scholarly journals SUN-937 A Case of Two Pathologies: Primary Hyperaldosteronism with a History of Pheochromocytoma

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Chukwuka Akamnonu ◽  
Sahil Parikh ◽  
Fredric Edward Wondisford
Keyword(s):  
Renal Cysts ◽  
Plasma Renin ◽  
Persistent Atrial Fibrillation ◽  
Normal Serum ◽  
Primary Hyperaldosteronism ◽  
Normal Range ◽  
Refractory Hypertension ◽  
Von Hippel Lindau ◽  
Subtotal Adrenalectomy ◽  
History Of

Abstract Background: In patients with system based diseases, multiple pathologies coexisting in the same organ can go undiagnosed. We present a case of a patient with long standing hypertension recently diagnosed with Primary hyperaldosteronism in the setting of known Pheochromocytoma. Case: A 43 year old male with a history of Von Hippel Lindau (VHL), Pheochromocytoma status post right subtotal adrenalectomy 18 years ago, renal cell carcinoma, prediabetes, bilateral renal cysts, and hypertension presented to the clinic for evaluation of suspected hyperaldosteronism. The patient had a history of medication refractory hypertension associated with hypokalemia requiring potassium supplementation. The patient’s VHL disease involved the pancreas, adrenal, brain and spine which required surgery to remove tumors in these areas. His Pheochromocytoma resolved status post subtotal adrenalectomy. Recent imaging and laboratory work up showed a stable left adrenal nodule (unchanged over the years), unremarkable right adrenal status post subtotal adrenalectomy with normal serum and urine metanephrines. In September 2019 he was seen by his Cardiologist for persistent Atrial Fibrillation and subsequent workup for suspected hyperaldosteronism showed a morning plasma aldosterone of 44.4ng/dL (normal range 4–21 ng/dL), plasma renin activity of <0.6 ng/ml/hr (normal range 0.6–4.3 ng/mL), 24 hour urine aldosterone of 21mcg/24hr (normal range 3–20 mcg/24hr), Urine sodium excretion of 236 mmol/24hr (normal range 40-217mmol/24hr), and potassium of 3.5mmol/L (normal range 3.6–5.0 mmol/L). The diagnosis of Primary hyperaldosteronism was confirmed and the patient was subsequently scheduled for adrenal venous sampling. Conclusion: This case demonstrates how multiple adrenal pathologies can present in the same individual. There is paucity of data in the literature as to the existence of both Pheochromocytoma and primary hyperaldosteronism in the same individual or the pathophysiological connection between them. When the diagnosis of a disease affecting a specific organ has already been made, the discovery of a secondary pathologic process affecting such organ may end up getting delayed. Clinicians should be aware of this possibility.

Hereditary Heparin Cofactor II Deficiency and the Risk of Development of Thrombosis

1987 ◽  
Vol 57 (02) ◽  
pp. 196-200 ◽  
Author(s):  
R M Bertina ◽  
I K van der Linden ◽  
L Engesser ◽  
H P Muller ◽  
E J P Brommer
Keyword(s):  
Liver Disease ◽  
Venous Thrombosis ◽  
Healthy Volunteers ◽  
Mean Value ◽  
Age Group ◽  
Normal Range ◽  
Heparin Cofactor Ii ◽  
History Of ◽  
Group 2 ◽  
Hereditary Nature

SummaryHeparin cofactor II (HC II) levels were measured by electroimmunoassay in healthy volunteers, and patients with liver disease, DIC, proteinuria or a history of venous thrombosis. Analysis of the data in 107 healthy volunteers revealed that plasma HC II increases with age (at least between 20 and 50 years). HC II was found to be decreased in most patients with liver disease (mean value: 43%) and only in some patients with DIC. Elevated levels were found in patients with proteinuria (mean value 145%). In 277 patients with a history of unexplained venous thrombosis three patients were identified with a HC II below the lower limit of the normal range (60%). Family studies demonstrated hereditary HC II deficiency in two cases. Among the 9 heterozygotes for HC II deficiency only one patient had a well documented history of unexplained thrombosis. Therefore the question was raised whether heterozygotes for HC II deficiency can also be found among healthy volunteers. When defining a group of individuals suspected of HC II deficiency as those who have a 90% probability that their plasma HC II is below the 95% tolerance limits of the normal distribution in the relevant age group, 2 suspected HC II deficiencies were identified among the healthy volunteers. In one case the hereditary nature of the defect could be established.It is concluded that hereditary HC II deficiency is as prevalent among healthy volunteers as in patients with thrombotic disease. Further it is unlikely that heterozygosity for HC II deficiency in itself is a risk factor for the development of venous thrombosis.

