Prevalence and spectrum of haemoglobinopathies in females of reproductive age group- A first tertiary care center experience in Punjab, North India.

Background: Thalassemia and hemoglobinopathies are a group of inherited conditions characterized by abnormalities in the synthesis or structure of hemoglobin (Hb). According to estimates, approximately 7% of the world population is a carrier of Hb disorders, leading to high morbidity and mortality. To reduce the burden of these highly prevalent monogenic disorders, detecting them in the carrier stage is crucial to prevent disease progression. Aim: We aimed to estimate the prevalence and spectrum of hemoglobinopathies in females in the reproductive (20-40 years) age group. Settings and Design: It was a retrospective observational study carried out for 2.5 years (from January 2018 till June 2020). Materials and Methods: All the females in the age group of 20-40 years age whose blood samples were received in the department for High-Performance Liquid Chromatography (HPLC) were included. The cases with abnormal HPLC findings were analyzed for hematological parameters including hemoglobin, RBC count, and RBC indices [mean corpuscular volume (MCV), mean corpuscular hemoglobin (MCH), mean corpuscular hemoglobin concentration (MCHC), & red cell distribution width - coefficient of variation (RDW-CV)]. Statistical Analysis: Statistical package for social science (SPSS) statistics 21 version for Microsoft Windows (Chicago, USA) was used for statistical analysis of data. The data were described in terms of range, mean ± standard deviation (SD), frequencies (number of cases), and relative frequencies (percentage) as appropriate. Results: During the study period, 72.2% of the females were affected with ß-thalassemia trait, followed by HbD Punjab trait (17.8%), HbQ India trait (2.9%), ß-thalassemia major (1.8%), and two cases (1.2%) each of HbS trait, HbD Iran trait, and compound heterozygous of HbD Punjab and ß-thalassaemia, whereas HbE trait, compound heterozygous of HbQ and ß-thalassemia, compound heterozygous of HbJ-variant and ß-thalassemia had one case each (0.6%). Conclusion: Preventive strategies are cost-effective and include population screening, premarital screening, screening of spouses, genetic counseling, and prenatal diagnosis. Educating the carrier females about the potential risk and various screening methods may help in controlling the disease.

Comparison of norepinephrine and phenylephrine infusions for maintenance of haemodynamics following subarachnoid block in lower segment caeserean section.

BACKGROUND AND AIMS: Phenylephrine is the vasopressor of choice in spinal anaesthesia-induced maternal hypotension. However, it results in reflex bradycardia and decrease in cardiac output (CO), an effect that is perhaps less evident with the use of norepinephrine. We sought to evaluate the effect of phenylephrine and norepinephrine infusion on maternal systolic blood pressure (SBP), heart rate (HR), intraoperative nausea vomiting (IONV) and fatal Apgar scores. METHODS: A randomised double-blind study was conducted on 200 American Society of Anesthesiologists (ASA) II-III parturients undergoing caesarean section under subarachnoid block (SAB) who were randomised to two groups A and B to receive variable rate, manually controlled infusions of phenylephrine and norepinephrine targeting maintenance of SBP to 100% of the baseline value. Maternal haemodynamics especially episodes of hypotension, IONV and vasopressor consumption were observed and recorded. RESULTS: A statistically significant trend of lower SBP was observed during the first 6 min following intrathecal injection in group A (P value - 0.000). Though a greater number of parturients experienced ≥1 episode of hypotension in Group A vs Group B (13% vs 9%), the difference was, however, statistically insignificant. The incidence of bradycardia was higher in group A than in group B (16% vs 1%) and was found to be statistically significant (P < 0.05). The episodes of hypertension, IONV, maternal vasopressor consumption and neonatal Apgar score were comparable among both the groups. CONCLUSION: A dilute solution of norepinephrine infusion is comparably efficacious to the current gold standard vasopressor phenylephrine in maintaining blood pressure following spinal anaesthesia for caesarean delivery, with a significantly lower incidence of bradycardia.

Clinicobiochemical Parameters and Predictors of Liver Disease in Hospitalized Asian Indian Pregnant Women in a Tertiary Care Center in Northern India.

