Important Criterion For Diagnosis Research Articles

Comparison of the cystocholedochal angle in patients with choledocholithiasis and only cholelithiasis.

This study aims to evaluate the relation between the cystocholedochal angle (SCA) and choledocholithiasis. The data of 3.350 patients were reviewed retrospectively and a total of 628 patients who met the criteria were included in the study. The patients included in the study were divided into three groups as patients with choledocholithiasis (Group I), patients with only cholelithiasis (Group II), and patients without gallstones as control group (Group III). Measurements of SCA, cystic, bile, and common hepatic ducts (CHDs) were made on magnetic resonance cholangiopancreatography (MRCP) images. Laboratory findings and demographic characteristics of the patients were also recorded. Of the patients included in the study 64.2% were female, 35.8% were male, and their age ranged from 18 to 93 (mean 53.37 ± 18.87 years). While the mean SCA values of all patient groups were 35.44° ± 10.44°, the mean length of cystic, bile and CHDs were 28.91 ± 9.30, 40.28 ± 12.91, 27.09 ± 9.68 mm respectively. All measurements were higher in Group I in comparison to other groups, whereas all measurements of Group II were higher than those of Group III (p< 0.001). Statistical analysis suggests that a SCA of 33.5° and above is an important criterion for diagnosis of choledocholithiasis. Increase of SCA raises the likelihood of choledocholithiasis, as it facilitates the passage of stones from gallbladder into the bile ducts. This is the first study to compare SCA in patients with choledocholithiasis and those with only cholelithiasis. Therefore, we think that this study is important and will be a guide for clinical evaluation.

Cephalometric evaluation and comparison of upper pharyngeal airway space in cleft lip and cleft palate patients treated with maxillary advancement and camouflage

Introduction: Pharyngeal airway space has become an important criterion for diagnosis and treatment planning and this has led to a paradigm shift towards soft tissue evaluation for orthodontic diagnosis and treatment planning, The aims and objectives of the study were to evaluate and compare upper pharyngeal airway space in Cleft Lip &amp; Cleft Palate patients who underwent orthodontic treatment under different treatment modalities like Camouflage treatment &amp; Maxillary Advancement. Methods: This is a retrospective cephalometric study in which 20 Pre-treatment Cephalograms and 20 Post-treatment Cephalograms of Cleft Lip and Palate patients were taken. Orthodontic treatment involving Camouflage therapy and Maxillary Advancement were studied in each respective group. The Cephalogram was traced on Matt acetate paper and the pharyngeal airway space was measured using the McNamara analysis method. Results: Statistically significant difference was observed in the Pre-treatment and Post-treatment mean values in both groups, where the Post-treatment mean was higher than the Pre-treatment means in both groups. A highly significant mean was noted in the Cleft Surgical group as compared to the Camouflage (xiii) group, where, the post-treatment upper airway mean was higher in the Cleft Surgical group than the Cleft Camouflage group. Conclusion: There is a significant increase in upper pharyngeal airway space in patients with CLCP treated with Maxillary Advancement and Camouflage modality. The significance is comparatively more in the Maxillary Advancement group than in the Camouflage group.

Adrenal cancer: relevance of different grading systems and subtypes

PurposeThe subclassification of adrenal cancers according to the WHO classification in ordinary, myxoid, oncocytic, and sarcomatoid as well as pediatric types is well established, but the criteria for each subtype are not sufficiently determined and the relative frequency of the different types of adrenal cancers has not been studied in large cohorts. Therefore, our large collection of surgically removed adrenal cancers should be reviewed o establish the criteria for the subtypes and to find out the frequency of the various types.MethodsIn our series of 521 adrenal cancers the scoring systems of Weiss et al., Hough et al., van Slooten et al. and the new Helsinki score system were used for the ordinary type of cancer (97% of our series) and the myxoid type (0.8%). For oncocytic carcinomas (2%), the scoring system of Bisceglia et al. was applied.ResultsDiscrepancies between benign and malignant diagnoses from the first thee classical scoring systems are not rare (22% in our series) and could be resolved by the Helsinki score especially by Ki-67 index (more than 8% unequivocally malignant). Since all our cancer cases are positive in the Helsinki score, this system can replace the three elder systems. For identification of sarcomatoid cancer as rarest type in our series (0.2%), the scoring systems are not practical but additional immunostainings used for soft tissue tumors and in special cases molecular pathology are necessary to differentiate these cancers from adrenal sarcomas. According to the relative frequencies of the different subtypes of adrenal cancers the main type is the far most frequent (97%) followed by the oncocytic type (2%), the myxoid type (0.8%) and the very rare sarcomatoid type (0.2%).ConclusionsThe Helsinki score is the best for differentiating adrenal carcinomas of the main, the oncocytic, and the myxoid type in routine work. Additional scoring systems for these carcinomas are generally not any longer necessary. Signs of proliferation (mitoses and Ki-67 index) and necroses are the most important criteria for diagnosis of malignancy.

