As Mark J Peters comments in his letter,we know that
hyperleukocytosis has been postulated as a factor for pulmonary
hypertension in Pertussis infection , but shortness of letter exposition
does not make it possible to report. Nevertheless,our patient never
reached these values of leucocytosis;it's possible, as in many other
diseases, that several pathogenic mechanisms contribute to pulmonary
hypertens...
As Mark J Peters comments in his letter,we know that
hyperleukocytosis has been postulated as a factor for pulmonary
hypertension in Pertussis infection , but shortness of letter exposition
does not make it possible to report. Nevertheless,our patient never
reached these values of leucocytosis;it's possible, as in many other
diseases, that several pathogenic mechanisms contribute to pulmonary
hypertension, making a concomitant treatment approach necessary.
As the professor Milton Diamond wrote it, words can wound, there are
cases where they can even kill.
As a counsellor, my reference is always the experience of my clients
and it is a personal, an ethical and a professional goal to understand
them as they understand themselves.
The intersexed people I know are all telling me that they got deeply
destructive messages during their c...
As the professor Milton Diamond wrote it, words can wound, there are
cases where they can even kill.
As a counsellor, my reference is always the experience of my clients
and it is a personal, an ethical and a professional goal to understand
them as they understand themselves.
The intersexed people I know are all telling me that they got deeply
destructive messages during their childhood. Instad of being welcomed,
respected and, if needed reassured, they got endless silence, lies and
manipulation. This behavior gave them the message that they were
dsysfunctional, inherently bogus and without value. This left deep wounds
in them, and it takes them years, sometimes decades to recuperate.
Regardless of their gender identity and their sexual orientation, the
ones who recover are deeply convinced of one thing: their body is not
disordered and they are not disordered. It is in no way a mistake, a bad
thing, or a disorder to be born intersexed.
We are currently seeing intersexed people recovering from the abuse
they suffered, taking power over their lives, meeting each others and
organizing themselves through various organisations, like the Organisation
Intersex International. In other words, they are freeing themselves of the
"disorder" model and they are freeing themselves of the people who want to
normalize them.
And suddenly, appears a medical group with a new name for intersexed
people. They are no more intersexed, now they are "disorders of sex
developpment". Beyond the fact that I have serious questions about the
real motivations of the people who created this new expression, I am
deeply convinced that this term will strongly hurt a large number of
children, who will receive a reinforced message according to which they
are inherently dysfunctionnal and bogus. And once again, they will take
years, sometimes decades to recover. I cannot consider this an improvement
and I cannot consider this as a respectful way to treat human beings.
We read with interest the article by Inward and Chambers proposing a
review on the current fluid management of DKA in the United Kingdom.[1] The
authors correctly state the importance of avoiding “rapid rates of fluid
infusion” and the need to “maintain serum sodium concentrations” in order
to reduce the administration of free water and hence reduce the risk of
cerebral oedema.
We read with interest the article by Inward and Chambers proposing a
review on the current fluid management of DKA in the United Kingdom.[1] The
authors correctly state the importance of avoiding “rapid rates of fluid
infusion” and the need to “maintain serum sodium concentrations” in order
to reduce the administration of free water and hence reduce the risk of
cerebral oedema.
It has been our experience over the last few years that children with
severe DKA admitted to our paediatric intensive care unit have, as
suggested by Inward and Chambers, received excessive amounts of
intravenous fluid, a factor which may potentiate the development of
cerebral oedema. Over the last 2 years, ten patients with severe DKA
(median pH 6.9, median pCO2 1.8 kPa) were admitted our PICU of which four
developed cerebral oedema. All four patients received between 50 and 85
ml/kg resuscitation within the first 5 hours of treatment. Two of the
patients with signs of brainstem herniation were receiving 0.45 % saline
maintenance fluid therapy. No patient had a corrected sodium value
recorded at any stage.
These excessive volumes of fluid are placing these children at an
unnecessary risk for brainstem herniation for the following reasons.
First, the initial fluid resuscitation volumes (first four hours) often
exceed the “50 ml/kg” threshold value identified by Mahoney as an
important risk factor for cerebral oedema.[2] As hypotension in DKA is both
unusual and uncommon, the indication for fluid boluses is often poor
capillary refill or failure of rapid improvement in acidosis. Only one of
the ten patients admitted to our PICU had hypotension which was
effectively treated with inotropes. Adopting the “maintenance plus
rehydration” strategy currently advocated by the British Society for
Paediatric Endocrinology,[3] in addition to the large volumes of
resuscitation fluid, commonly leads to the administration of fluid volumes
above the threshold value proposed by Duck of 4 L/m2/day as a risk factor
for cerebral oedema as dehydration is often corrected within 24 hours.[4]
In addition, the administration of free water in the form of 0.45 % saline
as maintenance fluid (half of which will distribute to the tissues as
electrolyte free water) may potentiate hyponatraemia, a
known risk factor
in the development of cerebral oedema.
