Search results for: Syed Adnan Qasim

Authors: Makda Aamir, Sood Aayushi, Syed Omar, Nihan Khuld, Iskander Peter, Ijaz Naeem, Sharma Nishant

Abstract:

Introduction:Glycogen Storage Disease Type-1 (GSD-1) is a rare phenomenon primarily affecting the liver and kidney. Excessive accumulation of glycogen and fat in liver, kidney, and intestinal mucosa is noted in patients with deficiency of Glucose-6-phosphatase deficiency. Patients with GSD-1 have a wide spectrum of symptoms, including hepatomegaly, hypoglycemia, lactic acidemia, hyperlipidemia, hyperuricemia, and growth retardation. Age of onset, rate of disease progression and its severity is variable in this disease.Case:An 18-year-old male with GSD-1a, Von Gierke’s disease, hyperuricemia, and hypertension presented to the hospital with nausea and vomiting. The patient followed an hourly cornstarch regimen during the day and overnight through infusion via a PEG tube. The complaints started at work, where he was unable to tolerate oral cornstarch. He washemodynamically stable on arrival. ABG showed pH 7.372, PaCO2 30.3, and PaO2 92.2. WBC 16.80, K+ 5.8, HCO3 13, BUN 28, Cr 2.2, Glucose 60, AST 115, ALT 128, Cholesterol 352, Triglycerides >1000, Uric Acid 10.6, Lactic Acid 11.8 which trended down to 8.0. CT abdomen showed hepatomegaly and fatty infiltration with the PEG tube in place.He was admitted to the ICU and started on D5NS for hypoglycemia and lactic acidosis. Per request by the patient’s pediatrician, he was transitioned to IV D10/0.45NS at 110mL/Hr to maintain blood glucose above 75 mg/L. Frequent accuchecks were done till he could tolerate his dietary regimen with cornstarch. Lactic acid downtrend to 2.9, and accuchecks ranged between 100-110. Cr improved to 1.3, and his home medications (Allopurinol and Lisinopril) were resumed. He was discharged in stable condition with plans for further genetic therapy work up.Discussion:Mainstay therapy for Von Gierke’s Disease is the prevention of metabolic derangements for which dietary and lifestyle changes are recommended. A low fructose and sucrose diet is recommended by limiting the intake of galactose and lactose to one serving per day. Hypoglycemia treatment in such patients is two-fold, utilizing both quick and stable release sources. Cornstarch has been one such therapy since the 1980s; its slow digestion provides a steady release of glucose over a longer period of time as compared with other sources of carbohydrates. Dosing guidelines vary from age to age and person to person, but it is highly recommended to check BG levels frequently to maintain a BG > 70 mg/dL. Associated high levels of triglycerides and cholesterol can be treated with statins, fibrates, etc. Conclusion:The management of hypoglycemia in GSD 1 disease presents various obstacles which could prove to be fatal. Due to the deficiency of G6P, treatment with a specialized hypoglycemic regimen is warranted. A D10 ½ NS infusion can be used to maintain blood sugar levels as well as correct metabolic or lactate imbalances. Infusion should be gradually weaned off after the patient can tolerate oral feeds as this can help prevent the risk of hypoglycemia and other derangements. Further research is needed in regards to these patients for more sustainable regimens.

Keywords: von gierke, glycogen storage disease, hypoglycemia, genetic disease

Procedia PDF Downloads 76

Authors: Sugam Kale, Arif Uzair Bin Mohammed Roslan, Cindy Lee, Syed Beevee Mohammed Ismail

Abstract:

