to disturbed glycogenolysis and gluconeogenesis, and patients with GSD Ia therefore depend completely on exogenous sources of glucose. Biochemically, it is characterized by the combination of astringent fasting hypoglycemia, hyperlactacidemia, hyperuricemia and hyperlipidemia.^1,ii Clinically, information technology is characterized by stunted growth, hepatomegaly, wasted muscles, delayed puberty and a haemorrhage tendency due to impaired platelet function. The primary aim of treatment is prevention of hypoglycemia past administering exogenous glucose either past frequent meals, uncooked cornstarch (UCCS), or continuous gastric drip feeding (CGDF).³

This lifelong dietary handling has dramatically improved the expected lifespan of patients with GSD Ia. More and more patients reach adult age, but several long-term complications have been observed such equally glomerular hyperfiltration and proteinuria leading to renal insufficiency, liver adenomas with a modest run a risk of malignant transformation, decreased os density, anemia, ovarian cysts in females and rarely pulmonary hypertension.⁴

With increased life expectancy, pregnancy is condign an of import upshot in female person GSD Ia patients. The complications described above pose unique risks during pregnancy including the occurrence of hypoglycemia, possible increases in size or numbers of adenomas due to hormonal changes⁵ or a further subtract in renal function during pregnancy, as is seen in diabetes mellitus patients.⁶ Furthermore, in normal pregnancy, cholesterol and triglyceride levels increment significantly and uric acid initially decreases and thereafter rises in the 2d one-half of pregnancy.^vii,8 These biochemical fluxes may pose additional pregnancy-related risks for GSD Ia patients, who are already predisposed to hyperlipidemia and hyperuricemia in the non-pregnant state.

Reports on pregnancies in women with GSD Ia are scarce.^9–14 Reports concerning the dietary management during pregnancy and the effect of pregnancy on adenomas and renal function in GSD Ia patients are not available at all. In this study, nosotros describe the class and management of 15 pregnancies in 11 GSD Ia patients, with special focus on dietary handling, metabolic command and the form of adenomas and renal function during pregnancy.

Fabric and Methods

We included all female person GSD Ia patients from the metabolic centers of Hamburg (Germany), Düsseldorf (Federal republic of germany), Florida (USA) and Groningen (the Netherlands), who had been under surveillance during their pregnancies. The diagnosis was confirmed by mutation analysis in all patients. The report was approved by the Institutional Review Boards at the respective institutions either as a prospective study (Florida, Düsseldorf) or every bit an exempt investigation because of the retrospective and anonymous drove of data, in accordance with Dutch law. Data were collected from the patient records by the treating dr. and anonymous patient record forms were filled in that could non be traced to the individual patients. Written informed consent from the patients was obtained.

Hypoglycemia is defined equally glucose concentrations < 63 mg/dl (three.v mmol/l). Blood glucose concentrations were measured at home by means of a portable glucose meter if symptoms of hypoglycemia occurred and occasionally during 24 hr glucose measurements.

Data on the diets of the patients before, during and after pregnancy were collected. In all centers dietary adjustments in pregnancy were based on clinical symptoms of hypoglycemia, 24 hr blood glucose measurements and the above described biochemical parameters. Basal saccharide requirements before, during and inside half dozen months after pregnancy were calculated from the nocturnal saccharide intake and were expressed in mg/kg/min.

No specific handling protocols for pregnant women with GSD Ia be. In 2002 guidelines were published for the management of GSD Ia patients in general.³ These guidelines contain biomedical targets for the follow-up of these patients too every bit recommendations for dietary and pharmacological treatment. In case of increased uric acid levels a xanthine oxidase inhibitor such as allopurinol® is recommended for prevention of gout and urate nephropathy. Angiotensin converting enzyme inhibitors such as enalapril® should be started in instance of persistent microalbuminuria for preventing further deterioration of renal function in patients.

Data on metabolic control and GSD Ia related complications before, during and after pregnancy were collected retrospectively. Patients were regarded to be in adept metabolic control if they met the biomedical targets every bit defined in the guidelines of the retrospective European study on GSD I.³ According to these guidelines patients are considered to be in good metabolic control when triglycerides are < 530 mg/dl (6.0 mmol/l) and uric acid concentrations < 7.6 mg/dl (450 µmol/l). Triglyceride and uric acid concentrations in plasma were investigated co-ordinate to standard laboratory procedures by colorometric and spectrophotometric analysis, respectively (Roche Modular).

