Interpupillary distance in a normal black population.

This report expands on a study by Pryor [Pryor HB. Objective measurement of interpupillary distance. Pediatrics 1969: 44: 973 977] that related normal values of inner canthal distance (ICD), outer canthal distance (OCD) and interpupillary distance (IPD) for Whites, Asians and Mexican Americans. To date, no similar values have been reported for Blacks. Utilizing a sample (n = 931: 485 males; 446 females) of black people (range, birth 24 years), OCD, ICD, and head circumference (HC) were measured and tabulated. We calculated mean IPD according to Pryor's formulation and report that the general mean OCD and ICD in our sample differed significantly from, and were consistently higher than, Pryor's reported measurements for White males and females at each age level (p < 0.001). However, ICD in our sample was significantly lower at birth in both sexes, appeared to increase at a more rapid rate relative to Whites during the first 3 months of life, and reached and maintained a higher value beyond the age of 3 months, with most age groups showing a significant difference in mean ICD measurements. At each age level, the mean IPD values in Whites and Blacks were significantly higher (p < 0.001). Based upon these findings, we suggest that interpupillary distance of Black children and adults be assessed according to the mean proportions for their race.

Nasopharyngeal carriage of penicillin-resistant Streptococcus pneumoniae in children with sickle cell disease.

OBJECTIVE: We studied the prevalence of nasopharyngeal (NP) carriage, antimicrobial susceptibilities, and serotypes of Streptococcus pneumoniae (SP) in children with sickle cell disease (SCD) in the Mid-South. In addition, we examined risk factors for NP carriage of penicillin-resistant SP (PRSP). STUDY DESIGN: Between July 1994 and December 1995, we obtained NP cultures from 312 children with SCD followed at the Mid-South Sickle Cell Center, 208 (67%) of whom were receiving penicillin prophylaxis. RESULTS: Among the 312 patients, colonization with SP occurred in 42 (13%), 30 (71%) of whom were receiving penicillin prophylaxis. Twenty-three of the 42 SP isolates (55%) were resistant to penicillin; 5 of the 23 (22%) were highly resistant. PRSP organisms were also resistant to cefotaxime (43%), trimethoprim-sulfamethoxazole (57%), and erythromycin (22%). Serotypes 6A, 6B, 14, 19A, and 23F accounted for 19 (90%) of 21 resistant strains. Children who were treated with antibiotics during the preceding month were more likely to carry PRSP than children who were not treated. CONCLUSIONS: There is a high prevalence of NP carriage of PRSP in children with SCD in the Mid-South, which raises concerns regarding the continued effectiveness of penicillin prophylaxis in these children. Further studies on the antimicrobial susceptibilities of resistant organisms and the relationship between NP carriage of SP and invasive disease are needed before developing new recommendations for prophylaxis and treatment.

Penicillin- and cephalosporin-resistant strains of Streptococcus pneumoniae causing sepsis and meningitis in children with sickle cell disease.

OBJECTIVE: We investigated the possibility that antimicrobial-resistant pneumococci were causing invasive disease in children with sickle-cell disease (SCD). STUDY DESIGN: Records of all children with SCD observed at the Mid-South Sickle Cell Center (MSSCC) at LeBonheur Children's Medical Center were reviewed from January 1990 to June 1994. Children with SCD and pneumococcal sepsis were identified. The Streptococcus pneumoniae isolates from these children were examined for serotype and antimicrobial susceptibilities. Two additional children not observed in the MSSCC had pneumococcal sepsis caused by penicillin-resistant isolates and were also included. RESULTS: Antimicrobial susceptibility testing of the six penicillin-resistant isolates revealed that four were resistant to trimethoprim-sulfamethoxazole, two to erythromycin, and one to clindamycin. The two isolates that were resistant to ceftriaxone also were multiply resistant. From the MSSCC, 26 children had pneumococcal sepsis during the 4 1/2-year period studied. Five of these children (19%) died. Four (15%), including one who died, were infected with penicillin-resistant strains. CONCLUSION: Pneumococcal sepsis, meningitis, and infections of other foci in children with SCD may be caused by S. pneumoniae that is resistant to one or more antimicrobial agents, including penicillin. The addition of vancomycin to the antibiotics currently used for initial management should be considered in areas where the antibiotic resistance of S. pneumoniae is prevalent.

