HbDPunjab is an uncommon variant hemoglobin that does not result in significant pathology when inherited as a homozygous disorder. When inherited with other hemoglobinopathies, it may result in varying disease phenotypes. HbSDPunjab has been rarely reported in Saudi Arabia, coexisting with alpha or beta thalassemia. In this report, we discuss the case of a 39 years old male who presented with severe anemia and renal injury and was later diagnosed with HbSDPunjab through electropheresis and genetic testing.
In the absence of specialized care centers for hemoglobinopathies in Saudi Arabia, there are insufficient data on prevalence of clinical complications in this population. This is a retrospective record review about the clinical complications associated with hemoglobinopathies at King Abdulaziz University Hospital, for patients followed between January 1st 2010 through June 30th 2016. A total of 349 patients were included, with a mean age of 25.45 years, including 266 with sickle cell disease and 80 with thalassemia. Of those receiving regular transfusions, 17.5% developed alloantibodies, 16.6% tested positive for hepatitis C virus antibodies and the mean ferritin level was 2487 ng/ml. Almost half of the patients were screened for renal disease, and more than 50% were found to have proteinuria. Of 203 patients with recent echocardiograms, 15.8% had pulmonary hypertension. Thirty-one (8.9%) patients died at a mean age of 28.5 years. In conclusion, the prevalence of disease related complications is high in hemoglobinopathies. Our results reiterate the need for specialized care hemoglobinopathy centers, which can further improve patient outcomes.
Anastomotic leak (AL) develops in 5–22% of cases following oesophagectomy, and results in additional morbidity, and reduction in overall survival. The management of AL has evolved significantly with advances in endoscopic therapies. Covered cylindrical metal stents have been increasingly used, and insertion techniques have improved over time. We aimed to investigate the effectiveness and safety of modern metal stents in the management of AL.
Methods
All patients who underwent oesophagectomy with thoracic anastomosis between 2006–2020, and subsequently developed AL were retrospectively identified. Initially, treatment details and patient outcomes were assessed for the subgroup of patients where the AL was initially managed using a stent. In all cases, these were cylindrical covered metal stents. Those patients that progressed to surgery after treatment with a stent were then excluded, with the remainder compared to the subgroup of patients that underwent conservative (i.e., non-interventional) treatment for AL.
Results
Of 63 patients with AL, primary management was with stents in 32 (57%). Technical success was achieved in all stenting procedures, although three patients (9%) developed complications (stent migration). After stenting, six cases (19%) subsequently required escalation to operative management (thoracotomy). The remainder (N = 23) were then compared to those managed conservatively (N = 26) and were found to have a significantly longer length of hospital stay (median: 37 vs. 20 days, p = 0.003). The AL healing rate was 96% in both groups (p = 1.000); one conservatively managed patient died before healing, and one stented patient refused further treatment.
Conclusion
Endoscopic treatment of oesophageal AL with cylindrical covered metal stents is safe, with a high technical success rate, and low rates of stent-related morbidity. Treatment with stents also avoids the need for operative management including oesophageal disconnection in the majority of cases, whilst leading to successful healing of the AL. However, treatment with stents does significantly increase hospital stay over conservative management, particularly where multiple procedures are required.
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