Eur J Pediatr (1995) 154:944-948

9 Springer-Verlag 1995

Stephan F6sel

Received: 28 December 1994

Accepted: 2 April 1995

I would like to dedicate this paper to Professor Dr. W. Toussaint in Koblenz for
having awakened my interest in neonatology
S. F6sel
Kinderklinik, Gustav-Adolf-Strasse 8,
D-97422 Schweinfurt, Germany

Transient and permanent neonatal diabetes

A b s t r a c t N e o n a t a l diabetes, which
m a y be transient or permanent, is
rare. M o s t patients are full-term but
small- for-date infants. Typical symptoms of diabetes mellitus occur within
the first 4 w e e k s o f life, requiring insulin therapy and v e r y strict b l o o d
glucose monitoring. Subsequent
growth and psychomotor development
are usually normal. In about 33% of
these patients the diabetes r e m a i n s
permanent; the transient cases, h o w ever, often develop permanent diabetes
mellitus later in life. Exocrine pancre-

Introduction
The yearly incidence of insulin dependent diabetes (IDDM)
is a p p r o x i m a t e l y 10-15 n e w cases per 100 000 children in
E u r o p e with c o n s i d e r a b l e regional differences (Finland
42.9/100 000-Northern G r e e c e 4.6/100 000) with increasing t e n d e n c y over the last 30 years. O n l y 0.5% o f paediatric patients develop diabetes during the 1st y e a r of life
[9, 35, 70, 90].
N e o n a t a l onset o f I D D M , called p e r m a n e n t neonatal
diabetes mellitus ( P N D M ) is e x t r e m e l y rare. M o r e c o m mon is a temporary form of I D D M called "transient neonatal diabetes m e l l i t u s " ( T N D M ) , also k n o w n as "transient
diabetes in infancy" [2], " t e m p o r a r y idiopathic neonatal
h y p e r g l y c a e m i a " [30] "neonatal p s e u d o d i a b e t e s m e l l i t u s "
[26] and similar synonyms. This disease resembles P N D M ,
but d i s a p p e a r s c o m p l e t e l y after several w e e k s or months.
T N D M and P N D M can only be distinguished by their
course [4, 5, 12].

atic insufficiency is present in some

patients. Neonatal diabetes differs
from t y p e - I diabetes in m a n y aspects
and seems to form a distinct entity o f
inborn pancreatic malfunction.
K e y w o r d s N e o n a t a l diabetes 9
Pancreatic m a l f u n c t i o n
A b b r e v i a t i o n s IDDM insulin
d e p e n d e n t diabetes mellitus (type I) 9
TNDM transient neonatal diabetes
mellitus 9 P N D M p e r m a n e n t neonatal
diabetes mellitus

Materials and methods

This article is based on the review of 139 cases of neonatal diabetes. We evaluated the reports for possible differences between
PNDM and TNDM. Excluded from analysis were:
1. Seventeen cases of secondary hyperglycaemia due to infections,
cerebral affections, glucose infusions etc. [50, 53, 58, 60, 63, 66,
72, 77, 82, 89, 105]. This phenomenon has been studied extensively [24, 34, 65, 76, 104] and differs clearly from "idiopathic"
neonatal diabetes.
2. Nine cases of permanent diabetes with onset of symptoms after
the 1st month of life [3, 29, 99, 101, 104].
3. Ten cases - nine permanent and one transient-associated with
complex syndromes, malformations or metabolic disorders [51,
62, 67, 78, 79, 88, 92].
4. Nine "undecided" cases: these children died early or the report
was incomplete, not allowing a detailed analysis [18, 20, 21, 44,
46, 54, 74, 81, 102].
This left us with 65 cases of neonatal diabetes designated as "transient" and 29 cases designated as "permanent". However, differentiation between "transient" and "permanent" sometimes remained
doubtful because many permanent cases were followed for only
6-12 months [1, 36, 37, 43, 47, 64], whereas the longest reported
duration of transient diabetes is 18 months [ 10, 49, 84]. Cases originally reported as "permanent" [103] were actually later found to
be temporary [48].

