Resistant anemia in a kidney transplant recipient: Pure red cell aplasia due to parvovirus B19 infection.

Anemia in kidney transplant recipients can stem from a diverse array of etiologies, including dietary deficiencies, inflammatory processes, allograft dysfunction, as well as viral and bacterial infections. We present a case of refractory anemia in a 49-year-old male patient occurring within the initial month following a kidney transplant, which persisted despite numerous transfusions, posing a formidable challenge. The patient was maintained on the standard immunosuppressant regimen-Tacrolimus, Mycophenolate, and Prednisolone. Diagnostic evaluations eliminated well-established causes such as dietary deficiencies, gastrointestinal losses, and prevalent infections. Subsequently, after viral PCR testing, a diagnosis of Pure Red Cell Aplasia (PRCA) due to infection with parvovirus B19 was made. Although the patient had a reduction in the immunosuppression drugs and received a course of Intravenous Immunoglobulins (IVIG) on two separate occasions spanning two months, the anemia relapsed. Subsequently, after an additional dose of IVIG with further modification and reduction of the immunosuppressant regimen, including stopping the mycophenolate and switching tacrolimus with cyclosporine, the patient ultimately achieved successful resolution of his symptoms and a significant decrease in viral load. Our case highlights the significance of unconventional etiologies when confronted with anemia in the setting of kidney transplantation. Furthermore, it also provides further insights into therapeutic avenues for addressing PRCA in kidney transplant recipients.

A systematic review of literature on Insulin-like growth factor-2-mediated hypoglycaemia in non-islet cell tumours.

INTRODUCTION: Insulin-like growth factor-2 (IGF-2)-mediated hypoglycemia is a rare yet clinically significant entity with considerable morbidity and mortality. Existing literature is limited and fails to offer a comprehensive understanding of its clinical trajectory, management and prognostication. METHODS: Systematic review of English-language articles reporting primary patient data on IMH was searched using electronic databases (PubMed, Scopus and Embase) from any date up to 21 December 2022. Data were analysed in STATA-16. RESULTS: The systematic review contains 172 studies, including 1 Randomised controlled trial, 1 prospective observational study, 5 retrospective observational studies, 150 case reports, 11 case series and 4 conference abstracts. A total of 233 patients were analysed, averaging 60.6 ± 17.1 years in age, with comparable proportions of males and females. The commonest tumours associated with Insulin-like Growth Factor-2-mediated hypoglycaemia were fibrous tumours (N = 124, 53.2%), followed by non-fibrous tumours originating from the liver (N = 21, 9%), hemangiopericytomas (N = 20, 8.5%) and mesotheliomas (N = 11, 4.7%). Hypoglycaemia was the presenting feature of NICT in 42% of cases. Predominant clinical features included loss of consciousness (26.7%) and confusion (21%). The mean IGF-2 and IGF-1 levels were 882.3 ± 630.6 ng/dL and 41.8 ± 47.8, respectively, with no significant correlation between these levels and patient outcomes. Surgical removal was the most employed treatment modality (47.2%), followed by medication therapy. The recovery rate was 77%, with chronic liver disease (CLD) significantly associated with a poor outcome (OR: 7.23, P: 0.03). Tumours originating from fibrous tissues were significantly associated with recovery (p < .001). In the logistic regression model, CLD remained a significant predictor of poor outcomes. CONCLUSION: This systematic review highlights that most non-islet-cell tumour-hypoglycaemia (NICTH) is due to fibrous tumours. NICTs demonstrate a variable prognosis, which is fair if originating from fibrous tissue. Management such as octreotide, corticosteroids, diazoxide, embolization, radiotherapy and surgical resection have disparate success rates.

Migraine in physicians and final year medical students: A cross-sectional insight into prevalence, self-awareness, and knowledge from Pakistan.

BACKGROUND: Despite its high prevalence, migraine remains underdiagnosed worldwide. A significant reason is the knowledge gap in physicians regarding diagnostic criteria, clinical features, and other clinical aspects of migraine. AIM: To measure the knowledge deficit in physicians and medical students and to assess the prevalence of migraine in the same population. METHODS: An online questionnaire was developed and distributed among physicians and final year medical students on duty in various medical and surgical specialties of Allied and DHQ Hospitals, Faisalabad, between October 2018 and October 2019. Inclusion criteria were public practicing physicians who experience headaches, while those who never experienced headaches were excluded. Different questions assessed respondents on their knowledge of triggers, diagnosis, management, and prophylaxis of the migraine headache. They were asked to diagnose themselves using embedded ICHD-3 diagnostic criteria for different types of migraine. Graphs, tables, and figures were made using Microsoft Office 2016 and Microsoft Visio, and data analysis was done in R Studio 1.4. RESULTS: We had 213 respondents and 175 fulfilled inclusion criteria, with 99 (52%), 58 (30%) and 12 (6.3%) belonging to specialties of medicine, surgery, and others, respectively. Both genders were symmetrically represented (88 male and 87 female). Fifty-two (24.4%) of our 213 respondents were diagnosed with migraine, with 26 (50%) being aware of it. Females had higher prevalence among study participants (n = 28, 32.2%) compared to males (n = 20, 22.7%, P = 0.19). A majority (62%) of subjects never consulted any doctor for their headache. Similarly, a majority (62%) either never heard or did not remember the diagnostic criteria of migraine. Around 38% falsely believed that having any type of aura is essential for diagnosing migraine. The consultation rate was 37% (n = 65), and migraineurs were significantly more likely to have consulted a doctor, and a neurologist in particular (P < 0.001). Consulters and migraineurs fared better in the knowledge of diagnostic aspects of the disease than their counterparts. There was no significant difference in other knowledge aspects between consulters versus non-consulters and migraineurs versus non-migraineurs. CONCLUSION: Critical knowledge gaps exist between physicians and medical students, potentially contributing to misdiagnosis and mismanagement of migraine.