Prevention of rapid osmotic shifts in DKA and the defence of plasma
tonicity are important goals to minimise the risk of cerebral oedema.
Hyponatraemia or a fall in the glucose corrected plasma sodium are
indicators of excessive free water retention and in the DKA patient with a
depressed level of consciousness, mandates treatment. Mannitol, although
effective at reducing cerebral oedema, may cause a large osmotic diuresis
that may complicate the fluid rehydration regime without correcting the
plasma sodium. In contrast to mannitol, hypertonic saline will increase
the plasma sodium (and hence tonicity) and simultaneously expand the
plasma volume which is itself an important target in maintaining adequate
cerebral perfusion pressure.[5] Hypertonic saline may also be required for
the treatment of cerebral salt wasting which has been proposed as a
mechanism of hyponatraemia in patients with DKA. It is our experience that
the use of 3 % saline in patients with DKA is increasing as clinicians
continue to prescribe large amounts of fluids for both initial fluid
resuscitation and ongoing maintenance which is often 0.45 % saline.
Although the final solution on how and why cerebral oedema develops
in DKA remains unsolved, a number of factors concerning current fluid
management place these children at unnecessary risk. We strongly support
the proposal of Inward and Chambers for review of the current guidelines
and as Intensive Care Specialists would especially like to see and be
involved in the development of guidelines for the ongoing management of
patients unfortunate enough to develop the most catastrophic complication
of DKA, cerebral oedema.
References
(1) CD Inward, TL Chambers, J Edge. Fluid management in diabetic
ketoacidosis. Arch Dis Child 2002;86:443-4.
(2) Mahoney CP, Vleck BW, DelAguila M. Risk factors for developing
brain herniation during diabetic ketoacidosis. Pedaitr Neurol 1999;21:721-7.
(3) British Society of Paediatric Endocrinology and Diabetis,
Recommended DKA guidelines. http://bspe.shef.ac.uk/dka.html, 2001.
(4) Duck SC, Wyatt DT. Factors associated with brain herniation in the
treatment of diabetic ketoacidosis. J Paediatrics 1988;113:10-4.
(5) Curtis JR, Bohn D, Daneman D. Use of hypertonic saline in the
treatment of cerebral edema in diabetic ketoacidosis Pediatric Diabetes
2001; 2:191-4.
Complications associated with the bacille Calmette-Guerin vaccination
We read with interest your article (July 2006) on complications
associated with BCG vaccination. We are treating a male infant who has
developed a similar severe suppurative adenopathy following BCG. He had
evidence of dissemination with high fevers, an unusual rash (Fig 1) and
hepatosplenomegaly with multiple hypoechoic hep...
Complications associated with the bacille Calmette-Guerin vaccination
We read with interest your article (July 2006) on complications
associated with BCG vaccination. We are treating a male infant who has
developed a similar severe suppurative adenopathy following BCG. He had
evidence of dissemination with high fevers, an unusual rash (Fig 1) and
hepatosplenomegaly with multiple hypoechoic hepatosplenic lesions on
ultrasound. Microbiological samples from the suppurative lesion and bone
marrow demonstrated acid fast bacilli and M. bovis BCG was isolated after
3 weeks of culture. Treatment was commenced with rifampacin, isoniazid,
ethambutol, pyridoxine and amikacin. Routine immunological investigations
were normal, as in your case.
Recently the essential role of the TH1-type cytokine pathway in the
containment of mycobacteria has been described in a number of
publications. Several deleterious mutations as well as partial defects in
genes that encode interleukin 12 (IL12) and interferon gamma (IFNƒ×ƒw or
their receptors, have been identified (Newport et al., 1996;Picard et al.,
2002;Dorman et al., 2004).
We investigated the TH1-type cytokine pathway in this patient by ex-
vivo stimulation experiments of whole blood, as previously described by
our group. In these assays, our patient showed decreased production of
IL12, indicating a potential defect in this gene. The genetic analysis to
identify the exact mutation in this patient is currently awaited.
His systemic signs resolved with quadruple antimycobacterial therapy,
but the axillary lesion progressed (Fig 2). We therefore added
subcutaneous injections of IFN£^ x3/week to the anti mycobacterial
regimen, substituting azithromycin and ciprofloxacin for amikacin. This
baby is now 6 months old, generally well and thriving. The IF- £^ dose has
been escalated to the point where he has mild post-dose fevers, at
175mcg/m2. The skin lesion remains prominent with a central 4 x 3 cm area
of granulation tissue, but the surrounding erythema and induration and the
associated swelling of his left arm have significantly decreased.
