Wernicke encephalopathy Clinical presentation and MR images in two nonalcoholic patients

The aim of the present study was to investigate the importance of and correlation between clinical presentations and magnetic resonance imaging （MRI） of two different cases of nonalcoholic Wernicke encephalopathy. Case l ： A 63-year-old man with a diagnosis of incomplete mechanical intestinal obstruction. His abdominal symptoms were improved by gastrointestinal decompression, but blurred vision, hypoacusis, dizziness, and unsteady gait were noted. His illness deteriorated to confusion on day seven. MRI showed hyperintense lesions in the medial thalami, tectum of the midbrain, and the periaqueduct region on T2- and diffusion-weighted images. Thiamine therapy was commenced immediately with good results. Case 2： A 22-year-old woman was admitted for sudden-onset confabulation and unsteady gait after hyperemesis gravidarum. She had no history of alcohol or any medication. Brain MRI was normal. The patient experienced relief after Vitamin B1 treatment. These results suggest that brain MRI can define characteristic abnormalities in Wernicke encephalopathy, and that diffusion-weighted imaging may improve the diagnosis sensitivity. In addition, the MRI images may be correlated to the clinical stage and severity of the disease. Nevertheless, the clinical features are essential for correct diagnosis.

Chlorfenapyr poisoning:mechanisms,clinical presentations,and treatment strategies

BACKGROUND:Chlorfenapyr is used to kill insects that are resistant to organophosphorus insecticides.Chlorfenapyr poisoning has a high mortality rate and is difficult to treat.This article aims to review the mechanisms,clinical presentations,and treatment strategies for chlorfenapyr poisoning.DATA RESOURCES:We conducted a review of the literature using PubMed,Web of Science,and SpringerLink from their beginnings to the end of October 2023.The inclusion criteria were systematic reviews,clinical guidelines,retrospective studies,and case reports on chlorfenapyr poisoning that focused on its mechanisms,clinical presentations,and treatment strategies.The references in the included studies were also examined to identify additional sources.RESULTS:We included 57 studies in this review.Chlorfenapyr can be degraded into tralopyril,which is more toxic and reduces energy production by inhibiting the conversion of adenosine diphosphate to adenosine triphosphate.High fever and altered mental status are characteristic clinical presentations of chlorfenapyr poisoning.Once it occurs,respiratory failure occurs immediately,ultimately leading to cardiac arrest and death.Chlorfenapyr poisoning is diflcult to treat,and there is no specific antidote.CONCLUSION:Chlorfenapyr is a new pyrrole pesticide.Although it has been identified as a moderately toxic pesticide by the World Health Organization(WHO),the mortality rate of poisoned patients is extremely high.There is no specific antidote for chlorfenapyr poisoning.Therefore,based on the literature review,future efforts to explore rapid and effective detoxification methods,reconstitute intracellular oxidative phosphorylation couplings,identify early biomarkers of chlorfenapyr poisoning,and block the conversion of chlorfenapyr to tralopyril may be helpful for emergency physicians in the diagnosis and treatment of this disease.

Recent Advances for Global Perspectives on Etiology, Pathophysiology, Clinical Presentations, and Management of Moyamoya Disease

Moyamoya disease (MMD) is a condition characterized by the gradual narrowing and blockage of blood vessels in the brain, specifically those in the circle of Willis and the arteries that supply it. This results in reduced blood flow and oxygen to the brain, leading to progressive symptoms and potential complications. The underlying pathophysiological mechanism remains elucidated. However, recent studies have highlighted numerous etiologic factors: abnormal immune complex responses, susceptibility genes, branched-chain amino acids, antibodies, heritable diseases, and acquired diseases, which may be the great potential triggers for the development of moyamoya disease. Its clinical presentation has varying degrees from transient asymptomatic events to significant neurological deficits. Moyamoya disease (MMD) shows different patterns in children and adults. Children with MMD are more susceptible to ischemic events due to decreased blood flow to the brain. Conversely, adults with MMD are more prone to hemorrhagic events involving brain bleeding. Children with MMD may experience a range of symptoms including motor impairments, sensory issues, seizures, headaches, dizziness, cognitive delays, or ongoing neurological problems. Although adults may present with similar clinical symptoms as children, they are more prone to experiencing sudden onset intraventricular, subarachnoid, or intracerebral hemorrhages. One of the challenges in moyamoya disease is the potential for misdiagnosis or delayed diagnosis, particularly when physicians fail to consider MMD as a possible cause in stroke patients. This review aims to provide a comprehensive overview of recent global studies on the pathophysiology of MMD, along with advancements in its management. Additionally, the review will delve into various surgical treatment options for MMD, as well as its rare occurrence alongside atrioventricular malformations. Exciting prospects include the use of autologous bone marrow transplant and the potential role of Connexin 43 protein treatment in the development of moyamoya disease.

