CF twin preg 11-3-16
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(1) A 33-year-old cystic fibrosis patient with homozygous delta F508 mutation underwent in vitro fertilization and developed a twin pregnancy. (2) At 26 weeks, she experienced edema, poor appetite, and elevated liver enzymes, eventually developing hyperemesis gravidarum requiring total parenteral nutrition. (3) She delivered twins via emergency C-section at 29 weeks after developing a bowel obstruction requiring surgery. (4) While twin pregnancies are not preferred for cystic fibrosis patients due to increased abdominal pressure, her pulmonary function and nutrition recovered after delivery with supportive care including nutrition.
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Ppt dub
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Multiple pregnancy
1) Multiple pregnancies occur when more than one fetus develops simultaneously in the uterus. The main types are dizygotic (non-identical) twins, which account for 80% of twins, and monozygotic (identical) twins, which make up 20%.
2) Risk factors for dizygotic twins include being between 30-40 years old, above average height and weight, previous pregnancies, and certain fertility treatments.
3) Managing a twin pregnancy involves regular monitoring for complications like prematurity, anemia, preeclampsia, and intrauterine growth restriction. Labour involves close fetal monitoring and the potential for a higher rate of interventions.
laparoscopic radical hysterectomy for carcinoma cervix Female Pelvic Anatomy ...
This document discusses complications that can occur during and after laparoscopic radical hysterectomy (LRH) and how to prevent and manage them. It notes that complications can be divided into intraoperative, immediate postoperative, and delayed. Intraoperative complications include vascular injuries, ureteric injuries, and bowel injuries. Immediate postoperative complications are mainly hemorrhagic due to issues like clip slippage or inadequate vessel sealing. Delayed complications include thromboembolic episodes which can be prevented with prophylaxis like leg strapping and heparin. The document emphasizes that thorough knowledge of pelvic anatomy and careful surgical technique are important to prevent complications during LRH.
Designing a good ivf service
Designing an effective IVF program requires a patient-centered approach, not just a focus on technology. The key factors to consider include:
1) The types of treatment offerings and how they impact costs and space requirements.
2) Dimensions of service quality like patient-centeredness, timeliness, safety, effectiveness and efficiency.
3) Design choices for layout, interiors, equipment and protocols that influence patient experience, embryo safety, and effectiveness of treatments.
4) Ensuring the infrastructure for airflow, electricity, and gas supports the clinical needs while maintaining quality, safety and cost-effectiveness.
Ovarian stimulation oral agents
This document discusses ovarian stimulation using clomiphene citrate. It notes that estrogen is the main hormone that regulates gonadotropin production, so clomiphene citrate acts as a selective estrogen receptor modulator (SERM) to stimulate follicle growth. Clomiphene citrate treatment involves monitoring follicular growth, endometrial thickness, and progesterone levels. The starting dose depends on body weight and cycle monitoring is needed to properly time human chorionic gonadotropin injection and assess treatment response. Increasing the number of mature follicles, improving endometrial quality, and using intrauterine insemination can boost pregnancy rates when using clomiphene citrate for ovulation induction.
Laparoscopy 4
This document summarizes evidence-based guidelines for investigating infertile couples. It finds that while many tests have been advocated, the necessity and cost-effectiveness of many have not been proven. It recommends conventional semen analysis, assessment of ovulation with progesterone tests, and HSG or laparoscopy to assess tubal patency as useful initial tests. Tests like post-coital testing, hysteroscopy, and sperm penetration assays provide little predictive value on pregnancy rates and are less useful. A simplified approach focusing on proven essential tests can significantly reduce time and costs in evaluating infertility.
How to Setup IUI and IVF Services
This document discusses setting up IUI and IVF services. It covers the key components needed, including good lab design, infrastructure, equipment, and personnel. For infrastructure, it recommends building from scratch in a pollution-free area for an embryo-friendly environment. It provides details on room classification, air handling units, electricity, and gas supply. Essential and desirable equipment are outlined for IUI and IVF labs. It emphasizes the importance of a cohesive team approach. Startup costs are estimated at 1-1.5 lakhs for IUI and 40 lakhs for IVF, with an ideal setup costing around 100 lakhs plus 40-50 lakhs for infrastructure. Effective service provision focuses
Imaging of the placenta
This document discusses imaging of the placenta. Sonography is the preferred imaging modality for evaluating the placenta due to its ability to characterize tissue without radiation exposure. Magnetic resonance imaging can provide additional information, particularly for invasive placental processes like placenta accreta. Computed tomography has a limited role due to poorer tissue characterization and radiation risk to the fetus. The document describes normal placental anatomy and variants, as well as placental pathologies that can cause antepartum hemorrhage like placenta previa, placental abruption, and placental hematomas.