We agree with the authors that we appear to be seeing more
complications of BCG vaccinations and that surveillance should be
instituted nationally. Treatment of BCG suppurative adenitis is
controversial (Goraya and Virdi, 2001). Where available, TH1-type cytokine
assays should be carried out in those with severe suppurative adenitis, as
adjuvant therapy with IFN£^ may be beneficial in the presence of host
defense defects.
KJ Fidler1, SS Struik1, H Lyall1, S.Walters1, G Tudor-Williams1,2, B
Kampmann1,2
1 Dept of Paediatric Infectious Diseases, St Mary¡¦s Hospital,
Paddington, London, UK
2 Dept of Paediatric Infectious Diseases, Imperial College, London UK
Conflict of interest; None
References:
1. Dorman, S.E., C.Picard, D.Lammas, K.Heyne, J.T.van Dissel,
R.Baretto, S.D.Rosenzweig, M.Newport, M.Levin, J.Roesler, D.Kumararatne,
J.L.Casanova, and S.M.Holland. 2004. Clinical features of dominant and
recessive interferon gamma receptor 1 deficiencies. Lancet 364:2113-2121.
2. Goraya, J.S. and V.S.Virdi. 2001. Treatment of Calmette-Guerin
bacillus adenitis: a metaanalysis. Pediatr. Infect. Dis. J. 20:632-634.
3. Newport, M.J., C.M.Huxley, S.Huston, C.M.Hawrylowicz, B.A.Oostra,
R.Williamson, and M.Levin. 1996. A mutation in the interferon-gamma-
receptor gene and susceptibility to mycobacterial infection. N. Engl. J.
Med. 335:1941-1949.
4. Picard, C., C.Fieschi, F.Altare, S.Al Jumaah, S.Al Hajjar,
J.Feinberg, S.Dupuis, C.Soudais, I.Z.Al Mohsen, E.Genin, D.Lammas,
D.S.Kumararatne, T.Leclerc, A.Rafii, H.Frayha, B.Murugasu, L.B.Wah,
R.Sinniah, M.Loubser, E.Okamoto, A.Al Ghonaium, H.Tufenkeji, L.Abel, and
J.L.Casanova. 2002. Inherited interleukin-12 deficiency: IL12B genotype
and clinical phenotype of 13 patients from six kindreds. Am. J. Hum.
Genet. 70:336-348.
Figure 1: Patient at presentation showing the rash and small non supparative axillary lymph node.
Figure 2: Patient 3 months after presentation, on anti- BCG therapy.
Debating the use of PSGs in diagnosing childhood sleep-related
breathing disorders (SBD) seems pointless when physicians lack the
clinical skills necessary to suspect the disease is present. My
impression is that a mass screening of all "normal" children using PSGs
would show the vast majority of children with SBD are viewed as normal
sleepers by clinicians who fail to ask about sleep or fail to diagn...
Debating the use of PSGs in diagnosing childhood sleep-related
breathing disorders (SBD) seems pointless when physicians lack the
clinical skills necessary to suspect the disease is present. My
impression is that a mass screening of all "normal" children using PSGs
would show the vast majority of children with SBD are viewed as normal
sleepers by clinicians who fail to ask about sleep or fail to diagnose
anything but behavioral sleep problems. Until physicians wake-up to the
fact "behavioral" sleep problems shouldn't be diagnosed until subtle
airway abnormalities leading to SBD have been excluded, these children
will go undiagnosed, and the last people to know about it will be the
sleep medicine specialists. If pediatricians believe all children who
sleep poorly have behavioral problems, which it seems they do, then the
likelihood of diagnosing a medically treatable sleep disorder is almost
nil. If these children see any specialists, it's the psychologists, not
the sleep doctors, who then mismanage them, and no one gets a sleep study
- worth doing or not.
Debate doing the diagnostic tests as long as you like. The reality is
that doctors lack the clinical skills needed to know the test should be
considered, so necessary or not, it doesn't get done.
Inward and Chambers provide a provocative description and discussion
of the continued confusion regarding the issues surrounding rehydration
and treatment of the pediatric patient with DKA.[1] They review some of the
key issues that link fluid therapy to complications from brain swelling,
and question the appropriateness of using a volume of fluid calculated by 'maintenance plus deficit', calling for...