Clinical Presentation and Treatment Outcomes of Pregnancy-Related Acute Kidney Injury among Pregnant Women Admitted at the Benjamin Mkapa Hospital in Tanzania

Background: Globally, PRAKI is among the leading causes of death in pregnant women. The prevalence, causes and outcome of this condition vary among countries due to differences in environmental, socioeconomic, and health delivery systems. The common causes that have been reported in several studies are PIH, Haemorrhages and Sepsis while the outcomes may be either complete renal recovery, progression to CKD and hence dialysis dependency or death. This study aimed at determining clinical presentation and treatment outcomes of Pregnancy-Related Acute Kidney Injury in Pregnant women admitted at the Benjamin Mkapa Hospital, Dodoma, Tanzania. Results: Out of 4007 pregnant women who were admitted to the maternity ward 51 pregnant women were found to have PRAKI. Of those with PRAKI, 74.5% were between 21 to 25 years. The leading causes of PRAKI were PPH 12 (23.53%), Eclampsia 12 (23.53%), and pre-eclampsia 12 (23.5%). Hemodialysis therapy was provided to 22 (43.1%) patients, 15 (29.4%) individuals recovered spontaneously with medical management and 14 (27.5%) missed haemodialysis therapy due to various reasons. The mortality due to PRAKI was 17 (33.3%). Conclusion and Recommendation: Pre-eclampsia/eclampsia and post-partum haemorrhage were found to be the main causes of PRAKI. The mortality related to PRAKI is high and Hemodialysis therapy is vital help to prevent deaths for pregnant women with PRAKI. Pregnant women who develop acute kidney injury should be followed closely and a nephrologist should be consulted early. Early referral should be done by the lower level facilities for all at-risk pregnant women to a specialized multidisciplinary health facility.

Clinical Presentation, Therapeutic Aspects and Results of Urogental Prolapse in Yaounde

Introduction: Urogenital prolapse is the descent of one or more elements of the abdominal-pelvic contents into the vaginal cavity. The anterior, middle and posterior anatomical compartments may be involved giving rise to several types of genital prolapse. However, there are various therapeutic modalities ranging from observational procedures to surgery. In Yaounde, urogenital prolapse appears to be a rare clinical entity, and its treatment has not been clearly stated. There is limited data about this subject in our context. Our aim was to study the clinical and therapeutic aspects of urogenital prolapse in Yaounde. Methods: We conducted a descriptive cross-sectional study with retrospective data collection at the Yaounde Gynaecological Obstetric and Pediatric Hospital (YGOPH) and the Yaounde Central Hospital (YCH) over a period of 05 months (from February 2022 to June 2022). All women aged ≥30 years and all women with documented urogenital prolapse admitted to the maternity and urology departments of these two health facilities were included. Data were collected using a structured questionnaire. Data was analyzed using IBM SPSS (statistical package for social sciences) software version 23.0 and the data were reported as mean, standard deviation, frequencies and percentages. Results: A total of 50 patients were recruited;the mean age was 45.8 ± 17 years. The age range was from 21 to 72 years. Majority of the patients were aged more than 55 years (32%). Most of the patients attended primary education (48%). Forty percent of the patients were housewives. The majority of the patients were from West Cameroon (26%). The most common reason for consultation was pelvic heaviness (54%). The most prevalent clinical sign was cystocele (56%), followed by hysterocele (54%). According to the Baden Walker classification, one-third of the patients were classified as first degree (36%). More than half (58%) of patients had the condition located at the first stage. Surgery was performed in 68% of cases while 48% received medical treatment. The average length of inpatient stay was 7.9 ± 3.7 days and the average length of transurethral catheterization was 3.02 ± 2.9 days. Most patients had a good short-term outcome, and the main postoperative complications were infection (8.8%) and hemorrhage (5.9%). Conclusion: Urogenital prolapse is a rare condition in our population. Diagnosis is essentially clinical. Cystocele and hysterocele are the predominant clinical forms. Surgery is the mainstay of treatment. Outcomes are satisfactory.

