bluuebunny/crossref_metadata_2025_split huggingface dataset:
P-14 A case of Paget's disease of bone presenting at early stages of life
Authors: Öner Hatice , Yavuz Dilek Gogas 
Published: January 2024

Abstract
Paget's disease of bone is the metabolic bone disease that affects 2-4% of individuals over age 55 in European nations. The disease may be asymptomatic and can be detected incidentally through x-rays or biochemical tests conducted for another condition. It can also manifest as bone pain, deformity, fracture, Here, we present a case of Paget's bone disease that manifested at a young age.
A 47-year-old female was referred to an endocrinology outpatient clinic due to a nonfunctional pituitary gland microadenoma. She has had constant pain in the right thigh region for the past two years, and no fractures have ever been reported. During the physical examination, the patient's right femur was sensitive to palpation. The results of the standard laboratory test were as follows: Creatinine: 0.61 mg/dl (0-1.2 mg/dl), Albumin: 50 g/L (35-52 g/L), Calcium: 9.8 mg/dl (8.8-10.6 mg/dl), Phosphorus: 4.2 mg/dl (2.6-4.5 mg/dl), PTH (Parathyroid Hormone): 33 ng/L (15-65 ng/L), Alkaline Phosphatase: 524 U/L (33-98 U/L) and 25-OH vitamin D is 43.7 mcg/L.
A bone scintigraphy was performed due to the patient's elevated alkaline phosphatase levels and bone pain. The scintigraphy revealed elevated osteoblastic activity in a sclerotic lesion expanding the right femoral cortex, and the results were reported in accordance with Paget's disease. A biopsy of the right femur lesion was performed due to the patient's young age and to rule out other diagnosis such as osteosarcoma or bone metastases. The pathology results revealed thickened and distinct cement lines along with calcified bone fragments, consistent with Paget's disease. The patient was administered 5 mg of IV zoledronic acid, and the alkaline phosphatase level was determined to be 123 U/L (Reference Range: 33-98) at the one-month control following treatment. Paget's disease can be diagnosed in young people. Therefore, it is important to take into account even a slight increase in ALP levels that is linked to early radiological abnormalities.
P-18 Dent's Disease as a rare cause of rickets
Authors: Akçay Seçkin , Kapan Ece , Yavuz Dilek 
Published: January 2024

Abstract

Introduction
Dent's disease is a rare recessive X-linked disorder characterized by hypophosphatemia, hematuria, proteinuria, hypercalciuria, nephrocalcinosis, and progressive renal failure. The majority of cases are caused by mutations in the CLCN5 or OCRL genes. The primary mechanisms of rickets in Dent's disease are a deficiency in phosphate absorption in the proximal tubules of the kidney and an increase in urinary calcium excretion. In order to draw attention to this rare disease, we present a patient diagnosed with Dent's disease while being evaluated for hypophosphatemic rickets.

Clinical Case
A 23-year-old male patient with Dent's disease was referred to an adult endocrinology clinic for follow-up after a CLCN5 mutation was identified during an examination for hypophosphatemic rickets and proteinuria at the age of three. Since the age of 18, he has been diagnosed with chronic kidney disease. At the age of 19, he has a history of hospitalization for osteomyelitis. No history of fracture. In his family history, both of his parents were cousins, that have short stature and Dent's disease mutation was found to be heterozygous. Both cousins had hypophosphatemic rickets diagnosis. On physical examination, he was mobile, 155 cm tall, weighed 55 kilograms, had bilateral genu valgum, and other system examinations revealed no pathology. The patient was taking 500 mg of phosphate three times per day in addition to 0.5 microgram of calcitriol. In laboratory evaluation, creatinine was 3.11 mg/ dL, GFR was 26 ml/min, PTH was 118 pg/mL, ALP was 82 IU/ml, phosphorus was 3.44 mg/ dL, and calcium was 10 mg/dL. 24-hour urine calcium: 195 mg/day; osteocalcin: 188 ng/mL; beta-crosslaps: 2 ng/mL. It was determined to be high-turnover chronic kidney disease-bone disorder. Urinary ultrasonography showed bilateral kidney diameters decreased and parenchymal thickness thinned, bilateral kidney echoes increased, and bilateral medullary nephrocalcinosis with high echogenicity and calcifications. Cataract and basal ganglia calcification were not detected.
Dual Energy X-Ray Absorbsiometry evaluation revealed that the L1-L4 BMD: 0.915 gr/cm2 Z score: -1.1 and the femoral neck BMD: 0.614 gr/cm2 Z score: -3.1 were lower than anticipated for age.
The patient's calcitriol dosage was reduced to three times per week after PTH suppression. Since the serum ALP concentration was normal, the oral phosphorus dose was not altered. A limited sodium diet was suggested for the patient. The patient with bilateral nephrocalcinosis was referred to the nephrology clinic for further evaluation.

