Iron status at birth and at 4 weeks in preterm-SGA infants in comparison with preterm and term-AGA infants.

OBJECTIVE: To determine the iron status at birth in preterm small for gestational age (SGA) in comparison with preterm appropriate for gestation (AGA) and term-AGA infants. METHODS: Mother-infant pairs with gestation of < 37 weeks, both SGA, and preterm-AGA and term-AGA as control were enrolled. Maternal, cord blood and infant blood samples at 4 weeks were obtained for various iron indices - cord serum ferritin, proportion of infants with "low" serum ferritin, serum ferritin at 4 weeks and correlation among maternal and neonatal iron indices - hemoglobin,serum ferritin and total iron-binding capacity. RESULTS: There were 50 mother-infant pairs in each group. Cord serum ferritin levels were less in preterm-SGA group as compared to preterm-AGA group (median [interquartile range]: 68 [30 113] vs. 120 [73 127], p = 0.002) and preterm-AGA had less cord ferritin than term-AGA (141 [63 259], p = 0.006). The proportion of the infants with "low" serum ferritin was more in preterm-SGA than in preterm-AGA (16 [32%] vs. 5 [10%], p = 0.01). The serum ferritin levels at follow-up were also less in preterm-SGA as compared to preterm-AGA (143.5 ± 101 vs. 235.1 ± 160, p = 0.004). Other cord blood iron indices and follow-up serum ferritin levels were similar. There was no correlation among various maternal and neonatal cord iron parameters. CONCLUSIONS: Preterm-SGA infants have lesser total iron stores as compared to gestation-matched AGA infants, which is again lesser than term infants. Future studies can be planned to look at iron status at 12 months as well as their neurodevelopmental outcome.

Iron status at birth and at 4 weeks in term small-for-gestation infants in comparison with appropriate-for-gestation infants.

OBJECTIVE: To determine body iron stores at birth in term small-for-gestational age (SGA) infants as compared to appropriate-for-gestational age (AGA) infants. METHODS: In this prospective study, mother-infant pairs with gestation of  ≥ 37 weeks and birth weight of at least 1.5 kg were enrolled. Asymmetric SGA infants were taken as cases and term AGA infants as controls. Maternal, cord blood, and infant blood samples at 4 weeks were obtained for measurement of various iron indices - cord serum ferritin, serum ferritin at 4 weeks, and correlation among maternal and neonatal iron indices - Hb, serum iron, ferritin, and total iron binding capacity (TIBC). RESULTS: There were 50 SGA and 50 AGA mother-infant pairs. Cord serum ferritin levels were low in SGA group as compared to AGA [median (IQR): 68 (30,136) vs. 141 (63,259), p = 0.007]. The proportion of infants with 'low' cord ferritin (< 40 µg/l) were more in SGA [p = 0.05]. There was no correlation among various maternal and neonatal cord iron parameters. The serum ferritin levels at 4 weeks were similar in both the groups (p = 0.16). CONCLUSIONS: Term SGA infants have lesser total iron stores as compared to AGA infants at birth. Future studies can be designed to look at long-term neurodevelopmental outcome of the SGA babies with low as well as normal ferritin and also the role of early iron supplementation in term SGA neonates.

Hyperfibrotic myelodysplastic syndrome: a report of three cases from north India.

Extensive fibrosis in myelodysplastic syndromes (MDS) is distinctly infrequent. Herein, we report three rare cases of hyperfibrotic MDS. This entity should be classified separately in the chronic myeloproliferative disease (CMPD)-MDS group due to variable clinical presentation and poor prognosis.

Multiple constitutional aetiological factors in bone marrow failure syndrome (BMFS) patients from north India.

BACKGROUND AND OBJECTIVES: A large number of patients diagnosed with bone marrow failure syndromes (BMFS), comprising aplastic anaemia (AA) and myelodysplastic syndromes (MDS), remain aetiologically uncharacterized worldover, especially in resource constrained set up. We carried out this study to identify a few constitutional causes in BMFS patients attending a tertiary care hospital in north India. METHODS: Peripheral blood lymphocyte cultures were performed (with and without clastogens) in a cohort of 135 consecutive BMFS patients, in order to detect Fanconi anaemia (FA), Down's syndrome (+21), trisomy 8 (+8) and monosomy 7 (-7). RESULTS: Constitutional factors were detected in 17 (12.6%) patients. FA defect was observed in 24.07 percent (13/54), 16.66 percent (1/6) and 2.85 percent (1/35) paediatric aplastic anaemia, paediatric MDS and adult MDS patients respectively. Down's syndrome was detected in 5.00 percent (2/40) adult aplastic anaemia patients. None of the patients revealed trisomy 8 or monosomy 7. INTERPRETATION AND CONCLUSION: Presence of an underlying factor determines appropriate management, prognostication, family screening and genetic counselling of BMFS patients. Special tests required to confirm or exclude constitutional aetiological factors are not available to majority of the patients in our country. Diepoxybutane (DEB) test yielded better results than mitomycin C (MMC) test in our experience.