Clinical Presentation, Management and Materno-Fetal Outcome of Patients with HELLP Syndrome at the Yaoundé Gyneco-Obstetrics and Pediatric Hospital

Background: HELLP (Hemolysis, Elevated Liver enzymes and Low Platelets) syndrome is a common complication of severe preeclampsia, with a high maternal and perinatal mortality rate. Data on HELLP syndrome is scanty in our setting. We sought to study the clinical presentation, management, and materno-fetal outcome of patients with HELLP syndrome at the Yaoundé Gyneco-Obstetrics and Pediatric Hospital (YGOPH). Methods: This was a cross-sectional, analytical study with a retrospective data collection of patients, diagnosed with HELLP syndrome at the YGOPH between 1st January 2020 and 31st July 2021. Data were analyzed using the software R version 4.0.2. Results are presented as mean ± standard deviation, frequencies and percentages. P values < 0.05 were considered statistically significant. Results: Of 254 cases of severe preeclampsia, 17 developed HELLP syndrome, giving us a frequency of 6.7%. One patient who presented with hepatitis B was excluded. The mean age was 27.19 ± 6.44 years. Most patients (56.3%) had poor follow up during antenatal contacts. The most common symptom was headache (93.8%). Most cases (56.3%) were diagnosed during post-partum, prepartum (25.0%), and per partum (18.8%). Obstetrical management was done by cesarean section (62.5%) and vaginal delivery (37.5%). Post-partum management of HELLP syndrome consisted of antihypertensive medication, anticonvulsants, sedatives, transfusion of blood and blood products, and fluid resuscitation. Of the 50% of patients who presented with acute kidney injury, only 12.5% (1) were referred for dialysis. Over 50% of our study participants presented severe complications after delivery, but 43.8% recovered, while 56.2% finally died. The fetal outcome was: still birth (31.2%), intra-uterine growth restriction/low birth weight (12.5%), term delivery (≥37 weeks) (31.3%), and preterm deliveries (<37 weeks) (68.7%). Three (18.8%) of the neonates delivered alive presented with a poor Apgar score at the 5th minute. Conclusion: Although rare, HELLP syndrome exists in our setting. Proper management in appropriate centers is key to improving maternal and fetal outcomes.

多参数3D MRI影像组学模型预测结直肠癌肝转移及相关临床危险因素的分析

目的探讨多参数3D MRI影像组学模型对于结直肠癌肝转移(CCLM)的预测价值及相关临床危险因素的分析。方法收集2019-2022年在本院术前行MRI检查且术后病理证实的198例结直肠癌患者的临床及影像资料。按照8:2的比例随机分为训练集及验证集(n=159及39)。对T_(2)WI、DWI、e-THRIVE+序列分别逐层勾画,得到3D VOI,提取组学特征并降维处理、构建影像组学模型。采用逻辑回归分析,筛选出CCLM的独立危险因素并构建临床模型。结果三序列联合的影像组学模型预测CCLM的效能高于单一序列模型,其训练集与验证集AUC值分别为0.919及0.896。逻辑回归分析得到CCLM的独立危险因素分别为CEA、CA199及NLR(P<0.05)。三者联合的临床模型效能亦高于单一临床模型,训练集与验证集的AUC值分别为0.800及0.791。结论多参数3D MRI影像组学模型对于CCLM具有较高预测效能。基于CEA、CA199、NLR的临床模型对于CCLM具有一定预测效能。

Clinical presentations of gastric small gastrointestinal stromal tumors mimics functional dyspepsia symptoms

AIM: To explore whether clinical presentations of gastric small gastrointestinal tumors (GISTs) mimics gastrointestinal dyspepsia symptoms.

Incidence and clinical presentation of portal vein thrombosis in cirrhotic patients

BACKGROUND: Portal vein thrombosis (PVT) is due to many risk factors, but its pathogenesis is still not clearly understood. To identify the risk factors for PVT, we analyzed the clinical characteristics and complications associated with PVT in cir-rhotic patients. METHODS: We studied patients with liver cirrhosis who were admitted to our unit from April 2009 to December 2014. The patients were divided into the PVT and non-PVT groups, and were compared by variables including gender, age, the etiology of cirrhosis, stage of cirrhosis, complications, imaging, and treatment. RESULTS: PVT was found in 45 (9.8%) of 461 cirrhotic pa-tients admitted to our hospital. Most patients (45.9%) had hepatitis B virus (HBV)-related cirrhosis, with a similar dis-tribution of etiologies between the groups. However, there was no positive relationship between PVT and etiologies of cirrhosis. Most patients (71.5%) were in the stage of hepatic decompensation. No statistically signiifcant differences were found in complications including esophageal varices, ascites, and hepatic encephalopathy between the groups. However, there was a signiifcant positive correlation between hepatocel-lular carcinoma (HCC) and PVT (P<0.01). In 30 patients with PVT, thrombosis occurred in the portal vein and/or portal branches, 37.8% were diagnosed on ultrasound. CONCLUSIONS: The incidence of PVT was 9.8%, mainly in patients with HBV-related cirrhosis. The development of PVT was associated with the severity of liver disease and HCC.

