Critical care emphasizes critical thinking, focuses on the triggers that lead to disease progression, and attaches great importance to early diagnosis of diseases and assessment of the compensatory capacity of vital organs. Pregnancy-associated atypical hemolytic uremic syndrome (P-aHUS) is relatively rare in the intensive care unit (ICU). Most cases occur within 10 weeks after delivery. Severe cases can be life-threatening. It characterized by microangiopathic hemolytic anemia, decreased platelet count (PLT), and acute kidney injury (AKI). Early clinical diagnosis is difficult due to its similarity to various disease manifestations. On January 28, 2024, a 26-year-old pregnant woman at 26⁺³ weeks gestation was transferred to the ICU 19 hours post-vaginal delivery due to abdominal pain, reduced urine output, decreased PLT, elevated D-dimer, tachycardia, increased respiratory rate and declined oxygenation. On the day of ICU admission, the critical care physician identified the causes that triggered the acute respiratory and circulatory events based on the "holistic and local" critical care thinking. The condition was stabilized rapidly by improving the capacity overload. In terms of etiological diagnosis, under the guidance of the "point and face" critical care thinking, starting from abnormality indicators including a decrease in hemoglobin (Hb) and PLT and elevated D-dimer and fibrin degradation product (FDP) without other abnormal coagulation indicators, the critical care physician ultimately determined the diagnosis direction of thrombotic microangiopathy (TMA) by delving deeply into the essence of the disease and formulating a laboratory examination plan in a reasonable and orderly manner. In terms of in-depth diagnosis, combining the disease development process, family history, and past history, applying the two-way falsification thinking of "forward and reverse" as well as "questioning and hypothesis", the diagnosis possibilities of preeclampsia, HELLP syndrome [including hemolysis (H), elevated liver function (EL) and low platelet count (LP)], thrombotic thrombocytopenic purpura (TTP), typical hemolytic uremic syndrome (HUS), and autoimmune inflammatory diseases inducing the condition was ruled out. The diagnosis of complement activation-induced P-aHUS was finally established for the patient, according to the positive result of the complement factor H (CFH). Active decision was made in the initial treatment. The plasma exchange was initiated early. "Small goals" were formulated in stages. The "small endpoints" were dynamically controlled in a goal-oriented manner to achieve continuous realization of the overall treatment effect through phased "small goals". On the 5th day of ICU treatment, the trend of microthrombosis in the patient was controlled, organ function damage was improved, and the patient was transferred out of the ICU. It is possible to reach a favorable clinical outcome for critically ill patients by applying a critical care mindset to quickly integrate diagnostic and therapeutic strategies, accurately identifying the triggers and causes that led to the progression of the disease, and using critical care medical techniques for early and effective intervention.
Humans ; Female ; Pregnancy ; Adult ; Atypical Hemolytic Uremic Syndrome/therapy* ; Intensive Care Units ; Pregnancy Complications, Hematologic/therapy* ; Critical Care

OBJECTIVETo summarize and review the clinical characteristics including clinical features, prenatal characteristics, diagnosis, treatments and short-term outcomes of the twin anemia-polycythemia sequence (TAPS) to improve the recognition of the disease.

METHODThe clinical data of one case with twin anemia-polycythemia sequence and the reports of 15 cases seen in the past 5 years were reviewed and analyzed.

