Name : Dr. ALI  HASAN DHARY  AL- JUMAILY

      Adress : IRAQBAGHDAD – Central Teaching Hospital For Children

     Email : ( ali_hassan562004@yahoo.com )  .

     

PREVALENCE OF HYPOCALCEMIA AMONG THALASSEMIC PATIENTS REGISTRED IN IBN

AL-BALADY HOSPITAL ( THALASSEMIC CENTRE)

BAGHDAD IRAQ

 

 Dr.Ali Hassan Al-Jumaily, Dr.Shaimaa Khider

 

SUMMARY:

 

The objective of this study is to through light on the prevalence

Of hypocalcaemia in thalassemic patients registered in Ibn Al-balady

Hospital (Thalassemic Centre) Baghdad Iraq

400 with thalassemia major were included in the study and randomly

selected at October 2001 they subjected to serum study for calcium ,

phosphorus and alkaline phosphatase  20 patients x-rayed for bones

only no facilities for studying serum ferritin ,parathyroid hormone

and densometry for bones

It was found that hypocalcaemia so prevalent 87 patients out of 400

Tab. 1 and more prevalent among age group 10 years and above tab.2

The above findings are discussed and comments are put forward in

The direction of finding clue(s) for such results

 

  INTRODUCTION:  

 

Hypocalcaemia is well known complication of iron overloaded  and/or anemia (1)

Iron overload occurs when iron intake is increased over a sustained

Period of time either from the transfusion of red blood cells or because there is increased absorption of iron from the digestive

tract. both of these occur in thalassemia. Blood transfusion being

the major cause in thalassemia major and increased iron absorption being more important in thalassemia intermedia.Because there is no mechanism in human to excrete the excess iron, this has to be removed by chelation therapy.

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Trasfusional iron overload in thalassemia major is fatal in the second decade of life usually from cardiac complications (8) iron overload also causes pituitary damage with hypogonadism and poor growth .Endocrine complications namely diabetes, hypothyroism and hypoparathyroism also seen. Liver diseases with fibrosis and eventually cirrhosis particularly if a Concomitant chronic hepatitis is present

.majority of hypocalcaemia Patients due to iron overload  show mild                                     

form of the disease accompanied by paraesthesia more sever cases may demonstrate tetany,seizures or cardiac failure (1,5,8)   

Hypocalcaemia is defined as total serum calcium<7.0mg/dl( 1.75mmol/L) or

Ionized calcium <3.5mg/dl.however mild symptoms can occur with a total calcium of 7.5mg/dl in neonate and 8.5mg/dl in older children.   

                                                Most of the calcium is in skeleton the plasma calcium, normally about 10mg/dl(mEq/L,2.5mmol/l) is partly bound to protein and partly diffusible

 Table1 distribution (mmol/L) of calcium in normal human plasma:

 

Diffusible                                                         1.34

    Ionized(calcium)1.18

    Complexed to HCO3Citrae…0.16

Non diffusible(protein-bound)                       1.16

    Bound to albumin 0.22

    Bound to globulin0.24

                                 Total plasma calcium                            2.5

 

Three hormones are primarily concerned with regulation of calcium metabolism. 1,25-Dihyroxycholecalciferol is steroid hormone formed from vitamin D by successive hydroxylations

In the liver and kidney.it increases calcium absorption from the

Intestine and bone. Parathyroid hormone which is secreted by the parathyroid glands ,mobilizes calcium from bone and increases urinary phosphate excretion .Calcitonin a calcium lowering hormone secreted by the thyroid gland,inhibits bone resorption.All 3 hormones operate in

Concert to maintain the constancy of the calcium level in the body fluids.(2,3,4,6,7)

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Aim:

 

the purpose of this study is  to estimate the prevalence of hypocalcaemia  among thalassemic patients in Ibn Al-balady Hospital(thalassemic centre).

 

Patients and methods:

 

400 patients with thalassemia major registered in Ibn Al-Balady Hospital(thalassemic centre) In Baghdad Iraq at October 2001

 were selected randomly for this study. After a full clinical examination all the patients were subjected to serum study for calcium, phosphorus and alkaline phosphatase.Bone x-ray were done only for 20 patients. Facilities for studying serum ferritin, parathyroid hormone and bone density (desometry) not available at that time.

