Siblings with pediatric sodium chlorite toxicity causing methemoglobinemia, renal failure and hemolytic anemia.

INTRODUCTION: Over the past decade, Miracle Mineral Solution (sodium chlorite) has been promoted as a cure-all for many conditions. CASE REPORT: A 9-year-old boy presented with his brother after they accidentally ingested a small amount of undiluted 22.4% sodium chlorite. Symptoms included nausea, vomiting, diarrhea, and dyspnea. Oxygen saturation remained 71% despite supplemental oxygen (15L/min). The patient was noted to have dark chocolate-appearing blood, minimal urine output, diffuse pallor and cyanosis. He developed methemoglobinemia, renal failure requiring renal replacement therapy and hemolysis requiring blood transfusion. DISCUSSION: These are the 7th and 8th reported cases of sodium chlorite toxicity by ingestion and the second and third in children. Takeaway for Physicians: Miracle Mineral Solution is a commonly purchased potentially lethal compound that can cause methemoglobinemia with respiratory failure, hemolytic anemia requiring transfusion and renal failure requiring dialysis.

Infantile Pyknocytosis: An Uncommon Cause of Newborn Hemolytic Anemia.

Infantile pyknocytosis is a rare cause of neonatal hemolytic anemia, which presents in the first few weeks of life. We report a classic case of infantile pyknocytosis that presented to our institution with rebound hyperbilirubinemia after receiving phototherapy. The infant was found to have a hemoglobin of 5.8 g/dL, requiring a total of 15 mL/kg of red blood cells (in 2 separate transfusions) before discharge. The diagnosis was ultimately made by a review of the peripheral blood smear. We review the literature and suggest pediatricians consider infantile pyknocytosis on their differential when hemolytic anemia presents in the newborn period.

Immunoglobulin for alloimmune hemolytic disease in neonates.

BACKGROUND: Exchange transfusion and phototherapy have traditionally been used to treat jaundice and avoid the associated neurological complications. Because of the risks and burdens of exchange transfusion, intravenous immunoglobulin (IVIg) has been suggested as an alternative therapy for alloimmune hemolytic disease of the newborn (HDN) to reduce the need for exchange transfusion. OBJECTIVES: To assess the effect and complications of IVIg in newborn infants with alloimmune HDN on the need for and number of exchange transfusions. SEARCH METHODS: We performed electronic searches of CENTRAL, PubMed, Embase (Ovid), Web of Science, CINAHL (EBSCOhost), Academic Search Premier, and the trial registers ClinicalTrials.gov and controlled-trials.com in May 2017. We also searched reference lists of included and excluded trials and relevant reviews for further relevant studies. SELECTION CRITERIA: We considered all randomized and quasi-randomized controlled trials of IVIg in the treatment of alloimmune HDN. Trials must have used predefined criteria for the use of IVIg and exchange transfusion therapy to be included. DATA COLLECTION AND ANALYSIS: We used the standard methods of Cochrane and its Neonatal Review Group. We assessed studies for inclusion and two review authors independently assessed quality and extracted data. We discussed any differences of opinion to reach consensus. We contacted investigators for additional or missing information. We calculated risk ratio (RR), risk difference (RD) and number needed to treat for an additional beneficial outcome (NNTB) for categorical outcomes. We calculated mean difference (MD) for continuous variables. We used GRADE criteria to assess the risk of bias for major outcomes and to summarize the level of evidence. MAIN RESULTS: Nine studies with 658 infants fulfilled the inclusion criteria. Term and preterm infants with Rh or ABO (or both) incompatibility were included. The use of exchange transfusion decreased significantly in the immunoglobulin treated group (typical RR 0.35, 95% CI 0.25 to 0.49; typical RD -0.22, 95% CI -0.27 to -0.16; NNTB 5). The mean number of exchange transfusions per infant was also significantly lower in the immunoglobulin treated group (MD -0.34, 95% CI -0.50 to -0.17). However, sensitivity analysis by risk of bias showed that in the only two studies in which the treatment was masked by use of a placebo and outcome assessment was blinded, the results differed; there was no difference in the need for exchange transfusions (RR 0.98, 95% CI 0.48 to 1.98) or number of exchange transfusions (MD -0.04, 95% CI -0.18 to 0.10). Two studies assessed long-term outcomes and found no cases of kernicterus, deafness or cerebral palsy. AUTHORS' CONCLUSIONS: Although overall results show a significant reduction in the need for exchange transfusion in infants treated with IVIg, the applicability of the results is limited because of low to very low quality of evidence. Furthermore, the two studies at lowest risk of bias show no benefit of IVIg in reducing the need for and number of exchange transfusions. Based on these results, we have insufficient confidence in the effect estimate for benefit of IVIg to make even a weak recommendation for the use of IVIg for the treatment of alloimmune HDN. Further studies are needed before the use of IVIg for the treatment of alloimmune HDN can be recommended, and should include blinding of the intervention by use of a placebo as well as sufficient sample size to assess the potential for serious adverse effects.