945

Incidence, geographical and genetic factors

Table 1 Conditions causing secondary hyperglycaemia in neonates

A)

Both TNDM and PNDM have been reported from all continents affecting all races [17, 25, 30, 33, 102]. Neonatal
diabetes is rare. The incidence in Sweden has been estimated to be in the order of 2-3 cases per 1 million births

B)

C)

[81].
In 40% of PNDM and in 31% of TNDM a positive
family history for diabetes was observed, but few reports
distinguish between type I and type II diabetes or state
the precise family relationship of the affected relative.
Twenty-six percent of PNDM and 28% of TNDM patients
had an affected sib, usually presenting the same type. Two
pairs of cousins [43, 68], however, were dissimilar, one
with PNDM and the other with TNDM. Maternal diabetes
during pregnancy is reported only occasionally [38, 57,
97].
HLA typing was positive for the DR3 or DR4 haplotype in four of ten cases of transient diabetes [7, 10, 25,
62, 71, 80, 96] and in four of ten cases of permanent diabetes [5, 40, 42, 45, 51, 81, 83, 100]. The "protective"
type of DR2 or B7 was also found [5, 7, 81].

Pathogenesis and etiology

Neonatal diabetes is caused by insulinopenia, resulting
from a poor response of the pancreatic [~-cell to hyperglycaemia and other stimuli [31, 41, 71, 83, 94]. Glucosuria,
polyuria and dehydration develop as in older patients, but
acidosis and ketonuria are not as severe. This has been related to "immature metabolic pathways" [ 16].
The ultimate cause of the malfunction of the [3-cell remains unclear. A number of hypotheses have been postulated, many of them with little scientific proof: cerebral
malfunction [4, 72], maternal hypoglycaemia [32], maternal hyperglycaemia [98], adrenocortical disturbance [26,
48, 50], and temporary immaturity of the hepatic systems
[28]. Some publications relate early postnatal hypoglycaemia to neonatal diabetes [4, 13]. However, small-fordate babies very often suffer from hypoglycaemia without
developing neonatal diabetes [16, 24], and more than 60%
of the cases of neonatal diabetes did not show hypoglycaemia despite their low birth weight.
The commonly accepted auto-immunity theory of typical juvenile IDDM cannot be applied to neonatal diabetes, because neither anti- islet antibodies not the typical
HLA types have been found and the intra-uterine period is
too short for the development of the auto-immune process
of IDDM [9].
Some authors refer to delayed [3-cell maturation [27,
71, 73, 75, 83] without conclusive answers for the cause
of delayed maturation.

D)

Metabolic disorders:
methylmalonic aciduria
alaninuria
Infusions of lipids or glucose:
especially in very small infants
Stress-induced hyperglycaemia:
surgical procedures, anaesthesia, sepsis, respiratory
distress, hypoxia, cerebral haemorrhage
Drugs:
Corticosteroids, coffein, theophyllin

Clinical observations, diagnosis

and differential diagnosis
Most children have a low or very low birth weight for gestational age, some full-term infants weighing less than 1.5
kg [5, 7, 13, 59, 81, 91]. The average birth weight and the
rate of prematurity are identical in TNDM and PNDM.
About 75% of either cases show their first symptoms within
10 days of birth, presenting with abnormal weight loss,
polyuria and dehydration. Glycosuria and hyperglycaemia
(often 600-1000 mg%) confirm the diagnosis. Levels of
C-peptide or plasma insulin are uniformly low in both
TNDM or PNDM [11, 12, 19, 22, 31, 40, 45, 56, 73], later
on an increase in C-peptide levels heralds the end of the
disturbance in transient cases [6, 39, 73]. No anti-islet
antibodies or anti-insulin antibodies have ever been detected [10, 33, 49, 73, 86]. Initial acidosis and ketonuria
rarely occur in TNDM, but mild degrees are quite common in PNDM.
Exocrine pancreatic dysfunction (steatorrhea, diminished production of bicarbonate and chymotrypsin) has
repeatedly been described in PNDM [11, 56, 62, 99, 100].
It has also been observed in cases of permanent diabetes
developing several years after TNDM [25].
Differential diagnosis of neonatal diabetes includes iatrogenic or stress-induced hyperglycaemia as well as inborn errors of aminoacid or organic acid metabolism [34,
65, 67, 76, 85, 88, 104]. A list of conditions associated
with symptomatic hyperglycaemia is presented in Table 1.

