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.

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.

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.

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.

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.