Inward and Chambers provide a provocative description and discussion
of the continued confusion regarding the issues surrounding rehydration
and treatment of the pediatric patient with DKA.[1] They review some of the
key issues that link fluid therapy to complications from brain swelling,
and question the appropriateness of using a volume of fluid calculated by 'maintenance plus deficit', calling for a second revolution in the
management of DKA. In the accompanying commentary, Edge makes several
statements concerning fluid therapy in DKA, including that 'DKA is
associated with severe fluid losses', that 'any guidelines for fluid and
electrolyte management must be simple to calculate', that administration
of base is a risk factor for intracranial complications, and that despite
published data and 'changes in protocols', there is no evidence that the 'incidence of cerebral oedema has changed over the past 20 years'.[1] It is
our opinion that the problem in the rehydration of the pediatric patient
with DKA does not lie in assigning a maintenance fluid allotment. Rather,
the source of error lies largely with failure to accurately estimate the
volume of deficit and the tendency to automatically assume a severe degree
of dehydration. From our experience with over 450 consecutive episodes of
moderate and severe DKA, and our weight gain data,[2] severe DKA (i.e.
severe ketoacidemia) does not necessarily mean severe dehydration; the
converse is also true.[2,3] The degree of dehydration ranges from
negligible (<_1 _="_" to="to" extreme="extreme"/>20 %).[3] Severe ketoacidemia,
however, does cause vasoconstriction which may be manifested peripherally
by cool, mottled skin, and Kussmaul breathing which leads to very dry oral
mucosa. The striking appearance of a parched mouth and the presence of
cool, even mottled skin without a critical assessment of vital signs and
examination of distal (foot) pulses often results in an erroneous
impression of shock and 'severe dehydration':[3] A method for estimation of
the volume of deficit was described in 1990 [2] and we continue to use this
approach successfully. Successful therapy requires not only gradual
deficit replacement (evenly over 48 hours) but an accurate estimation of
the volume of deficit along with critical monitoring of the clinical and
biochemical response.[3] If the deficit is assumed to be 10-15 % but is
actually only 3 %, that patient will receive excess water independent of
the more gradual timeframe and independent of the type of fluid given.
Guidelines that have proposed 'safe' limits to fluid volumes administered
such as 4 liters/m2/day [4] or 50 ml/kg body wt./4 hrs. [5] violate the
concept of the individualised assessment of the degree of dehydration and
will invariably overhydrate the mild to moderately dehydrated child; the
problem is compounded when actual body weight is used instead of ideal
body weight in fluid calculations for the obese patient. On the other
hand, certain patients, particularly those with complicating illness e.g.
septic shock, pancreatitis, may require more than 20 ml/kg of fluid
resuscitation in the first treatment hour and more than 50 ml/kg in the
first four hours. Setting arbitrary fluid volume limits per hour or per
day endanger particularly those patients at the mild and severe end of the
dehydration spectrum. Although the insult would be greater with hypotonic
fluid, overhydration occurs readily with isotonic fluid as well when water
requirements are overestimated.
DKA represents the effects of a complex disruption of normal
metabolism, which leads to metabolic death if left untreated. Shock
(decreased peripheral pulses, with or without hypotension), if present,
should be corrected rapidly. Insulin should be given preferably by
continuous, low dose, intravenous infusion, as soon as possible to begin
correction of ketoacidemia/ketoacidosis. Regardless of the serum
concentration of glucose, insulin is required to inhibit the hepatic fatty
acyl carnitine cycle leading to ketoacid formation.[6] A delay in insulin
administration only serves to enhance and prolong ketoacidemia thereby
extending the period of time during which the patient remains vulnerable
to central nervous system and other complications.
Our proposed management strategy may not satisfy the call for
simplicity but it is an easily learned approach. It requires an
understanding of relevant, known pathophysiology, the monitoring of serial
physical examinations and laboratory studies with special attention to
correction of acidemia and osmolality, and the anticipatory care that is
inherent in the care of the critically ill.[2,3,6-10,12]
Physiologic management was first described between 1988 [10] and 1990,[2] and set forth with additional detail and data in 1994. [3] It is rarely
described in its complete form when referenced in texts; mere portions of
our recommendations do not constitute what we have called physiologic
management. Not only is it unlikely that large numbers of patients
outside our own institution have been managed using our guidelines in
their entirety, but the recommendations simply are not old enough to be
reflected in data over the past 20 years. We suspect that physiologic
management is significantly underrepresented in the multicenter studies
conducted thus far, all of which compare variations of traditional therapy
(empiric volume resuscitation whether or not shock is present, assumption
of a large volume of deficit, planned rehydration in less than 48 hours
with either 0.45 % or 0.9 % NaCl, with or without urinary output
replacement). In a retrospective portion of our study in 1990 [2] we
compared these same therapies and also found that no form of traditional
therapy minimized the risk of brain herniation during treatment.
