Discrete Left Ventricle Outflow Tract Obstruction in Children: Incidence and Predictors of Recurrence. A Multi-Center Study.

OBJECTIVES: The purpose of this study is to measure the incidence of recurrence of discrete subaortic stenosis (DSS) after primary resection in two major cardiac centers in Saudi Arabia and to identify risk factors associated with recurrence. METHODS: Data on 234 patients who were diagnosed with DSS and underwent surgical resection between 1999 and 2018 were retrospectively reviewed. Patient demographics as well as echocardiographic, surgical, and pathological data were compared between patients with recurrence and non-recurrence. RESULTS: The overall recurrence incidence after primary resection was 44.87% (N = 105). Most patients were male (59%). The median age at the 1st operation was 60 months (range 3 months to 133 months). The presence of aortic stenosis at the time of diagnosis was significantly associated with recurrence (p-value = 0.002). The overall median peak gradient in which the primary resection was indicated is 60 mmHg (range 11 to 152 mmHg). The median peak gradient pre-operation and post-operation were significantly higher for the recurrence group (p-value=0.018 and p<0.001, respectively). We used univariate and multivariate analysis and controlled for the follow-up time, but there were no significant independent predictors of recurrence. CONCLUSION: The recurrence rate of DSS after the primary resection is relatively high in this study. Further prospective studies are needed to draw a definite conclusion on risk factors for recurrence after primary resection.

Allogeneic stem cell transplantation in hematological disorders: single center experience from Pakistan.

One hundred and fifty-four patients received allogeneic stem cell transplantations from HLA-matched siblings for various hematological disorders from July 2001 to September 2006. Indications for transplantation included aplastic anemia (n=66), beta-thalassemia major (n=40), CML (n=33), acute leukemia (n=8), and miscellaneous disorders (n=7). One hundred and twenty patients were males and 34 were females. Median patient age was 14 years (range, 1(1/4)-54 years). All patients achieved successful engraftment. Median time to engraftment (ANC>0.5x10(9)/L) was 14 days. Posttransplant complications encountered in our patients included acute graft versus host disease (GvHD) (grade II-IV) 28.5%, chronic GvHD 15.5%, hemorrhagic cystitis 9.7%, VOD liver 5.1%, acute renal failure 3.2%, bacterial infections 51.2%, fungal infections 15.0%, cytomegalovirus (CMV) infection 4%, herpes zoster 4%, tuberculosis 2.6%, Pneumocystis jirovicii infection 0.6%, malaria 0.6% patients, graft rejection 5.2% patients, and relapse in 4% patients. Certain unexpected and rare posttransplant complications were also observed in our patients. These included Hickman catheter embolization, Guillain-Barré (GB) syndrome, deep vein thrombosis, hemorrhagic pericarditis with clots leading to cardiac tamponade, idiopathic polycythemia, dengue fever, and cyclosporine-induced neurotoxicity. Mortality was observed in 27.2% patients. Major causes of mortality were GvHD, VOD, disease relapse, intracranial hemorrhage, acute renal failure, pseudomonas septicemia, tuberculosis, disseminated aspergillosis, and CMV infection. At 5 years, overall survival (OS) and disease-free survival (DFS) rates were 72.5% and 70.7%, respectively.

Cellular immune response in acute hepatitis B leading to chronic carrier state

Immunological studies among jaundiced patients revealed significant changes in T-helper and T-suppressor cells among chronic HBV cases from the acute and control groups. The chronic cases revealed a highly significant decrease in interleukin-2R expression but their low interferon-gamma values were statistically nonsignificant from the control group. The acute cases recorded the highest interleukin-2R and interferon-gamma values. Lymphocyte blastogenesis assay in response to different mitogens and antigens produced two groups: responders [acute cases] and nonresponders [chronic cases]. The responders revealed more intact specific and nonspecific cellular immune responses. Neither group differed with regard to their proliferative response to HBsAg, but vigorous response to HBcAg was a significant feature of the responders

Prostatic aspergillosis.

Prostatic aspergillosis is rare with only 3 cases reported previously. We report a case of localized invasive aspergillosis of the prostate in a nonimmunocompromised patient with chronic urinary retention and recurrent urinary tract infections. Transurethral resection followed by open prostatectomy was performed for massive prostatomegaly. No systemic antifungal therapy was required for cure. The literature is reviewed, and diagnostic and management options are discussed.

IgD myeloma with malignant pleural effusion.

We have described a patient with IgD myeloma and pleural effusions in whom biopsy and postmortem examination demonstrated myelomatous pleural involvement with IgD-positive plasma cells. Despite the presence of two uniformly poor prognostic features (IgD type and malignant effusion) at presentation, this patient survived for two years. This is the first case of IgD myeloma with documented malignant pleural effusion reported in the English literature.