Reduced sensitivity of PLASMIC and French scores for the diagnosis of thrombotic thrombocytopenic purpura in older individuals.

BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disorder characterized by thrombocytopenia, microangiopathic hemolysis, and ischemic organ failure. The PLASMIC and French TTP scores can help guide clinical decisions when ADAMTS13 testing is not immediately available. Older individuals often present atypically, but the impact of age on these tools is not known. STUDY DESIGN AND METHODS: We calculated the sensitivity and specificity of the PLASMIC and French TTP scores in patients enrolled in the Johns Hopkins thrombotic microangiopathy (TMA) registry. RESULTS: Of 257 patients with TMA enrolled in the registry, we excluded patients less than 18 years of age (n = 19), with prior TMA (n = 81) or who initially presented at another hospital (n = 25). The remaining 132 patients (75 with TTP and 57 with other TMA) were analyzed. Sensitivity of a French score of 2 decreased with age and was 72.2%, 61.5%, and 46.2% for ages 18 to 39, 40 to 59, and ≥ 60 years old, respectively. A PLASMIC score ≥ 5 had higher sensitivity than the French score but this also decreased with age; sensitivity was 91.4% (95% confidence interval [CI], 76.9-98.2), 78.3% (95% CI, 56.3-92.5), and 76.9% (95% CI, 46.2-95.0) for patients 18 to 39, 40 to 59, and ≥ 60 years old, respectively. Older patients had higher platelet counts and serum creatinine than the youngest group, contributing to the loss in sensitivity. CONCLUSION: The PLASMIC and French TTP scores have reduced sensitivity at age ≥ 60 years and are less reliable in identifying TTP in older patients. A high index of suspicion and availability of rapid ADAMTS13 assays is required to correctly diagnose all patients with TTP.

Reduced ADAMTS13 activity during TTP remission is associated with stroke in TTP survivors.

With timely and effective treatment, most patients with thrombotic thrombocytopenic purpura (TTP) survive the acute TTP episode. In addition to the risk of relapse, TTP survivors have higher all-cause mortality than the general population and increased rates of chronic morbidities, including hypertension, depression, and mild cognitive impairment. We conducted this retrospective-prospective cohort study to determine the incidence and prevalence of stroke after recovery from acute TTP and to test the hypothesis that lower ADAMTS13 activity after recovery from TTP is associated with an increased risk of stroke during remission. Of 170 consecutive patients treated for TTP at The Johns Hopkins Hospital from 1995 through 2018, 14 (8.2%) died during the index episode and 19 were observed for less than 1 month after recovery. Of the remaining 137 patients, 18 (13.1%) developed stroke unrelated to an acute TTP episode over a median observation period of 3.08 years, which is fivefold higher than the expected prevalence of 2.6% from an age- and sex-matched reference population (P = .002). ADAMTS13 activity during remission was measured in 52 patients and was >70% in 44.2%, 40% to 70% in 23.1%, 10% to 39% in 25%, and <10% in 7.7%. Stroke after recovery from acute TTP occurred in 0% (0 of 22) of patients with normal remission ADAMTS13 activity (>70%) and in 27.6% (8 of 29) of patients with low ADAMTS13 activity (≤70%; P = .007). In conclusion, stroke is common after recovery from TTP and is associated with reduced ADAMTS13 activity during remission.

Laparoscopic repair of ventral hernia.

OBJECTIVE: To determine the outcome of patients in terms of wound complications and follow-up, after laparoscopic ventral hernia repair. STUDY DESIGN: A case series study. PLACE AND DURATION OF STUDY: Department of Surgery, Dubai Hospital, Dubai, from January 2007 to December 2011. METHODOLOGY: All patients above 13 years of age, who underwent laparoscopic ventral hernia repair, were included. A proceed dual mesh was used for repair of hernia. Multiple parameters were analyzed, including demographic features, presentations, co-morbid conditions, duration of hernia, and defect size. The duration of surgery, postoperative complications, and follow-up of these patients in terms of suture site pain and recurrence of hernia were also analyzed. The data was expressed as frequency, percentages and mean ± standard deviation of values. RESULTS: There were 27 patients with mean age of 47 ± 10.3 years, including 20 female (74.07%) and 7 male (25.95%)patients. All patients presented with abdominal wall swelling. The hernia was partially reducible in 12 patients (44.44%), and completely reducible in 15 patients (55.55%). Seven patients (25.95%) had hypertension, 4 (14.81%) had ischaemic heart disease, and 4 (14.81%) had obesity as co-morbid conditions. All patients underwent laparoscopic hernia repair with proceed dual mesh. The mean defect size of the hernia was 6 cm, and mean duration of surgery was 94 minutes. Early postoperative complications included, seroma in 3 patients (11.11%), and haematoma in one patient (3.70%). The mean follow-up was 23 months. Four patients (14.81) had pain at suture site. CONCLUSION: Laparoscopic repair is an appropriate approach for ventral hernia repair. It results in good repair and low wound complications in terms of haematoma and wound infection. There was no recurrence of hernia in this study.