Emphysematous osteomyelitis caused by Salmonella typhi in beta thalassemia major.

There have been various cases of salmonella osteomyelitis reported in sickle cell anemia. We present a case of emphysematous osteomyelitis caused by Salmonella typhi in a 29-year-old beta thalassemia major patient. Diagnosis of emphysematous osteomyelitis was confirmed by computed tomography and magnetic resonance imaging, and culture of pus drained during surgical debridement confirmed the causative microorganism, Salmonella typhi. Antimicrobials were given according to microbiological sensitivity for a period of 8 weeks. Our patient also received hyperbaric oxygen therapy. At the end of therapy, he was afebrile and laboratory parameters normalized with a residual joint deformity which developed within 3 months.

Moyamoya syndrome in hemoglobin E-beta thalassemia: A rare presentation and association.

Moyamoya disease is an idiopathic, nonatherosclerotic, noninflammatory, chronic progressive cerebrovascular disease characterized by bilateral stenosis or occlusion of the arteries around the circle of Willis, typically the supraclinoid internal carotid arteries, followed by extensive collateralization, which are prone to thrombosis, aneurysm, and hemorrhage. Secondary moyamoya phenomenon or moyamoya syndrome (MMS) occurs in a wide range of clinical scenarios including prothrombotic states such as sickle cell anemia, but the association with other hemoglobinopathies is less frequently observed. We describe a case of a 25-year-old female with hemoglobin E-beta thalassemia who had a rare presentation of MMS in the form of choreoathetoid movements in the left upper and lower extremities. We describe this association, primarily to emphasize thalassemia as an extremely rare but a potential etiology of MMS. Since MMS is a progressive disease, it is important to diagnose and initiate treatment to prevent worsening of the disease and recurrence of stroke.

Management of opportunistic infections in pediatric HIV.

Human immunodeficiency virus (HIV) causes a chronic infection that leads to profound immunosuppression. A hallmark of this process is the depletion of CD4+ lymphocytes, which predisposes the patient to develop a variety of opportunistic infections and certain neoplasms. The CD4+ lymphocyte count is the best validated predictor of the likelihood of developing opportunistic infections (OI). Susceptibility to OI increases as HIV induced immunosuppression becomes more severe. The management of HIV infection hence involves not only inhibiting viral replication using antiretroviral drugs, but also treating OI. This article focuses on the management of such commonly occurring OI in HIV infected patients.

Clinical profile of HIV infection.

OBJECTIVE: To study the clinical profile of human immunodeficiency virus (HIV) infection in children. DESIGN: Prospective. SETTING: HIV clinic at a pediatric tertiary care center in an urban metropolis. METHODS: From August 1994 onwards, 285 HIV positive children were referred to the HIV clinic. These included those intramural deliveries born to HIV positive mothers, those referred from other centers with a positive HIV ELISA (enzyme-linked immunosorbent assay) test and those screened routinely at our center in view of transfusion dependence and found to be HIV positive. After informed consent from either parent, the HIV status of all referred patients was retested by ELISA. RESULTS: Two hundred and thirteen (74.73%) patients were below the age of five years. Vertical transmission as the route of infection was documented in 247 (86.66%), 33 (11.57%) were infected through blood and in 5 (1.75%), the mode of transmission could not be ascertained. The clinical features noted were protein energy malnutrition in 127 (44.56%), pulmonary and extrapulmonary tuberculosis in 84 (29.47%), hepatosplenomegaly in 82 (28.77%), persistent generalized lymphadenopathy in 67 (23.50%), skin lesions in 63 (22.10%), chronic diarrhea in 43 (15.08%), oral thrush in 42 (14.73%), pyrexia of unknown origin in 36 (12.63%), chronic lung disease in 32 (11.22%), chronic hypertrophic parotitis in 27 (9.47%), chronic ottorrhea in 26 (9.12%), recurrent lower respiratory tract infection in 24 (8.42%), neurological manifestations of non-tuberculous origin in 13 (4.56%) and Pneumocystis carinii pneumonia in 11(3.88%). Forty-eight (16.84%) were asymptomatic, 30 (10.52%) died of AIDS during the study period and 39 (13.68%) have been lost to follow up. CONCLUSION: Vertical transmission was the commonest mode of infection. Perinatally infected children become symptomatic by five years of age. Protein energy malnutrition, hepatosplenomegaly and persistent generalized lymphadenopathy were common presenting features. Tuberculosis was the major co-infection. Chronic hypertrophic parotitis and chronic lung disease were distinguishing features of this study. Encephalopathy was associated with poor outcome.