Introduction  During pregnancy, liver dysfunction is more frequent than expected and may require specialized care. For the early diagnosis, it is important to determine if changes in liver physiology may develop into liver disease. Liver disease during pregnancy may require intervention from a hepatologist for adequate monitoring of mother-fetus health outcomes. This study was aimed to evaluate the clinical profile and predictors of maternal mortality in patients with liver diseases among Asian-Indian-females. Methods We conducted a prospective, open-label, consecutive all-comers study of 2,663 pregnant Asian Indian women admitted in the hospital, which included 92 with liver dysfunction. The medical aspects of the pregnancy were then followed prospectively with laboratory and clinical data during the hospital stay and analyzed. The current study was approved by the Institutional Ethical Committee. Results We found that 92 out of 2,663 patients had liver dysfunction with a prevalence of 3.45%. Fifty-four (58.7%) patients had icterus followed by fever in 23 (25.0%), hypertension in 22 (23.9%), central nervous system manifestations in 21 (22.8%), abdominal pain in 19 (20.6%), vomiting in 19 (20.6%), and pruritus in six (6.5%). Predictors of maternal mortality were icterus (p = 0.04), hepatomegaly (p = 0.04), presenting serum-bilirubin greater than 10 milligram% (mg%) (p = 0.008). The most common etiology was acute viral hepatitis (45.6%), followed by a hypertensive disorder of pregnancy (29.3%), acute fatty liver of pregnancy (1.1%), cholestatic jaundice (9.8%), hyperemesis gravidarum (2.2%), septicemic hepatitis (3.3%), dengue immunoglobulin M (IgM), and plasmodium vivax malaria antigen positive in (2.2%) each. Four patients (4.3%) were leptospira IgM reactive and had co-infection with hepatitis E virus. There was one patient (1.1%) with underlying chronic liver disease. Idiopathic liver disease was present in 5.4% of patients. Conclusion Liver disease is relatively common in Indian pregnant women. It is associated with high maternal and perinatal mortality, even in a tertiary referral center. When managing pregnancy in a tertiary care center, for adequate follow-up of the disease and to prevent adverse consequences for mother and child, it is important to discard liver alterations early. For this purpose, liver disease during pregnancy needs early diagnosis for proper management. Furthermore, it is difficult to manage patients with preexisting liver disease, and it may require specialized intervention from a hepatologist and a gastroenterologist.

Vitamin D: Its Deficiency and Effect of Supplementation on Maternal Outcome.

OBJECTIVES: Vitamin D deficiency is on a rise globally and so are the maternal complications related to it. This deficiency can be easily detected and corrected by simple oral supplementation for a better health outcome in pregnancy. METHODS: Antenatal women with no history of Vitamin D intake and first antenatal visit at our hospital between 26 to 28 weeks of gestation or after 34 weeks were tested for levels of Vitamin 25(OH)D. Deficient women (< 30 ng/ml) between 26 to 28 weeks were supplemented and tested again before delivery (Group A). Deficient women after 34 weeks who did not receive supplementation before delivery constituted Group B. Maternal outcome was noted and compared in both the groups. RESULTS: Out of the 189 Vitamin D deficient women included in the study; 105(55.5%) were enrolled in Group A and 84 (44.4%) in Group B. 24 (12.7%) women were severely deficient (<4 ng/ml), 134 (70.9%) were deficient (<20 ng/ml) and 28(14.8%) were vitamin D insufficient (20-30 ng/ml). A statistically significant reduction (<0.001) was observed in vitamin D deficient women after supplementation in group A. 5.7% women developed preeclampsia in group A as compared to 28.5% in group B (p<0.0001). Higher (13%) incidence of gestational diabetes mellitus was observed in group B as compared to group A (6.6%) though the difference was not significant. A significantly higher incidence of preterm labor was observed in group B (p=0.007). CONCLUSION: Vitamin D deficiency is correlated with a higher incidence of preeclampsia, gestational diabetes mellitus and preterm birth. Maternal screening in targeted population and its supplementation is recommended to improve maternal outcome.

A rational approach to manage surgical procedures in COVID Era - A perspective based on experience in a private referral hospital.