The clinical characteristics and outcomes of patients with pulmonary hypertension in association with hyperthyroid state: A systematic review.

BACKGROUND: Pulmonary hypertension (PHTN) may occur in thyroid disorders, especially in hypothyroidism. However, there is increasing evidence of PHTN in hyperthyroidism (HTH). The etiology, clinical course, management, and factors associated with outcomes of PHTN in the setting of HTH are unascertained. This systematic review consolidates available evidence on patients with HTH who developed PHTN. METHODS: We conducted a systematic review on English articles from PubMed, Scopus, and Google Scholar reporting PHTN in patients with hyperthyroidism. Data were analyzed and reported in Microsoft Excel 2020, SPSS version 26, and Jamovi version 1.2. RESULTS: We identified 589 patients with PHTN in the setting of HTH. Etiologies included Grave disease 66.7%), toxic multinodular goiter (TMNG) (16.8%), drug-induced HTH (0.3%), thyroiditis(0.8%), and toxic adenoma(0.1%). Most patients did not receive any specific management for PHTN and were managed by antithyroid treatment (97.4%). Outcomes of PHTN were reported in 181 patients, with a 94% recovery rate. Pulmonary artery pressures (PAP) before and after HTH management ranged from 22.5 to 75 mm Hg and from 24 to 50 mm Hg, respectively. Outcome analysis performed on data from case reports and series with individually identifiable data revealed a 67.6% female preponderance. An estimated 73.5% of the patients had PHTN at the initial presentation of HTH, which was associated with a better resolution rate of PHTN(OR: 12, P-value: 0.048). TRAB was positive in 47% patients with no clinical difference in outcomes. antiTG AB was reported positive in 29.4%, all of whom had an improvement, compared to an 83.3% improvement rate in those with negative antiTG AB. Various etiologies and treatments did not have any significant differences in the outcome of PHTN. CONCLUSIONS: PHTN can be present at the initial diagnosis of HTH, which is associated with better outcomes of PHTN. There is a clear female preponderance in the development of PHTN. However, resolution rates seem to be better in males. Although TRAB is associated with the development of PHTN, it does not seem to affect the outcomes. PHTN in patients with HTH does not need any specific management, with >90% resolution with antithyroid therapy. Whether any specific antithyroid therapy has a better outcome in PHTN needs to be explored prospectively.

Extramedullary haematopoiesis in patients with transfusion dependent ß-thalassaemia (TDT): a systematic review.

INTRODUCTION: Around 5% of the world's population is expected to have some degree and type of thalassaemia. Beta thalassaemia (BT) occurs due to a deficient production of the beta-globin chain of haemoglobin. Extramedullary haematopoiesis (EMH) is one of the complications of BT, mainly observed in minor/intermedia subtypes. EMH is the production of blood cells outside the marrow as a compensatory response to longstanding hypoxia. Due to chronic transfusions, it is not expected in patients with beta-thalassaemia major (BTM). However, there are increasingly reported cases of EMH in BTM. The incidence of EMH in BTM is thought to be <1%. We aim to pool the available data and provide cumulative evidence on the occurrence of EMH in BTM patients. METHODS: This is a systematic review of case reports, series, and retrospective studies that presented data on the occurrence of EMH in BTM patients. Data were recorded and analyzed in Microsoft Excel 2016 and SPSS 26. The protocol has been registered in PROSPERO: CRD42021242943. RESULTS: Data from 253 cases of EMH in BTM patients were extracted with a mean age of 35.3 years. Mean haemoglobin at presentation with EMH was 8.2 mg/dL. Lower limb weakness was the most common presenting feature (N = 23) (paraspinal EMH). Magnetic resonance imaging (MRI) was the most widely used diagnostic modality (226). Overall, blood transfusion was the commonest reported treatment (30), followed by radiotherapy (20), surgery (15), hydroxyurea (12), steroids (6), and exchange transfusion (2). An outcome was reported in 20% of patients, all recovered, except one who died as a result of nosocomial infection. CONCLUSION: EMH is rare in BTM and can occur in any organ system with varied clinical features. MRI can effectively diagnose EMH, and conservative management has similar results compared to invasive treatments. Larger studies, focussing on outcomes may enhance guidelines on preventive and therapeutic strategies for managing EMH in BTM.KEY MESSAGESExtramedullary haematopoiesis is a rare complication in beta thalassaemia. Although it is more common in non-transfusion dependent thalassaemia, increasingly reported cases suggest a higher prevalence of EMH in TDT than what is known before.There are no clear guidelines on the management of EMH in TDT, with reported patients showing similar outcomes with conservative invasive treatment modalities.More extensive and preferably prospectively designed studies are required focussing on the management of EMH and its outcomes in patients with TDT to formulate evidence-based guidelines.