Neonatal management and outcome in alloimmune hemolytic disease.

INTRODUCTION: Hemolytic disease of the fetus and newborn (HDFN) occurs when fetal and neonatal erythroid cells are destroyed by maternal erythrocyte alloantibodies, it leads to anemia and hydrops in the fetus, and hyperbilirubinemia and kernicterus in the newborn. Postnatal care consists of intensive phototherapy and exchange transfusions to treat severe hyperbilirubinemia and top-up transfusions to treat early and late anemia. Other postnatal complications have been reported such as thrombocytopenia, iron overload and cholestasis requiring specific management. Areas covered: This review focusses on the current neonatal management and outcome of hemolytic disease and discusses postnatal treatment options as well as literature on long-term neurodevelopmental outcome. Expert commentary: Despite major advances in neonatal management, multiple issues have to be addressed to optimize postnatal management and completely eradicate kernicterus. Except for strict adherence to guidelines, improvement could be achieved by clarifying the epidemiology and pathophysiology of HDFN. Several pharmacotherapeutic agents should be further researched as alternative treatment options in hyperbilirubinemia, including immunoglobulins, albumin, phenobarbital, metalloporphyrins, zinc, clofibrate and prebiotics. Larger trials are warranted to evaluate EPO, folate and vitamin E in neonates. Long-term follow-up studies are needed in HDFN, especially on thrombocytopenia, iron overload and cholestasis.

Ceftriaxone-Induced Hemolytic Anemia in a Jehovah's Witness.

BACKGROUND Drug-induced immune hemolytic anemia (DIIHA) is a rare condition that may result from the administration of an antibiotic, most notably the cephalosporin class, commonly used in both the adult and pediatric populations. A delay in recognition by a provider may lead to continuation of the offending agent and possibly result in fatal outcomes. CASE REPORT We report the case of a 65-year-old woman on ceftriaxone infusions after being diagnosed with acute mitral valve endocarditis 3 weeks prior, which presented with severe anemia and bilateral transient vision loss. Being a Jehovah's Witness, the patient refused blood product transfusions and was managed with alternative therapies. The etiology of the symptoms was suspected to be a hemolytic anemia directly related to her ceftriaxone infusions. CONCLUSIONS This report demonstrates the importance of close vigilance while prescribing drugs known to cause hemolytic anemia. Although rare, drug-induced immune hemolytic anemia caused by ceftriaxone may be a potentially fatal condition, but with early recognition and withdrawal of the offending agent, successful treatment may ensue. Serological tests should be utilized to obtain a definitive diagnosis.

Neonatal outcome after fetal anemia managed by intrauterine transfusion.

UNLABELLED: In-utero transfusion is now well under control and improves the survival of foetuses monitored for fetal anemia with a survival rate of more than 80 %. The aim was to evaluate short-term neonatal outcome after fetal severe anemia managed by intrauterine transfusions. We did a retrospective study of all neonates born after management of severe fetal anemia (n = 93) between January 1999 and January 2013 in our regional center. The two main causes of anemia were maternal red blood cell alloimmunization (N = 81, 87 %) and Parvovirus B19 infection (N = 10, 10.8 %). In the alloimmunization group, phototherapy was implemented in 85.2 % of cases with a maximum level of bilirubin of 114.4 ± 60.7 (mg/dl). Transfusion and exchange transfusion were, respectively, required in 51.9 % and in 34.6 % of cases. One neonate presented a convulsive episode, and we observed three neonatal deaths. In the parvovirus group, none of the child had anemia at birth and no management was necessary. CONCLUSION: Contemporary management of Rhesus disease is associated with encouraging neonatal outcomes. In case of Parvovirus infection, no specific management is necessary at. But, in all cases of fetal anemia, children should be followed up with particular attention to neurologic development. WHAT IS KNOWN: • In-utero transfusion is now well under control and improves the survival of fetuses monitored for fetal anemia. • Limited studies are available on the effect of IUT on postnatal outcome in infants with a history of fetal anemia. What is New: • Contemporary management of severe Rhesus disease is associated with encouraging neonatal outcomes. • The majority of infants can be managed with phototherapy and a limited number of top-up transfusions and exchange transfusions. In case of Parvovirus infection, the short-term neonatal outcome is excellent.