Liver adenomas were monitored before, during and after pregnancy past performing ultrasonography of the liver. Nephropathy was diagnosed from the presence of microalbuminuria (defined as > 2.5 mg albumin/mmol creatinine) in morning urine samples. Estimated creatinine clearance was calculated from adjusted serum creatinine every bit described past Gault et al.^xv Glomerular filtration rates before pregnancy and within 5–10 months after pregnancy were measured by I¹²⁵ iothalamate and I¹³¹ hippuran clearance as described by Apperloo et al.¹⁶

Finally data with regard to conception and fetal outcome were obtained from the medical records. Neonatal hypoglycemia was defined equally glucose levels < 45 mg/dl (2.5 mmol/fifty).

Differences in carbohydrate requirements, creatinine clearance and glomerular filtration rates between the periods earlier, during and after pregnancy were analyzed with the paired-samples t-test, by apply of statistical software (SPSS 12.0.1).

Results

Patient characteristics

In total xv pregnancies in xi GSD Ia patients were included. The demographical data of these women are shown in tabular array i. Three patients have had multiple pregnancies. Two pregnancies occurred in two patients and one patient has had 3 successful pregnancies. Median age at conception was 29.3 years (range 21.0–34.7 years). Median weight before pregnancy was 62 kg (range 51–74 kg). Median Body Mass Index before pregnancy was 25.three (range 21.9 – 28.9).

Table 1

Patient characteristics.

Patient	Centre	Age at formulation (yrs)	Height (cm)	Weight at formulation (kg)	BMI at formulation (kg/10002)	Weight 3rd trimester (kg)	Weight gain in pregnancy (kg)
1	H	32.half-dozen	161	59	22.8	74	15
ii	H	34.0	175	74	24.2	84	x
3.1	Yard	29.3	167.v	63	22.five	eighty	17
3.2	G	34.0	167.5	62	22.i	85	23
4	G	31.iv	162	60	22.9	69	9
v	G	31.three	147	51	23.6	64	xiii
6	D	21.0	162	72	27.4	95	23
7	D	34.7	166	67	24.iii	78	11
8	D	26.vi	155	62	25.8	68	6
9.ane	D	22.0	150	57	25.iii	73	16
9.2	D	25.0	150	65	28.9	NA	-
x	F	31.0	154.4	65	27.3	NA	-
eleven.1	F	25.4	147	59	27.3	64	5
11.two	F	26.one	147	sixty	27.eight	NA	-
11.iii	F	28.5	147	59	27.three	NA	-

Conception

Out of the 11 included GSD Ia patients, five had delayed pubertal development, defined as reaching Tanner stage 4 later than the 95th percentile. In three out of seven investigated patients, polycystic ovaries were present. Of the 11 women included in our study, three failed to conceive after 12 months of unprotected intercourse. Ii of these three women had polycystic ovaries and one of these 2 women with polycystic ovaries was treated with follicle stimulating hormone.

Dietary treatment

Earlier pregnancy, UCCS was taken at night in xi of the 15 pregnancies, and in the other four cases CGDF. Patient 3 was initially treated with UCCS during the dark, but developed frequent hypoglycemia in the first trimester. Her nocturnal diet was inverse to CGDF in a hospital setting and connected throughout pregnancy; thereafter, no hypoglycemia was seen. During the second pregnancy of this patient, she was treated with CGDF at night throughout pregnancy, but frequent hypoglycemia again occurred in the commencement trimester. One episode of severe hypoglycemia required hospitalization and intravenous correction with an intravenous infusion of glucose.

Patient 4, one of the four patients treated with CGDF at night, suffered from astringent nausea and pyrosis in the third trimester leading to decreased intake and recurrent hypoglycemia. She was admitted to the infirmary and her diet was adjusted to CGDF 24 hours per twenty-four hours. Thereafter, no hypoglycemia was observed.

Figure 1 shows the basal carbohydrate requirements in 10 pregnancies of 8 GSD Ia patients, calculated as sugar intake in mg/kg/min at dark. The carbohydrate requirements in the first trimester of pregnancy are significantly higher compared to the requirements before pregnancy (p= 0.007) and after pregnancy (p= 0.05). Carbohydrate requirements in 2nd and third trimester did not differ from the requirements before and after pregnancy.

