Harish K. Pemde

Anemia in severe acute malnutrition

OBJECTIVES India has the highest prevalence of severe acute malnutrition (SAM). Severe anemia is one of the comorbidities responsible for increased mortality in severely malnourished children, yet it has not received the attention it should. The aim of the present study was to determine the prevalence and type of anemia and to evaluate the possible etiologies for severe anemia, in these children. METHODS A cross-sectional study of patients with SAM in a tertiary care hospital in northern India over a period of 12 mo from Sept. 1, 2010 to Aug. 31, 2011 was conducted. We observed the prevalence of severe anemia (hemoglobin < 7 g/dL), morphologic type of anemia, number of patients requiring blood transfusion, hematologic profile of mothers, nature of feeding, duration of exclusive breastfeeding, and the demographic profile of these patients. RESULTS Included in the study were 131 cases of SAM. The age group varied between 6 and to 59 mo. Of patients with SAM, 67.3% had severe anemia; 13.8% had moderate anemia. Of these patients, 25% required packed red blood cell transfusion. The most common type of anemia was microcytic (38.6%) followed by megaloblastic (30.5%). CONCLUSIONS A high incidence of severe anemia in SAM with a large proportion (25%) requiring blood transfusion is a pointer toward nutritional anemia being a very common comorbidity of SAM requiring hospital admission. Because megaloblastic anemia closely followed microcytic anemia, supplementation with vitamin B12 in addition to iron and folic acid would be recommended.

C-reactive protein in childhood meningitides.

Utility of C-reactive protein (CRP) latex agglutination test in meningitis was evaluated. Serum CRP test was positive in 100% cases of meningitic groups and 53% cases of “no meaningitis (NM)” group. Cerebrospinal fluid (CSF) CRP test was positive in 100% cases of pyogenic meningitis, whereas it was negative in 95% cases of tuberculous meningitis and 100% cases of NM group. CSF CRP test showed 100% sensitivity and negativbe predictive values, 95–100% specificity and 94–100% positive predictive values for various inter-group differentiations. This study conclluded that CSF CRP positive cases should be considered as pyogenic meningitis unless proved otherwise. Routine use of this simple, reliable and inexpensive test is recommended for rapid diagnosis and differential diagnosis f meningitis.

Antithymocyte globulin and cyclosporine in children with aplastic anemia: a developing country experience.

Background: Human leukocyte antigen-matched bone marrow transplant in the treatment of aplastic anemia is generally not feasible in developing countries due to lack of resources and expertise and immunosuppressive therapy (IST) has been used as an alternative. This study aims to report the long-term outcome of children with aplastic anemia treated with IST [antithymocyte globulin (ATG) and cyclosporine] in our hospital. Procedure: Case files of children with aplastic anemia who received IST from January 2001 to November 2009 were reviewed. Results: Thirty-five patients with aplastic anemia (14 very severe aplastic anemia; 21 severe aplastic anemia) were given IST. Seven patients expired within 3 months of therapy and were excluded. The analysis was done in 28 patients (24 male and 4 female; 12 very severe aplastic anemia and 16 severe aplastic anemia). The median age was 10 years (range, 5 to 12 y). Ten patients achieved partial response and 4 patients complete response at 1 year with overall response rate of 50%. Three nonresponders received a second course of ATG after 12 months out of which 2 responded. Hence, overall response including second course was 16 (57%). Three patients relapsed after a median interval of 23 months. The median duration of follow-up of 16 responders was 40 months (range, 15 to 119 mo). In the patients with long-term follow-up for >4 years (n=7), all were surviving and independent of transfusions. Conclusions: In a developing country setting, IST with ATG and cyclosporine seems to be a good alternative treatment for aplastic anemia in children.

Quality of Life of Adolescents With Transfusion-dependent Thalassemia and Their Siblings: A Cross-sectional Study.

Transfusion-dependent thalassemia (TDT) and its treatment affect the physical, emotional, and social functioning, impairing the quality of life (QoL). There are few studies on QoL of adolescent with TDT. Its effect on their siblings’ QoL has not been studied so far. In this cross-sectional study, 40 adolescents with TDT, 28 siblings, and 40 controls were studied to assess the QoL of adolescents thalassemics, and their siblings using the shorter version of World Health Organization Quality of Life instrument, the WHOQOL-BREF. Thalassemics had poor perception of their general health and scored significantly lower in all the subscales compared with the controls. Lowest mean subscale scores were for physical (57.7 vs. 72.4, P<0.001), and psychological domains (56.7 vs. 72.3, P<0.001). Their siblings also scored significantly less in environment domain. Prevalence of school dropout, short stature, and delayed puberty were significantly higher in thalassemics. Pretransfusion hemoglobin, age at onset of anemia, and chronological age were found to be significant predictors of total summary scores. This study showed that the concept of QoL is a cumulative reflection of individual and disease variables and highlights the negative impact of thalassemia on the patients’ QoL and some aspects of their siblings’ lives also.