Conclusion
Patients diagnosed with rickets should also be mindful of Dent's disease, which must be closely monitored alongside nephrology.Figure 1.Bilateral knee radiograph of the patient
P-25 Unusual cause for spontaneous hypoglycemia: Hirata's Disease in Iraqi patient
Authors: Khudhair Mahmood Shakir , Altaee Gelal Abidali , Sabri Khalid Abdulhussain 
Published: January 2024

Abstract

Introduction
Hirata's Disease, is a rare condition characterized by episodes of spontaneous hypoglycemia associated with hyperinsulinemia with positive insulin autoantibodies. Hypoglycemia is common in patients with diabetes mellitus especially those who are taking insulin therapy or insulin secretogogues oral medications but spontaneous hypoglycemia is rare and there is no clear figures about those patients in Iraq.
We receive a lot of persons claim to have hypoglycemia but most of them fail to fulfill the Whipple triad.
Although endogenous hyperinsulinemic hypoglycemia especially insulinoma is very rare condition, it is still concern for endocrinologist in patients presented with presumed hypoglycemia.

Clinical Case
A 43 year old female presented with frequent fainting attacks over several weeks. She has no past medical history a part of localized areas of vertigo for which she received no systematic treatment. Initial medical evaluation was unremarkable but with documented hypoglycemia below 40 mg/dl. She has normal heart, brain, kidney, liver, thyroid and adrenal function. She has multiple emergency admissions treatment with glucose infusion. She gains more weight as she eats more to avoid hypoglycemia.
Her specific lab during the attack shows high serum insulin level (141.10 mIU/ml) with high C-peptide 18.55 ng/ml
Work up for insulinoma shows normal MRI abdomen, normal endoscopic ultrasound and negative PET Gallium 68 scan.
There are no previous reports of Insulin autoimmune syndrome in Iraq. The insulin autoantibodies in this patient were very high (more than 300.00 U/ml) which highly suggestive of this syndrome after excluding other factors that may cause elevation of these autoantibodies.
Patient condition persist for about eight months and was advised for small multiple meals and diazoxide was not used much because it is not available in Iraq widely. Her condition resolved spontaneously after one year with dietary modifications.

Conclusion
Hirata syndrome is very rare but important cause of spontaneous hypoglycemia that should not be ignored. Up to our knowledge this is the first documented case in Iraq.
P-29 Metformin poisoning without lactic acidosis: Case report
Authors: Bivol Elena , Dumitraș Tatiana , Sîrbu Felicia , Gușanu Olesea , Vieru Daniela 
Published: January 2024

Abstract

Introduction
Metformin is the most widely used oral antidiabetic, which belongs to the biguanide pharmacological group. Metformin-associated lactic acidosis (MALA) is a rare event with an incidence of approximately 19 cases per 100,000 patient-years. The risk of developing lactic acidosis, when taking metformin is enhanced by several factors such as: age &gt; 65 years; concomitant diseases that have the ability to induce hypoxemia (chronic renal failure, congestive heart failure, cardiogenic shock, severe lung disease, sepsis, advanced liver disease, history of lactic acidosis); an excessive alcohol consumption; an administration of iodinated contrast agents.

Clinical Case
A 46-year-old man was urgently admitted with the following symptoms: drowsiness, repeated vomiting, lethargy, dizziness. The symptoms started after he administered outpatient and at own initiative 50 tablets of metformin, each tablet contains a dose of 500 mg (total dose 25g). Concomitant pathologies: diagnosed with Type 2 Diabetes for 8 years; Toxic liver cirrhosis, compensated Child-Pugh A (recently diagnosed); hypertension; CKD G3aA2 KDIGO. The patient's condition at admission was assessed as extremely serious, Glasgow scale - 11 points, mild hypertension, tachycardia, tachypnea. The paraclinical investigations revealed the presence of metabolic acidosis, but with lactate &lt;5 mmol/l; the progressive renal dysfunction with creatinine values in the first 24 hours up to 1184 µmol/l, urea – 24.5 mmol/l; a severe hypoglycemia - 1.68 mmol/l; a moderate cytolytic syndrome; a cholestatic syndrome, an inflammatory and hepatoprive syndrome; coagulation disorders; a pancreatic dysfunction. In the context of the evident increase of urea and creatinine, the decision was made to initiate the intermittent hemodialysis, a total of 14 sessions were required during hospitalization.
The particularity of this case is represented by the absence of lactic acidosis, despite metformin intoxication with altered neurological status, acute renal failure, developed due to chronic kidney disease, liver cirrhosis of toxic etiology and severe hypoglycemia.