Strategy for preventing vertical transmisssion of HIV : Bombay experience.

OBJECTIVE: To evaluate the efficacy of an interventional regime to reduce the perinatal mode of transmission of human immunodeficiency virus (HIV). DESIGN: Prospective. SETTING: Perinatal HIV clinic at a university affiliated maternity hospital. SUBJECT & METHODS: After adequate counseling, consenting HIV positive women were offered perinatal intervention: (i) administration of 400 mg of zidovudine (AZT) per day for the last 6 weeks of the antenatal period; (ii) delivery by elective Caesarian section before rupture of membrances; (iii) oral AZT powder in the dose of 8 mg per kilogram daily to the infant for the first 6 weeks of life; and (iv) avoidance of breast milk. The infants were scheduled for regular follow-up for at least 18 months. A definitive diagnosis of infectivity in the infant was ascertained by two positive enzyme-linked immunosorbent assays (ELISA) at the age of 9 months and between 15 to 18 months. RESULTS: Of the 107 mother-infant pairs enrolled, 22 infants were lost to follow-up, 15 were under 18 months of age at the time of this analysis and 2 infants died without a diagnosis. Of the remaining 68 infants followed up, 4 tested HIV positive at 18 months. Of the 229 women-infant pairs who did not receive perinatal intervention, 55 infants followed up to 15-18 months were found to be infected. CONCLUSION: This interventional strategy significantly reduced the mother to child transmission of HIV. However, the results need to be substantiated by larger studies.

Perinatally cotransmitted human herpesvirus 6 is activated in children born with human immunodeficiency virus infection.

OBJECTIVE: To evaluate the mother-to-child transmission profile of human immunodeficiency virus (HIV) as well as human herpesvirus 6 (HHV-6) and to examine active replication of HHV-6 in the HIV-infected mothers and their newborns. STUDY DESIGN/METHODS: This polymerase chain reaction (PCR)-based detection was done using DNA from peripheral blood mononuclear cells (PBMCs) and milk cells from the mothers, PBMC from the newborns, and DNA derived from plasma and cell-free milk fluid from mothers and plasma of the newborns. None of the mothers received antiretroviral treatment. RESULTS: HIV was transmitted to 50% newborns and, of 36 total mothers, 8 had actively replicating HHV-6 detectable in their plasma and 2 also had it in the lactosera. Among the neonates. HHV-6 was found in the PBMC DNA of seven and in the plasma fractions of five, the latter five newborns were all HIV-infected at birth. CONCLUSION: Perinatally cotransmitted HHV-6 was always activated in the neonates who were born with HIV infection. Also, HHV-6 can be detected in the milk cells and the activated virus may be present in the lactosera of some of these HlV-infected mothers.

Cellular and humoral factors in colostrum of HIV infected and uninfected lactating mothers.

OBJECTIVE: To compare the cellular and humoral factors in colostrum from HIV infected and uninfected lactating mothers. DESIGN: Cross sectional study. SETTING: Maternity Ward. METHODS: Colostrum was collected from 130 mothers (62 HIV seropositives and 68 HIV seronegatives). These colostrum samples were tested for total cell count, cell viability, differential count, phagocytic activity of macrophages, 'T' cell counts, IgA, IgM and IgG levels. RESULTS: There was a statistically significant decrease in the phagocytosis and 'T' cell number (p <0.001) and in the IgA and IgG levels (p<0. 05) in the colostrum obtained from HIV seropositive mothers as compared to HIV seronegative ones. CONCLUSION: Some of the cellular and humoral factors are reduced in colostrum samples obtained from HIV seropositives as compared to normals.

