No abstract

Background: Nailfold capillaroscopy is a major diagnostic tool for connective tissue disorders like systemic sclerosis. Microvascular complications in diabetes is the major cause of morbidity and mortality which if detected early can improve the quality of life of these patients. Materials and Methods: A prospective, cross-sectional study enrolled 30 diabetic patients for one year. An ophthalmologist examined all participants to rule out retinopathy. All patients subsequently underwent detailed nail fold capillaroscopy (NFC) examination of all ten fingernails with digital Dermatoscope. Data was recorded and presented with proportions. Results: The mean age was 45.88±11.49 years (p-value: 0.009) with 17 (56.1%) males and 13 (43.9%) females. Mean HbA1c was 7.1 (1.6) % (p-value: 0.074). One (1.3%) participant had abnormal nail plate, nail fold, and lunula on examination. In addition, 2 (6.7%) participants had abnormal (ragged) cuticles. The mean capillary density was 6.72±0.38 capillaries per mm (range 5.83-7.24). Tortuosity was the most common observed qualitative change (n=28; 93.3%) followed by meandering capillaries (n=23; 76.7%), capillary dilatation (n=15; 50.0%) and avascular areas (n=14; 46.7%). Conclusion:In diabetic patients without retinopathy, nail fold changes appear before microangiopathic complications like retinopathy. Nailfold capillaroscopy is a potential early screening tool for patients at risk of microangiopathic complications.

Pyruvate kinase deficiency (PKD) is an autosomal recessive defect of the enzyme pyruvate kinase (PK) which is involved in catalyzing a reaction that produces ATP in the glycolytic pathway. It is the most common defect of the glycolytic pathway associated with congenital anemia. Patients usually present with signs of chronic hemolytic anemia such as hyperbilirubinemia, splenomegaly, reticulocytosis, and gallstones; the presentation can vary by age. Diagnosis is usually made by demonstration of decreased PK enzymatic activity in a spectrophotometric assay and on the detection of mutations in the PK-LR gene. Management strategies vary from full splenectomies to hematopoietic stem cell transplants with gene therapies with transfusions and administration of PK-activators coming in between. Thromboembolic complications do occur in patients with splenectomy, but there are not much data regarding this for patients with PKD. We present a case of a patient with PKD who demonstrated priapism to be a thromboembolic complication. This differs greatly as priapism has been frequently reported in patients with other chronic hemoglobinopathies such as sickle cell disease, thalassemia, and G6PD with and without splenectomy. While it is still unclear how splenectomies can result in thrombotic events in PKD, there does appear to be a correlation between splenectomies with resultant thrombocytosis with increased platelet adhesion.