Treatment and outcome

With few exceptions [69, 81], neonatal diabetes has to be
treated with insulin. Attempts with oral antidiabetic agents
were usually unsuccessful [73, 75, 86]. Continuous infusions [41, 86, 93], repeated injections of short-acting insulin [8, 28, 39, 55, 61, 87], and intermediate or long-acting preparations [12, 14, 52, 81, 95] have been used with
comparable results. Usually a total daily dose of 0.2-1.0
IU/kg is sufficient to establish normoglycaemia. These
patients are very sensitive to insulin and therefore in dan-

946

Table 2 Comparison between cases of TNDM and PNDM

Total number
Male/female
Rate of prematurity
Small for gestational age
Average birth weight (kg)
Onset before 10 days
Postnatal hypoglycaemia
Initial acidosis
Ketonuria*
Positive family history
Affected sibling
HLA DR3 or DR4 positive

PNDM

TNDM

29
16/8
9%
94%
2.06 + 0.22
79%
22%
60%
54%
40%
3 1%
4/10

65
26/31 ~
10%
81%
2.14 + 0.31
70%
40%
34%
20%
31%
28%
4/10

aSome reports do not state the sex of the patient

* Statistical power of the differences was tested by chi square test
or t-test; only "ketonuria" proved to be significant (P smaller
0.025), all other items did not show a significant difference

ger of severe hypoglycaemic events. Mental retardation

and/or spasticity developed in some patients and was attributed to sustained hypoglycaemia events [15, 48, 61].
Frequent blood glucose monitoring is essential and mild
hyperglycaemic values can be tolerated. In severely dehydrated infants, water and electrolyte imbalances should be
corrected with isotonic electrolyte or glucose/electrolyte
solutions over a period of 2 4 - 4 8 h avoiding too rapid rehydration with its danger of cerebral oedema [ 1, 31, 41 ].
Oral feeding m a y be instituted as soon as possible. After
initiation o f insulin therapy, growth usually accelerates
and body weight increases [4, 6, 64, 83, 95].
T N D M usually has to be treated for 2 - 1 6 weeks and
only rarely for more than a year. However, several patients continued to show an impaired glucose tolerance
[33, 43, 98] and 13 patients developed permanent diabetes
5 - 2 2 years later [7, 14, 25, 33, 81, 84, 94, 96]. One of
them was non-insulin dependent [7].

Nosological aspects
T N D M and P N D M share m a n y features (Table 2) and
may be different manifestations of the same entity. In contrast, neonatal diabetes mellitus differs from I D D M in
many aspects raising the possibility of separate nosologi-

cal, i.e. aetiopathogenetic entities. Features separating

both forms o f neonatal diabetes from type 1 diabetes mellitus in older children include the following:
1. Patients are negative for anti-islet antibodies.
2. Usually they do not express the typical HLA-antigens
DR3/DR4.
3. Late complications such as nephropathy or retinopathy
have not been reported, even after more than 20 years
of treatment [5, 23].
4. There are often signs of exocrine pancreatic insufficiency.
To conclude, there are no clinical or laboratory differences between cases of P N D M and T N D M . We therefore
put forward the hypothesis that T N D M and P N D M are
not two distict entities, but are both caused by prenatal
pancreatic damage affecting endocrine, and sometimes
also exocrine functions. More severe cases m a y result in
permanent insulin dependence, occasionally after a transient partial recovery. The less severe cases result in "transient" neonatal diabetes, but m a n y continue to have impaired glucose tolerance and m a y later again b e c o m e insulin dependent. They then resemble P N D M in all aspects.
Heredity, possibly autosomal recessive, m a y play a
certain role, because about 25% of neonatal cases have an
affected sibling, with boys and girls equally affected.

Case report
The following patient illustrates the typical features of TNDM.
The girl was delivered at term by caesarean section weighing 2960
g. Two days later she was transfered to our paediatric unit because
of loss of weight, feeding difficulty, muscular hypotonia and dehydration. Laboratory tests revealed hyperglycaemia of 626 mg/dl,
glucosuria of 1000 mg/dl, moderate metabolic acidosis and ketonuria. Electrolyte studies and blood counts were normal. Islet
cell antibodies were negative, C-peptide levels initially low,
reached normal values at about 6 weeks. Results of tests for exocrine pancreatic function and analysis for other metabolic disorders gave normal results.
She was rehydrated and treated with continuous intravenous insulin, which was later changed to multiple subcutaneous injections. She required about 1 IU/kg body weight/day, being fed normal formula milk 6-8 times daily. After 10 weeks the insulin requirement decreased and after 90 days treatment could be stopped.
Meanwhile the girl is 3 years old with normal psychomotor development and normal glucose tolerance.

947

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