#hemoglobinosis

[jcrmhscasereports]

Sickle cell nephropathy, a complication not to be ignored, through a Moroccan case by Asmaa Biaz in Journal of Clinical Case Reports Medical Images and Health Sciences 

SUMMARY

Nephropathy is a major complication of sickle cell disease. Indeed, the kidneys are particularly sensitive organs to this disease.

We report a case of a patient with a major sickle cell syndrome; she was hospitalized in the nephrology department of Mohammed V Military Training Hospital, forend-stage renal failure. The family investigation revealed a composite S/O-Arab heterozygosity responsible for the severity of the clinical disorder.

Key words: Sickle cell nephropathy - End stage renal failure - Sickle cell major syndrome S/O-Arab.

INTRODUCTION

Sickle cell nephropathy (SCN) is a major complication of sickle cell disease. It manifest’s in various forms, including glomerulopathy, proteinuria, hematuria, and Renal tubular disorders, and frequently results in end-stage renal disease(ESRD). Hemolysis and vascular occlusion are the main factors promoting the manifestations of this disease. Dialysis and renal transplantation are the last resort for patient with SCN [1].

Through the case of a patient with a major sickle cell syndrome S/O-Arab complicated by end-stage renal failure, we will explain the pathophysiological mechanisms of this complication and emphasize the importance of biological monitoring.

CASE REPORT

The patient was 24 years old and was admitted to the nephrology department of the Mohammed V Military Training Hospital for incidental renal failure in the context of an impure nephrotic syndrome revealing sickle cell nephropathy. In his history, we retained a

follow-up since the age of 5 years in another hospital structure for a hemoglobinosis S treated by iterative transfusions with notion of acute renal failure during sickle cell crises.

The biological result showed an anemia at 7.7 g/dL, corrected serum calcium at 82 mg/L, serum phosphorus at 64 mg/L, intact parathyroid hormone 1-84 at 543 pg/L, Alkaline Phosphatase at 201 U/L.

Hemoglobin electrophoresis was ordered to this patient, but due to repeated transfusions, her electrophoretic profile remains uninterpretable (Figure 1). Therefore, hemoglobin electrophoresis (HBE) was performed in the parents as part of the hemoglobin phenotypic study. The HBE of both parents is performed on Capillarys (Sebia®) at alkaline pH followed by electrophoresis at acidic pH on Hydrasys (Sebia®) which showed a heterozygous Hb O-Arab variant in the mother (Figure 2) and a heterozygous hemoglobinosis S (A/S) in the father (Figure 3).

Figure 1: Electrophoretic profile of the patient uninterpretable due to recent transfusion

Figure 2: Electrophoretic profile of the mother showing hemoglobinosis O-Arab in the heterozygous state

Figure 3: Electrophoretic profile of the father showing hemoglobinosis S in the heterozygous state

Referring to the phenotypic study of Hb performed in the parents, it is concluded that the patient has a composite heterozygosity S/O-Arab explaining the severity of the renal manifestations.

The evolution was marked by the absence of improvement of her renal function and the aggravation of the uremic syndrome motivating her setting in peritoneal dialysis. The patient was treated with erythropoietin ARANESP 30µg/ per 2 weeks with a blood transfusion of 2 packed red blood cells on average every two months.

The patient died at the age of 26 years before benefiting from either a hemoglobin genotyping study or a renal transplant.

DISCUSSION

Sickle cell disease is the most common hereditary hemoglobinopathy in the world. An estimated 300,000 children are born with this disease each year, three quarters of whom are born in sub-Saharan Africa [2]. It is characterized by extreme variability in terms of clinical manifestations, the most serious of which are renal manifestations.

The association S/O Arab is responsible for a major sickle cell syndrome, as in the case of our patient. Indeed, Hb O Arab stabilizes the intracellular polymerization of Hb S and leads to an irreversible sickle cell disease of red blood cells, thus expressing by a more severe clinical disorder. The clinical and biological manifestation of this association is similar to homozygous sickle cell disease and the association Hb S / Hb D Punjab. The onset is usually early, in infancy, and is marked by the classic triad of chronic hemolysis: anemia, jaundice and splenomegaly. Anemia is usually moderate outside of hemolytic attacks (Hb = 7 - 10 g/dL). The evolution is often marked by sickle cell complications. Osteoarticular complications are the most frequent, such as vaso-occlusive crises, septic arthritis and osteoporosis. Pneumonia, leg ulcers and vesicular lithiasis are also reported [3].

Sickle cell nephropathy is a major complication of sickle cell disease. The kidneys are particularly sensitive organs to the disease. Sickle cell disease substantially alters the structure and function of the kidneys and is the cause of several renal diseases and syndromes. Renal damage is more severe in SS homozygous patients than in other major sickle cell syndromes [4]. Approximately 5-18% of patients have SCN, thus increasing the risk of morbidity and mortality of the disease [5].