Laparoscopic splenectomy in childhood.

The authors describe their technique for laparoscopic splenectomy in childhood. Five ports, including one 12-mm port for an endoscopic stapler, are placed. With the patient in the supine position, the short gastric vessels are divided between clips. The patient is then placed in the lateral decubitus position for mobilization of the splenic flexure of the colon, division of the posterolateral ligamentous attachments, and en masse transection of the splenic hilum using the EndoGIA stapler. The freed spleen is placed in a nylon reinforced Lap Sac, which is exteriorized at the neck. The spleen is morcellated and the sac removed. Concomitant cholecystectomy is performed in patients with hereditary spherocytosis who have cholelithiasis or sludge. The procedure has been performed without complication in six patients who had hematologic disorders. For another patient, the procedure was converted to an open splenectomy to achieve better hemostasis.

Laparoscopic splenectomy.

Splenectomy is easily amenable to laparoscopic technique. Compared with the open technique, its advantages include improved exposure, decreased pain, improved pulmonary function, shortened hospitalization, rapid return to unrestricted activities, and improved cosmetic appearance. These advantages are at the expense of prolonged operative time that, with experience and improved instruments, should diminish.

Auditory function in sickle cell anemia.

Auditory acuity and central auditory processing were assessed in 22 patients with sickle cell anemia, 13 of whom were chronically transfused, and compared with a control black population. Pure tone air conduction thresholds were within normal limits for all patients, and mean Speech Reception Threshold for each ear was normal at 10 dB. All subjects exhibited type A tympanograms. Central auditory processing was assessed by the Competing Sentence Test and Binaural Fusion Test. No significant differences were found among transfused, nontransfused, and control patients. Abnormal auditory function does not appear to be a common problem in patients with sickle cell disease.

Prophylaxis with oral penicillin in children with sickle cell anemia. A randomized trial.

Children with sickle cell anemia have an increased susceptibility to bacterial infections, especially to those caused by Streptococcus pneumoniae. We therefore conducted a multicenter, randomized, double-blind, placebo-controlled clinical trial to test whether the regular, daily administration of oral penicillin would reduce the incidence of documented septicemia due to S.pneumoniae in children with sickle cell anemia who were under the age of three years at the time of entry. The children were randomly assigned to receive either 125 mg of penicillin V potassium (105 children) or placebo (110 children) twice daily. The trial was terminated 8 months early, after an average of 15 months of follow-up, when an 84 percent reduction in the incidence of infection was observed in the group treated with penicillin, as compared with the group given placebo (13 of 110 patients vs. 2 of 105; P = 0.0025), with no deaths from pneumococcal septicemia occurring in the penicillin group but three deaths from the infection occurring in the placebo group. On the basis of these results, we conclude that children should be screened in the neonatal period for sickle cell hemoglobinopathy and that those with sickle cell anemia should receive prophylactic therapy with oral penicillin by four months of age to decrease the morbidity and mortality associated with pneumococcal septicemia.

Lymphocyte subsets in children with aplastic anemia.

Twenty-four patients with aplastic anemia (AA) were evaluated with immunologic studies. Patients with posthepatitis AA had decreased proportions of T4 cells, increased proportions of T8 cells, and decreased T4:T8 ratios. Patients with constitutional AA had varied immunologic findings. There were no significant abnormalities in lymphocyte subsets in patients with idiopathic AA. Of the 12 patients who were treated with antithymocyte globulin (ATG), six (50%) responded. Nine of these patients are living 3-27 months after treatment; three of the nine are transfusion dependent. T-cell subsets were not helpful in predicting the response to ATG. Measurements of other immunologic mediators may be more useful with regard to pathophysiology and prognosis in AA.

Lymphocyte phenotype and function in chronically transfused children with sickle cell disease.