Acute mechanical bowel obstruction: Clinical presentation, etiology, management and outcome

AIM: To identify and analyze the clinical presentation, management and outcome of patients with acute mechanical bowel obstruction along with the etiology of obstruction and the incidence and causes of bowel ischemia, necrosis, and perforation. METHODS: This is a prospective observational study of all adult patients admitted with acute mechanical bowel obstruction between 2001 and 2002. RESULTS: Of the 150 consecutive patients included in the study, 114 (76%) presented with small bowel and 36 (24%) with large bowel obstruction. Absence of passage of flatus (90%) and/or feces (80.6%) and abdominal distension (65.3%) were the most common symptoms and physical finding, respectively. Adhesions (64.8%), incarcerated hernias (14.8%), and large bowel cancer (13.4%) were the most frequent causes of obstruction. Eighty-eight patients (58.7%) were treated conservatively and 62 (41.3%) were operated (29 on the first day). Bowel ischemia was found in 21 cases (14%), necrosis in 14 (9.3%), and perforation in 8 (5.3%). Hernias, large bowel cancer, and adhesions were the most frequent causes of bowel ischemia (57.2%, 19.1%, 14.3%), necrosis (42.8%, 21.4%, 21.4%), and perforation (50%, 25%, 25%). A significantly higher risk of strangulation was noticed in incarcerated hernias than all the other obstruction causes. CONCLUSION: Absence of passage of flatus and/or feces and abdominal distension are the most common symptoms and physical finding of patients with acute mechanical bowel obstruction, respectively. Adhesions, hernias, and large bowel cancer are the most commoncauses of obstruction, as well as of bowel ischemia, necrosis, and perforation. Although an important proportion of these patients can be nonoperatively treated, a substantial portion requires immediate operation. Great caution should be taken for the treatment of these patients since the incidence of bowel ischemia, necrosis, and perforation is significantly high.

Characteristics and clinical presentations of pediatric 0retinoblastoma in North-western Iran

·AIM:To report the characteristics and clinical presentations of retinoblastoma in a series of pediatric patients from Iran. ·METHODS:In this retrospective study, profiles of pediatric patients with retinoblastoma archived in a referral eye hospital in north-west of Iran during 7 years (n =40 patients with 57 eyes) were reviewed. Demographics, as well as the laterality, clinical manifestations and the types of treatment were the major endpoints. The Student’s t test, Mann-Whitney U -test, Chi-square or Fishers’ Exact test was used for analysis where appropriate. ·RESULTS:There were 23 cases (57.7%) with unilateral and 17 cases (42.5%) with bilateral involvement. The male to female ratio was 1 to 1.4 with a mean admitting age of 24.0±11.3 (range:5-62) months. The mean diagnosis delay was (7.4±9.6) months (range:10 days to 13 months). The most common presenting sign was leukocoria (97.5%) followed by proptosis (7.5%), strabismus (7.5%), hyphema (5%), orbital cellulitis (5%) and glaucoma (2.5%). Enucleation was performed in 95.7% of the cases with unilateral involvement and at least one eye of the patients with bilateral disease. A second enucleation was performed in other 3 patients (17.6%) of the latter group. ·CONCLUSION:This is the first study evaluating a series of Iranian children with retinoblastoma.

1.5 T MRI与64排多层螺旋CT在脊柱骨折临床诊断中的作用

目的探讨1.5 T磁共振成像(magnetic resonance imaging,MRI)与64排多层螺旋计算机体层成像(computed tomography,CT)对脊柱骨折的诊断价值。方法方便选取2021年1月—2022年12月泰兴市第二人民医院收治的82例疑似脊柱骨折患者作为研究对象,均予1.5 T MRI与64排多层螺旋CT检查,以手术结果为金标准,评估两种技术对脊柱骨折的诊断效能,并分析其对脊柱骨折分型的诊断价值。结果1.5 T MRI对脊柱骨折诊断的准确度、灵敏度与64排螺旋CT比较,差异无统计学意义(P均>0.05)。两种方法与金标准一致性均较好(kappa值≥0.75)。1.5 T MRI对脊柱骨折A、B、C型分型诊断整体符合率为97.56%,高于64排多层螺旋CT的86.59%,差异有统计学意义(χ^(2)=7.111,P<0.05)。结论1.5 T MRI与64排螺旋CT对脊柱骨折诊断效能相当,但1.5 T MRI的分型诊断效能更高。