Physical growth in children with transfusion-dependent thalassemia

correspondence: Harish K Pemde Department of Pediatrics, Lady Hardinge Medical college, Kalawati saran children’s Hospital, Bangla sahib Marg, new Delhi 110001, india Tel +91 11 23363432 Fax +91 11 23365792 email harishpemde@gmail.com Objective: To describe physical growth and related factors in transfusion-dependent thalassemia patients. Methods: This is a cross-sectional analysis of the records of the patients registered at and being followed up by the Thalassemia Day Care Center (TDCC) at Kalawati Saran Children’s Hospital, New Delhi, India. Clinical and laboratory parameters were recorded on a spreadsheet for analysis. Clinical parameters included weight, height, sexual maturity ratings, and general and systemic physical examination. Laboratory parameters included pretransfusion hemoglobin (Hb), periodic serum ferritin, and tests for viral markers of human immunodeficiency virus (HIV) and hepatitis B and C. Z-scores for weight, height, and body mass index (BMI) were calculated using World Health Organization reference data. Statistical analysis was carried out using Microsoft Excel and Stata software. Results: Out of 214 patients registered at the TDCC since 2001, 154 were included in this study. The mean age of patients was 9.19 years (range 0.5–20 years). Pretransfusion Hb was well maintained (mean 9.21 g/dL; 95% confidence interval [CI]: 9.06–9.36), but the mean serum ferritin levels were approximately three times (3112 ng/mL) the desired value despite the patients being on deferiprone (72%) or deferasirox (25%). One-third (33.11%) of the patients had short stature, 13% were thin, and 10.82% were very thin (BMI z-score ,-3). No patient was overweight or obese. Linear regression coefficient showed that for every 1-year increase in age, the mean ferritin value increased by 186.21 pg/mL (95% CI: 143.31–228.27). Height z-scores had significant correlation with mean ferritin levels, whereas correlation with mean pretransfusion Hb was not significant statistically. Mean ferritin levels were significantly higher in patients with short stature than in the patients with normal height. Regression analysis showed that an increase of 3571 units of serum ferritin was associated with a decrease of one point in height z-scores. One-fifth (19.40%) of adolescent patients had delayed puberty. Conclusion: Approximately one-third (33.11%) of patients with transfusion-dependent thalassemia major were of short stature. In this group of patients with pretransfusion Hb levels maintained at desired levels, physical growth was correlated with status of iron overload.

Developing anticancer chemotherapy services in a developing country: Hodgkin lymphoma experience

Reporting on how the cancer treatment facilities were developed at a medical college hospital in India and the profile and outcome of patients with Hodgkin lymphoma (HL) at this new center were the objectives of the study.

Immunogenicity and safety of a DTaP-IPV//PRP∼T vaccine (Pentaxim) booster dose during the second year of life in Indian children primed with the same vaccine

ObjectiveTo evaluate the immunogenicity and safety of a pentavalent (diphtheria, tetanus, acellular pertussis, inactivated poliovirus, Hib polysaccharide-conjugate) combination vaccine booster dose.DesignMulticenter, open, Phase III clinical study.SettingTwo tertiary-care hospitals in Delhi and Vellore, India.Participants/patients207 healthy Indian children.InterventionThe DTaP-IPV//PR∼NT vaccine (Pentaxim) was given at 18–19 months of age to children who had been primed with the same vaccine at 6,10,14 weeks of age.Main outcome measuresImmunogenicity was assessed before and 1 month after the booster. Safety was evaluated from parental reports, and investigator assessments.ResultsAt 18–19 months of age, before boosting, the SP rates against diphtheria, tetanus, poliovirus and PRP were 82.3–100%; 90.0% of participants had anti-PRP ≥0.15 μg/mL. Anti-poliovirus titers were ≥1:8 dilution in 97.9–98.4% of participants. Anti-PT and FHA titers ≥5 EU/mL) were detectable in 82.5% and 90.8% of participants, respectively. One month after the booster dose, SP rates were 99.5% for PRP (≥1.0 μg/mL), 100% for diphtheria, tetanus (≥0.1 IU/mL) and polioviruses (≥8:1/dilution). Seroconversion (4 fold post-booster increase in anti-PT and -FHA concentration) occurred in 96.8% and 91.7%, respectively. Geometric mean concentrations (GMC) increased from 11.7 to 353.1 EU/mL and from 18.2 to 363.4 EU/mL for anti-PT and anti-FHA, respectively. Anti-PRP GMC increased from 1.75 to 70.5 μg/mL. Vaccine reactogenicity was low; severe solicited reactions were reported by <1.4% of participants.ConclusionThe DTaP-IPV//PRP-T vaccine booster at 18–19 months of age was well tolerated and induced strong antibody responses.

