Study of growth differentiation factor-15 in polytransfused children with ?-thalassemia

Background: Ineffective erythropoiesis is a predominant feature in ?-thalassemia major (?-TM), causing marked erythroid expansion leading to highly raised levels of growth differentiation factor-15 (GDF-15), which, in turn, suppresses hepcidin production in liver resulting in increased iron absorption from gut. We aim to study the serum GDF-15 in polytransfused ?-TM patients and its correlation with serum ferritin and serum hepcidin. Method: Thirty-nine polytransfused ?-TM children aged between 5 and 17 years and 33 age- and gender-matched healthy controls were enrolled in the study. Complete blood count, serum GDF-15, serum ferritin, and serum hepcidin were performed. Results: The mean serum GDF-15, serum hepcidin, and serum ferritin levels were 638.65 ± 306.96 pg/ml, 108.21 ± 191.30 ng/ml, and 2274.60 ± 1216.08 ng/ml, respectively, which were significantly higher than control group (P < 0.001, P = 0.003, P < 0.001, respectively). There was significant positive correlation of GDF-15 with blood transfusions (r = 0.415, P = 0.009), positive correlation with serum ferritin (r = 0.653, P = 0), and significant negative correlation with serum hepcidin (r = ?0.508, P = 0.001). Conclusion: The findings of the present study suggest that GDF-15 is an important regulator of hepcidin in ?-TM patients. GDF-15 and serum hepcidin together can be used to monitor iron overload and its related complications in such patients.

Validation of IMNCI Algorithm for Young Infants (0-2 months) in India.

Objective: To check the validity of Integrated Management of Neonatal and Childhood Illness (IMNCI) algorithm for young infants (0-2 months). Design: Prospective observational study. Setting: The outpatient department and emergency room of a medical college attached hospital. Methods: 419 infants (176 between 0-7 days, 243 between 7 days–2 months) underwent a detailed diagnostic assessment and treatment as per the standard protocol of treating unit. These infants also underwent assessment, classification and identification of treatment as per IMNCI algorithm. The diagnostic and therapeutic agreement between standard protocol and IMNCI was computed to assess the validity of IMNCI algorithm. Results: The IMNCI algorithm performed well in identifying sick young infants with sensitivity of 97%, 94% and 95%, and specificity of 85%, 87% and 87% in 0-7 days, 7 days–2 months and 0-2 months age groups, respectively. The algorithm covered majority (80%) of recorded diagnoses, and could identify bacterial infection with 88.5% sensitivity and 57.4% specificity. Complete diagnostic agreement with gold standard was seen in 50%; overdiagnosis and under diagnosis was seen in 13% and 19%, respectively. Low birthweight and upper respiratory infection were the main reasons for overdiagnosis whereas surgical conditions resulted in under diagnoses in majority. Conclusion: IMNCI algorithm for evaluation and management of young infants has good sensitivity and specificity for referring cases with severe illness.

A comparative study of injection placentrex and conventional therapy in treatment of pelvic inflammatory disease.

To compare the effect of placentrex injection given along with conventional therapy, with conventional treatment alone on the symptoms and signs of pelvic inflammatory disease (PID) ie, abdominal pain, dysmenorrhoea and adnexal tenderness, 50 out of 100 women with PID were randomly assigned to receive intramuscular placentrex injection along with two-week conventional therapy and 50 received conventional treatment only. Abdominal pain, dysmenorrhoea and adnexal tenderness were evaluated at the end of 2 months. There was marked reduction in the sign of adnexal tenderness in the placentrex group as compared to conventional treatment group (p < 0.001). Subjective symptoms of lower abdominal pain and dysmenorrhoea were also relieved better in placentrex group (p < 0.01 and 0.05 respectively). This study showed significant and persistent improvement of signs and symptoms of PID in women who received injection placentrex.

Pediatric HIV infection.