Endomysial antibodies predict celiac disease irrespective of the titers or clinical presentation

AIM:To investigate the association between serum antibody levels and a subsequent celiac disease diag-nosis in a large series of children and adults. METHODS:Besides subjects with classical gastrointestinal presentation of celiac disease,the study cohort included a substantial number of individuals with extraintestinal symptoms and those found by screening in at-risk groups.Altogether 405 patients underwent clinical,serological and histological evaluations.After collection of data,the antibody values were further graded as low[endomysial(EmA)1:5-200,transglutaminase 2 antibodies(TG2-ab)5.0-30.0 U/L]and high (EmA 1:≥500,TG2-ab≥30.0 U/L),and the serological results were compared with the small intestinal mucosal histology and clinical presentation. RESULTS:In total,79%of the subjects with low and 94%of those with high serum EmA titers showed small-bowel mucosal villous atrophy.Furthermore, 96%of the 47 EmA positive subjects who had normal mucosal villi and remained on follow-up either subsequently developed mucosal atrophy while on a glutencontaining diet,or responded positively to a glutenfree diet. CONCLUSION:Irrespective of the initial serum titers or clinical presentation,EmA positivity as such is a very strong predictor of a subsequent celiac disease diagnosis.

Primary localized gastric amyloidosis:A scoping review of the literature from clinical presentations to prognosis

Localized gastric amyloidosis(LGA)is a rare disease characterized by abnormal extracellular deposition of amyloid protein restricted to the stomach and it is confirmed by positive results of Congo red staining.Over decades,only a few cases have been reported and studies or research focusing on it are few.Although LGA has a low incidence,patients may suffer a lot from it and require proper diagnosis and management.However,the pathology of LGA remains unknown and no overall review of LGA from its presentations to its prognosis has been published.Patients with LGA are often asymptomatic or manifest atypical symptoms,making it difficult to differentiate from other gastrointestinal diseases.Here,we report the case of a 70-year-old woman with LGA and provide an overview of case reports of LGA available to us.Based on that,we conclude current concepts of clinical manifestations,diagnosis,treatment,and prognosis of LGA,aiming at providing a detailed diagnostic procedure for clinicians and promoting the guidelines of LGA.In addition,a few advanced technologies applied in amyloidosis are also discussed in this review,aiming at providing clinicians with a reference of diagnostic process.With this review,we hope to raise awareness of LGA among the public and clinicians.

1.5T MRI平扫联合DWI在宫颈癌诊断中的价值分析

目的:探究1.5T磁共振(magnetic resonance imaging,MRI)平扫联合扩散加权成像(diffusion weighted imaging,DWI)在宫颈癌中的诊断价值。方法:回顾性分析2020年8月至2022年7月我院收治的88例宫颈癌患者的临床资料,所有患者均行MRI平扫及DWI检查。将病理检查结果作为诊断“金标准”,对比不同病理分期下表观弥散系数(Apparent diffusion coefficient,ADC)值、转移淋巴结及非转移淋巴结的ADC值、分析MRI平扫及联合扫描在宫颈癌分期中的诊断价值。结果:不同病理分期下宫颈癌患者ADC值对比,均无统计学意义。经MRI检查共检出淋巴结102枚,其中转移淋巴结41枚,非转移淋巴结61枚。非转移淋巴结ADC值高于转移淋巴结(P<0.05)。MRI平扫+DWI检查Ⅰb和IIa期灵敏度及准确度均高于MRI平扫(P<0.05)。结论:1.5T MRI平扫联合DWI在宫颈癌鉴别诊断中具有较高的临床应用价值,于各分期诊断中准确度较高。

Clinical Presentation of Ovarian Tumors

Ovarian malignancy is a serious disease affecting women of all ages, more so above 50 years, and they are still difficult to treat, partly because no truly effective therapy has yet been developed although presentation is often vague and non-specific, the symptoms are definitely present. It is important to recognize the symptoms so far, there is no test yet available, which is truly specific and suitable for screening and early detection of epithelial ovarian carcinoma. So, it is concluded that for prognosis and patient survival, early detection and treatment is mandatory.