Relation between Single Nucleotide Polymorphism rs3738423 (C&gt;T) of NPHS2 Gene and some Biochemical Parameters in Pediatrics Nephrotic Syndrome Patients

Clinical biochemical parameters were known as important criteria for diagnosis and treatment of nephrotic syndrome (NS). Single nucleotide polymorphism (SNP) rs3738423 (C&gt;T) of NPHS2 gene encoding for podocin protein in globular membrane was proved associated to steroid resistant nephrotic syndrome. This study was to evaluate the relation between rs3738423 (C&gt;T) of NPHS2 gene and biochemical parameters of pediatrics NS patients in steroid- sensitive (SS), early (ESR) and late (LSR) steroid resistant groups. Blood and urine samples from 149 patients were collected for protein, albumin and protein/creatinine analysis; blood samples in EDTA tube were used for NPHS2 gene analysis. The results showed that CC and CT genotypes were mostly abundant in all three groups, whereas TT was minor appeared in only SS and KTM groups. Patients with the CT genotypes in the ESR group had a protein/creatinine index nearly twice as high as those patients with the CC genotypes. Meanwhile, those with CT genotypes in the SS and LSR groups had lower rates than the CC genotypes. Clinical biochemical parameters significantly contributed to the diagnosis, monitoring and treatment of pediatrics nephrotic syndrome, the changes of these parameters depended on the interaction between the response to corticoid and rs3738423 (C&gt;T) polymorphism of NPHS2 gene in pediatrics nephrotic syndrome patients.

Immunoglobulin G4-related diseases: Autoimmune pancreatitis and cholangitis

Autoimmune pancreatitis (AIP) and cholangitis are chronic inflammatory diseases characterized by the infiltration of lymphoplasmocytic cells into the pancreas and biliary tract and fibrosis. This is often accompanied by increased serum immunoglobulin G4 (IgG4) levels. An early and accurate diagnosis is extremely important because they display similar features to other malignant diseases of the pancreas and biliary tract and primary sclerosing cholangitis. In this study, we aimed to convey our clinic's experiences on this topic. The patients who were diagnosed with IgG4 (+)/(-) AIP and IgG4 (+) sclerosing cholangitis by excluding other causes with clinical, radiological, serological, and pathological examinations and treatment response between July 2014 and June 2020 were included in the study. The data of the patients were analyzed retrospectively. A total of 12 patients, 8 men and 4 women, were included in the study. The mean age of the patients was 39.4 (13-66) years. Abdominal pain of varying degrees was the main reason for admission in all patients. The time of diagnosis and follow-up was 19.2 (2-66) months on average. The average number of attacks during the follow-up was 2.9 (2-8) times. The average IgG4 level of the patients was calculated as 250.1 (133-409) mg/dL. All the patients were given 24-48 mg methylprednisolone at reduced doses for 6-8 weeks. High serum IgG4 levels are the key indicators for diagnosis, and an early and accurate differential diagnosis is extremely important for malignant diseases of the pancreas and biliary tract. A good response to steroids is an important criterion for diagnosis as well as treatment.

Characteristics and Histopathological Grading of Malignant Spiculated Mass in regards to Histopathological Grading of Breast Cancer Based on The Nottingham Grading System

Introduction: Spiculation in mammography is a typical finding for invading breast cancer and is an important criterion in diagnosis and in predicting prognostic and plays an important role in management. The purpose of this research is to determine the characteristics of malignant spiculating mass in mammography in regards to histopathological grading using The Nottingham Grading System.Methods: Patients whom had spiculation in mammography was reviewed using medical record data by two breast imaging consultants and then combined with The Nottingham Grading System criteria obtained from histopathological examination results of core biopsy and surgery specimen. There was 29 cases that met the inclusion criteria.Results: Of the 29 patients that met the inclusion, the spiculated masses grade I, II has mitotic index of 0-12, grade III has mitotic index of 13-25. Histopathologic grade II, III has the most tubular formation of &lt;10%, grade I has 10-75%. Grade I, II has moderate nuclear pleomorphism, grade III has severe nuclear pleomorphism. Most were grade III (44.8%), followed by grade II (37.9%), and minimally grade I (17.2%). Most patients are in stage 3 breast cancer.Conclusion: Malignant spiculated mass with grade I, II has low proliferation index (mitotic index 0-12), histopathological grade II, III had worse cellular differentiation (tubular formation &lt;10%), histopathological grade II, III has moderate to severe nuclear pleomorphism.