OBJECTIVE: The present cross sectional study was undertaken to study clinical profile of HIV infection in children in Northern India. METHODS: 64 children from newborn to eighteen years, presenting for confirmation of diagnosis of HIV infection or monitoring of CD4-CD8 counts in confirmed cases, were evaluated. Children were categorized as per CDC classification of Pediatric HIV. The diagnosis was confirmed by serological tests or PCR assay. CD4-CD8 counts were done by FACS Count. RESULTS: Majority of the children were between 18 months to 5 years. Adolescents comprised 24% of the case. 51.5% children were infected through the mode of mother to child transmission. 39% of the case was transfusion-mediated. Unsafe medical injections probably contributed to 6.2% and heterosexual promiscuity led to 3.1% cases. Clubbing, not described in Indian studies so far, was seen in 9.3% cases. CONCLUSIONS: HIV infection is a chronic childhood disease extending into adolescence, and contaminated blood and unsafe medical injections are still important routes of HIV transmission in India.

Continuous ambulatory peritoneal dialysis (CRPD)--an initial Indian experience.

Continuous ambulatory peritoneal dialysis (CAPD) is a widely accepted mode of renal replacement therapy. CAPD is largely underutilised in India. Twenty patients of chronic renal failure (CRF) were started on CAPD-10 on the standard straight set, 10 on the Y-set. The efficacy of CAPD was evaluated by assessment of laboratory parameters at the onset and after three months of CAPD. Significant improvements in blood, urea, serum creatinine, serum bicarbonate, serum calcium, serum phosphorous, haemoglobin level and blood pressure were observed. There was a significant deterioration in the serum lipid and protein levels on CAPD. Peritonitis was a major complication observed. On the standard set the incidence of peritonitis was one episode per 5.92 patient months compared to one episode per 19.33 patient months with the Y-set (P value = 0.05). Peritonitis occurred more frequently in the summer months. At the end of one year, 70% patients continued on CAPD, with 3 deaths, one each due to malnutrition, myocardial infarction and tunnel infection. Three patients switched over to other replacement therapies. To conclude, CAPD can emerge as a safe, viable mode of renal replacement in developing countries like India.

Neuroglial cyst: a rare occurence in abdomen and pelvis.

We hereby report a case of a child who presented with abdominal distension and urinary retention. Radiological investigations and exploratory laparotomy revealed a cystic mass in the sacral region pushing the urinary bladder anteriorly and upwards. Histopathological examination revealed neuroglial elements. Neuroglial cysts are uncommon outside the central nervous system and this is the first report of its occurrence in abdomen and pelvis.

Refractory anaemia with excess blasts (RAEB) in a child--an unusual clinicohaematological presentation--a case report.

Myelodysplastic syndrome (MDS) is a disease of elderly patients but, rarely can be encountered in pediatric age group. The present case of MDS (RAEB subtype) was reported in a child who presented with proptosis and unique and rare feature of haemophagocytosis by myeloid series of cells.

Pulmonary function tests in beta thalassemia.

To study pulmonary function tests (PFT) in multiple transfusion recipient thalassemics, PFTs were done for 30 thalassemics and 20 matched controls. Confirmed cases of thalassemia on regular transfusion therapy were the subject of study. Apart from history and physical examination of the thalassemics, serum ferritin estimation and spirometry were done. Parameters studied included lung volumes--functional residual capacity (FRC), forced vital capacity (FVC), residual volume (RV) and total lung capacity (TLC); and flow rates--forced expiratory volume in one second (FEV1), forced expiratory volume in 1 second/forced vital capacity (FEV1/FVC), peak expiratory flow 25-75 (PEF 25-75%) and peak expiratory flow rate (PEFR). Single breath carbon monoxide diffusing capacity (DLco) and arterial blood gas (ABG) were also analysed. The mean height and weight of thalassemics were below that of age matched controls. A restrictive abnormality in PFT was found in 86.6% cases. These patients were found to have a decrease in all the lung volumes namely FVC, FRC, RV and TLC with a proportional decrease in the flow rates, FEV1, PEF 25-75% and PEF with a normal (> 0.75) FEV1/FVC ratio. DLco was decreased in all the patients with restrictive lung disease and fall in DLco showed a good correlation (r = 0.7, P < .001) with the severity of restrictive disease suggesting that some intrapulmonary pathology is likely to be responsible for the restrictive pattern. None of the cases had an obstructive or mixed pattern of pulmonary dysfunction. No correlation was found between severity of restrictive disease and the serum ferritin levels. A negative correlation with degree of hepatosplenomegaly was found. No correlation was found between severity of the defect and age, number of blood transfusions received and hemoglobin at the time of doing the test. To conclude, restrictive lung disease is the predominant abnormality in multi-transfused thalassemics, which is probably due to pulmonary parenchymal pathology. The abnormality of PFTs is not directly related to iron overload.