Comments regarding the administration of base should be better
defined. Rapid administration or 'pushes' of hypertonic sodium
bicarbonate should not be given. On the other hand, there is no evidence
that administration of physiologic concentrations of base in the
rehydration solution are either harmful or undesirable. In our
experience, this practice mitigates the development of hyperchloremic
acidosis during treatment.
Since ours is a referral center, most of our patients have some form
of therapy initiated in outlying hospitals, sometimes in keeping with our
recommended approach, and sometimes with our recommendations instituted
only after initial telephone contact. In this setting, we have managed
certain patients with severe DKA who received resuscitation fluids in
excess of what their physical examination and laboratory data would
dictate. It is not unusual for such patients to require as little as a
typical maintenance allotment (without a deficit replacement component)
for the remainder of therapy; some patients required fluid restriction to
as little as two-thirds the usual maintenance volume.
Our approach has been criticized because of the incidence of mannitol
administration in our series. [1] In our mannitol recipients, several of
whom did not receive their initial management by us, there was no central
nervous system morbidity or mortality. In another large series of
patients there was a 50 % failure rate of mannitol to reverse a
deteriorating neurologic status even when mannitol was given prior to
respiratory arrest, with a near 100 % failure rate when mannitol was given
after respiratory arrest. [11] It is possible that not all of our mannitol
recipients actually had raised intracranial pressure. We believe,
however, that the key to our good outcome is that the fluid and
electrolyte therapy on which mannitol is superimposed is relevant to its
success. It is erroneous to assume that the 100 % success rate among our
mannitol recipients would be reproducible in the setting of a therapy that
violates the fundamental principles of rehydrating the hypertonic state of
DKA.
Drs Inward and Chambers ask 'do we have it right yet?' and convey
concern that certain recommendations do not, as of yet, 'have it right.'
We agree.
Our work regarding the management of the pediatric patient in
moderate to severe DKA has spanned 14 years [1,12] and nearly 500
consecutive prospectively managed episodes. We remain available to
participate in any endeavor to continue to improve the care of the
pediatric patient in DKA.
References
(1) Inward CD, TL Chambers, Edge J. Fluid management in
diabetic ketoacidosis. Arch Dis Child2002;86(6):443-5.
(2) Harris GD, et al.: Minimizing the risk of brain
herniation during treatment of diabetic ketoacidemia:
a retrospective and prospective study. J Pediatr 1990;117:22-4.
(3) Harris GD, Fiordalisi I. Physiologic management of
diabetic ketoacidemia. A 5 year prospective pediatric experience in 231 episodes. Arch Pediatr Adolesc Med 1994;148:1046-52.
(4) Duck SC, Wyatt DT. Factors associated with brain herniation in the treatment of diabetic ketoacidosis.
J Pediatr 1988;113:10-4.
(5) Mahoney CP, Vleck BW, DelAguila M. Risk factors for
developing brain herniation during diabetic ketoacidosis. Pediatr Neurol 1999;21:721-7.
(6) Fiordalisi I, Harris GD. Diabetic Ketoacidemia. In: Finberg L, Kleinman RE (Eds) Saunders Manual of
Pediatric Practice, 2nd ed. Philadelphia: WB Saunders Co., 938-46.
(7) Harris GD, Fiordalisi I, Yu C. Maintaining normal
intracranial pressure in a rabbit model during treatment of severe diabetic ketoacidemia. Life Sci 1996;59:1695-702.
(8) Harris GD, Lohr JW, Fiordalisi I, Acara M. Brain
osmoregulation during extreme and moderate dehydration in a rat model of severe DKA. Life Sci 1993;53:185-91.
(9) Bohn D, Daneman D. Diabetic ketoacidosis and
cerebral edema. Curr Opin Pediatr 2002;14(3):287-91.
(10) Harris GD, Fiordalisi I, Finberg L. Safe management
of diabetic ketoacidemia. J Pediatr 1988;13:65-7.
(11) Rosenbloom A. Intracranial crises during treatment
of diabetic ketoacidosis. Diabetes Care 1990;13:22-33.
(12) Fiordalisi I, Harris GD, Gilliland MGF. Prehospital
cardiac arrest in diabetic ketoacidemia. Why brain swelling may lead to death before treatment. J Diabet Compl 2002;16:214-9.
We just read the short report of Galanakis et al.[1]
We have been involving in periodic fevers management for many years.
PFAPA is not the only inherited cause of periodic fever, known at present.
It is an unclear nosological entity.
Pharyngitis, cervical lymphoadenopathies and oral aphthae are not
pathognomonic for PFAPA.
Among periodic fevers, cervical lymphoadenopathies and episodic fever can...