A clinical case of Wilson — Konovalov disease in the practice of gastroenterologist

Wilson — Konovalov disease (in the English literature known as Wilson’s disease) is a rare inherited disease with autosomal recessive type of inheritance, which is based on a genetic defect in copper metabolism. Since copper is a part of almost all foods and plays a role of coenzyme in many processes in the body, this element cannot be excluded from the diet. For individuals with absence of excretion of copper ions from the cell, a violation of the fatty acid structure of the cell membrane is inevitable. As a result, its integrity is lost, which leads to degeneration of organs and tissues. Symptoms may include psychiatric (depression, apathy) and neurological disorders (gait disturbance, tremor, irritability), idiopathic hepatitis and cirrhosis, hemolytic anemia, disorders of the cardiovascular and musculoskeletal systems. The insidiousness of this disease is that copper can accumulate for years and not cause severe symptoms until it reaches a certain threshold level, and then in a very short time causes irreversible changes in the body. This threshold level is individual for everyone. It is clinically confirmed that the signs of liver damage are ahead of neurological manifestations by 10 years. Liver damage has been reported in all patients with neurological manifestations. Another important manifestation of the disease is the formation of the Kaiser‑Fleischer ring due to the accumulation of copper in the descemet’s membrane of the cornea. It is found in 95 % of people with neurological manifestations, but is practically not found in children with the disease. An important criterion for diagnosis is psychiatric and behavioural changes, which often precede hepatic manifestations, but are written off at puberty. Such a wide range of disorders is stipulated by the fact that copper is a cofactor of many enzymes in the body. Predictive testing and timely administration of specific treatment will help to avoid such processes. Untreated disease progresses with the development of acute liver failure with the subsequent need for liver transplantation.The authors give an overview of recent data on the etiology, pathogenesis, clinical manifestations, methods of treatment of Wilson — Konovalov disease, and present their own clinical observation of a complex case of Wilson — Konovalov disease in a young girl of 20 years old.

Light Microscopy and Scanning Electron Microscopy of Colpocephalum nanum Piaget, 1890 (Phthiraptera: Amblycera: Colpocephalidae)

The aim of this study was to examine the morphological characteristics of Colpocephalum nanum (C. nanum) Piaget, 1890 using light microscopy (LM) and scanning electron microscopy (SEM). For this purpose, the C. nanum specimens collected from long-legged buzzards, Buteo rufinus (B. rufinus) (Accipitriformes: Accipitridae), in Turkey were examined under LM and SM for morphological characteristics. The specimens were fixed and kept in 70% ethanol, cleared and mounted on the slides in Canada balsam. They were examined for morphological characteristics under LM. Some of the samples were put in a plate on absorbing paper and kept overnight, for ether to evaporate. These samples were mounted on aluminum stubs to study the ventral surface by placing them on their dorsal or ventral surface on double-sided adhesive tape. They were sputter-coated three times with gold, each time for approximately 7 minutes and later viewed using SEM (Zeiss SUPRA 55 VP FE-SEM and Zeiss EVO lS 10). Parts of the specimens were photographed, and the obtained data about morphological characteristic were evaluated in detail. The LM and SEM photos of C. nanum were compared, and information about the important criteria for diagnosis and other morphological characteristics was obtained.

RAF near point rule for near point of convergence—a short review

RAF near point rule (RNPR) is a routinely used instrument in ophthalmology and optometry practice as well as for research purposes to measure the near point of convergence (NPC). The measurement of NPC is an important criterion for diagnosis and management of convergence insufficiency. The RNPR forms an important tool for ophthalmic clinicians however, only a very little is understood about it. This article tries to describe and review the designs, measurement techniques, merits and demerits of the RNPR and establish the need for its modification. It recommends that clinicians and researchers consider these findings while measuring NPC with the RNPR.

Measurement error in CT assessment of appendix diameter.

