Immune thrombocytopenic purpura.

Immune thrombocytopenic purpura (ITP) is the commonest cause of sudden onset thrombocytopenia in a healthy child. The condition is frequently preceded by a viral infection. The hematological parameters are essentially normal except a low platelet count. Bone marrow examination is not routinely indicated except in specific situations. The pros and cons of drug treatment have been discussed as the disease is benign with excellent prognosis in majority of the cases. The various treatment options including low and high dose steroids, intravenous immunoglobulins and anti D have been discussed at length with other modalities of treatment and role of splenectomy. Current therapeutic options with rituximab and other drugs for stimulating platelet production in chronic cases have also been included in discussion.

Autoimmune hemolytic anemia.

OBJECTIVE: To study the clinico-hematological profile and treatment outcome in children suffering from auto immune hemolytic anemia (AIHA). METHODS: Twelve children were diagnosed with auto immune hemolytic anemia over a period of four years. Direct antiglobulin test was positive in all the cases. Other causes of hemolytic anemia like thalassemia syndromes, hereditary spherocytosis, G6PD deficiency were excluded by appropriate tests. The children were followed up for 6 months to 4 years. RESULTS: The age ranged from 7 mth to 9 yr with a mean age of 4.51 yr. All patients had pallor as the presenting complaint followed by splenomegaly (83.3%), jaundice (66.7%), fever (50%) and bleeding manifestations (16.7%). 9 patients had primary disease and 3 had secondary disease. Tubercular infection was seen in 2 patients with secondary disease. Jaundice was seen equally in both the groups. Oral prednisolone produced remission in 83.3% cases. 4 patients (3 in primary and one in secondary group) had relapse after initial response. All responded to a second course of steroids but had subsequent relapses and developed a chronic course. CONCLUSION: Autoimmune hemolytic anemia is an uncommon cause of hemolytic anemia in children. Tubercular infection is an underlying pathology in cases of secondary autoimmune hemolytic anemia. Although oral steroids induce remission in most of the cases, relapses are common.

Correlation between clinical features and degree of immunosuppression in HIV infected children.

We conducted this study to find out correlation of CD4% with clinical status in 102 HIV infected antiretroviral naive children. Mean age of presentation was 4.8 years. Perinatal transmission was the commonest mode of transmission (94%). Fever (53%), chronic diarrhea (36%), and cough (29%) were the commonest presenting symptoms. Protein energy malnutrition was seen in 56.7% of children. 33.3% children were asymptomatic, whereas 45.1% were in WHO clinical stages III and IV at the time of presentation. The most common opportunistic infection was tuberculosis. CD4% correlated significantly with the deterioration of the WHO clinical stages (P<0.01) and increasing grades of protein energy malnutrition (P< 0.05).

A study of bone marrow failure syndrome in children.

Background: Bone marrow failure syndrome (BMFS), or aplastic anemia, includes peripheral blood single cytopenias, as well as pancytopenia due to inability of the marrow to effectively produce blood cells. Aim: To study the clinico-hematological profile and etiological factors of bone marrow failure syndrome in children. Setting and Design: This prospective study was carried out in the Department of Pediatrics of a university teaching hospital over 36 months. Materials and Methods: Children with pancytopenia (Hb 9 /L, platelet count < 100 x 10 9 /L) and bone marrow cellularity < 25% were included in the study. History of exposure to drugs, socioeconomic status, ethnicity and occupation of father were noted. Bone marrow aspiration; trephine biopsy; Ham test; viral studies for hepatitis A, B and C; and cytogenetic investigations were carried out. Statistical Analysis: Relative risk was estimated by odds ratio (OR) with 95% confidence interval (CI) in matched cases and controls. Results: Of the 53 children studied, 6 (11.3%) were diagnosed as Fanconi anemia. Two cases had features of myelodysplastic syndrome. Forty-five children were labeled as acquired aplastic anemia, of whom one had evidence of hepatitis B infection and two patients (5.8%) had paroxysmal nocturnal hemoglobinuria. Aplastic anemia was more common in children from family with lower socioeconomic status; in Muslims; and where the father's occupation was weaving, dyeing and painting. However, the number was small to make statistically significant conclusions. No correlation could be established with exposure to drugs. Conclusion: Fanconi anemia was responsible for approximately one-tenth of the cases of bone marrow failure syndrome. Majority of the patients had acquired aplastic anemia. Hepatitis B infection was an uncommon cause of acquired aplastic anemia.