Role of ethamsylate in preventing periventricular-intraventricular hemorrhage in premature infants below 34 weeks of gestation.

OBJECTIVE: To determine the role of ethamsylate in prevention of PVH-IVH in premature infants <34 weeks gestational age. DESIGN: Prospective, randomized, controlled study. METHODS: Infants less than 34 weeks gestational age were included in the trial. Neonates with congenital malformations, family history of bleeding disorders and with Apgar scores <5 at 5 minutes were excluded. Subjects were randomized into two groups--Group A infants received intravenous ethamsylate (12.5 mg/kg) six hourly for four days and Group B infants served as a control group. Regular cranial ultrasounds to detect the presence of PVH-IVH were done between days 3-5, 10-14 and 28-30 of post natal age, and before hospital discharge in all infants and weekly in infants detected to have PVH-IVH on earlier scans. Various antenatal and postnatal factors known to affect the incidence of PVH-IVH were recorded. RESULTS: A total of 192 infants underwent the trial, 93 in Group A and 99 in Group B. Antenatal corticosteroids (1 or 2 doses) were administered to 32 ( 34.4%) and 36 (36.3%) women in Group A and Group B, respectively. None of the mothers received phenobarbitone, vitamin K or indomethacin antenatally and none of the infants received phenobarbitone, vitamin E or indomethacin postnatally during the study period. PVH-IVH was seen in 26 infants in Group A, of which Grade I IVH occurred in 9, Grade II in 14, Grade III in 2 and Grade IV in one infant. Twenty-nine infants had PVH-IVH in Group B of which 11 had Grade I, 15 Grade II and 3 Grade III. None of the differences were statistically significant. CONCLUSION: Postnatal administration of ethamsylate did not decrease the incidence of PVH-IVH in the study infants.

Experience with intrauterine transfusions for severe Rh alloimmunization in a developing country.

This study reports our experience with 67 intrauterine transfusions (IUTs) carried out for 27 cases of severe Rh alloimmunization, which could be useful to other developing countries with similar situations. Most of the mothers were from sections of India other than Mumbai, their socioeconomic status was low, and they were referred during the second or third trimester. The mean gestation age at first IUT was 27+/-2.9 weeks and maternal anti-D titer ranged from 1:32 to 1:512. Ultrasonography (USG) was normal in eight cases, but showed minimal or gross ascites in 8 and 11 cases, respectively. The mean +/- SD hematocrit (HCT) in three groups defined by USG was 23.5+/-1.7, 15.9+/-4, and 12+/-5.9, respectively. Amniotic fluid analysis, which proved to be an important investigation, indicated IUT in eight cases having normal USG. Six cases were severely anemic (Hb deficit >7 g/dl). By fetal cell staining, the percentage of the donor's red cells in the fetal circulation was determined. Besides Hb, blood group, direct antiglobulin test, and mean cell volume, this parameter was also useful in assessing efficacy of IUT and the need for an exchange transfusion after birth. Of 11 fetuses having gross ascites, eight and one each from the remaining two groups, were stillborn. One death may be procedure related. Two neonates died due to hemorrhagic disorder and prematurity. The overall survival rate was 55.6%. Late referral, severe Rh alloimmunization, volume overload, delay in IUT because of nonavailability of blood and use of nonirradiated blood could be the reasons for the poor outcome. Strategies for improving results are discussed.

Antenatal diagnosis of congenital renal malformations using ultrasound.

