Icteric Skin Research Articles

An Exceptional Case of Cobalamin Deficiency that Presented with Extremely High Indirect Bilirubin Levels

Cobalamin deficiency anemia is a type of anemia that present with weakness, fatigue, icteric sclera and neuropathy. Main causes of cobalamin deficiency are low intake or decreased absorption (gastric and intestinal causes). In present case, we report a 65-year-old male who presented to the emergency department with signs and symptoms of cobalamin deficiency including bilateral peripheral neuropathy, icteric skin and sclera, and abdominal pain in right upper quadrant. He had low cobalamin and extremely high bilirubin levels (8mg/dL) in serum. After the diagnosis of cobalamin deficiency established, 1mg daily cobalamin treatment initiated for five days which would follow weekly and monthly intramuscular injections consequently. Hemolysis and other causes of elevated indirect bilirubin levels were excluded in differential diagnosis. Clinical and laboratory improvements were achieved after the treatment. In conclusion, physicians should kept in mind cobalamin deficiency even in subjects with unusual high levels of indirect bilirubin.

Neonate With Jaundice

A 3-day-old male infant presented to the emergency department for jaundice. He was born at 39 weeks of gestation via spontaneous vaginal delivery, with a weight of 3,056 g and Apgar scores of 8 and 9. The mother was 35 years old, and this was her second child after an uneventful pregnancy. He was breastfed 10 times per day, with 4 daily diaper changes. On examination, his vitals were normal, and he interacted well. He had icteric skin over the face, trunk, and legs, with subtle hyperpigmentation of the axilla and scrotum (Figure 1). Initial data demonstrated sodium levels of 133 mEq/L and potassium levels of 5.9 mEq/L. The pediatric emergency physician performed abdominal point-of-care ultrasonography (Figure 2).Figure 2Emergency abdominal ultrasonography shows an enlarged left adrenal gland above the left kidney apex (left panel arrowhead), with a highly coiled-cerebriform appearance resembling cerebral gyri in the suprarenal fossa (left panel arrow). The right adrenal gland was wrinkled and wavy, located above the right kidney apex (right panel arrowhead). Normal thin central echogenic stripe was absent, replaced by diffuse stippled echogenicity surrounded by a hypoechoic rim (right panel arrow).1Al-Alwan I. Navarro O. Daneman D. et al.Clinical utility of adrenal ultrasonography in the diagnosis of congenital adrenal hyperplasia.J Pediatr. 1999; 135: 71-75Abstract Full Text Full Text PDF PubMed Scopus (47) Google ScholarView Large Image Figure ViewerDownload Hi-res image Download (PPT) Congenital adrenal hyperplasia. The patient progressed to a sodium level of 131 mEq/L and a potassium level of 6.3 mEq/L the following day. On day 6, screening showed 17-hydroxyprogesterone 65 ng/dL. Additionally, physicians administered fludrocortisone and cortisone acetate with normalization of electrolyte derangement. Final data confirmed 21-hydroxylase deficiency, salt-wasting type congenital adrenal hyperplasia: with 17-OH progesterone levels of 21.9 ng/mL (reference range, 0.49-4.1 ng/mL), and adrenocorticotropic hormone levels of 585.8 pg/mL (reference range, 10-70 pg/mL). Infants with congenital adrenal hyperplasia present perinatally with ambiguous genitalia in females and life-threatening adrenal crisis in males. Although neonatal screening for 21-hydroxylase deficiency minimizes delays in diagnoses, one-third of infants with congenital adrenal hyperplasia were symptomatic when diagnosed,2Pang S.Y. Wallace M.A. Hofman L. et al.Worldwide experience in newborn screening for classical congenital adrenal hyperplasia due to 21-hydroxylase deficiency.Pediatrics. 1988; 81: 866-874PubMed Google Scholar with the most severe manifestation being cardiac arrest secondary to hyperkalemia.3Agarwal S. Deshpande G. Agarwal D. et al.Sudden cardiac arrest in a neonate with congenital adrenal hyperplasia.Pediatr Cardiol. 2005; 26: 686-689Crossref Scopus (3) Google Scholar In experienced hands, adrenal ultrasonography has a sensitivity of 92% and specificity of 100% for diagnosing congenital adrenal hyperplasia1Al-Alwan I. Navarro O. Daneman D. et al.Clinical utility of adrenal ultrasonography in the diagnosis of congenital adrenal hyperplasia.J Pediatr. 1999; 135: 71-75Abstract Full Text Full Text PDF PubMed Scopus (47) Google Scholar and is a useful adjunct for the rapid diagnosis of adrenal crisis in male infants with congenital adrenal hyperplasia.