We just read the short report of Galanakis et al.[1]
We have been involving in periodic fevers management for many years.
PFAPA is not the only inherited cause of periodic fever, known at present.
It is an unclear nosological entity.
Pharyngitis, cervical lymphoadenopathies and oral aphthae are not
pathognomonic for PFAPA.
Among periodic fevers, cervical lymphoadenopathies and episodic fever can
occur in patients with HyperIg D and periodic syndrome (HIDS), oral
aphthaeare a minor sign and isolated fever can be a frequent finding
in children affected by Familial Mediterranean Fever (FMF); lastly,
oral aphthae and recurrent febrile attacks characterise the onset of
Behcet disease (BD) in children. The efficacy of steroids does not confirm the diagnosis of PFAPA; Behcet disease is responsive to steroids,
too. The lack of familiar involvement is not a
criteria to exclude an inherited disorder, as FMF and HIDS are recessive
and BD is a multifactorial disease. Furthermore, the initial clinical
picture of these disorders can be atypical and incomplete and can change
during the clinical course.
So, considering the provenance of this series (Greece), we would
wonder if some
cases had BD and FMF, that will be recognised in the future.
Nowadays, with increased diagnostic sensitivity and multiethnic
societes, periodic fevers are being recognised outside their traditional area of incidence. Close follow-up is essential in further years, especially in patients
with incomplete picture of PFAPA. A possible genetic screening for gene
causing FMF, HIDS or immunological assay for HLA B51 could also be useful.
Reference
[1] Galanakis E, Papadakis CE, Giannoussi E, Karatzanis AD, Bitsori M, Helidonis ES. PFAPA syndrome in children evaluated for tonsillectomy. Arch Dis Child 2002;86:434-5.
We were interested to read the letter by Roberts-Harewood
and Davies.[1] We and other paediatricians [2] have been aware for a few years
of cases of pica in children with sickle cell disease (SCD). Parents do
not volunteer this embarrassing information but when asked "does your
child eat anything unusual?" will relate their child's specific penchant.
They are relieved to know that this is not unusual...
We were interested to read the letter by Roberts-Harewood
and Davies.[1] We and other paediatricians [2] have been aware for a few years
of cases of pica in children with sickle cell disease (SCD). Parents do
not volunteer this embarrassing information but when asked "does your
child eat anything unusual?" will relate their child's specific penchant.
They are relieved to know that this is not unusual in SCD and that the
children usually grow out of the habit. Little is known about the
aetiology of this unusual behaviour, but, as Harewood-Roberts and Davies
point out, it has been associated with developmental delay and brain
damage. Pica might be related to silent infarction which occurs in up to
25% of children with SCD,[3,4] often in the frontal lobes and associated
with cognitive deficits [5] and therefore potentially with unusual behaviour.
We have conducted a preliminary prospective survey to see how
commonly pica occurs in sickle cell disease in childhood. We have also
looked at any possible association with iron deficiency or MRI evidence of
brain damage.
Methods: In children attending the Paediatric Haematology clinic with
any type of sickle cell disease, cases of pica were identified by asking
routinely of every one who attended whether the child was eating unusual
things. Haemoglobin (Hb) concentration and ferritin levels were measured.
Ferritin levels assayed when the child was unwell were not used. Children
with sickle cell anaemia (HbSS) were compared with less severe forms of
sickle cell disease (HbSC, HbS/bthalassaemia) and population controls.
Controls were children attending the hospital, who needed a venepuncture
and who were not suffering from a febrile illness nor were known to be
suffering from a psychiatric disorder. Children in whom there were
concerns over possible neurological or developmental problems (stroke,
transient ischaemic attack, seizure or school problems) were offered MRI
and MRA.
Results:
Sickle cell anaemia (HbSS)
Other Sickle cell disease (HbSC or HbSB)
Controls
P for difference
Pica
No pica
21 (54%)
18 (46%)
3 (27%)
8 (73%)
14 (18%)
84 (82%)
*0.008
**1.0
***0.01
Hb (g/dl) median (range)
Pica
No pica
8.1 (6.0-10.8)
8.5 (5.7-12.7)
9.5 (9.3-11.2)
11.2(10-12.4)
12.5 (8.6-15.1)
11.9 (7.8-13)
****>0.05
Ferritin (ng/ml)
Median (range)
Pica
No pica
55 (21-120)
42 (24-54)
50 (37-77)
32 (22-38)
28 (4-72)
28 (2-84)
****>0.05
* Fisher's exact test for sickle cell anaemia compared with control
** Fisher's exact test for sickle cell disease (other) compared with
control
*** Fisher's exact test for sickle cell anaemia compared with sickle cell
disease (other)
**** Mann-Witney U-test for comparison between pica and no pica in each
group
Seventeen of the children with sickle cell disease had MRI scans, 7
with pica and 10 without.