Endometrial hyperplasia
The revised 2014 WHO classification of endometrial hyperplasia is recommended, distinguishing between hyperplasia without atypia and atypical hyperplasia. Diagnosis is typically via endometrial biopsy. For hyperplasia without atypia, initial treatment involves counseling and observation, with progestogen therapy or a levonorgestrel intrauterine device recommended if regression does not occur. For atypical hyperplasia or failure of medical treatment, total hysterectomy is usually recommended. Special considerations apply to fertility-preserving treatment and management in breast cancer patients on tamoxifen.
Ovulation induction protocols in ivf cycles. warda
This document outlines ovarian stimulation protocols for fertility treatments. It discusses using clomiphene citrate or gonadotropins like hMG or FSH with timed intercourse or IUI initially. For more advanced treatments, it describes using birth control pills followed by gonadotropins and a HCG trigger before retrieving eggs through retrieval. It also notes drawbacks of the short stimulation protocol and compares the composition and activity of different gonadotropin medications.
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Ovulation induction protocols in ivf cycles. warda
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Liver diseases in pregnancy
1. Liver disease is a common complication in 3% of pregnancies. Two types discussed are intrahepatic cholestasis of pregnancy (ICP) and acute fatty liver of pregnancy (AFLP).
2. ICP occurs more frequently in certain populations and is associated with genetic and environmental factors. It can cause pruritis and elevated bile acids posing risks to the fetus like prematurity. AFLP is caused by a fetal fatty acid oxidation defect and presents with hepatic failure requiring prompt delivery.
3. HELLP syndrome is a severe form of preeclampsia characterized by hemolytic anemia, elevated liver enzymes and low platelets. Risk factors include prior preeclampsia. Management involves delivery with cort
Jaundice in pregnancy
The document summarizes various liver conditions that can cause jaundice in pregnancy. It discusses physiological changes in the liver during pregnancy and various causes of jaundice related and unrelated to pregnancy. These include viral hepatitis, intrahepatic cholestasis of pregnancy (ICP), preeclampsia, HELLP syndrome, and acute fatty liver of pregnancy (AFLP). It provides details on pathogenesis, clinical presentation, management and prognosis for these conditions. Pregnancy-related liver disorders can affect both mother and fetus, so prompt diagnosis and treatment are important.
HEG work on latest obstrestic and gyane.pptx
This document discusses hyperemesis gravidarum, a condition characterized by severe nausea and vomiting during pregnancy. It covers the etiology, pathology, clinical features, complications, and management. Key points include:
- Hyperemesis gravidarum affects 0.8-1.6% of pregnancies and is caused by hormonal changes and factors like H. pylori infection.
- Complications include dehydration, electrolyte imbalances, and Wernicke's encephalopathy from thiamine deficiency. Fetal complications include growth restriction.
- Management involves rehydration, vitamin supplementation including B1 and B6, antiemetics, and total parenteral
Neonatal Cholestasis
A case of a 3 month old boy with jaundice and pale stool is presented. On examination, he was icteric with hepatomegaly but no other abnormalities. Laboratory tests found direct hyperbilirubinemia. The objectives of the discussion are to understand neonatal cholestasis, evaluate cases, understand the differential diagnosis, and discuss treatment options. Neonatal cholestasis is prolonged conjugated hyperbilirubinemia beyond the first 14 days of life. Causes include extrahepatic conditions like biliary atresia or intrahepatic conditions like idiopathic neonatal hepatitis. Evaluation and management aim to identify treatable causes and prevent progression of liver disease.
Failure to thrive
- A 10-month-old Saudi girl presented to the emergency room with fever and diarrhea for 2 days. She was born prematurely at 30 weeks and had a history of respiratory distress, brain hemorrhage, and necrotizing enterocolitis as a newborn. On examination, she had a high fever but was not dehydrated. Laboratory tests showed abnormalities including low thyroid function and low albumin levels. She was diagnosed with failure to thrive likely due to her prematurity and past medical history. She received nutritional management and thyroid replacement therapy.