Variable Clinical Presentations of ABO Incompatibility in Dizygotic Twins.

Newborns with ABO blood group incompatibility can have a spectrum of clinical presentations from remaining asymptomatic to severe hemolytic anemia with jaundice. This case presentation discusses dizygotic twins who demonstrated both ends of the clinical spectrum. Similar cases in which there is such extreme variation between twins were not attainable in the current literature, which prompted the authors to present it as a rare occurrence and one that was unexpected based on their past experience with ABO incompatibility both in singletons and in twins.

Plasma exchange for hemolytic crisis and acute liver failure in Wilson disease.

Wilson disease (WD) is a rare autosomal recessive disorder of copper metabolism which primarily involves the liver and the central nervous system. Rarely, WD can present as acute liver failure (ALF) and this disease is universally fatal in the absence of liver transplantation. The authors report a young girl with WD ALF, who showed signs of recovery after prompt initiation of plasma exchange (PE) and chelation therapy. Though liver transplantation could not be done in this child and the child died 8 d after stopping PE, this case highlights that PE can be a successful medical treatment in WD ALF and should be considered as a therapeutic measure to stabilize a patient by decreasing serum copper, reducing hemolysis, and helping to prevent renal tubular injury from copper and copper complexes until liver transplantation is possible.

Severe haemolytic anaemia due to ingestion of naphthalene (mothball) containing coconut oil.

Naphthalene, a widely used industrial and household chemical, has rarely been an agent of poisoning worldwide. Severe haemolysis from naphthalene poisoning is rare and can be a challenge to clinicians. We report a 22-year-old female, who accidentally ingested naphthalene mixed coconut oil and got admitted with recurrent vomiting, headache and passage of dark urine. Severe intravascular haemolysis with hypotension and neutrophilic leukocytosis was detected. She was treated with red blood cell transfusions, intravenous saline infusion and ascorbic acid.

Anaemia in pregnancy.

Anaemia in pregnancy, defined as a haemoglobin concentration (Hb) < 110 g/L, affects more than 56 million women globally, two thirds of them being from Asia. Multiple factors lead to anaemia in pregnancy, nutritional iron deficiency anaemia (IDA) being the commonest. Underlying inflammatory conditions, physiological haemodilution and several factors affecting Hb and iron status in pregnancy lead to difficulties in establishing a definitive diagnosis. IDA is associated with increased maternal and perinatal morbidity and mortality, and long-term adverse effects in the new born. Strategies to prevent anaemia in pregnancy and its adverse effects include treatment of underlying conditions, iron and folate supplementation given weekly for all menstruating women including adolescents and daily for women during pregnancy and the post partum period, and delayed clamping of the umbilical cord at delivery. Oral iron is preferable to intravenous therapy for treatment of IDA. B12 and folate deficiencies in pregnancy are rare and may be due to inadequate dietary intake with the latter being more common. These vitamins play an important role in embryo genesis and hence any relative deficiencies may result in congenital abnormalities. Finding the underlying cause are crucial to the management of these deficiencies. Haemolytic anaemias rare also rare in pregnancy, but may have life-threatening complications if the diagnosis is not made in good time and acted upon appropriately.

Fetal intraperitoneal injection of immunoglobulin diminishes alloimmune hemolysis.