Conclusion
Metformin should be administered with caution in patients with multiple comorbidities. Metformin intoxication can induce lactic acidosis with an increased risk of serious, potentially fatal adverse events. It is essential to identify metformin intoxication as early as possible, with prompt initiation of renal replacement measures and dynamic monitoring of biochemical parameters.
O-17 A case of severe and recurrent hypoglycaemia in a healthy patient
Authors: Samperi Irene , Bima Chiara , Radaelli Piero , Steffanini Mara , Persichetti Agnese , Cozzi Renato , Attanasio Roberto , Ponziani Maria Chantal 
Published: January 2024

Abstract

Introduction
Hypoglycaemia is a challenging condition for the endocrinologist.

Clinical Case
A 75 y/o man was admitted to ER due to severe symptoms of hypoglycaemia (weakness, blurring and sweating). His symptoms improved after eating some food and blood glucose was 50 mg/dL. He was treated with oral glucose and referred to our endocrinology clinic. His personal and family history was uneventful (no endocrine, autoimmune or neoplastic disease, no drug) and he did not complain any other symptoms besides hypoglycaemic ones. When going deeply into his medical history, he reported that back pain had occurred 4 weeks before ER access. His GP had thus prescribed supplements containing alpha lipoic acid (ALA) that he had assumed for 3 weeks and then discontinued for the lack of any benefit. He regarded this information as useless as ALA was “just a supplement”. Furthermore, he reported symptoms as shaking, anxiety, dizziness, sweating, and hunger that occurred 5-7 times per day far from meals and faded after eating.
A diet was prescribed with frequent eatings, high content of fibers and whole foods. He started flash glucose monitoring (FGM) with the alarm for hypoglycaemia set at 70 mg/dL and was trained for the management of hypoglycaemia. The laboratory alerted us due to the results of oral glucose tolerance test (Table 1). Hypoglycaemic episodes after the test were neither reported nor registered by FGM.
The differential diagnosis was among insulinoma, insulin resistance or insulin autoimmune syndrome (Hirata disease). The calculated molar ratio of insulin to C-peptide was 2.68, thus pointing to Hirata disease (to be suspected when the ratio is &gt;1). We opted for watchful waiting while continuing the monitoring.
Two weeks later the pending results became available: TSH 1.69 mU/L, HbA1c 5.2%, serum cortisol 14.8 μg/dL, ACTH 18.2 pg/mL, anti-endogenous insulin antibodies 5.8 U/ml (nv &lt;2.4), normal values of transaminases, lipase, and amylase.
The patient reported adherence to the diet, two symptomatic hypoglycaemias (in the afternoon, when he couldn't have the snack) as well as asymptomatic FGM alarms once daily, usually early in the morning.
We advised to add a snack before bedtime to shorten the fasting period. At the 3-month follow-up he had had no further hypoglycaemias, blood glucose was normal and anti-endogenous insulin antibodies were negative.

Conclusion
Hypoglycaemia can be a life-threatening condition and differential diagnosis is crucial. It is important to consider even rare conditions, such as Hirata syndrome that can be triggered by several factors and medications, the most common being ALA. Complete medical history is mandatory and tight follow-up essential. Most cases, as the one reported, resolve with a correct diet after the withdrawal of supplements.Table 1.Oral Glucose Tolerance Test
P-46 Panhypopituitarism in a patient with Acute Myeloid Leukemia
Authors: Cakir Ilkay , Evren Bahri , Arslan Oyku 
Published: January 2024

Abstract

Introduction
Panhypopituitarism with acute myeloid leukemia (AML) is a very rare condition. Few cases reported in the literature have been generally associated with poor prognosis.

