Severe combined immunodeficiency (SCID) represents a group of genetically heterogeneous disorders characterized by mutations that lead to profound defects in both humoral and cellular immunity. Transplacental maternal engraftment (TME) is a frequently observed complication in SCID. While most cases of SCID with TME exhibit no substantial impact on disease progression, a subset of patients may encounter diagnostic delays or therapeutic challenges due to TME interference. Furthermore, TME may predispose these individuals to graft-versus-host disease (GVHD) prior to hematopoietic stem cell transplantation, thereby increasing diagnostic complexity and treatment risks. This review systematically examines the etiology and clinical manifestations of SCID associated with TME, analyzes its implications for disease management, and evaluates current detection methodologies. The synthesized evidence provides a theoretical foundation for future research and offers potential insights into the clinical diagnosis and management of SCID associated with TME.
Humans ; Severe Combined Immunodeficiency/diagnosis* ; Pregnancy ; Female ; Hematopoietic Stem Cell Transplantation/adverse effects* ; Maternal-Fetal Exchange/immunology* ; Graft vs Host Disease/etiology* ; Animals ; Placenta/immunology*

Objective To investigate the difference in clinical features and chest HRCT findings between idiopathic nonspecific interstitial pneumonia(INSIP)and connective tissue disease-associated nonspecific interstitial pneumonia(CTD-NISP). Methods Totally 73 cases of NISP from 2011 to 2016 were retrospectively reviewed ,whose final diagnosis all were made after clinico-radiologic-pathologic discussion and 52 cases of them were diag-nosed as INSIP and 21 cases as CTD-NSIP. Clinical features ,lung function test results and chest HRCT findings of INSIP and CTD-NSIP were compared. Results Common underlying diseases of CTD-NSIP were poly-/dermato-myositis(PM/DM),rheumatoid arthritis(RA)and Sjogren syndrome(SS). The mean age of CTD-NSIP[(47.14 ± 9.24)y]was younger than that of INSIP[(59.09 ± 11.20)y](P<0.05). Compared to CTD-NSIP,expectoration was more common in patients with INSIP,while dry mouth/eyes,arthralgia and erythra were less common in INSIP (P < 0.05). Lung function test1 showed restrictive ventilatory dysfunction with dispersion function decline was found in both groups. There were no significant differences in lung function test results between INSIP and CTD-NSIP. In HRCT,the subpleural vertical line was more common in INSIP than that in CTD-NSIP,while patchy consolidation,subpleural curvilinear shadow,pleural effusion and esophageal dilation were less common in INSIP(P<0.05). Conclusions Specific difference of clinical and HRCT features between CTD-NSIP and INSIP are conducive to differentiating the two from each other.