Appendiceal diameter continues to be cited as an important criterion for diagnosis of appendicitis by computed tomography (CT). To assess sources of error and variability in appendiceal diameter measurements by CT. In this institutional review board-approved review of imaging and medical records, we reviewed CTs performed in children <18years of age between Jan. 1 and Dec. 31, 2010. Appendiceal diameter was measured in the axial and coronal planes by two reviewers (R1, R2). One year later, 10% of cases were remeasured. For patients who had multiple CTs, serial measurements were made to assess within patient variability. Measurement differences between planes, within and between reviewers, within patients and between CT and pathological measurements were assessed using correlation coefficients and paired t-tests. Six hundred thirty-one CTs performed in 519 patients (mean age: 10.9 ± 4.9years, 50.8% female) were reviewed. Axial and coronal measurements were strongly correlated (r = 0.92-0.94, P < 0.0001) with coronal plane measurements significantly larger (P < 0.0001). Measurements were strongly correlated between reviewers (r = 0.89-0.9, P < 0.0001) but differed significantly in both planes (axial: +0.2mm, P=0.003; coronal: +0.1mm, P=0.007). Repeat measurements were significantly different for one reviewer only in the axial plane (0.3mm difference, P<0.05). Within patients imaged multiple times, measured appendix diameters differed significantly in the axial plane for both reviewers (R1: 0.5mm, P = 0.031; R2: 0.7mm, P = 0.022). Multiple potential sources of measurement error raise concern about the use of rigid diameter cutoffs for the diagnosis of acute appendicitis by CT.

Cytogenetics in the management of "chronic myeloid leukemia": an update by the Groupe francophone de cytogénétique hématologique (GFCH).

Cytogenetic evaluation is one the most important criteria for diagnosis and response to treatment in chronic myeloid leukemia, and recent baseline prognostic factors including particular additional clonal cytogenetic abnormalities have been established. The French cytogenetic group in hematology GFCH proposes here an updating of recommendations for cytogenetic assessment of CML in the era of tyrosine kinase inhibitors.

Lead-related infective endocarditis: factors influencing the formation of large vegetations.

The presence of intracardiac lead vegetations (ILV) is one of the important criteria for diagnosis of lead-related infective endocarditis (LRIE). The objective of the present study was to evaluate risk factors of ILV and their impact on vegetation size. Clinical data of 500 patients with LRIE undergoing transvenous lead extraction in 2006-15 were retrospectively analysed. The study population consisted of 352 patients with the presence of vegetations (giant, >3 cm; large, 2.0-2.9 cm; moderate-sized, 1.0-1.9 cm; and small, <1 cm) and 148 patients without ILV. We identified risk factors for vegetation occurrence and ILV size. Intracardiac lead vegetations were found more frequently in younger patients (P < 0.05), slightly more often in women (P = 0.084), and less commonly in patients with atrial fibrillation (P < 0.05). Intracardiac lead vegetation occurred significantly more frequently in patients with intracardiac lead abrasion (OR 2.373; 95% CI [1.497-3.765]; P < 0.001) and much less frequently in the concomitant presence of pocket infection (PI) (OR 0.127; 95% CI [0.074-0.218]; P < 0.00). Large vegetations were significantly more common in patients with renal failure (RF) (P < 0.001), heart failure (P < 0.001), implantable cardioverter defibrillator (P < 0.05), and loops of the leads (P < 0.001). Intracardiac lead abrasion is one of the most common factors influencing the occurrence of ILV. Metabolic disorders in patients with RF, heart failure, defibrillation leads, and loops of the leads were found to contribute to the formation of large vegetations. In LRIE patients, ILVs were less frequently detected in the presence of concomitant PI, indicating a different mechanism of LRIE development in patients with and without vegetations.

Brucellosis Suspicion is the Most Important Criterion for Diagnosis Particularly in Endemic Regions.

