scholarly journals Dysgerminoma with Estrogen-Producing Functioning Stroma Presenting Precocious Puberty

2021 ◽  
Vol 2021 ◽  
pp. 1-4
Author(s):  
Shunsuke Nagase ◽  
Kanako Ogura ◽  
Karin Ashizawa ◽  
Nana Nakazawa-Tanaka ◽  
Masahiko Urao ◽  
...  
Keyword(s):  
Detection Limit ◽  
Precocious Puberty ◽  
Tanner Stage ◽  
Breast Development ◽  
Normal Range ◽  
Spindle Cells ◽  
First Case ◽  
The Right ◽  
Estrogen Biosynthesis ◽  
Sex Cord Stromal Tumors

Dysgerminoma is a malignant ovarian germ cell tumor, and unlike sex-cord stromal tumors, endocrine manifestation is considered rare. Here, we report the first case of dysgerminoma presenting precocious puberty. The patient is a 7-year-old girl who presented with a breast development in Tanner stage 3. Serum estradiol (E2) was markedly elevated while luteinizing hormone (LH) and follicle-stimulating hormone (FSH) were suppressed below the detection limit. Microscopically, the right ovarian mass displayed nests of large polygonal cells and fibrous septa which were focally concentrated by theca-like plump spindle cells. Immunohistochemistry revealed that the spindle cells expressed various steroidogenic enzymes involved in estrogen biosynthesis including P450 aromatase. The tumor was diagnosed with pure dysgerminoma with estrogen-producing functioning stroma. After the operation, serum E2 declined below the detection limit; LH and FSH returned within the normal range. This case demonstrates that even a conventional dysgerminoma can present endocrine manifestation through functioning stroma.

scholarly journals The association of primary hyperparathyroidism and primary ovarian failure: a de novo t(X; 2) (q22p13) reciprocal translocation

2008 ◽  
Vol 158 (2) ◽  
pp. 261-263 ◽  
Author(s):  
Gul Bano ◽  
Sahar Mansour ◽  
Stephen Nussey
Keyword(s):  
Primary Hyperparathyroidism ◽  
Serum Calcium ◽  
Reciprocal Translocation ◽  
Tanner Stage ◽  
Ovarian Failure ◽  
Breast Development ◽  
Chromosome 2 ◽  
Primary Amenorrhoea ◽  
First Case ◽  
A Genome

Case: A 40-year-old female presented with primary amenorrhoea at 17 years of age. She was tall at 98th centile for height with eunuchoidal body habitus. Her breast development was Tanner stage 3, pubic and axillary hair Tanner stage 4 with normal external genitalia. Her bone age was 13.4 years at a chronological age of 17.8 years. Gonadotrophins were elevated indicating primary ovarian failure. A diagnostic laparotomy revealed hypoplastic, infantile uterus with bilateral streak gonads. Chromosomal analysis showed a balanced reciprocal translocation 46X, t(X; 2) (q22 p13). She became pregnant by in vitro fertilization with egg donation at the age of 36 years. At 13 weeks of gestation, she presented with intractable vomiting. She had raised corrected serum calcium and parathyroid hormone concentrations consistent with the diagnosis of primary hyperparathyroidism (PHPT). She underwent parathyroidectomy at 24 weeks of gestation with removal of a large left inferior parathyroid adenoma which normalized her serum calcium. Multipoint linkage from a genome-wide screen has identified a region of suggestive linkage on chromosome 2p13.3–14 in some cases of familial isolated hyperparathyroidism (FIHP). Conclusion: To our knowledge, this is the first case of primary amenorrhoea due to reciprocal translocation involving chromosome 2 and the X chromosome associated with PHPT. PHPT in this case is most likely to be as a result of chromosome 2 involvement where a locus for FIHP has been identified. Identification of the gene involved on chromosome 2p13.3–14 will be of considerable interest.

scholarly journals Central Precocious Puberty in a Three-Year-Old Girl

2021 ◽  
Vol 27 (3) ◽  
pp. 341
Author(s):  
Suryani Jamal ◽  
Liong Boy Kurniawan ◽  
Suci Aprianti ◽  
Ratna Dewi Artati ◽  
Ruland DN Pakasi ◽  
...  
Keyword(s):  
Physical Examination ◽  
Precocious Puberty ◽  
Tanner Stage ◽  
Central Precocious Puberty ◽  
Bone Age ◽  
Breast Development ◽  
Hypothalamic Hamartoma ◽  
Secondary Sexual Characteristics ◽  
Pelvic Ultrasonography ◽  
Sexual Characteristics