A randomized, triple-blind, placebo-controlled trial of prophylactic oral phenobarbital to reduce the need for phototherapy in G6PD-deficient neonates.

OBJECTIVE: Decreased conjugation is probably more important than hemolysis for causing jaundice in G6PD-deficient neonates. The role of enzyme inducers, like phenobarbital, in G6PD deficiency is unclear. This randomized controlled trial was performed to evaluate Phenobarbital's role in reducing the need for phototherapy among G6PD-deficient neonates. STUDY DESIGN: This stratified, randomized, triple-blinded, placebo-controlled trial was conducted in a level III NICU. Consecutive babies with gestation >/=34 weeks and birth weight >/=1800 g were screened from cord blood. G6PD-deficient neonates, who were otherwise healthy, were enrolled. Rh isoimmunization, maternal Phenobarbital use and lack of parental consent were exclusion criteria. Subjects were randomly allocated to receive 5 mg/kg day of oral phenobarbital/ placebo for first 3 days. They were monitored daily for total serum bilirubin (TSB) until declining TSB was documented twice. The primary outcome was requirement for phototherapy and secondary outcomes were duration of phototherapy, need for exchange transfusion, peak TSB and adverse effects. Sample size of 56 could detect a decline in phototherapy requirement from 40 to 5% with 80% power and 5% error. RESULTS: Of 2370 babies screened, 63 were G6PD-deficient. Of them, 56 eligible babies were allocated to phenobarbital (n=27) or placebo (n=29). The mean age of administration of the first dose was 18.55+/-7.3 h. In total, 44% in phenobarbital group and 41% in placebo group required phototherapy (p=1.0). There was no significant difference in exchange transfusion rates (18.5 vs 10%, p=0.46). No baby had adverse reactions. CONCLUSION: Prophylactic oral phenobarbital does not decrease the need for phototherapy or exchange transfusions in G6PD-deficient neonates.

Hairy cell leukemia-variant--a case report.

Hairy-cell leukemia-variant (HCL-V) is a rare B-cell disorder which accountsfor 10% of HCL cases. The main features are splenomegaly, lymphocytosis and cytopenias without monocytopenia. The circulating cells have a morphology intermediate between prolymphocytes and hairy cells. The immunophenotype shows a mature B-cell phenotype with expression of B-cell antigens CD11c and CD103 but unlike typical hairy cell the cells are negative for CD25. The histology of bone marrow and spleen shows a pattern of infiltration similar to that in HCL. We present a case of HCL-V in a 66-year-old male. The bone marrow findings, immunophenotypic profile and electron microscopic features are described. The patient underwent splenectomy which also revealed infiltration by leukemia. Patients are resistant to alkylating agents and alpha-interferon (á-IFN). Splenectomy may be beneficial for long-lasting partial responses in some of the patients and is a good palliative treatment.

Myxomatous stromal changes and necrosis of bone marrow--a retrospective study of 3 years.

Myxomatous stromal changes and bone marrow necrosis (BMN) are uncommon histologic findings. These changes have been found in various conditions like disseminated carcinomatosis, postchemotherapy cases, chronic infections, infiltrative disorders of the marrow etc. The present study is a retrospective study of 3 years (Jan, 1999 to Dec. 2001) from Deptt. Of Hematology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh (India). During this period, 3740 bone marrow samples were examined. Myxomatous stromal changes and bone marrow necrosis were noted in 0.43% (16/3740) and 0.45% (17/3740) samples respectively. In addition to common causes of myxomatous stromal changes and bone marrow necrosis as described in the literature, this study highlights the association of these conditions with some of the rarer entities like hyperoxalosis, leishmaniasis, parvovirus induced marrow aplasia and cryptococcal infection. There is paucity of such associations in the literature.

Phenobarbitone prophylaxis for neonatal jaundice in babies with birth weight 1000-1499 grams.

Management of neonatal jaundice is simple but in sick, very low birth weight babies poses additional hemodynamic insult. Role of prophylactic postnatal phenobarbitone (two different dosage regimens) was evaluated prospectively on occurrence of neonatal jaundice and the need for therapy in 150 babies with birth weight 1000-1499 grams. Phenobarbitone in the dose of 10mg/kg given within 6 hours of life followed by 5mg/kg/day till day 5 of life intravenously significantly decreased the need for exchange transfusion and duration of phototherapy in babies with birth weight of 1000-1499 grams. This dosage schedule was better than dose of 5mg/kg for 5 days in significantly reducing the duration of phototherapy