Preemptive analgesia is an established concept in the modern practice of anaesthesia. To be most effective, it is best instituted earlier than the surgical stimulus and should last beyond the offset of surgically induced pain till healing is complete. Whereas the start of afferent pain blockade with regional anaesthesia is common, its effect often falls short to cover the entire period of pain impulses making their way to CNS in the post-operative period. We tried to use a combination of two regional anaesthetic techniques used sequentially to overcome this handicap. Madam S., a 56 year old lady, was scheduled for elective surgery for pancreatic cancer. She underwent laparotomy and distal pancreatectomy, splenectomy, bilateral salpingo oophorectomy, and sigmoid colectomy. Surgery was expected to be extensive, and it was presumed that the standard pain relief with PCA with opiates and oral analgesics would not be adequate. After counselling the patient pre-operative about the technique of regional anaesthesia techniques, including epidural catheterization and rectus sheath catheter placement, their benefits, and potential complications, informed consent was obtained. Epidural catheter was placed awake, and general anaesthesia was then induced. Epidural infusion of local anaesthetics was started prior to surgical incision and was continued till 60 hours into the postoperative period. Before skin closure, the surgeons inserted commercially available rectus sheath catheters bilaterally along the midline incision used for laparotomy. After 46 hours post-op, local anaesthetic infusion via these was started as bridging while the epidural infusion rate was tapered off. The epidural catheter was removed at 75 hours. Elastomeric pumps were used to provide local anaesthetic infusion with the ability to vary infusion rates. Acute pain service followed up the patient’s vital signs and effectiveness of pain relief twice daily or more frequently as required. Rectus sheath catheters were removed 137 hours post-op. The patient had good post-op analgesia with the minimal additional analgesic requirement. For the most part, the visual analog score (VAS) for pain remained at 1-3 on a scale of 1 to 10. Haemodynamics remained stable, and surgical recovery was as expected. Minimal opiate requirement after an extensive laparotomy also translates to the early return of intestinal motility. Our experience was encouraging, and we are hoping to extend this combination of two regional anaesthetic techniques to patients undergoing similar surgeries. Epidural analgesia is denser and offers excellent pain relief for both visceral and somatic pain in the first few days after surgery. As the pain intensity grows weaker, rectus sheath block and oral analgesics provide almost the same degree of pain relief after the epidural catheter is removed. We discovered that the background infusion of local anaesthetic down the rectus sheath catherter largely reduced the requirement for other classes of analgesics. We aim to study this further with a larger patient cohort and hope that it may become an established clinical practice that benefits patients everywhere.

Keywords: rectus sheath, epidural infusion, post operative analgesia, elastomeric

Procedia PDF Downloads 101

Authors: Muhammad Haris, Syed Kamran Ali, Mubeen Islam, Tariq Mehmood, Faisal Shah

Abstract:

Jacobabad Khairpur High, part of a Sukkur rift zone, is the separating boundary between Central and Southern Indus Basin, formed as a result of Post-Jurassic uplift after the deposition of Middle Jurassic Chiltan Formation. Habib Rahi Formation of Middle to Late Eocene outcrops in the vicinity of Jacobabad Khairpur High, a section at Rohri near Sukkur is measured in detail for lithofacies, microfacies, diagenetic analysis and sequence stratigraphy. Habib Rahi Formation is richly fossiliferous and consists of mostly limestone with subordinate clays and marl. The total thickness of the formation in this section is 28.8m. The bottom of the formation is not exposed, while the upper contact with the Sirki Shale of the Middle Eocene age is unconformable in some places. A section is measured using Jacob’s Staff method, and traverses were made perpendicular to the strike. Four different lithofacies were identified based on outcrop geology which includes coarse-grained limestone facies (HR-1 to HR-5), massive bedded limestone facies (HR-6 HR-7), and micritic limestone facies (HR-8 to HR-13) and algal dolomitic limestone facie (HR-14). Total 14 rock samples were collected from outcrop for detailed petrographic studies, and thin sections of respective samples were prepared and analyzed under the microscope. On the basis of Dunham’s (1962) classification systems after studying textures, grain size, and fossil content and using Folk’s (1959) classification system after reviewing Allochems type, four microfacies were identified. These microfacies include HR-MF 1: Benthonic Foraminiferal Wackstone/Biomicrite Microfacies, HR-MF 2: Foramineral Nummulites Wackstone-Packstone/Biomicrite Microfacies HR-MF 3: Benthonic Foraminiferal Packstone/Biomicrite Microfacies, HR-MF 4: Bioclasts Carbonate Mudstone/Micrite Microfacies. The abundance of larger benthic Foraminifera’s (LBF), including Assilina sp., A. spiral abrade, A. granulosa, A. dandotica, A. laminosa, Nummulite sp., N. fabiani, N. stratus, N. globulus, Textularia, Bioclasts, and Red algae indicates shallow marine (Tidal Flat) environment of deposition. Based on variations in rock types, grain size, and marina fauna Habib Rahi Formation shows progradational stacking patterns, which indicates coarsening upward cycles. The second order of sea-level rise is identified (spanning from Y-Persian to Bartonian age) that represents the Transgressive System Tract (TST) and a third-order Regressive System Tract (RST) (spanning from Bartonian to Priabonian age). Diagenetic processes include fossils replacement by mud, dolomitization, pressure dissolution associated stylolites features and filling with dark organic matter. The presence of the microfossils includes Nummulite. striatus, N. fabiani, and Assilina. dandotica, signify Bartonian to Priabonian age of Habib Rahi Formation.

Keywords: Jacobabad Khairpur High, Habib Rahi Formation, lithofacies, microfacies, sequence stratigraphy, diagenetic history

Procedia PDF Downloads 427