Precocious puberty is defined as the onset of secondary sexual characteristics before 8 years of age in girls and 9 years in boys. Central Precocious Puberty (CPP) is caused by early activation of the hypothalamic-pituitary-gonadal axis. Laboratory test of LH, FSH, and Estradiol is recommended for monitoring suppressive effects from GnRHa therapy in the early three months and every six months. This study aimed to report a case of CPP in a 3-year and 3-month-old girl. A 3-year and 3-month-old girl went to the hospital with vaginal bleeding (menstruation), breast development, and pubic and axilla hair for 7-month-old. Physical examination found moderately ill with obesity, body weight 20 kg, height 98 cm. Tanner stage was A2M3P2, café au lait was found in the left forehead with size 7x3.5 cm. In March 2015 before GnRHa therapy, LH, FSH and Estradiol level increased with levels of 4.32 mlU/mL, 6.01 mlU/mL, and 67 pg/mL, and after 3 months of the treatment was 0.87 mlU/mL, 2.51 mlU/mL and <20 pg/mL. Pelvic ultrasonography showed suggestive precocious puberty, bone age 5-year and 9-month (Greulich and Pyle), CT-Scan of the brain showed hypothalamic tumor suspected hypothalamic hamartoma. This patient was treated with a GnRHa injection every 4 weeks. Leuprorelin is a synthetic non-peptide analogue of natural GnRH. The diagnosis was based on medical history, physical examination, laboratory, and radiological findings. The prognosis of the patient was good.

scholarly journals Precocious Puberty – A Case Report

2016 ◽  
Vol 30 (2) ◽  
pp. 109-112
Author(s):  
Poly Begum ◽  
Dipti Rani Saha ◽  
Md Kamrul Hassan
Keyword(s):  
Precocious Puberty ◽  
Pubertal Development ◽  
Tanner Stage ◽  
Central Precocious Puberty ◽  
Female Child ◽  
Pubic Hair ◽  
Breast Development ◽  
Average Height ◽  
Hypothalamic Pituitary Axis ◽  
Hair Development

The parents of a 04-year-old girl bring her to a Gynaecologist because of breast development, appearance of pubic hair and periodic per vaginal bleeding. Her medical history is unremarkable. The parents are of average height, and the mother reports first menstruating when she was 11 years old. At physical examination, the girl is 100 cm tall , weighs 17 kg, and has a bodymass index of 17. Her pubertal development is classified as Tanner stage 3 breast development and Tanner stage 2 pubic hair development. She was diagnosed as a case of precocious puberity. Appearance of secondary sexual development before the age of 9 in a male child and before the age of 8 in a female child is called precocious puberty. When the cause of precocious puberty is premature activation of the hypothalamic-pituitary axis, it is called central or complete precocious puberty and she was a case of central precocious puberty. After proper consult she was treated by GnRHa suppressor of pituitary till 11 years of age.Bangladesh J Obstet Gynaecol, 2015; Vol. 30(2) : 109-112

scholarly journals A 4-Year-Old Girl with Precocious Puberty

2016 ◽  
Vol 6 (3) ◽  
pp. 161-163
Author(s):  
Poly Begum ◽  
Dipti Rani Saha ◽  
Md Kamrul Hassan
Keyword(s):  
Precocious Puberty ◽  
Vaginal Bleeding ◽  
Pubertal Development ◽  
Tanner Stage ◽  
Female Child ◽  
Pubic Hair ◽  
Breast Development ◽  
Average Height ◽  
Gonadotrophin Secretion ◽  
Hypothalamic Pituitary Axis

Appearance of secondary sexual development before the age of nine in a male child and before the age of eight in a female child is called precocious puberty. Precocious puberty due to premature activation of the hypothalamic-pituitary axis is called central or complete precocious puberty. Incomplete precocious puberty is called if ectopic gonadotrophin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex. Here we report a case of a 4-year-old girl brought to a gynecologist by her parents because of breast development, pubic hair and periodic per vaginal bleeding. Her medical history was unremarkable. The parents were of average height, and the mother reported first menstruation when she was 11 years. At physical examination, the girl was 100 cm tall, weighed 17 kg, and had a body mass index of 17. Her pubertal development is classified as Tanner stage 3 breast development and Tanner stage 2 pubic hair development.J Enam Med Col 2016; 6(3): 161-163

scholarly journals Precocious puberty in a 24 month old Nigerian girl: case report

2020 ◽  
Vol 47 (4) ◽  
pp. 358-360
Author(s):  
I.O. Oluwayemi ◽  
A.A. Afolabi ◽  
E.O. Adeniji ◽  
T.O. Ayeni
Keyword(s):  
Precocious Puberty ◽  
Elevated Serum ◽  
Tanner Stage ◽  
Bone Age ◽  
Pubic Hair ◽  
Breast Development ◽  
Endocrine Disorders ◽  
Essentially Normal ◽  
History Of ◽  
Age And Sex