BACKGROUND AND AIMS: COVID-19 disease has imposed challenges in caring for non-Covid elective surgical patients. As elective surgeries become essential, we propose to evaluate our approach and outcomes of surgical procedures performed during the initial period of COVID-19 pandemic so as to provide a road-map for safer approach. MATERIAL AND METHODS: We retrospectively evaluated outcomes in patients who underwent essential elective and emergency surgeries during the 5-week period between April 18, 2020 and May 28, 2020. All patients were screened at the front desk on their arrival to identify possible exposure to SARS- CoV-2. Nasopharyngeal swab of patients requiring hospital admission was tested for COVID-19 by quantitative RT-PCR. Patients needing essential elective surgery were taken up for surgery if they tested negative for COVID-19. Emergency procedures were undertaken in a demarcated theatre for COVID after taking level-3 protection without delay. The clinical data was reviewed and analysed. RESULTS: A total of 764 surgical procedures were conducted, of which 70.7% were elective essential surgeries, with 95.4% of these patients being discharged in stable healthy condition. Approximately 23% of the elective and 26% of the emergency surgeries was categorised in the surgical difficulty category III and majority of these were performed under general anesthesia. Postoperative mortality was 1.04%, but the overall mortality rate was approximately 2.5%. Only two patients (0.3%) tested positive for COVID-19 in our series. CONCLUSION: A robust preoperative screening and testing can enable safe scheduling of essential elective surgeries.

Successful management of abnormal uterine bleeding from uterine arteriovenous malformations with progesterone in postabortal patients.

AIM: To study the feasibility of conservative management with progesterone as a treatment option for postabortal patients with uterine arterio-venous malformations (AVMs). METHODS: This prospective observational study was conducted in the tertiary care teaching hospital over a period of 2 years. Postabortal patients with abnormal uterine bleeding were enrolled. Diagnosis was made by history, clinical and radiological examinations. Oral norethisterone was used (10 mg twice daily for 3 weeks, maximum of three cycles). Descriptive statistics was used to present the data. RESULTS: A total of 30 patients were included. Majority (n = 17) had complete resolution of symptoms after a single 3-week course of progesterone therapy. Rest (n = 13) remained symptomatic and required second course. Of the later, only three remained symptomatic after 2 months, and underwent CT angiography followed by embolization. There was no report of any serious adverse events. CONCLUSION: Oral norethisterone is a safe, effective and novel oral drug as an alternative to embolization or surgical therapy for patients with postabortal AVM bleed. Larger studies are required to confirm the findings of the present study.

Pregnancy Outcomes in Isolated Oligohydramnios during Second Trimester: A Case Series.

Oligohydramnios is associated with increased maternal and foetal morbidities. However, some of the recent studies have shown no adverse effect of isolated oligohydramnios on perinatal outcome and recommends continuation of pregnancy. Pregnancies between 18-28 weeks with isolated Oligohydramnios were included. History and physical examination was recorded in a pre-designed proforma. All the cases received care as per the protocol. A total of seven patients were recruited of which one was a twin pregnancy with Oligohydramnios in both sacs. The mean age at presentation was 30 years. Three patients went into spontaneous explusion at an average gestational age of 22-24 weeks. One patient with twins delivered vaginally at 32 weeks. Rest were delivered by caesarean section between 34-35 weeks (indication in majority of the cases was foetal distress and cord compression). None of the babies suffered any complication and were discharged in good condition. Isolated oligohydramnios during second trimester does not increase adverse perinatal outcome significantly (but increases the caesarean section rate) and therefore, should not be an indication for termination of pregnancy.

Evaluating the Efficacy of Levonorgestrel Intrauterine System and Danazol for Relief of Postoperative Pain in Endometriosis.

INTRODUCTION: Endometriosis is an oestrogen-dependent disorder, manifests during reproductive years and is associated with pain and infertility. There is considerable debate about the effectiveness of various interventions for pain relief. AIM: To evaluate the efficacy of Levonorgestrel Intrauterine System (LNG-IUS) and Danazol in postoperative pain relief for patients with endometriosis. MATERIALS AND METHODS: Hundred patients with diagnosis of endometriosis, who were treated laparoscopically, entered the study to receive either danazol (600 mg once daily) or LNG-IUS (inserted during immediate post operative period) postsurgery, for pain relief. Patients were analysed for pain relief according to VAS score and recurrence of disease using ultrasonography at third and sixth months of follow up. RESULTS: There were 50% patients in stage IV of endometriosis. Majority of them presented with complaint of infertility (49%) and pelvic pain (43%). It was observed that LNG-IUS was significantly more effective in relieving pain compared to danazol (65.2% vs 38.0%, p<0.05). Recurrence rate was significantly lower in LNG-IUS users compared to other group. CONCLUSION: LNG-IUS was found to be more effective in relieving pain compared to danazol.