A case of Fisher-Evans syndrome in a patient with type 2 diabetes mellitus after a COVID-19 infection

Patient A., female, aged 57, was admitted to the hospital with complaints of an increasing general weakness, low-grade fever, discomfort in the right hypochondrium, nausea, lack of appetite, icteric skin and sclera during the last week, the appearance of bruises on the body, hemorrhages that appear after injections or taking blood samples. Patient associated her condition with the COVID-19 infection 2 months before admission. The next day after admission, multiple hemorrhages appeared on the oral mucosa, blood pressure decreased, body temperature raised. She was consulted by a hepatologist and hematologist. Myelogram showed Fisher-Evans syndrome signs. Treatment started according to the protocol. On the third day, the patient showed a progressive deterioration, which was manifested by an increase in general weakness, motor aphasia, followed by the appearance of generalized tonic-clonic seizures with spontaneous resolution. Despite the intensive therapy, according to the recommendations of the hematologist (plasma and blood transfusion, glucocorticoids pulse-therapy) the patient’s condition progressively worsened. In the dynamics, hemoglobin and platelets decreased, signs of cerebral edema developed. On day 4 patient deceased. Conclusion Special attention should be paid to the prevention of recurrence of hemolysis and (or) thrombocytolysis in the event of infectious diseases, especially viral ones. The intake of drugs that block platelet function (for example, antiplatelet agents and anticoagulants) should be strictly controlled. In the presence of autoimmune pathology on an outpatient basis, clinical examination of such patients by hematologists, therapists and immunologists is necessary. Further study of the etiology and pathogenesis of the development of FES, observation of patients, identification of possible predictors of the severity of the disease and the development of an optimal therapy strategy are required.

A case of Fisher-Evans syndrome in a patientwith type 2 diabetes mellitus after COVID-19

A 57-year-old female patient with type 2 diabetes was admitted to the hospital with complaints of increasing general weakness, low-grade fever, nausea, lack of appetite, icteric skin and sclera during the last week, the appearance of bruises on the skin and hemorrhages after injections. The patient linked her condition with a history of COVID-19, 2 months before admission. The next day after admission, multiple hemorrhages appeared on the oral mucosa, the blood pressure decreased, and there was a rise in the body temperature. Laboratory data showed Fisher-Evans syndrome signs. Standard treatment was initiated. On the third day, the patient's condition showed progressive deterioration. Despite intensive therapy (plasma and blood transfusion, glucocorticoids pulse-therapy), the patient's status progressively worsened. Hemoglobin level and platelet count continued decreasing, and the signs of cerebral edema developed. On day 4 the patient deceased. The described clinical case is consistent with the literature data on the possibility of developing autoimmune disorders after COVID-19. As more and more patients are currently receiving antiplatelet and anticoagulant medications for various indications, it is important to remember that viral infections can provoke hemolysis.

Atresia de vías biliares extrahepaticas en pediatria y citomegalovirus como causante

La atresia de vías biliares es una alteración con gran importancia clínica, más frecuente en la primera infancia, secaracteriza por la obliteración proliferativa de los conductos biliares que progresa a fibrosis hepática, cirrosis einsuficiencia hepática en etapa terminal.El objetivo es presentar un caso clínico inusual y conocer la forma de actuar ante un caso similar. Se trata depaciente femenino de 4 meses de edad, con piel y mucosas ictéricas – verdosas, con hígado palpable debajodel reborde costal derecho, bazo palpable debajo del reborde costal izquierdo. Serología para Citomegaloviruspositivo. La ecografía abdominal reporto: Atresia de vías biliares.Se indicó una portoenterostomia de Kasai, sin embargo por la edad de la paciente, el procedimiento quirúrgicono se realizó.La atresia de vías biliares es una patología poco frecuente, su etiología es desconocida, pero es importante saberreconocerla durante los primeros 2 meses de vida, para evitar complicaciones.

Evans Syndrome: A Case Report

Evans syndrome, a combined clinical condition of autoimmune haemolytic anaemia (AHA) and idiopathic thrombocytopaenic purpura (ITP) and has non-specific pathogenesis. The clinical cases are extremely rare, since only 4% of AHA or ITP are incorporated with Evans. It is distinguished from differentials, such as lupus, IgA deficiency, and acquired immunodeficiency, by peripheral blood film, bone marrow, Coombs test, and coagulation profile. A case of adult female from Pabna, Bangladesh is documented in this report. She complained of high grade intermittent fever, exertional dyspnea, icteric skin and sclera. Other features included mild splenomegaly, dark urine, and profuse sweating after fever. Investigation reports were consistent with AHA and ITP, with normal coagulation and viral profile. However, the patient was treated with corticosteroids, platelet and blood transfusion. And in follow-up visits, there was a pattern of gradual decline in erythrocyte sedimentation rate (ESR) and reticulocyte count, with normalization of haemoglobin, red cell, and white cell count. No association with other diseases was found in this case.
 Bangladesh Med J. 2018 Jan; 47 (3): 37-40

Clinical Features and Laboratory Data Analysis of Glucose-6- Phosphate Dehydrogenase Deficiency