The Prevalence of Hereditary Antithrombin-III Deficiency in Patients with a History of Venous Thromboembolism

1985 ◽  
Vol 54 (04) ◽  
pp. 744-745 ◽  
Author(s):  
R Vikydal ◽  
C Korninger ◽  
P A Kyrle ◽  
H Niessner ◽  
I Pabinger ◽  
...  
Keyword(s):  
Venous Thrombosis ◽  
Antithrombin Iii ◽  
Positive Family History ◽  
Normal Range ◽  
The Family ◽  
History Of ◽  
Antithrombin Iii Deficiency ◽  
Recurrent Venous Thrombosis ◽  
Antithrombin Iii Activity ◽  
Slight Deficiency

SummaryAntithrombin-III activity was determined in 752 patients with a history of venous thrombosis and/or pulmonary embolism. 54 patients (7.18%) had an antithrombin-III activity below the normal range. Among these were 13 patients (1.73%) with proven hereditary deficiency. 14 patients were judged to have probable hereditary antithrombin-III deficiency, because they had a positive family history, but antithrombin-III deficiency could not be verified in other members of the family. In the 27 remaining patients (most of them with only slight deficiency) hereditary antithrombin-III deficiency was unlikely. The prevalence of hereditary antithrombin-III deficiency was higher in patients with recurrent venous thrombosis.

scholarly journals Parenchymal mucinous cystadenoma of the kidney: a case report and literature review

2021 ◽  
Vol 27 (1) ◽  
Author(s):  
Mahmoudreza Kalantari ◽  
Shakiba Kalantari ◽  
Mahdi Mottaghi ◽  
Atena Aghaee ◽  
Salman Soltani ◽  
...  
Keyword(s):  
Renal Cyst ◽  
Left Kidney ◽  
Previous History ◽  
Mucinous Cystadenoma ◽  
Single Layer ◽  
Renal Cysts ◽  
Cystic Mass ◽  
Flank Pain ◽  
History Of

Abstract Background Mucinous cystadenoma (MC) of the kidney is exceedingly rare. We found 22 similar cases in the literature. These masses are underdiagnosed due to radiologic similarities with simple renal cysts. Case presentation A 66-year-old man with a previous history of hypertension and anxiety was referred to our tertiary clinic with left flank pain. Ultrasound revealed a 60 mm-sized, complex cystic mass with irregular septa in the lower pole of the left kidney (different from last year's sonographic findings of a simple benign cyst with delicate septa). CT scan showed the same results plus calcification. Due to suspected renal cell carcinoma, a radical nephrectomy was performed. Postoperative histopathologic examination revealed a cyst lined by a single layer of columnar mucin-producing cells with small foci of pseudo-stratification, consistent with the MC’s diagnosis. The first follow-up visit showed normal blood pressure without medication and no flank pain and anxiety after a month. Conclusion It is quite challenging to distinguish the primary MC of the kidney from a simple renal cyst based on clinical and imaging findings. The radiologic features of these entities overlap significantly. Thus, complex renal cyst and renal cysts with mural nodules should be followed closely to detect malignancy earlier.

scholarly journals The Relationship between Scapular Upward Rotation and Shoulder Internal and External Rotation Isokinetic Strength in Professional Baseball Pitchers

Healthcare ◽  
2021 ◽  
Vol 9 (6) ◽  
pp. 759
Author(s):  
Byung Gon Kim ◽  
Seung Kil Lim ◽  
Sunga Kong
Keyword(s):  
External Rotation ◽  
Professional Baseball ◽  
Isokinetic Strength ◽  
Strength Ratio ◽  
Shoulder Injury ◽  
Isokinetic Dynamometer ◽  
Normal Range ◽  
History Of ◽  
Baseball Pitchers ◽  
The Relationship

This study aims to assess the relationship between scapular upward rotation (SUR) across varying humeral-elevation angles (HEAs) and shoulder isokinetic strength and ratio in professional baseball pitchers. The subjects were professional baseball pitchers (n = 16) without a history of shoulder injury in the last six months. The subject’s SUR angles were measured with the humerus elevated at HEAs of 0° (at rest), 60°, 90°, and 120° to the scapular plane. Shoulder isokinetic strength was evaluated for shoulder internal rotation (IR) and external rotation (ER) strength (PT%BW and TW%BW), and the ER/IR strength ratios were determined at 60, 120 and 180°/s using an isokinetic dynamometer. The SUR angle at an HEA of 0° was positively correlated with IR strength at 120°/s (r = 0.535) and 180°/s (r = 0.522). The SUR angle at an HEA of 60° was negatively correlated with the ER/IR strength ratios at 60°/s (r = −0.505) and 120°/s (r = −0.500). The SUR angle at an HEA of 90° was negatively correlated with the ER/IR strength ratios at 60°/s (r = −0.574; r = −0.554) and 120°/s (r = −0.521; r = −0.589) as well as with ER strength at 180°/s (r = −0.591, r = −0.556). The SUR angle at an HEA of 120° was negatively correlated with ER strength at 60°/s (r = −0.558), 120°/s (r = −0.504; r = −0.524), and 180°/s (r = −0.543) and the ER/IR strength ratio at 60°/s (r = −0.517). In this study, we found that the ratio of isokinetic strength between ER and IR became closer to the normal range on increasing the SUR angle. In particular, an HEA of 90°, which resembles the pitching motion, showed a clear relationship between SUR, shoulder ER, and the ratio of ER/IR isokinetic strength in professional baseball pitchers.