Introduction: Brucellosis is a zoonotic infectious disease that remains endemic in developing countries. The purpose of this study is to emphasize the need for considering brucellosis as a diagnosis, since this disease has a high risk of complications among young patients when not treated appropriately.Methodology: A total of 88 brucellosis cases with blood cultures that were positive for the pathogen were evaluated retrospectively in this study.Results:The patients included 33 males (37.5%) and 55 females (62.5%) with a median age of 8.9 years (range: 5-14 years). A total of 43.1% (n=38) of the cases included occupational exposure to animals as a possible infection source. The consumption of raw milk products, especially cheese, was present in 52.2% (n=46) of the cases. Clinically, 55 of the cases were acute (62.5%), 23 of the cases were subacute (26.2%) and 10 of the cases were chronic (11.3%). The distribution of the joint pain complaints was as follows: 62.5% (n=55) of patients reported hip pain, 22.7% (n=20) of patients reported knee pain, 11.4% (n=10) of patients reported lumbar-back pain and 3.4% (n=3) of patients reported pain in other joints. A total of 59.1% (n=52) of the cases had been examined by another doctor at least once and mistreated.Conclusion:Complication rates and the rate of chronic infection increase with delayed diagnosis, and clinical doubt is the most important criterion for diagnosis, particularly in endemic regions.

Effect of Percutaneous Coronary Intervention on Left Ventricular Diastolic Function in Patients With Coronary Artery Disease

Background:There is considerable disagreement over the effects of percutaneous coronary intervention (PCI) on left ventricular diastolic function that has necessitated the investigation of diastolic indices. The present study was conducted to evaluate left ventricular diastolic function and its indices, three months after performing the PCI procedure in patients with coronary artery disease (CAD).Methods:In a quasi-experimental clinical trial study (before and after), 51 patients with CAD scheduled for elective PCI were investigated provided that their Ejection Fraction (EF) was > 30%. Before and three months after PCI, echocardiography was carried out to evaluate left ventricular diastolic indices including the E/Ea as the most important criteria for diagnosis of diastolic heart failure (DHF).Results:Based on the E/Ea indices and after PCI, the number of patients with DHF decreased significantly: 40 patients (78.4%) before PCI versus 28 patients (54.9%) after PCI (p<0.05). The Mean and Standard error of deceleration time (DT), isovolumic relaxation time (IVRT), early diastolic mitral annulus velocity; Ea (E’), E/Ea and left ventricular ejection function (LVEF) indices underwent significant changes. In addition, MVA dur/PVA dur, PVs/PVd, and E/Ea indices had changed significantly after PCI in both genders. However, no significant difference was reported for the other indices.Conclusion:The E/Ea ratio as an important criterion for diagnosis of DHF was improved after PCI. Improvement of several other diastolic indices was observed after the PCI procedure. It can be concluded that PCI can be an effective treatment modality in patients with left ventricular diastolic indices.

Automatic segmentation and volumetry of multiple sclerosis brain lesions from MR images.

The location and extent of white matter lesions on magnetic resonance imaging (MRI) are important criteria for diagnosis, follow-up and prognosis of multiple sclerosis (MS). Clinical trials have shown that quantitative values, such as lesion volumes, are meaningful in MS prognosis. Manual lesion delineation for the segmentation of lesions is, however, time-consuming and suffers from observer variability. In this paper, we propose MSmetrix, an accurate and reliable automatic method for lesion segmentation based on MRI, independent of scanner or acquisition protocol and without requiring any training data. In MSmetrix, 3D T1-weighted and FLAIR MR images are used in a probabilistic model to detect white matter (WM) lesions as an outlier to normal brain while segmenting the brain tissue into grey matter, WM and cerebrospinal fluid. The actual lesion segmentation is performed based on prior knowledge about the location (within WM) and the appearance (hyperintense on FLAIR) of lesions. The accuracy of MSmetrix is evaluated by comparing its output with expert reference segmentations of 20 MRI datasets of MS patients. Spatial overlap (Dice) between the MSmetrix and the expert lesion segmentation is 0.67 ± 0.11. The intraclass correlation coefficient (ICC) equals 0.8 indicating a good volumetric agreement between the MSmetrix and expert labelling. The reproducibility of MSmetrix' lesion volumes is evaluated based on 10 MS patients, scanned twice with a short interval on three different scanners. The agreement between the first and the second scan on each scanner is evaluated through the spatial overlap and absolute lesion volume difference between them. The spatial overlap was 0.69 ± 0.14 and absolute total lesion volume difference between the two scans was 0.54 ± 0.58 ml. Finally, the accuracy and reproducibility of MSmetrix compare favourably with other publicly available MS lesion segmentation algorithms, applied on the same data using default parameter settings.