Cystic fibrosis
Cystic fibrosis is a genetic disease caused by mutations in the CFTR gene that result in a defective chloride channel protein. This leads to thick, sticky mucus in the lungs and other organs. In the lungs, the mucus clogs the airways and traps bacteria, causing chronic lung infections. While treatments can help manage symptoms, the only cure would be gene therapy to replace the defective gene or provide the normal protein before damage occurs. The goal of treatment is to clear the lungs of mucus and control infections.
Gastrocon 2016 - Pregnancy & Liver Disease
Dr Anil Arora address the liver diseases that are specific during pregnancy. The presentation contains case discussions on diagnosis, treatments & take home messages
Hypertensive disorders in pregnancy dr. betha fe m. castillo 102413
Hypertension is a common complication of pregnancy, affecting 5-10% of pregnancies. It includes gestational hypertension and preeclampsia. Gestational hypertension is defined as new hypertension after 20 weeks without proteinuria, while preeclampsia includes hypertension and proteinuria. Risk factors for preeclampsia include prior preeclampsia, chronic hypertension, diabetes, and family history. Symptoms involve the cardiovascular, renal, hepatic, and neurological systems. Treatment involves monitoring and delivery, with expectant management for mild cases and aggressive control of hypertension for severe preeclampsia.
Cystic fibrosis of the GIT
This document provides an overview of gastrointestinal complications of cystic fibrosis (CF), including distal intestinal obstruction syndrome, meconium ileus, gastroesophageal reflux disease, malignancies, pancreatic insufficiency and cystic fibrosis-related diabetes. It discusses the genetics, pathogenesis, clinical presentation, diagnosis and treatment of these conditions. Key points include that CF is caused by mutations in the CFTR gene, pancreatic insufficiency affects 85% of patients, and annual screening for cystic fibrosis-related diabetes is recommended beginning at age 10.
Pregnancy Dermatoses
Includes physiological skin changes in pregnancy, specific dermatoses such as intrahepatic cholestasis of pregnancy, polymorphic eruption of pregnancy (pruritic urticarial papules and plaques of pregnancy - PUPP), pemphigoid gestationalis and atopic eruption of pregnancy, as well as non-specific dermatoses ranging from infections, infestations, inflammations and immune disorders.
Powerpoint made by Dr. Jerriton, second year MD post graduate in DVL, SVMC, Pondy.
Seminar on neonatal cholestasis
This document provides information about evaluating a neonate presenting with cholestatic jaundice. It discusses performing a history and physical exam to identify potential etiologies. Key lab investigations are outlined to establish cholestasis and severity of liver injury. Imaging studies like ultrasound and hepatobiliary scintigraphy can help differentiate between extrahepatic and intrahepatic causes. The document reviews various etiologies of neonatal cholestasis including biliary atresia, idiopathic neonatal hepatitis, choledochal cyst, galactosemia, and TPN-related cholestasis. Timely evaluation is important to diagnose treatable conditions and identify those requiring surgical intervention.
Ghunhsl
This document describes the case of a 24-year-old pregnant woman admitted to the hospital with absence of fetal movements and symptoms of malaise, nausea and vomiting. Upon examination, she was found to have jaundice, mild leg edema, and abnormal liver function tests. Ultrasound revealed demise of the 34-week fetus and a sonographically normal liver. Further tests showed elevated liver enzymes and bilirubin consistent with liver disease, but serology tests for viral hepatitis were negative. This raises the possibility of pregnancy-related liver conditions like intrahepatic cholestasis of pregnancy or acute fatty liver of pregnancy. The next steps would be additional tests and imaging to determine the cause and guide management of the woman
Aflp
Acute Fatty Liver of Pregnancy (AFLP) is a rare but serious condition that affects 1 in 7,000-11,000 pregnancies. It is characterized by fatty infiltration and cellular dysfunction in the liver during late pregnancy or early postpartum. Prompt delivery is the recommended treatment as the condition does not typically improve until after delivery, and maternal and fetal mortality rates are high if not treated properly. Diagnosis is based on clinical presentation and lab tests in the absence of a definitive causative agent or diagnostic test. Close monitoring of future pregnancies is advised for women previously affected by AFLP.
cystic fibrosis
- An 8-month-old girl presented with cough, shortness of breath, and diarrhea since birth. She had recurrent lung infections and was failing to thrive.
- Testing found a sweat chloride level above normal, indicating cystic fibrosis. Genetic testing found a homozygous mutation in the CFTR gene.
- She was treated with antibiotics, pancreatic enzymes, airway clearance techniques, and nutritional supplementation. Her weight improved with treatment but she continued to have pulmonary exacerbations requiring hospital admissions. Future treatments may include gene and cell therapies as cystic fibrosis management continues to advance.