Clinical Case
Here we present A 57-year-old female patient with AML-M2. In the first year of her follow-up, she was diagnosed with central diabetes insipidus (DI)- Arginine vasopressin deficiency, when she was referred to the endocrinology clinic with complaints of fatigue, polydipsia and polyuria. Hypopituitarism was found following further investigations including pituitary hormones (Table 1). Magnetic resonance imaging (MRI) of the pituitary gland revealed a a heterogeneous contrasting lesion of approximately 15×11×21 mm, which is isointense with gray matter. Pituitary stalk was observed thickened. Differentiation between lymphocytic hypophysitis and macroadenoma could not be made (Figure 1). Visual field examination was normal. When clinical, laboratory and radiological findings were evaluated together, panhypopituitarism was considered and empirical treatment was started including prednisolone 5 mg daily, levothyroxine 75 mcg daily, and Desmopressin acetate hydrate (DDAVP) 0.600 twice daily without performing a fluid restriction test.
The patient had relocated because of social reasons. She applied to another endocrinology out-patient clinic for follow-up controls and reported that she first reduced the desmopressin to half dose and then stopped because she felt amelioration of symptomes. Her urine output during this admission amounted to 2 liters/day without receiving desmopressin. Laboratory workup also supported recovery of DI - Arginine vasopressin deficiency (Table 1). Since basal cortisol values were found to be within normal limits, steroid was also reduced and discontinued, but the need of replacement for hypothyroidism continued. In the control pituitary MRI, the changes of pituitary gland and stalk observed in the prior imaging was seen disappeared (Figure 1).
Although the patient did not receive any specific treatment for hypophysitis other than AML treatment, clinical and radiological improvement was observed. She is still doing well on levothyroxine treatment. Cytarabine (ara-c) and idarubicin, hematopoietic stem cell transplantation and venetoclax treatments were applied for AML, respectively and the patient was considered as remission. Resolution of hypophysitis may be associated with remission of AML.Figure 1.Pituitary MRI at diagnosis and follow-up Table 1.Laboratory findings of the patient at baseline and follow-up
P-53 Resistant macroprolactinoma
Authors: Berkovskaya Marina , Grigoriev Andrey 
Published: January 2024

Abstract

Introduction
Dopamine agonists (DAs) are considered to be the mainstay treatment for prolactinomas. Primary DA-resistance has been reported in approximately 20–30% of the patients on bromocriptine, and in around 10% of those on cabergoline. The DA-resistance is usually defined as a failure to achieve normal prolactin levels on maximally tolerated doses of DAs for at least 3-6 months and failure to achieve 30-50% reduction in tumor size in case of macroprolactinoma.
Most of resistant prolactinomas have shown to be large and/or invasive tumors. We present a clinical case of microprolactinoma, resistant to cabergoline.

Clinical Case
A 21-year old woman reported of a 2-year history of amenorrhea. Primary examination revealed hyperprolactinemia up to 361 ng/ml (bioactive prolactin 256 ng/ml). MR imaging with contrast enhancement showed the ovoid microadenoma in the right half of the adenohypophysis up to 10 mm without invasion to cavernous sinus (Figure 1). Hormone tests excluded the overproduction of ACTH, GH and TSH by the tumor (Table 1), and revealed no signs of hypopituitarism. The patient was prescribed cabergoline (0.5 mg per week), which was followed by the initial decrease of prolactin level to 116 ng/ml. However, subsequent increase of cabergoline dose up to 4,5 mg per week did not lead to further prolactin decrease and normoprolactinemia and there was no resumption of menstrual function. On the follow-up MR images (6 months after the first one) no dynamics was revealed: the tumor was up to 10 mm in size without invasive spreading to cavernous sinus or chiasm.
Taking into account the resistance to cabergoline, young age and pregnancy planning, the patient underwent transnasal debulking of the tumor. A week after the operation, normalization of prolactin level was noted, and there were no any signs of hypopituitarism. Within a month after operation, the patient reported of resumption of regular menstrual cycle. r4 months later the woman got pregnant. The pregnancy proceeded without complications and ended in timely delivery.Figure 1.MR image of pituitary microadenoma (contrast enhanced) Table 1.Primary hormone examination
P-80 A rare case of papillary thyroid carcinoma within a mature cystic ovarian teratoma
Authors: Tekin Selin , Ünlütürk Uğur 
Published: January 2024

Abstract

Introduction
Mature teratoma is the most common ovarian germ cell neoplasm. Teratomas can contain various tissue groups, such as skin, hair, and thyroid tissue. Teratomas in which more than 50% of the tumor tissue is composed of thyroid tissue are defined as Struma Ovarii. Malignant transformation of Struma Ovarii has been reported at a rate of 0.5-5%, most commonly presenting as thyroid papillary and follicular carcinomas. Metastasis is observed in 5% of malignant cases.