Newer diagnostic tests for bacterial diseases.

In diagnosing bacterial infections, the rapid identification of bacteremia at an early stage of the disease is critical for a favorable outcome. Furthermore, it is important that exact information be obtained on the stage of the disease rapidly in order to choose and initiate the appropriate therapy. In recent years many new techniques have been added in the diagnostic tools. During the past decade, there has been unprecedented progress in molecular biology as well as in the application of nucleic acid technology to the study of the epidemiology of human infection. Highly sensitive molecular techniques are found to be capable of detecting minute amounts of specific microbial DNA sequences and their complex genetic variations. Moreover, altered levels of biomarkers such as procalcitonin, C-reactive protein, tumor necrosis factor alpha and several interleukins are also found to be promising to define systemic inflammatory response syndrome as indirect evidences of bacterial infections. Lastly, many rapid culture methods are coming up to achieve faster bacterial diagnosis. In this review we will focus on these three newer methods for the early diagnosis of bacterial infections. These approaches will help to expedite the diagnosis of especially early infections and might be a further step towards the improvement of therapeutic methods.

Paroxysmal nocturnal hemoglobinuria in childhood: an uncommon presentation.

An eight year old boy presented with severe anemia and bleeding spots. Complete blood count showed pancytopenia. There was mild reticulocytosis. Bone marrow was hypocellular with normoblastic erythroid hyperplasia. Ham's test (acidified serum test) was positive which confirmed the diagnosis of Paroxysmal nocturnal hemoglobinuria (PNH). Although PNH is rare in childhood, it should be considered as a diagnostic possibility in cases of aplastic anemia as the two conditions can coexist. The presence of PNH in association with aplastic anemia can influence the outcome of the latter.

Abdominal tuberculosis with autoimmune hemolytic anemia.

An eight-year-old male child presented with progressive distension of abdomen, fever, pallor and jaundice with a history of tubercular contact. Investigations were suggestive of abdominal tuberculosis with autoimmune hemolytic anemia. The child responded well to a course of oral steroids with antitubercular treatment. A literature search did not reveal any previous case report of an association between tuberculosis and autoimmune hemolytic anemia.

Perceptual development in relation to nutritional status.

The present study was conducted on 180 children in the age group 5-10 years. These children were divided accordingly their ages into 3 sub-groups i.e. 5-6, 7-8 and 9-10 years respectively. In each group 60 children were studied. For the assessment of perceptual skills each child was tested with the help of Picture Ambiguity Test. Responses to the ambiguous cards were scored with respect to time taken to react in each card and ability of the child to perceive figure and ground relationship i.e. centration and decentration. The observations showed that with increase in age, centration effect reduces and majority of the children start decentring their perception by middle childhood. Further, children in higher age group took lesser time to respond on different ambiguous cards. When the responses of well-nourished and undernourished children were compared for perceptual flexibility in terms of part-whole perception i.e. centration and decentration; no difference was observed between the two groups. There was a significant difference in the performance of well-nourished and undernourished children when time to respond on ambiguous card was compared. Well-nourished children took lesser time to respond on different ambiguous cards. These observations in general suggest that poor nutrition may result in impaired perceptual abilities in children.

Aplasia cutis congenita.

Two cases of aplasia cutis congenita are reported here. One of the cases also had pre- and postaxial polydactyly and other digital anomalies.

Meconium stained amniotic fluid: antenatal, intrapartum and neonatal attributes.