A number of studies have focused on this pathology, its evolution includes several stages; it starts with hyperfiltration, then the occurrence of microalbuminuria, then macroalbuminuria and finally the progression to renal failure. The prevalence of these complications increases with the age of the patients but can also be seen from a young age.

Two models have been proposed to explain the pathophysiology of SCN. Becker et al. showed that prostaglandin release following ischemic injury causes an increase in glomerular filtration rate (GFR). This increase leads to glomerular injury and eventually manifests as proteinuria and glomerulosclerosis [6]. Alternatively, Nath and Katusic [7] classified the manifestations of SCN into two different phenotypes: the hemolysis-endothelial dysfunction phenotype and the viscosity-vaso-occlusive phenotype. The hemolysis-endothelial dysfunction phenotype affects the renal cortex and leads to hyperfiltration and glomerulopathy; heme released due to intravascular hemolysis predisposes to proteinuria through its accumulation on the glomerular filtration barrier, which disrupts membrane selectivity by exerting cytotoxic effects on podocytes and endothelial cells [8]. On the other hand, the viscosity-vaso-occlusive phenotype is responsible for hematuria, papillary necrosis, and tubular acidosis [9].

Microalbuminuria, reflecting the early stages of renal damage, should be routinely sought in the follow-up assessment in this category of patients.

Decreased GFR, which suggests loss of kidney function, occurs with the progression of sickle cell disease and may be a sign of uncontrolled disease. Sickle cell patients have a higher risk of developing chronic kidney disease compared to the general population.

Specific treatment with an angiotensin-converting enzyme inhibitor or an angiotensin II

receptor antagonist should be considered in these patients in order to slow the progression of the renal disease. The prevention of microthrombosis and thus of renal damage requires the maintenance of a hemoglobin A level of more than 50% by regular blood transfusion as soon as sickle cell disease is diagnosed. The role of hydroxyurea in the prevention and/or treatment of renal function abnormalities in sickle cell disease remains to be studied [10].

CONCLUSION

Sickle cell nephropathy is a major complication of sickle cell disease. It must be systematically and early sought in all sickle cell patients to reduce the risk of morbidity and mortality of this disease.

The development of new biomarkers has become increasingly essential for the early detection of sickle cell disease in order to improve the survival of patients with sickle cell disease.

Declaration of interest:

The authors declare no conflict of interest.

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[giresearch]

Adult Hemoglobinopathy Testing Market by Major Key Players,Competitive landscape and Forecast to 2030

On 2024-7-26 Global Info Research released【Global Adult Hemoglobinopathy Testing Market 2024 by Manufacturers, Regions, Type and Application, Forecast to 2030】. This report includes an overview of the development of the Adult Hemoglobinopathy Testing industry chain, the market status of Consumer Electronics (Nickel-Zinc Ferrite Core, Mn-Zn Ferrite Core), Household Appliances (Nickel-Zinc Ferrite Core, Mn-Zn Ferrite Core), and key enterprises in developed and developing market, and analysed the cutting-edge technology, patent, hot applications and market trends of Adult Hemoglobinopathy Testing. According to our (Global Info Research) latest study, the global Adult Hemoglobinopathy Testing market size was valued at USD 186150 million in 2023 and is forecast to a readjusted size of USD 215380 million by 2030 with a CAGR of 2.1% during review period. The Adult Hemoglobinosis Test is a set of tests used to determine the presence and relative amount of abnormal forms of hemoglobin to screen or diagnose hemoglobinopathies and to adults. According to our research, the global market for medical devices is estimated at US$ 603 billion in the year 2023, and will be growing at a CAGR of 5% during next six years. The global healthcare spending contributes to occupy 10% of the global GDP and is continuously rising in recent years due to the increasing health needs of the aging population, the growing prevalence of chronic and infectious diseases and the expansion of emerging markets. The medical devices market plays a significant role in the healthcare industry. The market is driven by several factors, including the increasing demand for advanced healthcare services globally, advancements in medical technology, growing geriatric population, rising healthcare expenditure, and increasing awareness about early disease diagnosis and treatment. The Global Info Research report includes an overview of the development of the Adult Hemoglobinopathy Testing industry chain, the market status of Hospital (HPLC Detection, Hb Electrophoresis), Medical School (HPLC Detection, Hb Electrophoresis), and key enterprises in developed and developing market, and analysed the cutting-edge technology, patent, hot applications and market trends of Adult Hemoglobinopathy Testing. Regionally, the report analyzes the Adult Hemoglobinopathy Testing markets in key regions. North America and Europe are experiencing steady growth, driven by government initiatives and increasing consumer awareness. Asia-Pacific, particularly China, leads the global Adult Hemoglobinopathy Testing market, with robust domestic demand, supportive policies, and a strong manufacturing base. Market segment by Type： HPLC Detection、Hb Electrophoresis、Mass Spectrometry、Other Market segment by Application：Hospital、Medical School、Other Major players covered： Bio-Rad Laboratories、Thermo Fisher Scientific、PerkinElmer、Chromsystems Instruments & Chemicals GmbH、BioMedomics、CapitalBio Technology、Trinity Biotech、Streck