Four children had infarcts on MRI , one with pica and three without
(Fisher's exact test, p=0.6). Six children had cerebrovascular disease on
MRA, 3 with pica and 3 without (Fisher's exact test, p=0.6).
Conclusions: Pica is common in childhood sickle cell disease and is
not obviously associated with iron deficiency. Although haemoglobin was
lower in those with pica, this was not statistically significant; a larger
American study did find an association.[2] Pica was discovered in over half
the children with sickle cell anaemia, a higher proportion than the
American study,[2] which might reflect differences in patient population in
terms, for example, of age, cultural background or nutritional status.
There was no evidence for an association with cerebral infarction or
cerebrovascular disease in the small number of symptomatic children
studied. The possibility of an association with sleep disorders6 will be
investigated in our randomised controlled trial of oxygen supplementation,
funded by the Stroke Association. Other haematological and
neurodevelopmental factors that might account for this phenomenon should
also be investigated, as many parents find it distressing and it might be
a marker for more pervasive cognitive or behavioural problems that could
be effectively treated or prevented.
M A Rossiter
MAS Ahmed
A Yardumian
Departments of Paediatrics and Haematology
North Middlesex University Hospital NHS Trust, London N18 1QX
F Kirkham
Neurosciences Unit
Institute of Child Health (University College London), London WC1N 1EH
References
(1) Roberts-Harewood M and Davies SC Pica in sickle cell disease: "She ate
the headboard". Arch Dis Child 2001;85:510-11.
(2) Ivascu NS, Sarnaik S, McCrae J, Whitten-Shurney W, Thomas R, Bond
S. Characterization of pica prevalence among patients with sickle cell
disease. Arch Pediatr Adolesc Med 2001 Nov;155:1243-7.
(3) Miller ST, Macklin EA, Pegelow CH, Kinney TR, Sleeper LA, Bello
JA, DeWitt LD, Gallagher DM, Guarini L, Moser FG, Ohene-Frempong K,
Sanchez N, Vichinsky EP, Wang WC, Wethers DL, Younkin DP, Zimmerman RA,
DeBaun MR. Silent infarction as a risk factor for overt stroke in children
with sickle cell anemia: a report from the Cooperative Study of Sickle
Cell Disease. J Pediatr 2001;139:385-90.
(4) Saunders DE, Bynevelt M, Hewes DKM, Cox TC, Chong WK, Evans JP,
Kirkham FJ. MRI in children with sickle cell disease without overt stroke. Dev Med Child Neurol 2001;43 suppl 90:27.
(5) Watkins KE, Hewes DKM, Connelly A, Kendall BE, Kingsley DPE, Evans
JPM, Gadian DG, Vargha-Khadem F, Kirkham FJ. Cognitive deficits associated
with frontal lobe infarction in children with sickle cell disease. Dev Med
Child Neurol 1998;40:536-43.
(6) Stein MA, Mendelsohn J, Obermeyer WH, Amromin J, Benca R. Sleep
and behavior problems in school-aged children. Pediatrics 2001;107:E60.
The original article gives a message of caution in the use of Renal
Ultrasound as a preliminary test in deciding on a V(M)CUG test to diagnose
VUR. The study looked at 2 definite investigations carried out in all
children under the age of 1, however the RCPCH (UK) guidelines recommend
DMSA, Renal U/S & MCUG in all children with a proven UTI under the age
of 1.
The original article gives a message of caution in the use of Renal
Ultrasound as a preliminary test in deciding on a V(M)CUG test to diagnose
VUR. The study looked at 2 definite investigations carried out in all
children under the age of 1, however the RCPCH (UK) guidelines recommend
DMSA, Renal U/S & MCUG in all children with a proven UTI under the age
of 1.
The RCPCH (UK)guidelines for children between 1 and 7 years of age
are to carry out Renal U/S, DMSA scan, Abdominal Xray & MCUG if
either DMSA or Renal U/S are abnormal(or if there is positive family
history/recurrent UTI/Pyelonephritis).
The age group studied in the Canadian children was under 5 years and
the majority of those were under the age of 1 ( Note: Median age of the
subjects: 85 days, with males being much younger than females presenting
with symptomatic UTI and needing hospitalisation).
If the same study were applied to children with symptomatic UTI
admitted to hospitals in the UK, all those under the age of 1 would need
all 3 Renal Imaging investigations (i.e. Renal U/S, MCUG & DMSA scans)
and the combination of Renal U/S & DMSA in 1-7 year olds, which will
help decide the need for an MCUG.