Idiopathic pulmonary hemosiderosis.

Two cases of idiopathic pulmonary hemosiderosis in children are reported. Both cases presented with a combination of acute/recurrent respiratory symptoms along with iron deficiency anemia. In one case diagnosis was delayed for 18 months after onset of symptoms. After initial stabilisation with corticosteroid therapy, both cases were put on chloroquine therapy and showed improvement. Various other forms of therapy and outcome are discussed. It is suggested that in cases with recurrent respiratory symptoms, idiopathic pulmonary hemosiderosis should also be considered after excluding infectious etiology.

Sonographic assessment of renal volume in Indian children.

In an attempt to making norms of renal volume (RV) in Indian children, 240 children (137 boys and 103 girls) below 5 years of age were subjected to sonographic examination in supine position. Left RV in female children increased from 18.04 mm3 in early infancy to 48.36 mm3 in 4-5 year age group. Similar increase was observed in the right RV. There was no significant left vs. right difference in RV in boys and girls (p > 0.05). Similarly, the RV did not differ significantly in both sexes (p > 0.05, in all age groups except 4-5 years, p = 0.04).

An epidemic of dengue hemorrhagic fever and dengue shock syndrome in children in Delhi.

OBJECTIVE: To study clinical profiles and outcome of children of dengue hemorrhagic fever (DHF) and dengue shock syndrome (DSS) during 1996 Delhi epidemic. DESIGN: Retrospective study. SETTING: Hospital based study. METHODS: Children hospitalized from September to November 1996 were studied. All patients were diagnosed, managed and monitored according to a standard protocol. RESULTS: One hundred and thirty four children (80 (60%) males and 54 (40%) females) were studied. Sixty (45%) children were less than 6 years of age of which 12 presented during infancy. There were 92 (67%) cases of DHF and 42 (33%) cases of DSS. Common symptoms were fever (93%), abdominal pain (49%) and vomiting (68%). The commonest hemorrhagic manifestation was hematemesis (39%) followed by epistaxis (36%) and skin bleeds (33%). Hepatomegaly was observed in 97 (72%) cases and splenomegaly in 25 (19%). Serology was positive (IgM hemaglutination antibody titres > 1: 160) for dengue type 2 in 31 (80%) of 39 patients in whom sera was tested during the acute phase of illness. Mortality was 6%. Hematocrit > 40% was observed in only 25 (18%) patients and hence the management protocol was based on clinical signs and symptoms and not on hematocrit. CONCLUSIONS: A management protocol of DHF/DSS in which fluid therapy is not based on haematocrit values needs to be formulated.

Double blind randomized comparative evaluation of nimesulide and paracetamol as antipyretics.

OBJECTIVE: To compare the efficacy and safety of nimesulide and paracetamol as antipyretic agents. DESIGN: Double blind, randomized clinical trial. METHODS: Hospitalized patients having fever due to a variety of infections were given either drug in a randomized manner. (Nimesulide group = 49, Paracetamol group = 50). Serial axillary temperature was recorded after drug administration and side effects monitored. RESULTS: The mean temperature after one hour of drug administration was significantly lower in nimesulide group (p < 0.05). Significantly fewer doses of nimesulide were required to bring down the temperature on the first day (p < 0.001). The mean maximum temperature recorded on second and third day was significantly lower in the nimesulide group (p < 0.05). Adverse reactions were seen in the form of epigastric pain and vomiting in one patient in Nimesulide group and three in paracetamol group. CONCLUSION: Nimesulide is more effective than paracetamol as an antipyretic agent and is safe for use in infants and children.