JNMA; journal of the Nepal Medical Association
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JNMA J Nepal Med Assoc · Mar 2019
ReviewA Simplified Understanding of the Black Swan: Anti-phospholipid Antibody Syndrome.
Anti-phospholipid Antibody Syndrome or Hugh's syndrome is a heterogeneous disorder, first fully described in 1980s. The syndrome is caused by the presence of specific antibodies against phospholipid binding plasma proteins in the serum of the patient, with or without underlying autoimmune diseases, that causes prolongation of tests of coagulation. High index of clinical suspicion is required for diagnosis of Anti-phospholipid Antibody Syndrome. ⋯ Steroids, intravenous immunoglobulin and immunosuppressant are reported to be effective in severe cases of catastrophic antiphospholid syndrome characterized by rapid small vessel thrombotic involvement of multiple organ systems. Studies are evaluating the efficacy of direct thrombin inhibitors in the management of refractory cases. Keywords: anticoagulants; anti-phospholipid syndrome; obstetric APS; thrombotic APS.
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JNMA J Nepal Med Assoc · Mar 2019
Accuracy of Glucose Meter among Adults in a Semi-urban Area in Kathmandu, Nepal.
Glucose meters are gaining popularity in monitoring of blood glucose at household levels and in health care set-ups due to their portability, affordability and convenience of use over the laboratory based reference methods. Still they are not free of limitations. Operator's technique, extreme temperatures, humidity, patients' medication, hematocrit values can affect the reliability of glucose meter results. Hence, the accuracy of glucose meter has been the topic of concern since years. Therefore, present study aims to evaluate the analytical and clinical accuracy of glucose meter using International Organization for Standardization 15197 guideline. ⋯ Glucose meter readings were within clinically acceptable parameters despite discrepancies on analytical merit. Possible sources of interferences must be avoided during the measurement to minimize the disparities and the values should be interpreted with caution.
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JNMA J Nepal Med Assoc · Mar 2019
Case ReportsPersistent Mullerian Duct Syndrome with Polysplenia and Short Pancreas: A Case Report.
Persistent Mullerian duct syndrome is a rare entity and usually presents with common symptoms of undescended testis and hernia. The syndrome is caused by an insufficient amount of Mullerian inhibiting substance or due to the insensitivity of the target organ to Mullerian inhibiting substance. Polysplenia is a rare congenital disease manifested by multiple small accessory spleens. ⋯ He was diagnosed as Persistent Mullerian duct syndrome with unilateral cryptorchidism, polysplenia and short pancreas, coincidentally detected while evaluating for ureteric colic. He underwent Ureteroscopic Lithotripsy with stenting for ureteric calculus, however, he refused laparotomy with excision of mullerian structures. Keywords: cryptorchidism; persistent mullerian duct syndrome; short pancreas.
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JNMA J Nepal Med Assoc · Mar 2019
Case ReportsExtra Hepatic Portal Vein Obstruction with Solitary Left Kidney: A Case Report.
Extra Hepatic Portal Vein Obstruction in individual with solitary left kidney is rare occurence. Though there is no etiological association between Extra Hepatic Portal Vein Obstruction and solitary left kidney but the solitary left kidney decides the modality of treatment. Eighteen year lady referred to our institute with menorrhagia for 5 years and ultrasonography finding of splenomegaly and atretic right kidney. ⋯ Patient with Extra Hepatic Portal Vein Obstruction present mainly with recurrent episodes of variceal bleeding, splenomegaly and hypersplenism. Splenectomy and esophagogastric devascularisation is an effective modality of treatment for patient with Extra Hepatic Portal Vein Obstruction with solitary kidney. Keywords: cavernoma; modified Hassab's operation; Portal vein; unilateral renal agenesis.
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JNMA J Nepal Med Assoc · Mar 2019
Case ReportsCoexistence of Gonadal Dysgenesis and Mullerian Agenesis in a Female with 46 XX Karyotype: A Case Report.
Gonadal dysgenesis is a rare genetically heterogeneous disorder characterized by underdeveloped ovaries with consequent, impuberism, primary amenorrhea, and hypergonadotropic hypogonadism. Mullerian agenesis or Mayer‑Rokitansky‑Kuster‑Hauser syndrome is characterized by congenital aplasia of the uterus and the upper part (2/3) of the vagina in a woman with normal development of secondary sexual characteristics and a normal 46 XX karyotype. The association of gonadal dysgenesis and Mayer-Rokitansky-Kuster-Hauser syndrome is very rare and appears to be coincidental. ⋯ Internal genitalia could not be identified on the pelvic ultrasound and pelvic MRI. There were no other morphological malformations. Keywords: Gonadal dysgenesis; Mayer Rokitansky Kuster Hauser syndrome; Mullerian agenesis; primary amenorrhea; 46,XX.