OBJECTIVES: To find out the incidence, outcome as well as antenatal, intrapartum and neonatal attributes of meconium stained amniotic fluid (MSAF). DESIGN: Prospective study. SETTING: Neonatal Unit of Hospital. SUBJECTS: 1426 live births occurring in 1500 consecutive deliveries, over one year period. INTERVENTIONS: In all babies born through MSAF, thorough oropharyngeal suction as soon as the head was delivered followed by immediate intratracheal intubation and suctioning in infants depressed at birth. RESULTS: 204 (14.3%) deliveries had MSAF of which thick meconium was present in 141. Hepatitis in mother, fetal distress during labor and intrauterine growth retardation were significant factors associated with MSAF. One fifth of babies born through MSAF suffered severe birth anoxia compared to 5.6% in non-MSAF group. The consistency of meconium had direct bearing on the neonatal outcome. Severe birth asphyxia (SBA) occurred in 27.0 and 6.3% of babies with thick and thin meconium staining, respectively. Meconium aspiration syndrome was observed in 9 babies of thick meconium group and 8 of these were depressed at birth. All deaths occurred in thick meconium group and were associated with SBA. CONCLUSIONS: Selective approach can be adopted for babies with MSAF reserving intratracheal suctioning at birth for depressed neonate or evidence of fetal distress in utero. Rest of the neonates only need careful observation after thorough oronasopharyngeal suctioning.

Genital standards for south Indian male newborns.

One hundred and thirty-five male newborns in JIPMER hospital were studied. The penile length (stretched and unstretched), width as well as testicular length and breadth were measured. The influence of the gestational age and intrauterine growth on these measurements were studied. The mean stretched penile length for term babies was 3.57 cms (+/- 0.46), the unstretched length 3.26 cms (+/- 0.41), and the width was 1.04 cms (+/- 0.15). There was a linear increase in the above measurements with increasing gestational age. The right testicular length was 1.39 cms (+/- 0.28) and breadth was 0.98 cm (+/- 0.15) while the length and breadth for left testis were 1.32 cms (+/- 0.24) and 0.95 cm (+/- 0.14) respectively for term babies. The prepucial type was tubular in 103 (76.3%) babies and of ring type in 32 (23.7%). In 72 (53.3%) babies the prepuce could be retracted enough to visualise external urinary meatus.

An outbreak of multidrug resistant. Salmonella typhimurium in a nursery.

A nursery epidemic caused by multidrug resistant Salmonella typhimurium is reported. In total, 21 infants developed symptomatic illness; of these, 17 had septicemia (7 blood culture positive) and 4 had diarrhea alone. Asymptomatic carrier state was identified in 13 infants. Male sex and birth asphyxia increased the risk for symptomatic illness. Fever, lethargy, and diarrhea were the most common clinical features. Amongst the septicemic infants there was no difference in clinical profile whether the blood culture was positive or negative for S. typhimurium. In the symptomatic group, S. typhimurium was isolated from feces in 19 cases and from blood in 7 cases. In both symptomatic and asymptomatic infants, all isolates of S. typhimurium, whether obtained from feces and/or from blood, were resistant to ampicillin, chloramphenicol, and trimethoprim, and a significant number (almost one-fifth) of them also showed resistance to third generation cephalosporins. More than 90% of isolates were sensitive to aminoglycosides and ciprofloxacin. On a combination of third generation cephalosporin (cefotaxime or ceftriaxone) and amikacin, 17 (81%) infants recovered, 2 succumbed to their illness, and 2 failed to improve and required ciprofloxacin. The origin of epidemic was traced to a carrier staff nurse working in nursery.

Immunoglobulin G and complement C3 levels in pregnancy induced hypertension.

The immunoglobulin G (IgG) and complement C3 (C3) were measured in the maternal as well cord blood sera of 30 cases of pregnancy induced hypertension (PIH) as well as 9 controls with normotensive pregnancy. A depression of IgG as well as C3 level was observed in the maternal as well as cord sera of the mothers with PIH. These findings suggest decreased immunological status of both mother and her offspring in PIH, irrespective of the gestation and intrauterine growth status.

Audit in maternal and child health.

Audit in medicine is a well tried means of assessing the quality of practice by using acceptable measures of outcome. Audit in maternal and child health has been limited to fatal outcomes such as maternal and perinatal deaths. The outcome of audit is of interest to the providers, the health authorities and the consumers. The utility of audit lies in effective use of data to improve quality of service. Quality control of instruments and education of junior staff are some other benefits of audit. The limitations of manpower, money, means, fear of litigation and above all dislike of clinicians for handling data are hurdles in the way of effective audit. The concept of 'Standard Primipara' and 'Total Fetal Wastage' are likely to add new dimension to perinatal audit.