Basal carbohydrate requirements during pregnancy

Nocturnal carbohydrate intake in mg/kg/min for x pregnancies in eight patients with glycogen storage disease type Ia.

Metabolic command in pregnancy

In 6 out of xv pregnancies, an increase in frequency and/or severity of hypoglycemia was observed. In three pregnancies, hospitalization was needed for adjusting the diet to CGDF during mean solar day and night or treatment with glucose infusion.

Triglyceride and uric acid concentrations before pregnancy and maximal levels during pregnancy are shown in table ii. While two patients had a triglyceride concentration above 530 mg/dl earlier pregnancy, both showed acceptable triglyceride levels during pregnancy. One patient with triglycerides below 530 mg/dl before pregnancy, adult a hypertriglyceridemia in the second trimester. After pregnancy, triglycerides were elevated in three patients. One of these 3 patients did not take elevated triglycerides before or during pregnancy.

Table two

Metabolic control and complications earlier and during pregnancy.

Patient	Centre	TG earlier pregnancy (mg/dl)	Max TG in pregnancy (mg/dl)	Max UA in pregnancy (mg/dl)	Adenomas before (number / size of the largest)	Course adenomas during pregnancy	Microalbuminu ria (>2.5 mg/mmol creatinine)
1	H	332	344	4.half dozen	4 / 5.7 cm	↓ 2.vii cm	No
2	H	187	240	6.3	No	-	No
three.1	M	174	313	four.4	No	-	No
three.ii	G	193	250	two.7*	No	-	No
four	Thousand	631	480	five.nine*	mult / ii.eight cm	status quo	Yes
five	G	472	438	9.9	mult / vi.6 cm	1 extra	No
half dozen	D	746	301	6.eight	No	-	Yes
seven	D	396	857	5.9	3 / 8 cm	status quo	Yes
8	D	NA	NA	NA	mult / 3.four cm	status quo	Yes
9.one	D	347	NA	seven.9	No	-	No
nine.2	D	320	NA	NA	No	-	No
x	F	NA	NA	eight.v	mult / 7.three cm	status quo	No
11.i	F	NA	443	NA	No	-	No
11.2	F	397	NA	3.ix	1 / 1 cm	status quo	No
11.3	F	310	NA	NA	1 / i cm	status quo	No

In 12 cases, allopurinol was used earlier pregnancy and was discontinued earlier conception in x pregnancies. One patient had a uric acrid concentration > vii.vi mg/dl before pregnancy during allopurinol treatment and continued to take increased concentrations during pregnancy without allopurinol. Two patients showed an elevation of uric acid during pregnancy after allopurinol treatment was discontinued. In two patients treated with allopurinol before pregnancy and with normal uric acid levels earlier and during pregnancy, uric acid was elevated after pregnancy.

GSD Ia complications during pregnancy

All patients were screened for adenomas before, during and after pregnancy by means of ultrasound investigations of the liver. In seven of 15 cases, i or more adenomas were present prior to pregnancy. In patient iv, a partial liver resection had been performed two years before pregnancy because of a hemorrhage in one of the adenomas, but this patient yet had multiple adenomas. Follow-up during and after pregnancy showed that one of the seven patients developed an actress adenoma during pregnancy. In five out of seven patients, the size and number of adenomas remained unaltered. In ane patient, a decrease in size of the adenoma was observed (tabular array 2).

In four out of xv pregnancies nephropathy with microalbuminuria was present prior to the pregnancy. In five patients, enalapril® was used. The enalapril® was discontinued in these patients prior to formulation. Estimated creatinine clearance values were available in seven of the 15 pregnancies and showed a significant increase in the second trimester, when compared to the values before pregnancy and afterward pregnancy (p=0.04, p=0.04). In iv pregnancies of three patients glomerular filtration rates were bachelor before and after pregnancy. In three of these four pregnancies a decrease in GFR after pregnancy was observed (figure 2). No increase in microalbuminuria was seen. Ane patient without nephropathy adult severe proteinuria in the second trimester and hypertension in the tertiary trimester, but completely recovered after pregnancy with no proteinuria documented for ii years.

