Familial primary calcific band-shaped keratopathy with late onset systemic disease: a case series and review of the literature

Abstract Background Familial calcific band-shaped keratopathy (BSK) is a very rare disease, with no underlying cause. There is no underlying disease in this form of the disease. This article introduces a family with seven children, three of whom were diagnosed with familial primary calcific BSK. One of them developed a systemic disease 38 years after ocular manifestation. Case presentation In this case report, three Iranian siblings from a family with familial calcific band-shaped keratopathy (BSK) are introduced. Systemic and ocular examinations performed on these patients indicated the occurrence of chronic kidney disease in the older child, a 41-year-old woman, 38 years after ocular manifestation. The examinations conducted on the other two siblings revealed no pathological findings. The 41-year-old sister and 37-year-old brother underwent unilateral deep anterior lamellar keratoplasty (DALK), while the 33-year-old sister underwent bilateral superficial keratectomy (SK). Conclusion Considering the late onset of systemic disease in one of the siblings diagnosed with familial calcific band-shaped keratopathy (BSK), it is crucial to emphasize the necessity of long-term follow-up for these patients and their families

Serum Level of the Angiotensin-Converting Enzyme in Patients with Idiopathic Acute Optic Neuritis: A Case-Control Study

Purpose. To evaluate the serum level of angiotensin-converting enzyme (ACE) as an important component of the renin-angiotensin system (RAS) in optic neuritis (ON) compared to the healthy control group in the context investigating the possible role of ACE in ON pathogenesis. Methods. This case-control study was conducted on patients with ON and healthy controls. Serum ACE levels were assessed and compared between the two groups by using commercially available kits by ELISA for ACE. Results. Sixty-five ON patients (75.4% female, mean age 29.70 ± 8.30 years) and 65 controls (75.4% female, mean age 29.66 ± 8.36 years) were enrolled. The median serum ACE levels were 33.5 U/L (range: 25–540) and 26 U/L (range: 22.3–72) for the ON patients and controls, respectively. Serum ACE levels were significantly higher in the patients than in the control group (P65 U/L) was present in 9 (13.8%) patients with ON and 2 (3.1%) controls. Conclusion. Our results indicated that the serum level of ACE appeared to be significantly higher in acute ON than in normal controls