Immunologic studies were performed on mononuclear cells from ten chronically transfused children with sickle cell disease, and the results were compared with those from five other groups: 21 sickle cell patients who were not receiving regular transfusions, 6 chronically transfused children with other forms of refractory anemia, 10 previously splenectomized children, 5 boys with hemophilia A, and 27 normal adult controls. The helper:suppressor T cell ratios (T4:T8) in all groups except hemophiliacs were normal, were unrelated to the number of units transfused, and were not suggestive of findings reported for patients with the acquired immune deficiency syndrome (AIDS) or groups at risk for the syndrome. Percentages of T3, T4, and T8 cells were low in sickle cell and splenectomized patients, but not in chronically transfused patients with other anemias. Serum IgG was frequently elevated, and IgG synthesis in vitro was increased relative to IgM synthesis in sickle cell patients. Coculture experiments indicated that such findings may stem from a selective increase in IgG synthesis by B cells. Thus, transfused sickle cell patients have a particular pattern of immunologic abnormalities that is distinct from that seen in AIDS.

Hemothorax and hemomediastinum in patients with hemophilia.

Three patients with hemophilia presented with hemothoraces which resolved within 1-3 weeks following treatment with factor concentrates. Drainage of blood was not necessary. Eight previously reported cases are reviewed, and therapy and complications are summarized. We suggest that treatment of this rare complication of hemophilia with factor concentrates and avoidance of surgical intervention is indicated.

Serial cell culture and cytogenetic studies of marrow cells from a patient with monosomy 7 syndrome.

Serial bone marrow cell culture studies, with cytogenetic characterization, were performed in an 18-month-old boy with monosomy 7 syndrome--a condition that usually converts to nonlymphoblastic leukemia. Over a 12-month course, his marrow demonstrated: decreased colony formation and increased cluster formation in vitro with disease progression (consistent with preleukemia); persistence of only the monosomy 7 karyotype in direct marrow studies; and in contrast, proliferation of both 46,XY and monosomy 7 cells in in vitro marrow cultures. The findings indicate that certain dormant marrow cell populations may be expressed under in vitro culture conditions and suggest the need for determining factors which would allow their expression in vivo.

Cyclic ultrastructural abnormalities in human cyclic neutropenia.

The ultrastructure of neutrophils from two brothers with cyclic neutropenia was studied at various time intervals of the neutrophil cycle, which varied from 15 to 21 days. During the nonneutropenic portion (middle third) of the cycle, when the blood neutrophil counts approached normal, the majority of blood and marrow segmented neutrophils appeared morphologically normal, whereas marrow promyelocytes demonstrated increased autophagy with decreased condensation of primary granules. During the early neutropenic portion of the cycle (first third), increased autophagy and granule abnormalities persisted in promyelocytes, myelocytes, and the few segmented neutrophils present. During the late neutropenic portion (last third) of the cycle, which preceded neutrophil recovery, most promyelocytes appeared morphologically normal, whereas the few segmented neutrophils present frequently contained increased autophagic and/or heterophagic vacuoles and decreased cytoplasmic granules. Abundant neutrophil debris was present in marrow macrophages consistent with dysgranulopoiesis and intramedullary destruction of abnormal myeloid cells. Similar changes were not observed in monocytes, eosinophils, or basophils. These results demonstrate a cyclic insult which has its initial morphologic impact on the promyelocyte of patients with cyclic neutropenia.

Immunoregulatory abnormalities in Evans syndrome.

Immune function in six patients with Evans syndrome (Coombs-positive hemolytic anemia and immune thrombocytopenia) was compared to that in seven with chronic ITP. The two groups differed in measurements of T-cell subsets and immunoglobulin production. Evans syndrome patients had decreased T4 (T-helper) (P = 0.025), increased T8 (T-suppressor) (P = 0.008), and a decreased ratio of T4:T8 cells (P = 0.0009) when compared to controls. Results in chronic ITP patients were similar to those in controls. Serum IgG, IgM, and IgA levels and in vitro synthesis of IgG and/or IgM were decreased in most Evans syndrome patients. Diminished in vivo and in vitro immunoglobulin synthesis in Evans syndrome is consistent with the decreased T4:T8 ratio in these patients. The altered T4:T8 ratio may represent an unsuccessful response to an autoimmune process in which the trigger is unknown.