Oral Squamous Cell Carcinoma: Epidemiology, Clinical Presentation and Treatment

Squamous cell carcinoma accounts for 90% of all oral cancers. It may affect any anatomical site in the mouth, but most commonly the tongue and the floor of the mouth. It usually arises from a pre-existing potentially malignant lesion, and occasionally de novo;but in either case from within a field of precancerized epithelium. The use of tobacco and betel quid, heavy drinking of alcoholic beverages and a diet low in fresh fruits and vegetables are well known risk factors for oral squamous cell carcinoma. Important risk factors related to the carcinoma itself that are associated with a poor prognosis include large size of the tumour at the time of diagnosis, the presence of metastases in regional lymphnodes, and a deep invasive front of the tumour. Squamous cell carcinoma is managed by surgery, radiation, and chemotherapy singularly or in combination;but regardless of the treatment modality, the five-year survival rate is poor at about 50%. This can be attributed to the fact that about two-thirds of persons with oral squamous cell carcinoma already have a large lesion at the time of diagnosis.

The clinical significance of detection of specific CK-20 mRNA in peripheral venous blood from patients with bladder carcinoma

Objective To determine the diagnostic significance of detecting the specific epithelial keratin CK-20 mRNA in peripheral venous blood from patients with bladder carcinomas. Methods Reverse transcription coupled with two-step polymerase chain reaction (nested RT-PCR) was used to detect CK-20 mRNA expression in the peripheral blood from patients with blodder carcinomas. Results Detection of CK-20 mRNA expression was positive in 37 of 91 patients with bladder carcinoma (41 % ). Among 20 patients with distant metastasis, 17 were positive (85 % ). CK-20 mRNA was not detectable in the blood samples from 25 normal individuals. The frequency of positive CK-20 mRNA expression was signficantly higher in those with distant metastasis. Conclusion The presence of CK-20 mRNA expression in peripheral blood may be used as an early indicator of hematogenous metastasis of bladder carcinoma cells. 6 refs,1 tab.

ANAPC5 mRNA水平表达上调在肝细胞肝癌中的临床价值及相关通路分析

目的探讨ANAPC5表达趋势及临床价值以及该分子在肝细胞癌(HCC)进展中参与的潜在生物学机制。方法联合来自世界范围内的HCC基因芯片及mRNA测序数据,使用Wilcoxon检验,通过合并标准化平均差(SMD)分析ANAPC5在肝细胞癌组织中的综合表达状况,评估其在肝细胞肝癌组中的临床价值及潜在参与的相关生物学通路。结果通过分析3820个HCC样本和3091个正常样本的高通量数据的结果,SMD=0.71(95%CI:0.55~0.88)。SROC曲线下面积为0.81,根据诊断试验结果,敏感度为0.69(95%CI:0.60~0.77),特异度为0.81(95%CI:0.73~0.88),阳性似然比为3.78(95%CI:2.63~5.47),阴性似然比为0.39(95%CI:0.29~0.48)。富集分析结果显示,ANAPC5与核糖体、剪接体、细胞周期等通路相关。结论ANAPC5上调在HCC中可能发挥促癌作用。

Severe Variant of Sickle Cell Intrahepatic Cholestasis a Difficult Approach Diagnosis Clinical Presentation and Outcome in Adult Population in the Congo

Background: There are no consensual diagnosis criteria to make diagnosis of sickle cell intrahepatic cholestasis and identify its 2 variants. Here we delineate the frequency of the complication in its severe form and describe its clinical features. Methods: We included in the study all the patients with sickle cell disease who were presenting fever, jaundice, pain in the upper right quadrant and filling up the biological criteria of the severe sickle cell intrahepatic cholestasis (serum direct bilirubin ≥ 78.5 mg/dL). Patients with evidence of viral hepatitis or gallstones were not included in the study. Results: Sixty-two patients with an average age of 21.5 years (range 18 and 25 years) meet the inclusion criteria, minimum serum direct bilirubin: 82 versus 528 for the maximum. The liver cytolysis was major and ranged from 310 to 1550 UI/L. Forty-nine patients (83.9%) did not develop any organ failure. The symptoms and biological abnormalities resolved with supportive treatment within 7 days. The global lethality was 16.1% associated with liver failure. Conclusion: Severe sickle cell intrahepatic cholestasis is more frequent than we thought and was mainly represented by borderline clinical feature. Early intervention may reduce the progression to organ failure stage and ultimate death.