Determinants of nutritional anemia in adolescents

ObjectiveTo associate the severity of nutritional anaemia with serum levels of ferritin, vitamin B12 and folate; and to determine demographic, socio-economic and nutritional correlates for nutritional anemia in adolescents.MethodsCross-sectional hospital-based study among 200 adolescents (10-18 y) with anemia. Dietary intake (24-h recall), and serum levels of folate, vitamin B12 and ferritin were estimated.ResultsIron, folate and vitamin B12 deficiency was present in 30.5% 79.5% and 50% of adolescents, respectively. Statistically significant association was observed between severity of anemia and serum vitamin B12 levels, iron intake, folate intake, Vitamin B12 intake, vegetarian diet, attainment of menarche and history of worm infestation.ConclusionFolate and vitamin B12 deficiencies are more common than iron deficiency in anemic adolescents. Low dietary intake of these nutrients seems to be a significant determinant of their deficiencies.

Enteric Fever presenting as secondary hemophagocytic lymphohistiocytosis.

Sir, Hemophagocytosis is the pathologic finding of aggressive, but non-malignant proliferation of macrophages and histiocytes [1]. Hemophagocytic Lymphohistiocytosis (HLH) has been associated with a variety of infections and malignancies [2, 3]. However, its association with enteric fever is uncommon. We describe a case of enteric fever with an uncommon complication of secondary HLH (sHLH), responding completely with antibiotic treatment alone. A 10-y-old boy presented with complaints of fever for 5 d and altered consciousness for one day. His younger sibling was admitted to a hospital for the past 10 d as a case of enteric fever. Examination revealed severe pallor, petechie over body, hepatomegaly (5 cm below right costal margin), splenomegaly (3 cm in splenic axis), altered sensorium (E3M1V2) and meningeal signs. Initial diagnosis of meningoencephalitis was kept. Investigations revealed pancytopenia (Hb 6.9 g%, TLC 800/μL, ANC 384/μL, MCV 86.3 fL, platelets010×10/ μL, DLC 0 N48L48E0M4, and normocytic normochromic RBCs). Rest of the investigations, including lumbar puncture were normal. In view of pancytopenia, bone marrow examination was performed which showed features of hemophagocytosis (Fig. 1). His serum triglycerides (265 mg/dl) and serum ferritin (1,791 ng/ml) were high. Thus the diagnosis was established as infection associated hemophagocytosis (IAHS). A widal test done on the seventh day of fever showed positive titres (TO >1:320, TH >1:320) rising from baseline tires (TO <1:40, TH <1:40) done at day two, done from outside. Repeat blood culture and bone marrow cultures were sterile. The patient was managed as a case of enteric fever with encephalopathy and secondary HLH. He received multiple transfusion supports and ceftriaxone for fourteen days. He became asymptomatic by fifth day of antibacterial therapy, his blood counts normalised and he was discharged after completion of antibiotic therapy. Association of sHLH with enteric fever has only been reported in a few cases till date in the English literature [4, 5]. Fatality rate of 50% is present in children with sHLH. The treatment includes etoposide, dexamethasone, and cyclosporine. However our case report stresses the fact that management of underlying infection in cases presenting with severe forms of HLH with enteric fever may obviate the need of intensive treatment. Cytopenias are known in enteric fever due to marrow suppression. However, hemophagocytosis is described as an uncommon extra-intestinal complication of enteric fever [4].

Idiopathic Fanconi's syndrome with nephrogenic diabetes insipidus in a child who presented as vitamin D resistant rickets--a case report and review of literature.

Abstract Fanconi’s syndrome is a complex of multiple tubular dysfunctions of proximal tubular cells occurring alone or in association with a variety of inherited (primary) or acquired (secondary) disorders. It is characterized by aminoaciduria, normoglycemic glycosuria, tubular proteinuria without hematuria, metabolic acidosis without anion gap and excessive urinary excretion of phosphorous, calcium, uric acid, bicarbonate, sodium, potassium and magnesium. Diabetes insipidus is a disease of collecting tubules and a child mainly presents with dehydration and hypernatremia. We report the first case of idiopathic Fanconi’s syndrome along with nephrogenic diabetes insipidus (NDI) in a child who presented to us as resistant rickets. Medline search did not reveal any case of nephrogenic diabetes insipidus associated with idiopathic Fanconi’s syndrome. We hypothesized that the NDI may be due to severe hypokalemia induced tubular dysfunction. The child was treated for hypophosphatemic rickets with severe metabolic acidosis and the treatment for NDI was also given. Now he has healed rickets and normal blood pH, sodium and osmolarity.