BRIEF REPORTS

Epilepsy. Proposal for revised classifica- 7. Chandy MJ, Rajshekar V, Ghosh S, tion of epilepsies and epileptic syn- Prakash S, Joseph T, Abraham J, et al. Sin- dromes. Epilepsia 1989; 30: 389-399. gle small enhancing CT lesions in Indian 2. Rajshekar V, Haran RP, Prakash GS, patients with epilepsy. Clinical, radiologi- Chandy MJ. Differentiating solitary small cal and pathological consideration. J cysticercus granulomas and tuberculomas Neurol Neurosurg Psychiatry 1991; 54: 702-705. in patients with epilepsy. Clinical and computerized tomographic criteria. J 8. Zini D, Farrell VJR, Wadee AA. The rela- Neurosurg 1993; 78: 402-407. tionship of antibody levels to clinical 3. Senanayake N, Roman GC. Epidemiology spectrum of human neurocysticerosis. J of epilepsy in developing countries. Bull Neurol Neurosurg Psychiatry 1990; 53: WHO 1993; 71: 247-258. 556-561. 4. Scarpa P, Carassini P. Partial epilepsy in 9. Jabbari B, Gunderson CH, Wippold F, childhood clinical and EEG study of 261 Citrin C, Sherman J, Bartoszek D, et al. cases. Epilepsia 1982; 23: 333-341. Magnetic resonance imaging in partial 5. Wadia RS, Makhale CN, Kelkar AV, complex epilepsy. Arch Neurol 1986; 43: Grant KB. Focal epilepsy in India with 869-872.

special reference to lesions showing ring and disc like enhancement on contrast 10. Blume WT. Clinical profile of partial sei- computed tomography. J Neurol zures beginning at less than 4 years of Neurosurg Psychiatry 1987; 50: 1298- age. Epilepsia 1989; 30: 813-819. 1301. 11. Commission on Tropical Diseases of 6. Sethi PK, Kumar BR, Mohan VS, Mohan International League Against Epilepsy. V. Appearing and disappearing CT scan Relationship between epilepsy and abnormalities and seizures. J Neurol tropical diseases. Epilepsia 1994; 35: 89- Neurosurg Psychiatry 1985; 43: 866-869. 93.

nonspherocytic hemolytic anemia, drug- Glucose-6-Phosphate induced hemolytic anemia or hemolytic Dehydrogenase Deficiency in disease of the newborn. Neonatal Hyperbilirubinemia in a G-6-PD deficiency is the most prevalent South Indian Referral Hospital enzyme deficiency worldwide. Routine screening of children and adults in various Kurien Anil Kuruvilla parts of India indicates that the prevalence Shaji T. Sukumar Atanu From the Neonatology Unit, Christian Medical Kumar Jana College Hospital, Vellore 632 004, Tamil Nadu. Reprint requests: A.K. Jana, Neonatology Unit, Christian Medical College Hospital, Vellore 632 Glucose-6-phosphate dehydrogenase 004, Tamil Nadu. (G-6-PD) is essential to maintain stability of Manuscript received: March 26, 1997; red blood cells(l). The inherited deficiency Initial review completed: May 13,1997; of this enzyme may manifest as congenital Revision accepted: August 19,1997

