Madu Rao

Effect of transfusion in acute chest syndrome of sickle cell disease

OBJECTIVE To study the effects of transfusion on the clinical course and oxygenation indexes of children with sickle cell disease and acute chest syndrome. METHODS During a 2-year period, 36 children with sickle cell disease admitted with a total of 40 episodes of acute chest syndrome were examined. Patients were given a clinical severity score indicative of the degree of respiratory distress. Arterial blood gas values were determined 4 to 24 hours before and 12 to 24 hours after transfusion, and indexes of oxygenation were calculated; six patients who were not given transfusions also had blood gases measured on admission and approximately 24 hours later for comparison. RESULTS Blood transfusion was administered during 27 episodes (67.5%); 20 children received a simple packed cell transfusion, four had a partial packed cell exchange transfusion, and three had a simple transfusion followed by whole blood exchange transfusion because of worsening clinical symptoms. Although there was no significant change in oxygenation indexes for the six patients not treated with transfusion, there was significant improvement in all indexes after transfusion. The transfused group had more severe disease on admission, but there was no significant difference in duration of fever, tachypnea, retractions, or hospital stay between the transfusion and the nontransfusion groups. CONCLUSION Blood transfusion, even simple transfusion of packed erythrocytes, significantly improves oxygenation in children with acute chest syndrome and is a valuable adjunct to therapy.

Clinical manifestation and outcome of tuberculosis in children with acquired immunodeficiency syndrome.

BACKGROUND Atypical clinical manifestations and rapid progression of tuberculous disease (TB) are well-recognized in adults with the acquired immunodeficiency syndrome (AIDS). There are few reports of children with AIDS and TB. We report the manifestations, clinical course and outcome of 12 pediatric patients with AIDS and TB. METHODS The charts of all children admitted to our institution, from 1989 through 1994, with the diagnoses of AIDS and culture-proved TB were reviewed. RESULTS Twelve children between the ages of 2 months and 13 years fit the criteria. The mean time between the diagnosis of AIDS and TB was 20 months. The most frequent presenting symptoms were fever (75%) and tachypnea (33%). All had negative Mantoux tests (5 tuberculin units of purified protein derivative). Extrapulmonary TB was present in 3 (25%). A source case was identified for 4 (33%). Previous pulmonary disease was present in 7 (58%). Chest roentgenograms were abnormal in 11 (91%), with diffuse interstitial infiltration the most common finding. Susceptibility tests were performed on 10 strains, 3 of which were resistant to 1 or more antituberculosis drugs. Three patients (25%) died of TB, 1 of whom was appropriately treated with antituberculosis drugs but had a strain resistant to isoniazid and rifampin. CONCLUSION Children with AIDS and TB most frequently present with atypical manifestations of TB. A high index of suspicion is needed to correctly diagnose TB in this group of children. Early diagnosis is important because most respond well when treated appropriately.

Allergen Sensitivity and Asthma Severity at an Inner City Asthma Center

The objective of this study was to examine the relationship of allergen sensitivity to asthma symptoms among inner-city asthmatics seen at our Brooklyn, NY, asthma center. We hypothesized that asthma severity would increase for adults and children with increased cockroach and dust mite allergen sensitivity. Data were gathered from retrospective chart review for all patients who were treated at the center with a diagnosis of asthma and had undergone skin-prick testing (SPT) for allergen sensitivity during 1998 (pediatric, n = 79; adult, n = 29). Asthma severity (determined by National Heart, Lung and Blood Institute [NHLBI] asthma severity class) was examined in relation to allergen sensitivity. Allergen sensitivity was measured by percent positive to skin-prick testing as well as by relative mean diameter of skin prick test wheals. For adults, mite sensitivity prevalence was 61% and cockroach sensitivity prevalence was 41%. For children, mite sensitivity prevalence was 49%; cockroach sensitivity prevalence was 42%. For adults, asthma severity correlated significantly with sensitivity to Cladosporium, tree, and grass as measured by percent positive skin tests and by increasing mean diameter of skin test wheals. There was a significant correlation with severity for adult dust mite sensitivity only as measured by increasing mean wheal diameter. Ragweed sensitivity showed a significant correlation with severity only as measured by percent positive skin tests. There was a significant positive association for adults between increasing asthma severity and total number of allergen sensitivities per subject. There was no significant correlation for children between asthma severity and total number of allergen sensitivities per subject. Among children, no specific allergen sensitization showed a significant positive association with asthma severity. By both measures of allergen sensitization, there was a significant negative association for children between Cladosporium and asthma severity. Among our inner-city asthmatic population significant correlation between mite sensitivity and asthma severity was found only in adults. No significant association was seen with cockroach. However, outdoor allergen sensitivity (Cladosporidium, tree, ragweed, and grass) significantly correlated with asthma for adults in this inner city population.

A prospective appraisal of pulmonary hypertension in children with sickle cell disease.