Market segment by region, regional analysis covers: North America (United States, Canada and Mexico), Europe (Germany, France, United Kingdom, Russia, Italy, and Rest of Europe), Asia-Pacific (China, Japan, Korea, India, Southeast Asia, and Australia),South America (Brazil, Argentina, Colombia, and Rest of South America),Middle East & Africa (Saudi Arabia, UAE, Egypt, South Africa, and Rest of Middle East & Africa). The content of the study subjects, includes a total of 15 chapters: Chapter 1, to describe Adult Hemoglobinopathy Testing product scope, market overview, market estimation caveats and base year. Chapter 2, to profile the top manufacturers of Adult Hemoglobinopathy Testing, with price, sales, revenue and global market share of Adult Hemoglobinopathy Testing from 2019 to 2024. Chapter 3, the Adult Hemoglobinopathy Testing competitive situation, sales quantity, revenue and global market share of top manufacturers are analyzed emphatically by landscape contrast. Chapter 4, the Adult Hemoglobinopathy Testing breakdown data are shown at the regional level, to show the sales quantity, consumption value and growth by regions, from 2019 to 2030. Chapter 5 and 6, to segment the sales by Type and application, with sales market share and growth rate by type, application, from 2019 to 2030. Chapter 7, 8, 9, 10 and 11, to break the sales data at the country level, with sales quantity, consumption value and market share for key countries in the world, from 2017 to 2023.and Adult Hemoglobinopathy Testing market forecast, by regions, type and application, with sales and revenue, from 2025 to 2030. Chapter 12, market dynamics, drivers, restraints, trends and Porters Five Forces analysis. Chapter 13, the key raw materials and key suppliers, and industry chain of Adult Hemoglobinopathy Testing. Chapter 14 and 15, to describe Adult Hemoglobinopathy Testing sales channel, distributors, customers, research findings and conclusion.

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[csadhina]

Glycosylated Hemoglobin Assay Kit

The HbA1c (glycosylated hemoglobin) Glycosylated Hemoglobinal Assay Kit is designed to measure the concentration of glycosylated hemoglobin in human whole blood. Its results reflect the glucose metabolism status of diabetic patients within two months. The test can be used for the early detection of diabetes or to monitor the occurrence of chronic complications of diabetes.

This glucose-sensitive glycosylated hemoglobin assay kit detects the presence of beta-N-1-deoxy-F-HbA1c. This measurement is  Glycosylated Hemoglobin Assay Kit especially useful to monitor the blood sugar control of pre-diabetics and those with diabetes. This test can also detect glycosylated hemoglobin in human serum and cell culture supernatants.

A Glycosylated HemoglobIN Assay Kit measures the amount of glycosylated hemoglobin in blood. This measurement will show whether you have maintained a stable blood glucose level in the last two to three months. The result will help you determine whether your diabetes is under control or needs to be treated. You can use this test to monitor your progress in managing your condition.

This test measures the concentration of glycosylated hemoglobin in human serum or plasma. The test is very sensitive and specific. It can determine glycated hemoglobin in both human blood samples and cell culture supernats. You can use it for both research and clinical purposes. This kit is recommended for patients with diabetes. It provides additional information about a patient's blood sugar control than a fasting test can provide.

A Glycosylated Hemoglobulin Assay Kit is used for the quantitative measurement of the concentration of Glycosylated Hemoglobin in serum and plasma. The test has high sensitivity and excellent specificity, allowing it to accurately diagnose diabetes. The test is very accurate and can be used as a guide for detecting glycosylated Hemoglobin in serum.

This test is highly sensitive and specific for Glycosylated Hemoglobiin. It measures the beta-N-1-deoxy-F-HbA1c concentration in serum and plasma. The kit is available in different countries. You can get the kit from your laboratory. It is easy to use and provides high sensitivity. You can also analyze the data on glucose and other compounds with the help of the ELISA.

Glycosylated Hemoglobinosis is a common blood condition and is a symptom of diabetes. Higher levels of HbA1c are a good indicator for the onset of glycosylated Hemoglobin-related diseases, such as heart disease, retinopathy, and nephropathy. The test has the ability to determine the level of glycated Hemoglobin in the blood.

The HbA1c Assay Kit is a highly sensitive and specific test for measuring HbA1c in serum and plasma. It can be used for research purposes only and is not for therapeutic applications. The kit allows for the quantitative determination of HbA1C in serum and plasma. For this purpose, the kit is suitable for the measurement of glucose levels in the blood of diabetic patients.