To explore clinical features and laboratory data of glucose-6-phosphate dehydrogenase（G6PD）deficiency and to investigate the relationship between them. Clinical data of 43 patients with G6PD deficiency was analyzed, the statistical method was applied to investigate the relationship between clinical features and laboratory data. Among 43 patients，neonatal jaundice occurred as the first symptom in 10 cases，while acute hemolytic anemia occurred as the first symptom in 23 cases. The major clinical symptoms of G6PD deficiency included icteric skin and/or sclera，dark urine，fever，gastrointestinal symptoms，fatigue and lethargy. Symptoms of 26 patients were caused by obvious inducement，including fava beans（61.5%），infection（34.6%）and miocardial infarction（3.8%）. All of 43 patients showed decreased G6PD activity，while the level of their indirect serum bilirubin（IBIL）was positively correlated with reticulocyte percentage（Ret%，r=0.5881，P=0.013） and mean corpuscular volume（MCV，r=0.6854，P=0.0024）. Patients with neonatal jaundice as the first symptom，showed higher level of Ret%（P<0.01）and MCV（P<0.001）and low RBC count（P<0.01）and low Hb level（P<0.01）. as compard with patients with acute hemolytic anemia as first symptome. Neonatal jaundice and acute hemolytic anemia are common clinical features of G6PD deficiency. Laboratory results of IBIL，Ret% and MCV have auxiliary value to evaluate the severity of hemolysis induced by G6PD deficiency. Patients with neonatal jaundice as their first symptom show more severe hemolysis than those only suffered from acute hemolytic anemia.

HBV and HCV coinfection associated with warm-type autoimmune hemolytic anemia: a case report.

Received/Gelis tarihi : December 15, 2012 Accepted/Kabul tarihi : April 30, 2013 To the Editor, The hepatitis virus may play an important role in autoimmune hemolytic anemia (AIHA). We herein report the case of a patient presented to our hospital with hepatitis B virus (HBV) and hepatitis C virus (HCV) coinfection who developed warm-type AIHA. The anemia improved, followed by declined viral levels, after rituximab plus antiviral therapy. To the best of our knowledge, this is the first reported case of HBV and HCV coinfection associated with warm-type AIHA. Written informed consent was obtained from the patient. A 66-year-old woman presented to the department of internal medicine of our hospital on 31 August 2010 with jaundice, inappetence, dizziness, palpitation, and fatigue. A month before admission, the patient complained of fatigue and palpitation, particularly aggravated after exercise, but she did not pay attention to it due to relief of these symptoms at rest. Three weeks later, the patient felt inappetence and dizziness, and her fatigue and palpitation did not go into remission at rest. Her daughter noticed her jaundice at this point and directed her to our hospital. She did not have past medical history of diffuse connective disease, lymphoproliferative disease, transfusion, favism, or allergic contact dermatitis. She had no related social history of cigarette, alcohol, or drug use. Her general physical examination revealed icteric skin and sclerae. Her abdominal examination showed a slightly increased spleen located in the left-upper abdomen. Complete blood count showed severe decrease of red blood cell count (1.39x1012/L), hemoglobin (53 g/L), and platelet count (73x109/L), in addition to significantly increased reticulocyte percentage (19%). Blood biochemistry revealed elevated total bilirubin (6.62 mg/dL), unconjugated bilirubin (4.95 mg/dL), and lactic dehydrogenase (528 U/L), with remarkable decrease of haptoglobin (0.3 g/L). The immunological test displayed

On the inverse problem in transcutaneous bilirubinometry

Transcutaneous bilirubinometry based on reflectance spectroscopy technique requires the development of suitable optical models of icteric skin and the solution of the inverse problem to derive pigment concentrations from spectral measurements. An algorithm is presented which allows the evaluation of bilirubin concentration during phototherapy. The present analysis shows that quantitative evaluation of bile pigment concentrations during phototherapy can be obtained with a good degree of accuracy by an inverse best-fit procedure; moreover, the knowledge of pigment kinetics makes this method almost independent of other conditions of the skin. The present results refer to the initial part of the phototherapy cycle and are based on a simple model of icteric skin; they may help to extend the application range of commercial transcutaneus bilirubinometer and to investigate the photoconfigurational transient of phototherapy.

Isolated splenic vein occlusion. Review of literature and report of an additional case.

In 1866, Gretsel described a syndrome consisting of primary splenomegaly, secondary anemia, and chronicity. Griessinger termed this splenic anemia. 1 In 1894, Banti isolated his syndrome from this broad entity. 1 Banti defined three clinical features: (1) a period lasting from three to five years and characterized by splenomegaly and anemia; (2) a short period characterized by hepatomegaly and oliguria; and (3) a final period lasting one to two years and characterized by ascites and a small liver. He also noted that the syndrome was frequently associated with hemorrhages from the mouth and bowel, an icteric skin hue (without true icterus), and a secondary anemia distinguished by leukopenia and relative lymphocytosis. Pathologically, Banti noted that this entity demonstrated (1) endophlebitis and calcification of the splenic vein; (2) splenic reticular fibrosis with narrowing of the splenic vein and capsular thickening; (3) presence of cirrhosis of the liver; and (4) finding of