Objectives Pulmonary hypertension (PHT) is a life-threatening complication of sickle cell disease that occurs in 20% to 40% of adults. Measurement of maximal tricuspid regurgitant jet velocity (TRV) by echocardiography provides a noninvasive screening tool; TRV values ≥2.5 m/s are correlated with PHT and increased mortality. Our objective was to estimate the prevalence of PHT in our pediatric sickle cell population and its possible association with various clinical and laboratory findings, including obstructive sleep apnea and/or pulmonary dysfunction. Study Design Eligible children had measurement of the TRV. Clinical data were collected, including detailed history with a standardized sleep apnea questionnaire; those with suggestive histories had polysomonography. Pulmonary function was assessed using whole body plethysmography. Results Of 48 subjects (79% homozygous sickle cell anemia; median age 12 y; 11 receiving chronic transfusion) enrolled in the study, 4 (8.3%) had TRV >2.5 m/s; all had homozygous sickle cell anemia and 1 was receiving hydroxyurea after 3 years of transfusion for secondary stroke prevention. Subjects with elevated TRV had higher indirect bilirubin levels; we found no association between elevated TRV and obstructive apnea or pulmonary function abnormalities. Conclusions Elevation of TRV was relatively uncommon in our pediatric patients as compared with prevalence reported in adults and may be associated with increased hemolysis. There was no association with obstructive sleep apnea or abnormal pulmonary function.

Persisting Perfusion Defects After Bronchoscopic Removal or Spontaneous Expulsion of Aspirated Foreign Objects

Follow-up lung scans were obtained in 9 children to determine whether perfusion lung scanning could be used to verify completeness of foreign-body removal. In 4 patients, extensive perfusion defects were present 4-19 days after removal of the object. In 1 patient, the area of hypoperfusion was larger than the territory served by a persistently obstructed lobar bronchus. Follow-up lung scans are of limited value for verifying completeness of foreign-body removal and localization of retained fragments.

Improved morbidity with the use of nasal continuous positive airway pressure in I-cell disease

Patients with I‐cell disease (mucolipidosis II) present with progressive morbidity failure to thrive, cardiomegaly, and recurrent respiratory tract infections leading to progressive deterioration and early death. We evaluated use of nasal continuous positive airway pressure (NCPAP) for 6 months in a 2‐year‐old girl with I‐cell disease, obstructive sleep apnea (OSA), and craniofacial anomalies. We observed a marked decrease in hospitalizations for respiratory problems and a marked improvement in arterial blood gases with the use of NCPAP. In patients with I‐cell disease, anatomical defects with superimposed upper respiratory tract infections cause worsening of OSA, and OSA contributes significantly to morbidity. In such patients NCPAP can lessen morbidity and can improve the quality of life. Pediatr. Pulmonol. 1998; 25:128–129.

Relationship of Air Pollution to Attack Rate of Asthma in Children

An inverse relationship between daily levels of smoke shade in ambient air and number of visits of children to an emergency clinic for acute episodes of asthma was noted over a period of six months—October 1, 1970 to March 31, 1971. The patient visits were not affected by variation in sulphur dioxide levels, although there was a significant correlation between the latter and the levels of smoke shade.

The use of intravenous isoproterenol in the treatment of status asthmaticus

The use of intravenous isoproterenol in nineteen children with status asthmaticus who did not respond to standard anti-asthmatic therapy is reported. Six of the fourteen patients who responded favorably had normal blood gases within six hours ten within twelve hours and twelve within eighteen hours; all fourteen of the patients who responded favorably had normal blood gases within twenty-two hours after the drug was begun. Clinically, eleven of the fourteen patients who responded favorably showed improvement in respiratory distress within twelve hours of the institution of therapy. All patients who responded favorably showed some improvement within twenty-eight hours of treatment. There were five treatment failures; two were due to cardiovascular toxicity of isoproterenol, and three to progression in symptomatology that necessitated continuous ventilation with respirators.

Electrophysiological Changes in the Heart of Children with Bronchial Asthma during the Acute Attack and Later during Remission

Electrocardiographic and vectorcardiographic changes in the heart were evaluated in 14 children with bronchial asthma during the acute attack and later during remission. Many more abnormalities were noted on the vectorcardiograms as compared to electrocardiograms. The abnormalities noted were in closer relationship to the particular attack rather than to the severity of asthma as revealed by history.Three patients showed evidence of right atrial enlargement during the acute attack, and all of them returned to normal during remission.Ten patients had abnormal orientation of QRS loop on vectorcardiogram during the acute attack, and of these, seven patients continued to have the abnormality during remission. Of the ten patients, five had evidence suggestive of left ventricular enlargement during the acute attack, and all returned to normal during remission. This finding was very intriguing and the possible explanations are discussed.

The Effect of Intradermal and Subcutaneous Route of Administration on Variation in PPD Sensitivity

From the Department of Pediatrics, State University of New York, Downstate Medical Center and Kings County Hospital Center, Brooklyn, NY. * Fellow in Pediatric Chest Diseases; †Assistant Professor of Pediatrics; ‡Assistant Professor of Pediatrics. Correspondence to M. S. Victoria, M.D., Department of Pediatrics, Kings County Hospital Center, 451 Clarkson Ave., Brooklyn, NY 11203. HE INTRADERMAL MANTOUX TEST is the most accurate and reliable method of tuberculin testing. However, a false negative result may be obtained in spite of infection with t~rl. txcbereiclosis.’~~ One of the many reasons given for obtaining a false , negative reaction has been that the test material was erroneously given subcutaneously. 3.7,8 We here report on a comparative study of the skin reactions obtained from the simul-