Glomerular filtration rates before and after pregnancy

Glomerular filtration rates before and subsequently pregnancy for 4 pregnancies in 3 patients with glycogen storage disease type Ia.

I of two GSD Ia patients with colitis experienced an aggravation of symptoms in the postpartum period.

Perinatal menstruation

In twelve out of 15 pregnancies, a caesarean section was performed for diverse obstetric reasons, not related to GSD Ia. All patients were treated with a glucose infusion during labor. I patient developed intermittent hypoglycemia and a mild lactic acidosis during delivery. During the 2d delivery of this patient, once again mild lactic acidosis was seen. Another patient suffered from severe hyperlactacidemia (31 mmol/l) and metabolic acidosis during delivery despite loftier-normal glucose levels. She developed a systemic inflammatory response syndrome with multi-organ failure and preterminal renal insufficiency. Hemodialysis was required, and she had to be admitted to an intensive care unit for 41 days. No information on metabolic control during pregnancy are available for this patient, but pre-pregnancy laboratory values showed expert metabolic control. Of note, withal, this patient has had a course complicated by epilepsy and mental retardation due to previous hypoglycemia, and she is being evaluated for a liver transplant due to hepatic adenomas, anemia and liver cirrhosis with hypoalbuminemia.

Offspring

The neonatal outcome of all 15 pregnancies is summarized in table 3. Two cases of prematurity, one case of fetal growth retardation and one case of macrosomia was observed. Hypoglycemia was documented in four of the15 neonates including the two neonates with macrosomia or fetal growth retardation. In three of these iv cases, hypoglycemia was transient (< 24 hours) except for the dysmature kid, who needed temporary gastric drip feeding because of feeding difficulties. The two premature children also were treated with gastric drip feeding because of prematurity, just showed no hypoglycemia. One of the premature children was built-in to the critically sick female parent with hyperlactacidemia and had depression APGAR scores (6/five/5 after 1/five/10 minutes). This child also showed delayed psychomotor development: walking at age ii years and i month, and speaking 3–four words at age iii years. All other children had normal psychomotor development at the age of one year.

Tabular array 3

Event offspring of 15 pregnancies in 11 GSD Ia patients.

Patient	Gestational historic period	Birth weight (grams)	Glucose < 45 mg/dl	PM development
ane	41+4	4320	Yes	Adept
2	xl+6	3015	No	Proficient
iii.1	38+4	3165	Yes	Good
3.two	38+4	3530	No	Proficient
4	38+3	3260	No	Skilful
5	38+0	2510	Yep	Skilful
six	37+4	3860	No	Good
7	37+0	2990	No	Good
8	34+6	2400	No	Delayed
nine.1	38+3	3520	No	Good
9.2	38+0	3000	Yep	Good
10	35+4	1705	No	Good
11.1	42+0	3905	No	Practiced
eleven.2	41+5	3232	No	Good
11.3	40+0	3680	No	Practiced

Annotate

Successful pregnancies are possible in GSD Ia patients, although specific GSD Ia related risks are present. Saccharide requirements showed large intra- and inter-individual variations. In the first trimester of pregnancy a significant increase in carbohydrate requirements was observed with a gradual return to pre-pregnancy sugar requirements afterward in pregnancy. Although two patients in this sample had two pregnancies, in all pregnancies an increment in carbohydrate requirements in the first trimester of pregnancy was establish. In normal pregnancy no increase in carbohydrate requirement is seen in early on pregnancy, but an increase in peripheral insulin sensitivity in early pregnancy has been described.¹⁷ The increase in peripheral insulin sensitivity possibly causes a diminished glucose availability in blood, that tin be compensated past an increased endogenous glucose production in times of need in healthy women. Because of the increase in carbohydrate requirement early in pregnancy and due to risks associated with hypoglycemia regular blood glucose measurements are recommended in GSD Ia patients throughout pregnancy.

Although an increase in uric acrid and/or triglyceride levels was observed in some patients, both during pregnancy and in the postnatal menstruum, the majority of our patients showed good metabolic command during pregnancy. This leads to the conclusion that good metabolic control during pregnancy can exist achieved, although frequent follow-upwards of the biochemical targets is warranted. Lactate concentrations can increase during pregnancy due to hypoglycemia, hormonal changes and stress of pregnancy. Monitoring of lactate concentrations with a portable lactate meter¹⁸ may also exist beneficial particularly in the start and 3rd trimesters in an effort to also maximize metabolic control.

