Pediatric pulmonary multisystem langerhans cell histiocytosis: does lung lesion severity affect the outcome?

BACKGROUND: The pediatric pulmonary multisystem Langerhans cell histiocytosis (PPM LCH) is associated with either low risk or high risk organ(s). The nodulo-cystic lung lesions although pathognomonic, yet are very variable in severity and remain a source of controversy in certifying pulmonary LCH diagnosis. The study aimed to examine the prognostic value of clinical respiratory manifestations and radiological lung lesions severity. This is through associating a CT chest triad of bilateral, extensive and diffuse lesions. It is a retrospective study of 350 LCH patients who received systemic treatment at Children's Cancer Hospital Egypt during the period from 2007 to 2020. RESULTS: Sixty-seven patients (67/350-19.1%) had PPM LCH at presentation. Severe lung lesions were present in 24 of them. The median follow-up period was 61 months (IQR: 3.4-8.3). The 5-year overall survival (OS) and event free survival (EFS) was 89% and 56.6% respectively. The EFS, for severe radiological lesions triad was 38% ± 20.7 versus 66% ± 16.2 for non-severe lesions triad p 0.002, while for presence of chest X-ray changes 27% ± 22.344 versus absence of chest X ray changes 66% ± 14.7 p 0.001, for clinical respiratory manifestations 13% ± 13.9 versus none 62% ± 22.9 p < 0.001, for RO- with severe lung lesions 47% ± 30.4 versus RO- without severe lung lesions 69% ± 5.9 p 0.04. There was a tendency for the independent prognostic impact of severe lung involvement; aHR = 1.7 (95% CI 0.92-3.13, p = 0.09). CONCLUSION: Although the lung is a low -risk organ per se in LCH, our study demonstrates a non negligeable prognostic impact of severe lung involvement in the risk stratification of pediatric LCH. This warrants further study and external validation.

Influenza A (H1N1) virus infection and TNF-308, IL6, and IL8 polymorphisms in Egyptian population: a case-control study.

BACKGROUND: The importance of influenza is increasing mainly because of the appearance of novel pandemic strains such as swine and avian. Each year, influenza has spread around the world causing about 250,000-500,000 deaths and more than 5 million cases of severe illness.The objective is as follows: evaluating the outcomes of patients with influenza A (H1N1) virus in relation to certain TNF-308, IL6, and IL8 polymorphisms and identifying the associated factors with the severe outcome. SUBJECT AND METHODS: This is a case-control study. The cases were patients confirmed by real-time polymerase chain reaction (RT-PCR) to be influenza A (H1N1) virus infected. The controls were healthy individuals. Medical history and outcome of the disease was registered. In all study participants, polymorphisms of TNF rs1800629, IL6 rs18138879, and IL8 rs4073; odds ratio (OR); and the 95% confidence interval (95% CI) were calculated. RESULTS: Infection with influenza A (H1N1) virus was associated more with the following genotypes: TNF-308 AA (OR = 4.041; 95% CI = 1.215-13.4) and IL8 AA (OR = 3.273; 95% CI = 1.372-7.805). According to our study results, HCV (OR = 3.2, 95% CI 1.2-8.5), renal disease (OR = 3.4, 95% CI 0.9-13.6), cancer (OR = 3.1, 95% CI 0.3-31.1), TB (OR = 8.4, 95% CI 1.8-39.7), ICU (OR = 2.9, 95%1.2-7.1), and mortality (OR = 7.9, 95% CI 0.9-67.4) are considered as risk factors for influenza A (H1N1)-infected patients. CONCLUSIONS: Our findings concluded that TNF-308 (AA) and IL8 (AA) polymorphisms may increase the susceptibility to be infected with H1N1influenza virus.

Langerhans Cell Histiocytosis (LCH) in Egyptian Children: Does Reactivation Affect the Outcome?

OBJECTIVE: To report a single centre outcome of management of Langerhans cell histiocytosis (LCH), a clonal disease with involvement of various body systems. METHODS: Retrospective analysis of 80 pediatric LCH patients at Children Cancer Hospital-Egypt between July 2007 and December 2011 was performed. Patients were stratified and treated according to LCH III protocol. The median follow up period was 42 mo (range: 1.18 to 71 mo). RESULTS: At wk 6 and 12, 'better' response was obtained in 61 (76 %) and 74 (93 %) patients respectively. Afterwards, reactivation occurred in 25 patients (38 %), of them multiple episodes occurred in 5 patients (6.25 %), managed by repetition of 1st line treatment for once or more. The 5 y overall survival (OS) and event free survival (EFS) was 96.3 and 55 % respectively. At last follow up, better status was reached in 70 patients, 3 in each 'intermediate' and 'worse' status. Three high risk patients died and one patient was lost to follow up. CONCLUSIONS: In a single Egyptian pediatric LCH experience, the response to treatment is satisfactory and survival remains the rule except in high risk organs disease that still needs a new molecule for salvage. However in multiple reactivations, patients do well with repetition of the 1st line of treatment with or without methotrexate.

Genotyping of Toxoplasma gondii strains from female patients with toxoplasmosis.

Thirty-eight female patients with abortion and intrauterine fetal death were selected from the Obstetric and Gynecology Emergency, Ain Shams University Hospitals, with positive PCR results for toxoplasmosis in previous study. In this study, a rapid and efficient procedure was used for genotyping of T. gondii isolates based on PCR-RFLP assay at SAG2 locus. On the basis of the alleles identified at SAG2 locus, the isolates were grouped into three lineages. Type I was determined by resistance of the 3' & 5' end nested product of the SAG2 locus to cleavage by HhaI & Sau3AI respectively. Resistance of 5' end of SAG2 locus to cleavage by HhaI determined type II. Type III was determined by resistance of the 3' end nested-PCR products of SAG2 locus to cleavage by Sau3AI. Of the 38 isolates, type II was the most prevalent genotype found in 33 (87%). Type I was found in 5 (13%) of the isolates, whereas genotype III was not never found.

Evaluation of semi-quantitative PCR and IgG & IgM ELISA in diagnosis of toxoplasmosis in females with miscarriage.

One hundred female (age 20-42 yrs) patients were classified into group I: 40 patients presented with abortion in the first trimester; group II: 33 patients with abortion in the second trimester and group III: 27 patients with intrauterine fetal death (IUFD). The positive percentages of semi-quantitative PCR and both IgG & IgM ELISA were 38% and 35% respectively. Ten (26.3%) cases out of 38 were positive for toxoplasmosis by both PCR and ELISA-IgG, while 5 (13.2%) cases out of 38 were positive by both PCR and ELISA-IgM, whereas 16 (42.1%) cases out of 38 PCR positive cases were positive by both ELISA IgG & IgM. Sensitivity and specificity of both ELISA IgG and IgM were 81.57% & 93.54% respectively. False negative by ELISA were found in 7 cases out of 38 positive toxoplasmosis cases detected by semi-quantitative PCR. Three cases out of the 7 cases with false negative by ELISA were detected with a trophozoite copy load of 10(1) trophozoite /mL in the blood sample by semi-quantitative PCR. So, the semi-quantitative PCR detected low levels of parasite DNA recommending its usefulness especially in the early stages of the disease when low amount of antibodies can't be detected by serological method or even by the conventional PCR.