52 INDIAN PEDIATRICS VOLUME 35-JANUARY 1998

of G-6-PD deficiency ranges widely from as Results low as 0.2% to as high as 19%(2-8). But Two hundred and twelve neonates apart from the high prevalence noted in with hyperbilirubinemia not caused by some ethnic groups(6,8), in most parts of ABO or Rh incompatibility were enrolled the country the prevalence is less than in the study. Twenty five (11.8%), were 6%(2-7). Neonates with hyperbilirubinemia found to have deficiency of G-6-PD. All constitute a high-risk group in whom the babies were born at Christian Medical prevalence of G-6-PD deficiency is likely to College and Hospital, Vellore, except one be high. However, there is no data from who was born elsewhere and transferred South India to indicate the extent to which with severe jaundice on the fourth G-6-PD deficiency contributes to neonatal postnatal day. Of the G-6-PD deficient hyperbilirubinemia. The present study was neonates, 16 (64%) were males and 9 (36%) undertaken to ascertain the role of G-6-PD were females; 4 (16%) were preterm and deficiency in neonatal hyperbilirubinemia low birth weight. The frequency of G-6-PD in South India, to determine whether this deficiency was similar in children of subset of children need to be routinely different birth weight, gestational age and screened for G-6-PD deficiency. religious background. The hematological profile of neonates Subjects and Methods with hyperbilirubinemia who were investi- This study was conducted among neo- gated for G-6-PD deficiency is shown in nates with hyperbilirubinemia admitted in Table I. the Christian Medical College and Hospi- Jaundice was not detectable within the tal, Vellore. From February to December first 24 hours in any baby; the mean age of 1996, we consecutively investigated all onset of jaundice was 61.4 (± 21.6) hours. term babies with serum bilirubin levels Thirteen (52%) patients had a peak serum more than 12 mg/dl (>205 µmol/1) and bilirubin level in the range of 15-20 mg/dl. preterms with serum bilirubin exceeding Only the outborn baby transferred with se- 15 mg/dl (<255 µmol/1) for G-6-PD defi- vere jaundice had a serum bilirubin of 32 ciency after ruling out ABO and Rh incom- mg/dl and required an exchange transfu- patibility by blood grouping, direct sion; in all other cases jaundice improved Coomb's test, reticulocyte count, and pe- with phototherapy alone. The mean dura- ripheral blood smear. They were also test- tion of photothrapy was 3.4 (± 1.1) days. ed for hemoglobin concentration, total and None of the babies developed features of direct bilirubin, random blood sugar, and kernicterus. G-6-PD levels. For the G-6-PD assay, 1 ml of anticoagulated blood in a glass bottle Discussion was sent and analyzed in the laboratory Several studies done across India by within two hours. Determination of the ac- screening of all newborns have shown that tivity of G-6-PD was done by spectropho- the prevalence of G-6-PD deficiency is less tometry(9). When low G-6-PD levels were than 6%(2-7). But in ethnic groups such as encountered (<120 U/L), the test was re- the Parsees and Bhanushalis, it was as high peated on a fresh sample to confirm defi- as 16 and 19%, respectively(6,8). However, ciency of the enzyme. Statistical analyses the studies done in Chandigarh, Delhi and was done using the Chi-square and Z-test Thailand investigating the contribution of to compare differences between G-6-PD G-6-PD deficiency to neonatal hyper- deficient and non-deficient babies. bilirubinemia reveal a uniformly similar

53 BRIEF REPORTS

contribution of 12%(10-12). Similarly, in potentially harmful drugs are not adminis- this study from South India, 11.8% of new- tered to these infants and their nursing borns investigated for hyperbili-rubinemia mothers. Thus, we would recommend that were found to have G-6-PD deficiency. a more appropriate strategy would be to identify those with G-6-PD deficiency by A majority of G-6-PD deficient babies focusing our efforts on neonates with had features mimicking exaggerated physi- significant jaundice. ological jaundice. The onset of clinical jaun- dice was always after 24 hours, the peak se- REFERENCES rum bilirubin rarely exceeded 25 mg/dl, and there was almost universal response to 1. Beutler E. The glutathione instability of phototherapy alone. Only by investigating drug sensitive red cells. A new method the G-6-PD level was it possible to make a for the in vitro detection of drug sensitivi- diagnosis of G-6-PD deficiency, in a popu- ty. J Lab Clin Med 1957; 49: 84-91. lation where this disorder has not been 2. DaCosta H, Pattani J, Mehandle K, Desai well recognized earlier. M, Merchant SM. G-6-PD defect in Indi- The incidence of G-6-PD deficiency an children. Indian Pediatr 1967; 4: 163- among infants with hyperbilirubinemia is a 168. measure of the public health aspect of the 3. Gupta S, Ghai OP, Chandra RK. G-6-PD problem. Thus, in developing countries like deficiency in the newborn and its relation India, where the prevalence of G-6-PD defi- to serum bilirubin. Indian J Pediatr 1970; ciency varies widely in the large popula- 37:169-175. tion, routine screening of all newborns is 4. Khanduja PC, Agarwal KN, Julka S, not feasible or cost-effective except in se- Bhargava SK, Taneja PN. Incidence of lected ethnic groups where a high preva- G-6-PD deficiency and some observations lence of G-6-PD deficiency has been report- in patients with hemoglobinuria. Indian J ed. Yet the importance of identifying G- Pediatr 1966; 33: 341-344. 6-PD deficiency in the newborn period 5. Verma M, Singla D, Crowell SB. G-6-PD cannot be overlooked so that appropriate deficiency in neonates: A prospective counselling is provided to the family and study. Indian J Pediatr 1990; 57: 385-388.