RESULTThere was an increasing number of reports of cases with TAPS. Prenatal manifestation: among the 16 cases, TAPS occurred in 13 cases naturally and in 3 cases occurred after laser treatment. Amniotic fluid volume showed no significant difference in 16 cases. Middle cerebral artery peak systolic velocity (MCA-PSV) > 1.5 multiples of the median (MoM) in the donor were 11/16 cases and 3/16 cases were not tested. MCA-PSV < 1.0 MoM in the recipient were seen in 10/16 cases and in 3/16 cases MCA-PSV was not tested. Hydrops fetalis was found in 6/16 cases. Intrauterine intervention: intrauterine blood transfusion was performed in 4/16 cases, fetoscopic laser occlusion of chorioangiopagus vessels was performed in 4/16 cases, umbilical cord occlusion selective feticide was done in 2/16 cases and intrauterine hemodilution in the recipient was performed in 1/16 case. Postnatal manifestation: average hemoglobin concentration in the anemic neonate was 95 g/L and in the polycythemic one was 208 g/L, intertwin Hb difference was > 80 g/L in 10/16 cases and < 80 g/L in 2/16 cases (after intrauterine laser treatment). Intertwin reticulocyte count ratio was > 1.7 in 5/16 cases and < 1.7 in 1/16 case (after intrauterine laser treatment). Postnatal treatment: 9/16 cases of donor had anemia, among them, 6/16 cases were given blood transfusions, 6/16 cases of recipient with hyperviscosity underwent partial exchange transfusions. Neurodevelopmental follow-up during neonatal period was normal in 11/16 cases, in our case, neurodevelopmental follow-up at the corrected gestational age 3 months was normal.

CONCLUSIONTAPS is a new atypical form of twin-twin transfusion syndrome (TTTS) that presents as a large intertwin hemoglobin difference with one twin developing anemia and the other developing polycythemia, without oligohydramnios-polyhydramnios sequence that is required for the diagnosis of TTTS. We suggest that routine doppler studies and MCA-PSV measurements should be performed during each follow-up visit in all uncomplicated monochorionic twin pregnancies, in order to find out the cases required intrauterine intervention to decrease neonatal mortality rates and improve the prognosis.

Anemia ; diagnosis ; etiology ; therapy ; Blood Flow Velocity ; Blood Transfusion, Intrauterine ; Female ; Fetofetal Transfusion ; complications ; diagnosis ; Gestational Age ; Hemoglobins ; analysis ; Humans ; Infant, Newborn ; Laser Coagulation ; Male ; Middle Cerebral Artery ; diagnostic imaging ; physiopathology ; Polycythemia ; diagnosis ; etiology ; therapy ; Pregnancy ; Pregnancy Complications, Hematologic ; diagnosis ; therapy ; Prognosis ; Twins, Monozygotic ; Ultrasonography, Prenatal

INTRODUCTIONPregnancies in women with thrombophilia are associated with a higher risk of obstetric complications. We systematically reviewed the findings of relevant randomised controlled trials (RCTs) with the aim of investigating the effectiveness of low-molecular-weight heparins (LMWHs) in pregnant women with inherited thrombophilic disorders and its effect on the incidence of live births in these patients.

METHODSThe MEDLINE-PubMed and Cochrane CENTRAL databases from 2000 to 2010 were searched using a combination of keywords, including low-molecular-weight heparin, enoxaparin, pregnancy, live birth and thrombophilia. Studies were included if they were RCTs assessing the effect of anticoagulant treatment on live birth rates in women with a history of miscarriage without apparent causes other than thrombophilic disorder. Interventions included LMWH, with or without aspirin, aspirin alone or placebo controlled for the prevention of adverse pregnancy outcome.

RESULTS43 articles with seven RCTs were retrieved following the initial search, of which four studies had to be excluded as they assessed thromboembolic events as the final outcome (n = 1), focused on idiopathic recurrent miscarriages (n = 1), compared efficacy and safety of two doses of enoxaparin (n = 1), and examined patients with or without thrombophilic disorder (n = 1). Pooled data from the remaining three RCTs showed no significant difference in the improvement of live birth rates following LMWH interventions (p = 0.15).

CONCLUSIONAt present, the use of LMWH in women with inherited thrombophilia with recurrent pregnancy loss is not indicated. Large randomised placebo-controlled trials are further needed to prove the effectiveness of LMWH in these patients.

Female ; Heparin, Low-Molecular-Weight ; therapeutic use ; Humans ; Pregnancy ; Pregnancy Complications, Hematologic ; drug therapy ; Pregnancy Outcome ; Thrombophilia ; complications ; drug therapy ; Treatment Outcome

OBJECTIVETo study the relationship between thrombocytopenia in pregnancy associated with various causes and neonatal outcomes.