We report a case of severe fetal anemia associated with maternal anti-M antibody that was treated by direct injection of pooled human immunoglobulin into the fetal abdominal cavity. Four treatments at a dosage of 2 g per-kg estimated fetal body weight were performed, and no side effects were observed. A healthy baby girl was delivered transvaginally at 38 weeks, with neither exchange transfusion nor phototherapy required. Follow-up over 12 months found no indications of anemia or developmental delay in the child. This is believed to be the first report of fetal anemia in a blood-type-incompatible pregnancy being treated successfully with only direct immunoglobulin injection into the fetus. The immunoglobulin may have functioned as a neutralizing antibody causing the anemia to improve.

[ABO hemolytic disease and developing of significant hyperbilirubinemia in term newborns: early predictive factors]. / Enfermedad hemolítica por incompatibilidad ABO y desarrollo de ictericia grave en recién nacidos de término: factores predictivos precoces.

UNLABELLED: Early hospital discharge has increased the risk of severe jaundice in term neonates with ABO incompatibility and hemolytic disease. AIMS: a) To identify predictive factors of severe hyperbilirubinemia (requiring phototherapy) in the first week of life; b) to determine the serum unconjugated bilirubin (UB) level cutoff at 24-36 hours that better predicts severe hyperbilirubinemia. METHOD: After parental consent was obtained, lab tests were measured at 24-36 hours, 3rd, 4-5th, 6-7th days of life. Predictive capacity of the serum UB level was assessed through the ROC curve analysis and estimation of the sensitivity, specificity and positive and negative predictive values of different serum UB level cut-offs. RESULTS: ABO incompatibility was identified in 172 (13.6%) of 1.263 healthy term newborns; 126 babies were included, 28 of them (22%) developed severe hyperbilirubinemia; 46 were excluded (33 did not grant consent, 11 were lost to follow up and 2 received NICU's care). These last had higher UB level at 24-36 hours than those that did not develop the condition during the first week of life. A serum UB value of 8.75 mg% at 24-36 hours showed the best performance: sensitivity 78%, specificity 83%, positive predicted value 45% and negative 95%. CONCLUSIONS: Serum UB at 24-36 hours of life might contribute to identify those term newborns with ABO incompatibility that have the highest risk of developing severe jaundice.

Role of vitamin E supplementation on serum levels of copper and zinc in hemolytic anemic patients with G6PD deficiency.

Vitamin E scavenges free radicals and may prevent destruction of RBC in Glucose6-phosphate dehydrogenase (G6PD) deficient hemolytic anemia, where changes in copper (Cu) and zinc (Zn) may act as additional contributory factors for hemolysis. In the present study changes in serum Cu and Zn and role of vitamin E supplementation on these changes were observed in hemolytic anemic patients with G6PD deficiency. This study was conducted in the Department of Physiology, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka during July 2005-June 2006. For this, 102 subjects with age ranged 5-40 years of both sexes were included in the study. Among them 68 were G6PD deficient patients, of whom 34 were in supplemented group and 34 were non-supplemented group. The supplemented group received vitamin E for 60 consecutive days at a dose of 800 IU/day for adult and 400 IU/day for children < or =12 years (4 times daily). Age and sex matched 34 apparently healthy subjects with normal G6PD level were taken to observe the base line data (healthy control) and also for comparison. All the G6PD deficient patients were selected from the Out Patient Department (OPD) of Hematology, BSMMU, Dhaka, and all the healthy subjects from personal contact. Blood G6PD level was done by spectrophotometric method and serum Cu, Zn levels by atomic absorption spectrophotometric method. To observe the availability of binding proteins serum total protein, albumin, globulin and A:G ratio were done by standard laboratory techniques. All parameters were measured on day 1 of their 1st visit and also on day 60 in deficient groups. Data analysis was done by appropriate statistical method. Serum Cu was significantly (p<0.001) higher but serum Zn, total protein, albumin, A/G ratio were significantly (p<0.001) lower in G6PD deficient groups in comparison to those of healthy control on day 1. After vitamin E supplementation, values of these parameters were comparable with those of healthy control in supplemented group in comparison to those of their pre-supplemented and non-supplemented groups both on day 1 and day 60. So, vitamin E supplementation has got its effective role in restoration of normal serum concentration of Cu and Zn in this group of patients.

[Infantile pyknocytosis: a rare form of neonatal hemolytic anemia. 5 case-studies]. / La pyknocytose infantile : une anémie néonatale mal connue à propos de 5cas.

