Puberty development among children and adolescents with chronic disease in Saudi Arabia

Increasing numbers of children with chronic health conditions are now surviving into adolescence and adulthood because of advancing health care. These chronic health conditions are generally known to impact a child's growth and development, including pubertal development. In Saudi Arabia, chronic diseases are prevalent, yet no reports of pubertal onset and its relation to chronic illness are available. The aim of this study was to explore pubertal development among Saudi children and adolescents with a chronic illness. Cross-sectional study conducted at schools in Riyadh, Saudi Arabia in 2006. Those students whose parents reported that their son/daughter had a chronic illness and/or was taking a long-term medication underwent a physical examination to determine sexual maturity rating and growth parameters. Of 1371 students who participated in the study, 155 [11.3%] had a chronic illness. Of those, 79 [51%] were male, and the mean SD age of all the students was 11.4 [2.4] years. Ninety [58%] students were taking medication for their health condition. Bronchial asthma was reported to be the most common chronic condition [n=66; 42.6%], followed by blood disorders [n=41; 26.5%]. Fifty-three [34%] students were overweight or obese. For male gonadal [G] development, the mean age of boys with G stage 2 was 11.7 years; stage 3: 13.5 years; stage 4: 14.1 years; and stage 5: 14.6 years. For female breast [B] development, the mean age of girls with B stage 2 was 10.7 years; stage 3: 11.3 years; stage 4: 12.4 years; and stage 5: 14.1 years. The pubic hair development for both boys and girls was similar to the corresponding gonadal or breast development, respectively. The age of onset of pubertal development for both boys and girls with a chronic illness are within normal limits. The high prevalence of overweight and obesity may contribute to this phenomenon, yet further studies should consider the effects of disease severity and chronicity and medication use as possible confounders

Cytomegalovirus infections in unrelated cord blood transplantation in pediatric patients: incidence, risk factors, and outcomes

Stem cells from umbilical cord blood [CB] have increasingly become a viable alternate source of progenitor cells for hematopoietic cell transplantation [HSCT] Cytomegalovirus [CMV] is thought to contribute significantly to HSCT morbidity and mortality. Retrospective case-control study in patients at tertiary care center. We determined the incidence, risk factors and outcomes for CMV infection and disease after unrelated cord blood transplantation [UCBT] in children. Between 2003 and 2007, 73 pediatric patients underwent UCBT and 68% of recipients were CMV seropositive. The overall incidence of CMV infection, early and late CMV infection was 58.9% [43/73], 62.8% [27/43], and 37.4% [1 6/43], respectively. In patients with early CMV infection, 6 of 27 [22%] patients progressed to develop CMV end-organ disease including pneumonitis and retinitis. High levels CMV antigenemia >70 infected cells by pp65 antigenemia assay + PMNs, P-.237 were associated with a higher risk of progression to CMV disease. The development of CMV infections was higher in CMV-seropositive recipients [P<.001] and in those who developed graft-versus-host-diseases [GVHD] [P<.001]. Other risk factors for CMV infection include the use of high-dose corticosteroids [P<.001] and older age of the recipient at the time of transplant [P<.002]. Late CMV infection was strongly associated with a previous history of early CMV infection [P<.001]. CMV infection is a significant complication in UCBT recipients in pediatric patients and is associated with an increase in transplant-related morbidity and mortality. Risk factors for CMV infections after UCBT include GVHD, use of corticosteroids, underlying diseases [hematologic malignancies] and older age. Late CMV infection was strongly associated with a previous history of CMV infection

Morphologic, immunphenotypic and clinical discriminators between T-cell / histiocyte-rich large B-cell lymphoma and lymphocyte-predominant Hodgkin lymphoma

Features of T-cell/histiocyte rich large B-cell lymphoma [THRLBCL] overlap with those of lymphocyte predominant Hodgkin lymphoma [LPHL]. The two lymphomas may represent a spectrum of the same disease, and differentiation between the two can sometimes be difficult. We looked at histomorphologic, immunophenotypic and clinical information that may help differentiate the two entities. Cases of THRLBCL and LPHL were blindly reviewed and studied for histological pattern [nodular vs. diffuse], nuclear features and pattern of expression of CD20, CD30, CD57, epithelial membrane antigen [EMA] and Epstein-Barr virus [EBV]. A score encompassing diffuse histology, high nuclear grade, CD20 single-cell pattern, CD30+, CD57-, EMA-, and EBV+ was estimated for the diagnosis of TCHRLBCL. There were 58 cases, including 30 cases of TCHRLBL and 28 cases of LPHL. The median age was 36 years for TCHRLBCL and 21 years for LPHL [P=0.0001]. Three types of nuclei were identified [lymphocytic/histocytic, Reed-Sternberg and centroblast-like]. The latter two high-grade nuclei were suggestive of TCHRLBCL. TCHRLBCL and LPHL, respectively, showed diffuse histology, 90% vs. 4% [P=0.001], single CD20+ cells, 93% vs. 3.5% [P=0.00004], CD30+ cells, 30% vs. 0% [P=0.01], CD57+ cells, 41% vs. 93% [P=0.008], EMA+ cells, 27% vs. 60% [P=0.113], EBV+ cells, 24% vs. 0% [P=0.117], high nuclear grade, 70% vs. 0% [P=0.001], total score 2-7 [mean 4.68] vs. 0-2 [mean 0.72] [P=0.001], high stage, 86% vs. 7% [P=0.0001]. Our findings indicate that a combination of multiple parameters can help differentiate between the two diseases. Two cases originally diagnosed as LPHL were re-assigned the diagnosis of THRLBCL

