April 1988, Volume 38, Issue 4

Case Reports


Fatema JawadJawad  ( Sughrabai Millwalla Hospital, North Nazirnabad, Karachi. )

06-PD STATUS AND NEONATAL HYPERBILIRU­BINAEMIA. Ahrned, P., Ahmed, A.J., Ahmed, K.N. Pakistan Paediatric Journal, 1986; 10:241-244
282 newborns which included 80 icteric babies were randomly selected for the trial. A detailed history along with the drug history was obtained. Methaemoglobin reduction test was done in all cases whereas serum bilirubin, haemo­globin and a peripheral smear was examined in the jaundiced babies. The parents and siblings of the deficient neonates were also screened for enzyme deficiency. Eleven babies showed G-6-PD deficiency. Of these 8 were jaundiced and 3 were non-icteric. None of the enzyme deficient babies had ABO or RH incompatibility, septicaemia, prematurity or exposure to any oxidant group of drugs and chemicals. Jaundice appeared on the second or third day of bbth and haemoglobin was within normal limits. Six mothers and fpur fathers of the deficient babies were screened and four mothers and one father was found lacking in the enzyme. This study confirms that among hyperbi­lirubinaemic newborns G-6-PD deficienby plays an important role in causing haemolysis. A plausible reason given by Friers is that generalised tissue deficiency of the enzyme involves the liver which leads to defective metabolism and clearance. This causes accumulation of meta­bolites capable of oxidising red cells and G-6-PD deficient erythrocyte may be destroyed due to it.
Conversion reaction or psychological stress. when unbearable and manifested as somatic sym­ptoms either motor or sensory is still observed in Pakistani children. This is due to the strong in­hibitory effects of the socio-cultural influences. 8 female and 6 male children with average ages being 9 and 11 years respectively were included in the study. All were school going with average 10 of 100. Five boys complained of fits and spasmodic attacks or tremors of the limbs. One had difficulty in breathing. 3 girls had painful limbs and joints, 2 had fits and 2 had fainting spells and one had headache and occasional blind­ness. Looking into the family dynamics it was concluded that interpersonal relationship within the family existed in all the cases. 8 children had overprotective, overanxious mothers which contributed towards regression in the child. 10 cases had an excessively dominant father having little communication with the wife or children. In two cases the mother played the dominant role. In the remaining two cases the father was absent, one deceased and one away for employment purposes. Another contributing factor towards conversion reaction in Pakistan is that parents have high expectations and place great importance on the child’s educational achievements which leads to stress and causes extreme anxiety in the child. Studying the precipitating factors it was assessed that in four cases the cause was school work, five wanted to escape household chores and another four wanted to gain attention from parents. Treatment was carried out with the concept of sick role. Techniques of behaviour modification with psychotherapy and family work proved successful. Once the perception of the parents towards the child changes from a chroni­cally ill to a potentially healthy individual they support the child’s escape with honour.
BLOOD TRANSFUSION REACTIONS IN CHILDREN. Ali, A., Khan, F.M., Imran, M. Pakistan Paediatric Journal, 1986;, 10:267-272.
Untoward reactions occurring in 350 blood transfusions in children are presented. 20 to 30 cc per Kg body weight whole blood or 10-15 cc per Kg body weight packed cells, as indicated, were transfused, being donated preferably by the parents. The total blood was given in 6 to 8 hours. In cases of cardiac failure injection Lasix was administered prior to the transfusion. 194 children were between the ages of 0 — 2 years, 87 between 2 to 5 years and 69 were above 5 years. There were 203 males and 147 females. The maximum number of children belonged to blood group B followed by Group A and 0. The indications for blood transfusion were anaemias of infection and nutrition, Thallasaemia major, Hodgkin’s disease and leukaemias, Purpura group and a miscellaneous group consisting of rheumatic fever, liver abscess, aplastic anaemia, rheumatoid arthritis and nephrotic syndrome.76 cases developed untoward reactions with 30 having mild fever and 30 hyperpyrexia with temperature above 40°C. Nine children developed rashes, 6 had itching and one had haemolysis leading to jaundice and haemoglo­binuria and died in 36 hours. This was the only mismatched blood transfusion. In 20 cases the transfusion had to be discontinued due to the severe reaction. The blood was sent for re-typing and was re-transfused in 15 patients. In 4 cases the blood was found to be old and clotted in tubes. 20 cases were given antihistamines and 8 had to be administered steroids concomitantly. The mild reactions observed could be attributed to the fact that the blood bank facilities available were not highly sophisticated and screen­ing for antibodies could not be done accurately. To prevent reactions and transmission of diseases it is of utmost importance that modern blood transfusion technology should be available in all hospitals.
CHILDHOOD NEPHROTIC SYNDROME. Raza, S., Khan, F.M., Imran, M. Pakistan Paediatric Journal, 1986; 10:273—278.
Fifty patients diagnosed as Nephrotic Syndrome in the Paediatric Department of Lady Reading Hospital, Peshawar were included in a study which is presented. The diagnosis was based on the clinical history, physical findings and laboratory results of urine proteins, serum pro­teins, and serum cholesterol. Compliment esti­mation, selectivity of proteins in the urine and renal biopsy could not be done due to lack of facilities. The ages of the children included in the study ranged between 1 and 12 years with the maximum cases being of 5 years of age. There were 31 males and 19 females. All had oedema, proteinuria, hypoproteinuria and hype rcholestero­laemia. Four cases had hypertension and 4 had haematuria. 20 children had pyuria but a positive culture was obtained in 5 only. Prednisolone was given in a dose of 2 mg per Kg body weight daily till the patient had protein free urine for three consecutive days. 48 cases responded to Steroids whereas 2 were non-responsive. 38 patients were early responders (2 weeks of steroid therapy), 5 showed a response in 4 weeks and 5 in 8 weeks. Of the two non-responders, one left against medical advice and one died after no response to cyclo­phosphamide therapy. 18 patients of the steroid responsive group had complete remission, 18 relapsed and 12 were lost to follow up. All cases with first relapse were treated again with steroids in full doses followed by alternate day treatment for 6 months. 8 of these cases had full remission and the remaining 10 relapsed again and were treated with cyclophos­phamide. Two of these cases were lost to follow up, 3 died and 5 responded completely. This study is comparable to Western studies except the age group here is slightly higher. The relapse was usually preceded by infection. It is also concluded that a renal biopsy should be done especially in cases who are resistant to steroids.

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