54 INDIAN PEDIATRICS VOLUME 35-JANUARY 1998

6. Anand JS, Baxi AJ, Nirmal RJ, Adalja MR, 10. Yachha SK, Marwaha RK, Narang A, Anand AK. A study of G-6-PD activity in Mohanty D. G-6-PD isoenzyme pattern Bhanushali and non-Bhanushali children. and evaluation of screening methods for Indian Pediatr 1981; 18: 385-388. G-6-PD deficiency in neonatal hyperbilirubinemia. Indian Pediatr 1987; 7. Deshmukh VV, Sharma KD. Deficiency of 24:1099-1103. erythrocyte G-6-PD as a cause of neonatal jaundice in India. Indian Pediatr 1968; 5: 11. Madan N, Sundaram KR, Bhargava SK, 401-405. Sood SK. G-6-PD deficiency and neonatal 8. Baxi AJ, Balakrishnan V, Undevia JV, hyperbilirubinemia. Indian J Med Res Sanghvi LD. G-6-PD deficiency in Parsee 1989; 90: 306-313. community in Bombay. Indian J Med Sci 12. Sasanakul W, Hathirat P, Jeraporn K, 1963; 17: 493-497. Tejavej A, Siripoonya P, Isarangkura P. 9. Kornberg A, Horecker BL. Methods in Neonatal jaundice and G-6-PD deficiency. Enzymology. Vol. 1: New York, Aca- J Med Assoc Thai 1989; 72 (Suppl 1): 130- demic Press, 1955; pp 323-325. 132.

Clinico-Hematological Profile of ease assumes great clinical importance Megaloblastic Anemia since it responds exceedingly well to treat- ment. The present study evaluates the varying clinico-hematological manifesta- Sunil Gomber tions in 29 patients diagnosed as megalo- Kusum Kela blastic anemia over a three year period. Neelam Dhingra Subject and Methods

Megaloblastic Anemia is one of the im- Twenty nine children (age range 3Vi months to 12 years) diagnosed as megalo- portant causes of anemias in children. It is blastic anemia over a period of three year not an infrequent entity in poor socio- (March 1993 to March 1996) were prospec- economic condition. This condition has tively studied. All anemic children admit- protean manifestations in childhood, some- ted with or without bleeding manifesta- times mimicking a hematological malig- tions had their peripheral blood smear nancy like leukemia. Diagnosing this dis- examined. Complete hemogram including platelet count and mean corpuscular From the Departments of Pediatrics and Pathology, University College of Medical Sciences and volume (MCV) were also carried out in Guru Tegh Bahadur Hospital, Shahdara, Delhi each child using Coulter T860 particle 110 095. counter. The platelet count obtained from Reprint requests: Dr. Sunil Gomber, F-4(A) Vijay Coulter counter was always confirmed by Nagar, Delhi 110 009. peripheral smear examination. Cases with Manuscript received: March 10, 1997; macrocytic blood picture on smear exami- Initial review completed: May 7,1997; nation were subjected to bone marrow Revision accepted: August 12, 1997 examination to confirm the diagnosis of

5 5