There is no debate in that, the MCUG, is the best diagnostic test for
the diagnosis of VUR, and that the Renal U/S has limitations in picking up
anomalies of Renal Morphology (dilatation) suggestive of VUR. However, the
combination of Renal U/S & DMSA scans should be more helpful.
This practical guideline in the UK may well lead to lesser number of
MCUGs done in children over 1 and thereby reduce significantly the need
for the invasive nature, infection risk & radiation exposure to all
children who will have MCUGs done in Canada.
We read with interest the paper by Lewendon et al. [1]
regarding the blood lead levels (BLL) of children. They
reported that children with behavioural and/or
developmental problems had higher BLL than controls (mean:
40.7 µg/l vs. 29.2 µg/l).
We would like to know the smoking style of their parents,
because there have been a few reports describing that
passive smoking causes a rise of B...
We read with interest the paper by Lewendon et al. [1]
regarding the blood lead levels (BLL) of children. They
reported that children with behavioural and/or
developmental problems had higher BLL than controls (mean:
40.7 µg/l vs. 29.2 µg/l).
We would like to know the smoking style of their parents,
because there have been a few reports describing that
passive smoking causes a rise of BLL of children.
Cigarettes contain considerable amount of lead (0.96-2.00 µ
g lead/cigarette in Japan),[2] therefore, cigarette smoke
causes lead contamination of environmental air. We
reported that preschool children (1 to 6 years of age) with
parents who smoked in the same room had a significantly
higher BLL than those with parents who never smoked (mean:
41.5 µg/l vs. 30.6 µg/l; p<_0.01.3p/>
It was of interest
that the BLL of school children (6 to 15 years of age) with
parents who smoked in the same room was not significantly
different from that of school children with parents who
never smoked (mean: 29.7 µg/l vs. 25.6 µg/l). It was clear
that passive smoking caused a rise of BLL only in preschool
children in Japan. That was probably because preschool
children spent much more time with their parents and had
much more contact with environmental tobacco smoke than
school children, and additionally, young infants had a
limited ability to excrete lead from the body.[3]
Ballew et al. also reported that children (1 to 5 years of
age) with smokers in household had a significantly higher
BLL than children without smokers (mean: 43.6 µg/l vs. 32.9
µg/l; p<_0.0001.4 an="an" inverse="inverse" association="association" has="has" been="been" demonstrated="demonstrated" between="between" low="low" level="level" lead="lead" exposure="exposure" and="and" iq.5="iq.5" according="according" to="to" these="these" data="data" it="it" is="is" obvious="obvious" that="that" passive="passive" smoking="smoking" causes="causes" a="a" rise="rise" of="of" bll="bll" children="children" subsequently="subsequently" deteriorates="deteriorates" their="their" neurobehavioural="neurobehavioural" development.="development." we="we" agree="agree" with="with" behavioural="behavioural" or="or" developmental="developmental" problems="problems" should="should" be="be" routinely="routinely" screened="screened" for="for" because="because" intoxication="intoxication" treatable="treatable" moreover="moreover" preventable="preventable" some="some" extent="extent" by="by" protecting="protecting" from="from" environmental="environmental" tobacco="tobacco" smoke.="smoke." p="p"/>References
(1) Lewendon G, Kinra S, Nelder R, Cronin T. Should
children with developmental and behavioural problems be
routinely screened for lead? Arch Dis Child 2001;85:286-
288.
(2) Watanabe T, Fujita H, Koizumi A, Chiba K, Miyasaka M,
Ikeda M. Baseline level of blood lead concentration among
Japanese farmers. Arch Environ Health 1985;40:170-176.
(3) Kaji M, Gotoh M, Takagi Y, Masuda H. Blood lead levels
in Japanese children: Effects of passive smoking. Environ
Health Prev Med 1997;2:79-81.
(4) Ballew C, Khan LK, Kaufmann R, Mokdad A, Miller DT,
Gunter EW. Blood lead concentration and children's
anthropometric dimensions in the Third National Health and
Nutrition Examination Survey (NHANES III), 1988-1994. J
Pediatr 1999;134:623-630.
(5) Banks EC, Ferretti LE, Shucard DW. Effects of low
level lead exposure on cognitive function in children: a
review of behavioural, neuropsychological and biological
evidence. Neurotoxicology 1997;18:237-282.
Masayuki Kaji, MD, PhD.
Division of Endocrinology and Meabolism
Shizuoka Children's Hospital
860 Urushiyama, Shizuoka 420-8660, Japan
TEL +81-54-247-6251
FAX +81-54-247-6259
E-mail makaji@jun.ncvc.go.jp
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