Precocious puberty refers to the appearance of signs of puberty at an earlier age than is considered normal. It occurs ten times more commonly in  girls than in boys. The overall incidence ranged from 1/5000 to 1/10,000 children. The cause is idiopathic in 90% of cases of female precocious  puberty. We present BA a 24 month old female toddler who presented with one year history of progressive breast development and 6 month history of pubic hair growth. There was associated increasing weight, height and vaginal secretion. There was no similar occurrence in the family. Mother attained menarche at 14 years of age. Essential finding at presentation revealed a toddler who is heavy and tall for age with a weight of 17kg (>95th percentile for age and sex), height of 90.5cm (90th percentile for age and sex), Occipito-frontal circumference of 49cm (normal). Her sexual maturityrating was Tanner stage 3 for breasts and stage 2 for pubic hair. An assessment of precocious puberty was made. Her investigation result showed an advanced bone age of 5 years; elevated serum gonadotrophins in the pubertal range; and essentially normal cranial CT. Abdomino-pelvic USS showed an enlarged uterus for age, and a dominant right follicle with internal echo measuring 17.1mm X 15.2mm. Parents were counseled on the need for treatment to arrest the progression of precocious puberty but yet to respond because of financial constraint after 2 years of diagnosis. Female precocious puberty is ten times more common than male precocious puberty. The aetiology is idiopathic in 90% of cases and It is amenable to treatment. Integration of the investigation and treatment of childhood endocrine disorders into the National Health Insurance scheme will be a great panacea to the challenge of prompt management in developing countries. Keywords: Precocious, puberty, 24 months old, female, idiopathic, poverty, Nigeria 

A Comparison of Patients with Central Precocious Puberty Who Have a Pubertal versus Prepubertal Ultrasensitive LH at Presentation

2021 ◽  
pp. 1-5
Author(s):  
Lauren A. Logan ◽  
Erica A. Eugster
Keyword(s):  
Luteinizing Hormone ◽  
Precocious Puberty ◽  
Chart Review ◽  
Retrospective Chart Review ◽  
Tanner Stage ◽  
Central Precocious Puberty ◽  
Bone Age ◽  
Breast Development ◽  
Advanced Development ◽  
Minimal Information

<b><i>Background:</i></b> A random ultrasensitive luteinizing hormone (LH) (LH-ICMA) ≥0.3 mIU/L is highly accurate in confirming a diagnosis of central precocious puberty (CPP). However, a prepubertal value does not exclude the diagnosis. The clinical differences between patients with CPP who have a pubertal versus prepubertal LH-ICMA have not been clearly defined. Furthermore, there is minimal information regarding the utility of this test in boys with CPP. The objective of this study was to analyze differences between patients diagnosed with CPP who had a pubertal versus prepubertal LH-ICMA, including a cohort of boys. <b><i>Methods:</i></b> A retrospective chart review of children diagnosed with CPP within the last 10 years who had a baseline LH-ICMA obtained was performed. Variables analyzed included sex, age, ethnicity, bone age, BMI, etiology, Tanner stage (TS), testicular volume, and menarchal status. <b><i>Results:</i></b> Of 27 boys and 126 girls who qualified for the study, the LH-ICMA was pubertal in 87% and prepubertal in 13%. Girls with a pubertal LH-ICMA had higher baseline estradiol concentrations (<i>p</i> &#x3c; 0.001) and more advanced breast development (<i>p</i> = 0.015) compared to girls with a prepubertal LH-ICMA. Of girls with a prepubertal LH-ICMA, 74% had at least TS 3 breast development and 1 was post-menarchal. The LH-ICMA was pubertal in 96% of the boys with CPP in this study. <b><i>Conclusions:</i></b> The LH-ICMA can be prepubertal even in girls with advanced development. To our knowledge, ours is the largest cohort of boys in whom the accuracy of a random LH-ICMA has been reported.

Adult-to-Adult Living-Donor Liver Transplantation

Swiss Surgery ◽  
2003 ◽  
Vol 9 (5) ◽  
pp. 227-236 ◽  
Author(s):  
Majno ◽  
Mentha ◽  
Berney ◽  
Bühler ◽  
Giostra ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Living Donor ◽  
Living Donor Liver Transplantation ◽  
Therapeutic Option ◽  
Donor Liver ◽  
Donor Liver Transplantation ◽  
Multidisciplinary Program ◽  
First Case ◽  
Living Donor Transplantation ◽  
The Right

Living donor liver transplantation is a relatively new procedure in which the right side of the liver is harvested in a healthy donor and transplanted into a recipient. After the first case in 1994, over 3000 cases have been done worldwide. This review summarizes the reasons why the procedure is needed, describes its main technical aspects, highlights the boundaries in which it can be done safely, summarizes the current experience worldwide and describes the main points of the program in our unit. We argue that living-donor transplantation is a viable alternative to a long time on the waiting list for several patients, and it can be performed safely and successfully provided that all precautions are undertaken to minimize the risks in the donor and to increase the chances of a good outcome in the recipients. If these prerequisites are met, and within the framework of a structured multidisciplinary program, we believe that living-donor liver transplantation should be funded by health insurances as a recognized therapeutic option.