This report has shown no show for increases in size or number of adenomas in this group of 15 pregnancies. Rupture of adenomas during pregnancy has been described in the past and poses a high mortality risk for both mother and child. Therefore Terkivatan et al. have recommended resection of big (≥ 5 cm) or growing symptomatic adenomas before pregnancy.⁵ Due to the modest number of patients in our study, nonetheless, no conclusions tin be drawn concerning this recommendation.

A significant increase in the estimated creatinine clearance was observed during pregnancy. This is compatible with the course in normal pregnancy, where a 40–65% increase in GFR has been described.^nineteen In three out of four investigated patients, a decrease in GFR was observed later pregnancy, compared to the pre-pregnancy values. In pregnant patients with moderate –to-severe renal insufficiency due to diabetic nephropathy, a transient worsening of renal function in pregnancy was seen in 27% of patients and a permanent decline in 45%.⁶ Especially diabetes patients with a significantly low creatinine clearance before pregnancy are at risk.²⁰ Nephropathy in GSD Ia patients resembles the grade of diabetic nephropathy.²¹ Possibly, due to hemodynamic changes in pregnancy and the discontinuation of enalapril before and during pregnancy, a further decrease in GFR might develop in GSD Ia patients with known nephropathy, somewhen leading to glomerular hypofiltration and possible renal insufficiency in some of the GSD Ia patients. It is generally brash to cease treatment with ACE inhibitors preconceptionally considering of agin effects for the fetus, such as hypotension, renal issues and growth restriction.²² Angiotensin II receptor antagonists, however, might be an alternative for GSD Ia patients with nephropathy, as described in pregnant patients with diabetic nephropathy.²³ Whether the decreases in GFR described in our patients would have occurred in the absence of pregnancy and despite enalapril® treatment is not clear. Withal, the progressive deterioration of renal function in pregnancy should at least be considered in GSD Ia patients with depression creatinine clearance values prior to pregnancy.

In three pregnancies, lactate acidosis developed during delivery with severe multi-organ failure in 1 instance. In some cases higher glucose amounts might prevent lactic acidosis, although glucose levels in the severe case were high-normal. Metabolic deterioration during delivery can be a serious take chances for the child, as lactic acidosis in the mother can cause serious issues in the neonate. Therefore frequent glucose controls and adequate treatment with glucose infusion according to the European guidelines³ are recommended during delivery.

The bulk of the children born to GSD Ia mothers are healthy and show a skilful psychomotor evolution. Transient hypoglycemia may occur in these neonates, merely this could well be accounted for by normal variation.

The fact that more healthier individuals are more likely to attempt pregnancy is not seen in the group of patients that nosotros describe. Some of these patients even had multiple complications.

In decision good metabolic control is possible in GSD Ia patients during pregnancy. Physicians should be enlightened of the apparent increased carbohydrate requirements during the first trimester of pregnancy and possibly well-nigh the end of pregnancy. No significant increase in size or number of liver adenomas is observed in our patients. However, a farther deterioration of renal function in GSD Ia patients, especially those with a low creatinine clearance prior to pregnancy, might be an important pregnancy-related take a chance in these patients. The development of hypoglycemias and lactic acidosis during pregnancy and delivery could pose an additional risk for impaired growth and delayed psychomotor evolution in children of GSD Ia mothers. This risk tin be controlled by closely monitoring metabolic control during pregnancy and past glucose measurements and continuous glucose infusion during delivery.

Acknowledgements

We thank Christina E Morgan, Catherine E. Correia (University of Florida), Greet van Rijn and Esmee Udema (University Medical Center Groningen) for their help in preparation of the paper.

GRANT SUPPORT: These investigations were supported in part by NIH General Clinical Inquiry Heart Grant M01 RR 00082 (Florida) and NIH Mentored Career Award K23 RR 017560 (DW).

Footnotes

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Presented in part at the Annual Symposium of the Society for the Study of Inborn Errors of Metabolism, Paris, France, Sept. 6–9, 2005.

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Does Glycogen Storage Disorder Cause Large for Gestational Age Babies

Source: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3812528/

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