METHODSMedical records of 140 pregnant women with thrombocytopenia in pregnancy and the neonatal outcomes from January 2009 to December 2010 were reviewed retrospectively. The pregnant women were classified into four groups according to the causes of thrombocytopenia: gestational thrombocytopenia (GT; n=94), pregnancy with immune thrombocytopenic purpura (ITP; n=30), pregnancy with other hematological disease (aplastic anemia or myelodysplastic syndrome; n=12), and other causes (n=4): pregnancy induced hypertension syndrome, pregnancy with systemic lupus erythematosus, and pregnancy with alcoholic cirrhosis. The neonatal outcomes in the four groups were compared.

RESULTSThe premature birth rates in the GT and the ITP groups were 11.3% and 16.7%, respectively. There was no significant difference between the two groups. The premature birth rate in the other hematological disease group was 53.8%, which was significantly higher than that in the GT (P<0.01) and the ITP groups (P<0.05). Congenital passive immune thrombocytopenia was found in 2 neonates (2%) in the GT group and in 4 neonates (13%) in the ITP group (P<0.05). In addition, other diseases were also observed in neonates in the ITP group, including 1 case (3%) of ITP and 1 case (3%) of Evans syndrome. Intracranial hemorrhage occurred in one neonate (8%) in the other hematological disease group. Neonatal lupus syndrome was found in 1 case (25%) in the other causes group.

CONCLUSIONSThrombocytopenia in pregnancy associated with different causes may result in different neonatal outcomes.

Adult ; Female ; Humans ; Infant, Newborn ; Pregnancy ; Pregnancy Complications, Hematologic ; Pregnancy Outcome ; Premature Birth ; epidemiology ; Prognosis ; Retrospective Studies ; Thrombocytopenia ; complications ; drug therapy

Jr(a) is a high-frequency antigen found in all ethnic groups. However, the clinical significance of the anti-Jr(a) antibody has remained controversial. Most studies have reported mild hemolytic disease of the newborn and fetus (HDNF) in Jr(a)-positive patients. Recently, fatal cases of HDNF have also been reported. We report the first case of HDNF caused by anti-Jr(a) alloimmunization in twins in Korea. A 33-yr-old nulliparous woman with no history of transfusion or amniocentesis was admitted at the 32nd week of gestation because of vaginal bleeding caused by placenta previa. Anti-Jr(a) antibodies were detected in a routine laboratory examination. An emergency cesarean section was performed at the 34th week of gestation, and 2 premature infant twins were delivered. Laboratory examination showed positive direct antiglobulin test and Jr(a+) phenotype in the red blood cells and the presence of anti-Jr(a) antibodies in the serum in both neonates. The infants underwent phototherapy for neonatal jaundice; this was followed by conservative management. They showed no further complications and were discharged on the 19th postpartum day. Preparative management to ensure the availability of Jr(a-) blood, via autologous donation, and close fetal monitoring must be performed even in cases of first pregnancy in Jr(a-) women.
Adult ; Blood Group Antigens/immunology ; *Blood Group Incompatibility ; Diseases in Twins/diagnosis/*immunology ; Erythroblastosis, Fetal/*diagnosis/immunology ; Female ; Gestational Age ; Humans ; Infant, Newborn ; Isoantigens/immunology ; Jaundice, Neonatal/complications/immunology/therapy ; Male ; Phenotype ; Phototherapy ; Pregnancy ; Pregnancy Complications, Hematologic/diagnosis/*immunology ; Twins