231629962-Cystic-Fibrosis-1
- An 8-month-old girl presented with cough, shortness of breath, and diarrhea since birth. She had recurrent lung infections and was failing to thrive.
- Initial workup found low albumin and elevated sweat chloride. Genetic testing found a homozygous mutation in the CFTR gene.
- She was diagnosed with cystic fibrosis and started on airway clearance techniques, pancreatic enzymes, vitamins, antibiotics, and nutritional support.
- Cystic fibrosis is a genetic disease caused by mutations in the CFTR gene resulting in thick mucus in the lungs and pancreas. Management focuses on airway clearance, nutrition, and treatment of infections to improve quality and length of life.
Vitamins and nervous system
Vitamin deficiencies can cause neurological manifestations. Cobalamin (vitamin B12) deficiency results in subacute combined degeneration, presenting with paresthesias, weakness, and spinal cord lesions. Folate deficiency causes similar symptoms. Vitamin E deficiency leads to spinocerebellar degeneration. Pellagra due to niacin deficiency can cause diarrhea, dermatitis, dementia, and other neurological symptoms. Pyridoxine deficiency can cause seizures in infants or neuropathy in adults. High doses of pyridoxine supplementation itself can cause neuropathy. Timely treatment of deficiencies can improve or stabilize neurological symptoms.
Vitamins and nervous system
Vitamin deficiencies, especially B12, folate, and vitamin E, can cause neurological manifestations. B12 deficiency results in subacute combined degeneration, presenting with paresthesias, weakness, and spinal cord involvement. Laboratory tests show elevated methylmalonic acid and homocysteine. Treatment involves high dose B12 injections. Folate deficiency causes similar symptoms and is treated with oral folate supplements. Vitamin E deficiency presents as a spinocerebellar syndrome with ataxia, areflexia, and sensory loss, treated with high dose vitamin E.
Pregnancy_Physiology-IUGR-PIH-edited14-09-10.ppt
The document discusses various physiological changes that occur during pregnancy including changes to body water metabolism, cardiovascular system, respiratory system, hematologic system, endocrine system, and other organ systems. It also discusses conditions like intrauterine growth restriction (IUGR) and hypertension that can arise during pregnancy. IUGR is defined as birth weight below the 10th percentile and can be symmetrical or asymmetrical. Hypertension in pregnancy includes chronic hypertension, gestational hypertension, and preeclampsia.
Malnutrition
The document outlines a lecture plan on malnutrition (hypotrophy) and protein-energy malnutrition. It begins with definitions of malnutrition and dystrophy. It then discusses the etiology and pathophysiology of malnutrition as well as classifications such as marasmus and kwashiorkor. The clinical manifestations are described including trophic disorders, gastrointestinal disorders, CNS dysfunctions, and immunological disorders. Laboratory tests and treatment/prevention are also mentioned. Globally, malnutrition remains a major health problem, especially in developing countries, and is a primary cause of childhood mortality.
Seminar on Neonatal Cholestasis.pptx
The document provides information on evaluating a neonate presenting with cholestatic jaundice. It discusses taking a thorough history and conducting a physical exam. Important investigations include liver function tests to establish cholestasis and assess severity, and tests to identify treatable conditions like infection or galactosemia. Imaging like ultrasound and hepatobiliary scintigraphy can differentiate between extrahepatic and intrahepatic causes. A liver biopsy further aids diagnosis and management of neonatal cholestasis. Common etiologies include biliary atresia, idiopathic neonatal hepatitis, and metabolic disorders. Timely evaluation is important to diagnose treatable conditions and avoid unnecessary surgery.
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CF twin preg 11-3-16
- 1. Nutritional Support Challenges for a Cystic Fibrosis Patient with Twin Pregnancy Michael M. Rothkopf, MD, FACN, Lisa Haverstick RD, Eleni Pellazgu, APN, Stanley Fiel, MD
- 2. Cystic Fibrosis - Overview • Autosomal recessive disease; 1 of 2,500 newborns • Cystic fibrosis transmembrane conductance regulator (CFTR) • c-AMP regulated ion channel - Cl, Na, H2O • Apical membrane of epithelial exocrine cells • CFTR defect chromosome 7, position q3 1.2 – Thickened, desiccated respiratory secretions – Pancreatic insufficiency and malabsorption – Increased RMR due to work of breathing – Diabetes and cirrhosis in older patients.