Late-onset malignant glaucoma

2020 ◽  
Vol 3 ◽  
pp. 1
Author(s):  
Ramiro José Daud ◽  
Horacio Freile ◽  
Mauricio Freile ◽  
Soledad Mariano
Keyword(s):  
Intraocular Pressure ◽  
Case Report ◽  
Acute Pain ◽  
Late Onset ◽  
Slit Lamp ◽  
Normal Range ◽  
Slit Lamp Examination ◽  
Complete Occlusion ◽  
Malignant Glaucoma ◽  
The Right

A case report on a 49-year-old female with diagnoses of ocular hypertension in her left eye (LE) treated with 250 mg/day acetazolamide for 2 years. During the slit-lamp examination, complete occlusion of both iridocorneal angles was detected. Intraocular pressure (IOP) was 10 and 35 mmHg in the right eye and LE, respectively. Phacotrabeculectomy was performed in the LE. After 1 month of the procedure, the patient developed a slowly progressive miopization from −1 to −3 diopters (D) the following months. Approximately 3 months after surgery, the patient developed an episode of acute pain, athalamia, and IOP 45 mmHg in her LE. Late-onset malignant glaucoma was suspected and the patient was treated with topical hypotensive and cycloplegic agent until a prompt vitrectomy was performed. Deepening of the anterior chamber and restoration of IOP to normal range was obtained after surgery.

scholarly journals Primary Gastrointestinal Stromal Tumor of the Liver with Cystic Changes

2019 ◽  
Vol 13 (1) ◽  
pp. 58-65
Author(s):  
Takashi Tashiro ◽  
Fumihiro Uwamori ◽  
Yukiomi Nakade ◽  
Tadahisa Inoue ◽  
Yuji Kobayashi ◽  
...  
Keyword(s):  
Liver Tumors ◽  
Past History ◽  
Brain Infarction ◽  
Arterial Phase ◽  
First Case ◽  
Positron Emission ◽  
Cystic Changes ◽  
Multiple Bone Metastases ◽  
The Right ◽  
Cells Of Cajal

Gastrointestinal stromal tumors (GISTs) are known to originate specifically from the intestinal cells of Cajal located in the gastrointestinal mesenchyme. GISTs developing outside of the digestive tract have barely been reported. We encountered a first case of large primary GISTs in the liver with cystic changes. A 63-year-old man with a past history of brain infarction visited our hospital. The computed tomography (CT) revealed a 6-cm and a 10-cm mass in the right and the caudal lobe of the liver, respectively. These tumors have marginal enhancement in the arterial phase; however, they presented as hypodense in the internal tumor sites. Both liver tumors had cystic changes. Gastrointestinal examinations using endoscopy revealed no other gastrointestinal tumors, and [18F]-fluoro-2-deoxy-D-glucose positron emission tomography/CT revealed multiple bone metastases in addition to the liver tumors. The liver tumor specimens were composed of spindle cells, and the immunohistochemical staining for c-Kit and for DOG1, as discovered on GIST, was positive. The patient was diagnosed with primary hepatic GIST with cystic changes.

scholarly journals Emphysematous pyelonephritis with air bubble in the inferior vena cava

2020 ◽  
Vol 26 (1) ◽  
Author(s):  
Tiffany A. Perkins ◽  
Alberic Rogman ◽  
Murali K. Ankem
Keyword(s):  
Septic Shock ◽  
Inferior Vena Cava ◽  
Inferior Vena ◽  
Vena Cava ◽  
Rare Presentation ◽  
Emphysematous Pyelonephritis ◽  
Case Presentation ◽  
Air Bubble ◽  
First Case ◽  
The Right

Abstract Background Emphysematous pyelonephritis (EPN) with gas in the inferior vena cava (IVC) is a rare presentation and to our knowledge, this is the first case report in the urologic literature. Case presentation A 35-Year-old obese diabetic Hispanic female presented to the emergency room with a clinical picture of septic shock. Prompt computerized tomography scan revealed EPN with gas throughout the right renal parenchyma and extending to the right renal vein, IVC, and pulmonary artery. She died before surgical intervention Conclusion This case demonstrates that patients presenting with severe EPN have a high mortality risk and providers should acknowledge that septic shock, endogenous air emboli, or a combination of both could result in cardiovascular collapse and sudden death.

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