18 years follow-up results of mitral balloon valvuloplasty in 531 consecutive patients and predictors of long-term outcome

Long-term echocardiographic follow-up studies of mitral balloon valvuloplasty [MBV] are scarce. The study aim was to assess the long-term results [up to 18 years] of MBV and to identify predictors of restenosis and event-free survival. The immediate and long-term clinical and echocardiographic results for 531 consecutive patients [mean age 31 +/- 11 years] who underwent successful MBV for severe mitral stenosis [MS] and were followed up for a mean of 8.5 +/- 4.8 years [range: 1 .5 to 18 years] after MBV are reported. Immediately after MBV, the mitral valve area [MVA] was increased from 0.92 +/- 0.17 cm[2] to 1.95 +/- 0.29 cm[2] [p< 0.0001]. Restenosis occurred in 165 patients [31%], and was less frequent [19%] in patients with a low mitral echo score [MES 8 [p< 0.001]. Event-free survival [death, redo MBV, mitral valve replacement, NYHA class III or IV] at 10, 15 and 18 years was 88 +/- 1%, 53 +/- 4%, and 21 +/- 5% respectively, and was significantly higher for patients with MES 8 [p< 0.0001] and previous surgery [p= 0.043] as predictors of restenosis, and MES >8 [p< 0.0001] and baseline atrial fibrillation [p=0.03] as predictors of combined events. MBV provides excellent long-term results for selected patients with MS. The long-term outcome of this procedure can be predicted from the baseline clinical and echocardiographic characteristics of the mitral valve

Grading of follicular lymphoma using flow cytometry

The treatment and prognosis of follicular lymphoma [FL] is dependant on the grade of the disease. In the World Health Organization classification of lymphoma, grading of FL into low grade [1 and 2] and high grade [3] is recommended. Grading of FL is possible in excision biopsy; histological grading is subjective and inconsistent Grading is extremely difficult in needle core biopsies and fine needle aspirates. We attempted to grade FL using flow cytometry [FCM] and CD 19/forward scatter. Cases of FL seen in our institution and submitted for FCM were evaluated for the percentage of cells detected beyond the 500-channel mark [on a 1024 scale] on a CD19/forward scatter dot plot. We hypothesized that these cells most likely represent centroblasts and their percentage would reflect the grade of the disease. Histological grading of the lymphoma on the open biopsies constituted the reference for FL grade. Thirty-six cases of FL, including 22 males and 14 females, ranging in age from 19 to 92 years [median, 42 years], were studied. There were 17 cases of low grade [grade 1; n=10 and grade 2; n=7] and 19 cases of high grade [grade 3] FL The percentage of cells identified beyond the 500-channel mark on CD19/forward scatter dot plot ranged from 0.12% to 12.55% [median, 4.9%] in low grade [grade 1 and 2] whereas the percentage of those cells in high grade FL ranged from 6.22% to 51.95% [median, 21%; p=0.00001]. Our findings suggest that using a CD19/forward scatter dot plot can help identify centroblasts in FL making grading possible on FCM, especially in small biopsies and fine needle aspirates

Biological markers in Helicobacter pylori-associated gastritis and carcinoma : the value of a scoring system

Background: Helicobacter pylori-associated gastritis has been linked to the pathogenesis of gastric adenocarcinoma [GA], especially when associated with intestinal metaplasia [IM] and atypia/dysplasia [A/D]. We examined p53 expression, ploidy and proliferative activity and assessed H. pylori infection in relationship to IM and/or A/D in cases of gastritis not associated with GA and in cases of GA

Methods: We examined 53 gastric biopsies from patients with gastritis not associated with GA, including patients with gastritis not associated with IM and/or A/D [n=35] and with gastritis associated with IM and/or A/D [n=21]. Thirty-six distal gastrectomy specimens from patients with GA constituted a third group of patients. A scoring system that encompassed the presence or absence of H. pylori, degree of gastritis, IM and/or A/D, p53, MIB-1 proliferative index [MPI] and ploidy was estimated in the cases of gastritis and in cancer-associated mucosa [CAM] and the adenocarcinoma from patients with GA

Results: Patients with GA had a higher median age than those with gastritis without IM and more were males [ratio, 2.2:1]. H. pylori was detected in 75% [40/53] of gastritis specimens and in 55% [20/36] of GA cases. There was a statistically significant difference between the incidence of gastritis without IM and/or A/D and CAM [P=0.01]. p53 expression was seen in 67% of cases [14/21] of gastritis with IM and/or A/D and in only 5% [2 cases] of gastritis without IM [P=0.0005]. A statistically significant difference in MPI was seen between CAM and GA [P=0.01] and gastritis without IM and/or A/D and gastritis with IM [P=0.004]. Cases of gastritis without IM and/or A/D had a median score of 8 while cases of gastritis with IM and/or A/D had a median score of 12 [P=0.0003]. CAM had a median score of 13, which was significantly different than gastritis without IM and/or A/D [P=0.0003]

Conclusion: The presence of IM and/or A/D can be used in H. pylori-associated gastritis as a starting point to further investigate high-risk lesions. Those showing p53 expression, high proliferative activity and aneuploidy require closer follow up and perhaps additional biopsies. Although aneuploidy is commonly seen in GA, its presence in cases of gastritis as an isolated finding should not indicate a high-risk lesion