- 3. Delta F 508 CFTR Mutation • >1,900 CF mutations • delta F 508 most common (70%) • Deletion of 3 thymine nucleotides (TTT) spanning positions 507 and 508 of CFTR gene • Loss of a single codon for phenylalanine (F); • Abnormal CFTR folds improperly • CFTR protein can not escape endoplasmic reticulum
- 4. More CF Women are Getting Pregnant • CF survival markedly improved from 1969 • U.S. median age at death was 8.4 years of age vs 40 in 2011. • Pregnancy among CF females has also risen steadily • First case report in 1960 • CF Foundation Patient Registry (CFFPR) now reports 211 pregnancies/year
- 5. Fertility in Cystic Fibrosis • CFTR - expressed in reproductive organs • CF men – 97% infertile – vas deferens absent/abnormal • CF females – 20% infertile – thick cervical mucous – delayed menarche – small ovaries/uterus – aberrant cycles – decreased ovulation rates Endocrinology. 2008 Jun; 149(6): 2790–2797.
- 6. In-Vitro Fertilization in Cystic Fibrosis • First case report 2000 • Preimplantation genetic diagnosis (PGD) of the delta F508 deletion is possible through rapid DNA analysis • Biopsy cleavage-stage embryo • Amplification of DNA from single embryonic cells • Singlet pregnancy is goal • Twin pregnancy should be avoided
- 7. Biopsy of Embryos • Removal of one or more cells from the early preimplantation embryo • Embryos are cultured for 3 days, or until the blastocyst stage (5th/6th day of culture) • Removal of one or two blastomeres from each embryo • Biopsy does not negatively affect implantation or gestational development
- 8. Pregnancy Issues in CF • Reduced FRC, FVC • Difficult Chest PT • Increased pneumonia • Chronic hypoxia • Pulmonary HTN • Cor Pulmonale • Increased nutritional requirements • Gestational DM • Fatty Liver of Pregnancy • Bowel obstruction • Hyperemesis
- 9. Case Report - 33 year old female • ND, homozygous dF508 • In vitro twin pregnancy • Pre-pregnancy - good health, athletic, no nutritional deficiencies, weight 108, height 61”, FVC = 2.11 (62%), FEV1 = 1.19 (42%) • No initial exacerbation for her CF • At 26 weeks, developed bilateral lower extremity edema, poor PO intake and elevated transaminases • Dx’d with acute fatty liver of pregnancy, treated with ursodeoxycholic acid • Progressed to hyperemesis gravidarum with intractable nausea and vomiting • Started TPN - (AA) 120 grams, (150 % of req), Dext 70, no lipids 1,500 ml • Developed bowel obstruction, emergency C-section, SB resection • Successfully delivered a boy and girl at 29 weeks, 3 days • Nutritional status recovered after delivery • FVC = 2.1 (62%), FEV1 = 1.2 (42%) post discharge
- 10. Discussion – CF Twin Pregnancy • Despite impact of twins on abdominal pressure, PFTs were stable during pregnancy • LFT elevations – AFLP?, extrinsic hepatic compression?, intra-abdominal sepsis? • Bowel obstruction due to meconium ileus at birth/bowel surgery • TPN support essential but complex given pulmonary, hepatic and glycemic issues
- 11. Conclusion – CF Twin Pregnancy • CF patients do not necessarily suffer from pulmonary or nutritional crises during pregnancy • Twin pregnancy in CF is not preferred but can be managed with close monitoring • Liver disease in CF pregnancy may be multifactorial • Nutritional support is essential if PO intake is compromised
- 12. Thank You!
Editor's Notes
- Morphology and histology of ovaries, uteri, and cervix of 7-wk-old wild-type and Cftr−/− females. A, Gross morphology of reproductive tracts of wild-type (left) and Cftr−/− females (center and right). Bar, 500 mm. Note the overall size reduction in Cftr−/− female tracts as well as the thinner uteri. B and C, Histology of ovaries from wild-type (B) and Cftr−/− females (C). Bar, 500 μm. Wild-type ovaries displayed CL (marked by asterisk), whereas the markedly smaller Cftr−/− ovaries rarely contained CLs, even though other follicle stages were present. D–F, Histology of cervices from wild-type (D) and Cftr−/− females (E and F). Of the 15 Cftr−/− females examined, the Cftr−/− mouse in (F) was the only one with obvious physical blockage in the cervix (marked by arrowhead). Bar, 250 μm.