Propylthiouracil (PTU) is known to be a potential cause of antineutrophil cytoplasmic antibody (ANCA) positive small vessel vasculitis, resulting in glomerulonephritis and diffuse alveolar hemorrhage (DAH). Herein, we describe a 25-year-old pregnant woman who developed a perinulcear ANCA (p-ANCA) and myeloperoxidase ANCA (MPO-ANCA) positive DAH during PTU therapy. The patient improved after corticosteroid therapy and discontinuation of the PTU. Methimazole was prescribed in spite of the risk of recurrence of DAH because of the pregnancy. The patient is currently free from pulmonary problems. Our case shows that the alternative agent, methimazole, can be used to treat hyperthyroidism in a pregnant patient with PTU associated DAH.
Tomography, X-Ray Computed ; *Pulmonary Alveoli ; Propylthiouracil/*adverse effects/therapeutic use ; *Pregnancy Complications, Hematologic ; Pregnancy ; Hyperthyroidism/blood/complications/*drug therapy ; Humans ; Hemoptysis/*chemically induced/diagnosis/immunology ; Female ; Diagnosis, Differential ; Bronchoscopy ; Antithyroid Agents/*adverse effects/therapeutic use ; Antibodies, Antineutrophil Cytoplasmic/*blood ; Adult

BACKGROUND: Idiopathic thrombocytopenic purpura (ITP) is a condition that often develops in young women and, consequently, physicians should frequently manage and monitor pregnant patients with this disorder. METHODS: We reviewed the charts of 30 women with chronic ITP delivered in 31 pregnancies from January 1995 to December 2003. RESULTS: Fifteen patients were diagnosed with ITP before pregnancy and sixteen patients were diagnosed during pregnancy. The mean platelet counts before pregnancy, during pregnancy, and at delivery were 70, 040/mm3, 83, 960/mm3, and 62, 680/mm3, respectively. The symptoms of hemostatic impairment were not noted in most of the pregnancies (77%, 24/31). During pregnancy and at delivery, most of the women (61%, 19/31) received various kinds of treatment to raise platelet counts. At delivery, the most commonly used therapy was platelet transfusion (48.4%, 15/31). Seven pregnancies (22.6%) were treated with corticosteroids during pregnancy and at delivery. Five pregnancies (16.1%) were treated with IV IgG during pregnancy and at delivery. Fifteen deliveries (51.7%) were performed by cesarean section and fourteen (48.3%) with vaginal delivery. Bleeding was uncommon at delivery. There were no cases of infants with any clinical signs of hemorrhage. CONCLUSION: Our current results suggest that ITP in pregnancy can proceed safely with low hemorrhagic risk in both infants and mothers, and that mothers with ITP can deliver healthy infants without serious hemorrhagic complications.
Adult ; Chronic Disease ; Comparative Study ; *Delivery, Obstetric/methods ; Female ; Glucocorticoids/therapeutic use ; Humans ; Immunoglobulin G/administration & dosage/therapeutic use ; Immunoglobulins, Intravenous/therapeutic use ; Infant, Newborn ; Platelet Count ; Platelet Transfusion ; Pregnancy ; *Pregnancy Complications, Hematologic/blood/diagnosis ; *Pregnancy Outcome ; *Purpura, Thrombocytopenic, Idiopathic/blood/diagnosis/therapy ; Retrospective Studies

A 27-yr-old woman who had been taking warfarin for 10 yr after mitral valve replacement became pregnant. After knowing her pregnancy, she received heparinization for nine weeks instead of warfarin, and took oral anticoagulant again. At 24 weeks of gestation, fetal ultrasound and MRI showed a left subdural hematoma, and the pregnancy was terminated. Subdural hematoma was demonstrated on autopsy. Fatal bleeding of the fetus is a rare complication of maternal warfarin medication, occurring mostly in the second or third trimester. There is no alternative regimen available, so that regular monitoring by fetal ultrasound and strict control of warfarin dose with regular measurement of prothrombin time are the best way to prevent intrauterine fetal death due to bleeding.
Adult ; Anticoagulants/*adverse effects ; Ductus Arteriosus, Patent/surgery ; Female ; Fetal Diseases/*chemically induced ; Heart Valve Diseases/therapy ; Hematoma/chemically induced ; Heparin/adverse effects ; Human ; Intracranial Hemorrhages/*chemically induced ; Maternal Exposure ; Pregnancy ; Pregnancy Complications, Hematologic ; Prothrombin Time ; Warfarin/*adverse effects