Results:

 

All the results obtained from 400 thalassaemic patients are tabulated:

Table 2 shows that : among the 400 patients included in this study 87 (21.75%) detected with total serum calcium less than 7.5mg/dl(mmol/l).

The clinical findings of these patients were:

55 patients(63%) with carpopedal spasm.

20 patients(22.9%) with irritability,sever headache, calf muscle spasm and paraesthesia.

10(11.4%)with latent tetany.

1 patient with seizure.

1 patient with combined findings.

81 patients with serum alkaline phosphatase above 5k.a.u

79 patients with serum phosphorus above 5mg/dl.

Table 3 shows the age distribution of the patients with hypocalcaemia included in this study:

52% above 10 years of age.

43% from5-10 years.

5% less than 5 years.

 

 

 

 

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Results of Investigation

 

      1. No. of patients                      2. S.Alk.ph.>5K.A.U.                  3. S. Ph. > 5mg / 100ml            4. S.Ca. <7mg /100ml

 

 

 

 

 

 

 

 

 

 

(  Table 2 )

 

 

 

 

 

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( Table   3 )

 

 

 

 

 

 

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Discussion:

The results obtained in the present study clearly show that hypocalcaemia is so prevalent in thalassemia major this obvious from the number and percentage(87 ,21.75% respectively)table2.also show that a higher prevalence rate among age groups of 10 years and above(52%) table 3 

This finding correlated with the previous study by (Desantis1995) which showed that hypocalcaemia due to

Hypoparathyroidism in thalassaemia is a recognized later

Complication ( age 16 years and above).

However in our study we demonstrated hypocalcaemia earlier than that detected by (Desanctis1995) we attributed

This to :

1.  Delay in diagnosis of thalassaemia in our society with

       subsequent delay in the treatment which leads to early

     and sever complication.

2.  poor patient compliance due to poor education

       about the disease

3   Therapy for thalassaemia is not always available.

4   communications between the the thalassaemic centers

       and the patients are not always easy.

It is difficult now and at the time of the study to differentiate between the different causes of hypocalcaemia due to lack of

facilities as mentioned above.

This is a preliminary study and a further study is needed

to know the causes later.

Conclusion:

It is ultimately concluded that hypocalcaemia is so prevalent in thalassaemc patients and more prevalent in age group10 years

and above(tab.2,3).

Recommendations:

. Screening tests are very essential serum calcium and1

     phosphorus must be done for the thalassaemic patients

     periodically especially for age group 10 years and above. 

2.  Best results were found with intensive desferal therapy

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     and vitamin D(one alpha) along with calcium rich diet

     oral administration of vitamin D or one of its analogous

    Some patients require high doses of vitamin D to normalize

         their serum calcium levels. This should be carefully monitored

hypocalcaemia is a common complication Of this treatment      as   

.Tetany and cardiac failure due to sever hypocalcaemia require  intravenous administration of calcium under careful cardiac

       monitoring followed by oral vitamin D

3.  More governmental support is required focusing on availability

     of therapy , facilities for discovering the cases

     antenatal diagnosis special attention must be payed for training

staff in this field. On the top of all education about the      the

     Thalassaemia is very important

4.  screening for thalassaemia must be included with  other tests as

     premarriage requirements

5.  Social supports for the patients and their families including

      psychological support .

  

ńReferences:

 

          1. Desantctis 1995

2. Clinical manual of emergency paediatrics Ellen f.Crain,Jeffry

    C.Gershel pp154-156 3rd ed.

3. Review of medical physiology W.FGanong 10th ed.pp308

4. Dorland 27th ed.

5. Thalassaemia international Federation(TIF)2000

6. Gentner JM:Disorders of calcium and phosphorus

    homeostasis.PediatrClin North Am 37:1441-1466,1990

7. Aarskog D,Harrison H: Disorders of calcium, phosphate,

   PTH and vitamin D, in Kappy Ms,Blizzard RM,Migeon

   CJ(eds):Wilkins Diagnosis and treatment of Endocrine

   Disorders in childhood and adolescence(4thed).Springfield,

   CCThomas,1994,pp1050-1067

8. Zurlo 1989                                                                                                

 

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