Our objectives were to determine the accuracy of antenatal sonography for the detection of congenital renal malformations and to characterize the type of malformations, seen in a 3-year prospective study at a university-affiliated maternity hospital. Participants were 31,217 pregnant women, during the study period, and subjects were 65 fetuses in whom renal malformations were detected on antenatal ultrasound. Pelvic ultrasound scans were performed at least once between 20 and 37 weeks' gestation on all pregnant women attending the antenatal clinic of the hospital for the detection of renal malformations. Fetal urinary sampling, diversion procedures, or termination of pregnancy were carried out as required in those detected to have renal anomalies. Postnatal diagnosis was confirmed by sonography or autopsy. Diagnostic procedures and renal surgery were performed postnatally if indicated. Sixty-five fetuses (0.2 per cent) were diagnosed to have congenital renal malformation antenatally at a mean gestational age of 28.4 weeks. A dilated urinary system was seen in 39, cystic renal disease in 15, agenesis/hypoplasia in six, combined lesions in four, and a horseshoe kidney in one. Oligohydramnios was noted in 20 (31 per cent) pregnancies. Multiple congenital malformations associated with renal anomalies were detected in 12 pregnancies. Termination was carried out at 20 weeks in two pregnancies for lethal malformations; fetal urinary sampling was done in two fetuses with obstructed uropathy, and a vesicoamniotic shunt inserted in one. Postnatal ultrasound confirmed a dilated urinary system in 32, cystic renal dysplasia in 15, renal aplasia/hypoplasia in five, combined lesions in six, and a horseshoe and an ectopic kidney in one each. Five infants were found to be normal. There were seven stillbirths and seven neonatal deaths. Radionuclide scans showed obstruction in nine, decreased renal function in six, and absent renal functions in 10 infants. Micturating cystourethrography demonstrated reflux in 11 and a non-refluxing non-obstructive dilated renal system in five babies. Renal surgery was performed in nine infants. The conclusions drawn from this study were that antenatal detection of renal disease is fairly accurate, even in an extremely busy hospital and certain types of malformations reported in other studies were not observed, despite a large cohort.

Viral infections in newborns through exchange transfusion.

Preprocedure sera of thirty one neonates requiring exchange transfusion were tested for serological markers of HBV, HCV, CMV, HIV and LFT. All the babies were investigated for these parameters one week and two months after transfusion to evaluate the risk of transmission of viral infection. Serological markers for these viral infections were also studied in the mothers and donors' blood to establish the route of infection. Donors' blood used for transfusion was pretested for HBsAg, VDRL and anti-HIV. HBsAg was detected one week post exchange in one baby and two months post exchange in two babies. Exchange transfusion was implicated in two of them, where one donor had HBsAg and the other anti-HBc. Vertical transmission accounted for the remaining one. Out of these HbsAg positive cases, one showed evidence of recently acquired CMV infection. Vertical transmission of anti-HCV was observed in one case. None of the neonates, mothers and donors were positive for anti-HIV. In view of probable serious consequences of HBV and HCV infections, blood used for exchange transfusion ought to be screened for anti-HBc and anti-HCV, besides routine HBsAg, VDRL and anti-HIV screening.

Nursery outbreak of neonatal fungal arthritis treated with fluconazole.

Eight preterm infants with mean gestational age of 31.6 +/- 1.16 weeks and a mean birth weight of 1310 +/- 201.7 g presented at a mean postnatal age of 26 +/- 11.4 days with knee joint swellings and pedal oedema. There was no other clinical, haematological or microbiological evidence of bacterial sepsis. Fungal cultures yielded growth of Candida spp. from blood in five, from urine in four, from cerebrospinal fluid in one, and from all the three babies in whom the joints were aspirated. Radiographic changes of metaphysitis of the involved joints were noted in all. All infants had received prior antibiotic therapy. No infant had received total parenteral nutrition or had central lines inserted. All infants were treated with fluconazole in doses of 7.5 mg/kg/day for 6 weeks. Six of eight were thriving well at 3 months of age without any evidence of residual joint disease. One infant succumbed to disseminated disease and one was lost to follow-up. Candidial arthritis is an uncommon presentation of neonatal candidiasis. Fluconazole therapy proved effective.