Clinical Case
A 45-year-old female with a history of Hashimoto's thyroiditis presented in July 2022 with complaints of abdominal pain. Abdominal contrast-enhanced tomography revealed a hypo dense lesion 5 cm in diameter the right adnexal region, and in the magnetic resonance images 5 cm mass was observed in the right adnexal area. Serum Ca-125 level was 212 U/mL (0-35) at admission. Due to high suspicion of malignancy ovarian cystectomy has been performed. Pathology of specimen revealed a 0.5 cm thyroid papillary carcinoma with follicular variant in infiltrative features but no lymphovascular invasion. Subsequently, the patient underwent a total abdominal hysterectomy and bilateral salpingo-oophorectomy due to the unverifiable risk of malignancy. Pathological examination showed no malignancy. The patient presented to our clinic in June 2023, neck ultrasound revealed a 13×12 mm heterogeneous hypoechoic thyroid nodule with thick and thin septations and occasional internal vascularity in the left thyroid lobe. Fine-needle aspiration biopsy of the thyroid was performed, and cytology results were reported as benign. The serum thyroglobulin level was found to be 83.7 ng/mL. In terms of the treatment plan, BRAF and K-RAS gene analysis was planned based on the pathology preparations. The patient will be evaluated for total thyroidectomy/radioactive iodine ablation/no treatment based on the results. Follow-up is ongoing, and test results are awaited.

Conclusion
Malignant transformation of mature teratoma is rare and thyroid papillary carcinoma being the most frequently seen malignancy. There is no consensus on treatment and follow-up, but it is recommended to classify patients based on their risks and consider options such as total thyroidectomy/radioactive iodine ablation/no treatment for follow-up. Literature suggests the need for guidelines and treatment algorithms for the management of malignant struma ovarii. Some authors recommend whole-body iodine scanning in determining risk and follow-up, asserting that iodine-based treatment and follow-up methods would be meaningless without thyroidectomy. There is also an approach advocating for only salpingo-oophorectomy in low-risk patients and subsequent follow-up. Thus, a consensus on guidelines and treatment algorithms for malignant struma ovarii is needed.
Benchmarks in Liver Resection for Intrahepatic Cholangiocarcinoma
Authors: Alaimo Laura , Endo Yutaka , Catalano Giovanni , Ruzzenente Andrea , Aldrighetti Luca , Weiss Matthew , Bauer Todd W. , Alexandrescu Sorin , Poultsides George A. , Maithel Shishir K. , Marques Hugo P. , Martel Guillaume , Pulitano Carlo , Shen Feng , Cauchy François , Koerkamp Bas Groot , Endo Itaru , Kitago Minoru , Pawlik Timothy M. 
Published: January 2024

Abstract
Introduction
Benchmarking in surgery has been proposed as a means to compare results across institutions to establish best practices. We sought to define benchmark values for hepatectomy for intrahepatic cholangiocarcinoma (ICC) across an international population.

Methods
Patients who underwent liver resection for ICC between 1990 and 2020 were identified from an international database, including 14 Eastern and Western institutions. Patients operated on at high-volume centers who had no preoperative jaundice, ASA class <3, body mass index <35 km/m2, without need for bile duct or vascular resection were chosen as the benchmark group.

Results
Among 1193 patients who underwent curative-intent hepatectomy for ICC, 600 (50.3%) were included in the benchmark group. Among benchmark patients, median age was 58.0 years (interquartile range [IQR] 49.0–67.0), only 28 (4.7%) patients received neoadjuvant therapy, and most patients had a minor resection (n = 499, 83.2%). Benchmark values included ≥3 lymph nodes retrieved when lymphadenectomy was performed, blood loss ≤600 mL, perioperative blood transfusion rate ≤42.9%, and operative time ≤339 min. The postoperative benchmark values included TOO achievement ≥59.3%, positive resection margin ≤27.5%, 30-day readmission ≤3.6%, Clavien-Dindo III or more complications ≤14.3%, and 90-day mortality ≤4.8%, as well as hospital stay ≤14 days.

Conclusions
Benchmark cutoffs targeting short-term perioperative outcomes can help to facilitate comparisons across hospitals performing liver resection for ICC, assess inter-institutional variation, and identify the highest-performing centers to improve surgical and oncologic outcomes.
ADHD in adults in a nutshell
Authors: Rzeska Ewa , Jurasz Karolina , Podgórska Dominika , Sanecki Miłosz , Tomczyk Karolina , Chojnacka Natalia , Klarycki Jakub , Cymer Radosław 
Published: January 2024

ADHD (attention-deficit hyperactivity disorder), primarily only considered as a childhood condition, remains an underdiagnosed condition that also affects adult patients. High comorbidity and similarities to different psychiatric disorders impedes the diagnosis. Despite this, it is important for medical practitioners to detect ADHD symptoms and implement proper treatment to enhance patients’ life quality. Treatment should be multimodal and involve both behavioral therapy and pharmacotherapy.