Optic neuropathy as a presenting feature of vitamin B-12 deficiency: A systematic review of literature and a case report.

INTRODUCTION: Vitamin B12 (VitB12) deficiency rarely manifests with visual symptoms. Optic nerve damage in VitB12 deficiency is thought to be via degeneration. However, optic neuritis, though infrequent, has been reported secondary to VitB12 deficiency. MATERIAL AND METHODS: We conducted a systematic review of all the reported cases of VitB12 deficiency with optic nerve involvement in Pubmed, Cochrane, and Google Scholar any date up to September 6, 2020. We have discussed the findings and compiled the available information on ophthalmological manifestations of VitB12 deficiency. We aim to provide a unified knowledge about the evidence related to types of optic neuropathies reported to date secondary to VitB12 deficiency. We also present a case of bilateral optic neuritis secondary to VitB12 deficiency. PRESENTATION OF CASE: We present a 29-year-old previously healthy male with progressive, painful, bilateral, but asymmetric visual deterioration for forty-five days. A detailed history, examination, and laboratory workup were carried out. He was diagnosed as having optic neuritis secondary to VitB12 deficiency. He showed partial improvement with the replacement of VitB12. CONCLUSION: We suggest promptly identifying and replacing VitB12 in patients with optic neuritis with proven VitB12 deficiency to prevent permanent damage to the optic nerve. Patients with VitB12 deficiency should have a baseline fundoscopic exam to rule out subclinical optic nerve damage. Moreover, patients who present with visual disturbances should be screened for VitB12 deficiency, especially the vegan population.

A retrospective study on the association of gastrointestinal symptoms in children with low lactase activity and low activity of other disaccharidases.

BACKGROUND: Disaccharides such as lactose and sucrose are sugars commonly found in human diet. They are broken down by mucosal disaccharidases in the duodenum. Previous small studies found no associations between gastrointestinal (GI) symptoms and combined low disaccharidase activity. We aim to explore the associations of low activity of disaccharidase and combinations of low activity of different disaccharidases with general GI symptom presentations in a large cohort of pediatric patients. METHODS: We examined a cohort (0-21 yrs.) who have undergone esophagogastroduodenoscopy and received disaccharidase activity assay from duodenal biopsy in the time period 2010 to 2012. Disaccharidase assays tested for activity of lactase, sucrase, maltase, and palatinase. GI symptoms were grouped into four categories, abdominal pain, diarrhea, weight loss, and gastroesophageal reflux. RESULTS: Of the 347 subjects, we found an association between low lactase activity and abdominal pain (OR = 1.78; 95% CI = 1.07-2.97; p < 0.05). Subjects with a lactase/sucrase ratio < 0.2 were found to be associated with abdominal pain (OR = 2.25; 95% CI = 1.25-4.04; p < 0.05), Subjects with low pandisaccharidase may be correlated with abdominal pain and have a unique frequency of GI symptoms due to low frequency of diarrhea and weight loss, but they were not statistically significant. CONCLUSIONS: Low activities of certain disaccharidase combinations may be associated with GI symptoms in subjects; a prospective study may be needed to investigate further.

A challenge for diagnosing acute liver injury with concomitant/sequential exposure to multiple drugs: can causality assessment scales be utilized to identify the offending drug?

Drug-induced hepatotoxicity most commonly manifests as an acute hepatitis syndrome and remains the leading cause of drug-induced death/mortality and the primary reason for withdrawal of drugs from the pharmaceutical market. We report a case of acute liver injury in a 12-year-old Hispanic boy, who received a series of five antibiotics (amoxicillin, ceftriaxone, vancomycin, ampicillin/sulbactam, and clindamycin) for cervical lymphadenitis/retropharyngeal cellulitis. Histopathology of the liver biopsy specimen revealed acute cholestatic hepatitis. All known causes of acute liver injury were appropriately excluded and (only) drug-induced liver injury was left as a cause of his cholestasis. Liver-specific causality assessment scales such as Council for the International Organization of Medical Sciences/Roussel Uclaf Causality Assessment Method scoring system (CIOMS/RUCAM), Maria and Victorino scale, and Digestive Disease Week-Japan were applied to seek the most likely offending drug. Although clindamycin is the most likely cause by clinical diagnosis, none of causality assessment scales aid in the diagnosis.