Suture Granuloma Mimicking Renal Cell Carcinoma: Magnetic Resonance Imaging (MRI) and Pathologic Correlation

OZET Solid renal masses are generally distinguished with contrast enhancement and intratumoral fatty foci by radiological examinations. The present of enhancement is most important criteria for diagnosis of malignant lesions. Generally, a contrast enhanced solid mass in kidney is accepted as a neoplasm. Foreign body granuloma is an extraordinary cause of enhanced solid renal mass. This case of a renal suture granuloma demonstrated peripheral enhanced exophytic renal mass mimic renal cell carcinoma, and underwent surgery. At the solid renal mass with different radiological features, biopsy is an option to determining the necessity of surgery as well as the surgical approach.

Comparing Verb Fluency With Verbal Fluency in Patients With Alzheimer's Disease

Background: Alzheimer's disease (AD) is the most common type of dementia. Patients with AD have impaired semantic knowledge of nouns and verbs as well as the ability to verb retrieval. Objectives: The aim of this study was to compare verbal fluency and verb fluency and to investigate the association between severity and types of fluency (verbal-verb) in patients with AD. Patients and Methods: This cross-sectional study was performed on 30 patients (14 males, 16 females) with AD. The participants were assessed using the Mini Mental State Examination (MMSE) and a neurologist made the diagnosis of AD. In order to examine verb and verbal fluency, the subjects were asked to name any words that they remember in one minute. Scoring was based on the correct number of items and data analysis was performed by SPSS 16. Results: There was a significant positive correlation between letter fluency and verb fluency (P = 0.013), letter fluency and semantic fluency (P < 0.001), and verb fluency and semantic fluency (P = 0.002) in patients with AD. The results showed significant differences between letter fluency (P < 0.001), verb fluency (P = 0.034), and semantic fluency (P < 0.001) among patients with different severity of AD. In addition, the most frequently produced words in letter fluency were /a/, /s/, and /f/, consecutively. The mean ratio of semantic fluency to letter fluency was 3:5. Conclusions: In patients with AD, verb fluency was impaired more than semantic fluency was. Verb fluency task is an important criterion in diagnosis of early dementia. Therefore, the investigation of verb fluency in susceptible individuals with dementia might prevents the progression of the disorder.

Basiswissen Histologie

Very commonly skin biopsies are crucial for the correct diagnosis of patients with skin diseases. The dermatopathology report is an important tool for communication between the clinical dermatologist and the dermatopathologist. This article explains the method of histopathological assessment of skin biopsies and summarizes the most important criteria for diagnosis of common skin diseases. The application of additional histochemical, immunohistochemical, and molecular biological methods is elucidated. The clinical dermatologist requires basic knowledge of dermatopathology to decide on the appropriate biopsy procedure. It also enables him to understand the dermatopathology report and to identify settings in which clinicopathological correlation is required.

Allergic Bronchopulmonary Aspergillosis (ABPA): Relevance of Absolute Eosinophil Count (AEC) in Peripheral Blood

PURPOSE: There is neither uniform consensus on the number of criteria needed for diagnosis nor the specific cut-off values for the various parameters employed in diagnosis of ABPA. Traditionally an AEC of >1000 cells/μL is considered an important criteria for diagnosis of ABPA. This study attempts to delineate the significance of peripheral blood eosinophil counts in the diagnosis of ABPA, and its relationship with lung function, immunological and radiological parameters.

Standard clinical practice underestimates the role and significance of erythropoietin deficiency in sickle cell disease

In sickle cell disease (SCD), vigorous reticulocytosis is required to partially compensate for chronic hemolytic anaemia. Consequently, early renal damage, insufficient to cause azotemia but sufficient to cause erythropoietin deficiency and chronic relative reticulocytopenia (chRR), could have severe clinical consequences. chRR was defined as reticulocytes <250×10(9) /l despite haemoglobin <9 g/dl on ≥ two occasions ≥4 weeks apart. The influence of multiple variables including chRR on time from first clinic visit to death was evaluated in 306 SCD patients. In univariate analyses, fetal haemoglobin, indices of renal damage (serum creatinine, proteinuria), chRR and age, were associated with rate of death. In multivariate analysis, only age and chRR (Hazard ratio 3·6, 95% CI 2·049-6·327, P<0·0001) were significant, underlining that chRR could be an early and important clinical consequence of renal damage. Even in chRR patients with normal serum creatinine levels, low haemoglobin and low reticulocyte counts were associated with low erythropoietin levels. In the general population, evaluation of erythropoietin levels is prompted by the combination of anaemia and abnormal serum creatinine. In SCD patients, this standard approach can miss a substantial risk factor for early death. chRR could be a practical and important criterion for diagnosis of erythropoietin deficiency in SCD.