UNLABELLED: Infantile pyknocytosis (IP) is a rare hematological entity of newborns. It is a form of hemolytic anemia with unusual red cell morphology: the red blood cells are distorted, irregular, and small with many projections. Spontaneous resolution usually occurs by 4-6months of age. OBSERVATION: We describe the clinical features and biological parameters of 5 cases of IP. The first symptoms were always early jaundice, which required phototherapy. Anemia was severe in all babies and red blood cell transfusion was needed. CONCLUSION: IP is a rare cause of neonatal anemia whose diagnosis is based on a careful peripheral blood smear examination. In our study, anemia was severe and required red blood cell transfusion. Ethnic specificity and familial occurrence are reported in our experience.

Cabot rings as a result of severe dyserythropoiesis in a dog.

An 11-year-old female Dachshund was presented with depression, diarrhea, weight loss, and radiographic evidence of masses involving the liver, spleen, and cranial lobe of the right lung. Results of a CBC included severe nonregenerative anemia (HCT 14.2%, hemoglobin, 4.3 g/dL, reticulocytes 66,000/microL) with marked metarubricytosis (nucleated RBCs 6.39 x 10(3)/microL). Examination of the peripheral blood smear revealed marked erythroid dysplasia, including marked anisocytosis with a prevalence of macrocytes, Howell-Jolly bodies, diffuse basophilic stippling, and multinucleated and atypical nucleated RBCs. Neutrophil hypersegmentation and giant forms were also noted. Numerous erythrocytes, particularly polychromatophilic cells, contained inclusions consistent with Cabot rings, which appeared as delicate red-purple ellipsoid or figure 8 structures. Rarely, Cabot rings were observed extracellularly. The dog was treated symptomatically with blood transfusions, prednisone, erythropoietin, and vitamin supplementation, but the anemia progressively worsened. The dog was euthanized 2 months after presentation. Bone marrow aspirate and core biopsy specimens obtained at the time of euthanasia revealed marked dysplastic changes in all cell lines, especially dyserythropoiesis, along with infiltrating carcinoma cells. A necropsy was performed, and histologic examination revealed poorly differentiated adenocarcinoma of the lung with multiple metastases to the marrow, spleen, and liver. The final diagnosis was marked myelodysplasia secondary to metastatic adenocarcinoma. Cabot rings are found rarely in humans with myelodysplasia, but have not been described previously in dogs. Based on the findings in this case, Cabot rings may occur rarely in dogs with severe dyserythropoiesis.

Plasmapheresis for hemolytic crisis and impending acute liver failure in Wilson disease.

Wilsonian crisis is fatal unless copper removal is initiated early and liver transplantation is performed for patients that fulfill criteria for a poor outcome. We report a patient presenting with severe hemolysis and impending acute liver failure that made a rapid recovery with prompt initiation of plasmapheresis and chelation therapy. Rapid copper removal by plasmapheresis alleviated hemolysis and liver injury. A review of the literature was performed examining the use of plasmapheresis and albumin dialysis with continuous veno-venous hemodialysis or molecular adsorbents and recirculating system.

[Hemolysis following valve surgery]. / L'emolisi dopo chirurgia valvolare.

Hemolysis is a frequent complication of the implant of prosthetic valves, and is conditioned by a variety of factors, most of which related to the type of valve implanted and to the hemodynamic conditions following implantation. If sufficiently severe, it may lead to varying degrees of hemolytic anemia. The following laboratory tests are useful to diagnose and assess the severity of hemolytic anemia: hemoglobin levels; reticulocyte count; the demonstration of schistocytes on a blood smear; serum levels of lactic dehydrogenase, haptoglobin and iron. Treatment of hemolysis includes the supplementation of iron and folate when their deficiency is evident. Transfusions are necessary only in cases of severe anemia refractory to treatment. The use of beta-blockers appears to decrease the severity of hemolysis, likely because of the induction of bradycardia and of their negative inotropic effects. Some cases have been described of erythropoietin treatment for hemolytic anemia in these conditions, with favorable outcome. However erythropoietin use should currently be restricted to patients with severe hemolytic anemia in whom surgical repair or transfusions should be avoided or deferred. The recognition and the estimation of severity of hemolysis after valve implantation are important steps in the patients' follow-up and the premise for a rational treatment.