Hemangioblastoma diagnosis and surveillance in von Hippel–Lindau disease: a consensus statement

2021 ◽  
pp. 1-6
Author(s):  
Kristin Huntoon ◽  
Matthew J. Shepard ◽  
Rimas V. Lukas ◽  
Ian E. McCutcheon ◽  
Anthony B. Daniels ◽  
...  
Keyword(s):  
Age At Onset ◽  
Benign Tumors ◽  
Expert Committee ◽  
Level Of Evidence ◽  
Von Hippel Lindau ◽  
Assessment Development ◽  
History Of ◽  
Von Hippel Lindau Disease ◽  
The Central Nervous System ◽  
Cancer Network

OBJECTIVE Hemangioblastomas are a frequent underlying cause of neurological morbidity and death in patients with von Hippel–Lindau disease (VHL). Although these benign tumors can cause significant neurological debility when undetected and untreated, unified evidence-based surveillance recommendations for VHL patients have not been established. To develop consensus recommendations, the VHL Alliance established an expert committee, named the International VHL Surveillance Guidelines Consortium, to define surveillance recommendations. METHODS The Central Nervous System (CNS) Hemangioblastoma Subcommittee of the Guidelines Consortium was formed as a multidisciplinary team of experts in the diagnosis and management of hemangioblastomas. Recommendations were formulated using the Grading of Recommendations, Assessment, Development and Evaluation (GRADE) and National Comprehensive Cancer Network Categories of Evidence and Consensus categorization after a comprehensive literature review. RESULTS Published studies (n = 49) that discussed age at onset, MRI frequency, natural history of VHL, and the risks and benefits of surveillance were analyzed. Based on this analysis, the authors recommend that clinical evaluation (yearly) be used as the primary screening tool for hemangioblastomas in VHL. The subcommittee suggests that screening be performed between the ages of 11 and 65 years, or with the onset of symptoms, for synchronicity with other testing regimens in VHL. The subcommittee also recommends that baseline MRI be first performed at the age of 11 years (suggested 2B, level of evidence D) or after identification of neurological symptoms or signs (if earlier) and continue every 2 years (recommended 2A, level of evidence A). CONCLUSIONS The CNS Hemangioblastoma Subcommittee of the International VHL Surveillance Guidelines Consortium here proposes guidelines that aim to increase the early detection of VHL-associated hemangioblastomas to reduce their morbidity and mortality.

LONG-TERM EFFECTS OF OTITIS MEDIA A TEN-YEAR COHORT STUDY OF ALASKAN ESKIMO CHILDREN

PEDIATRICS ◽  
1973 ◽  
Vol 52 (4) ◽  
pp. 577-585 ◽  
Author(s):  
Gary J. Kaplan ◽  
J. Kenneth Fleshman ◽  
Thomas R. Bender ◽  
Carol Baum ◽  
Paul S. Clark
Keyword(s):  
Hearing Loss ◽  
Otitis Media ◽  
Verbal Ability ◽  
Significant Loss ◽  
Long Term Effects ◽  
Normal Range ◽  
History Of ◽  
Conductive Component ◽  
Verbal Development

Histories of ear disease, otoscopic examinations, and audiologic, intelligence, and achievement tests were obtained from a cohort of 489 Alaskan Eskimo children who have been followed through the first ten years of life. Seventy-six per cent had experienced one or more episodes of otitis media since birth. Of these, 78% had their first attack during their first two years of life. Perforations and scars were present in 41%. A hearing loss of 26 decibels or greater was present in 16%, and an additional 25% were in the normal range but had a measurable air-bone gap. Children with a history of otitis media prior to 2 years of age and a hearing loss of 26 decibels or greater had a statistically significant loss of verbal ability and were behind in total reading, total math, and language. In addition, children who had an early onset of otitis media but now had normal hearing with a conductive component were also adversely affected in verbal areas. The number of otitis media episodes was related to tympanic membrane abnormalities, hearing loss, and low verbal and achievement scores. These findings indicate that otitis media has been a significant cause of morbidity in Alaskan Eskimo children, and its onset during the critical years of language development as well as the number of episodes play an important role in impairing verbal development.

MO427SAFE USE OF ALLOPURINOL TO TREAT ACUTE URIC ACID NEPHROPATHY IN PREGNANCY: A CASE REPORT

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Fahd Khan ◽  
Aizaz Ali ◽  
Jamie Willows ◽  
Didem Tez
Keyword(s):  
Uric Acid ◽  
Case Report ◽  
Serum Creatinine ◽  
Serum Uric Acid ◽  
Renal Tubules ◽  
Normal Range ◽  
History Of ◽  
Uric Acid Nephropathy ◽  
Acute Uric Acid Nephropathy ◽  
In Pregnancy

Abstract Introduction Acute uric acid nephropathy (UAN) is characterized by acute kidney injury (AKI) due to uric acid crystal precipitation within the distal tubules and collecting ducts. We present a young woman, with a history of hyperuricaemia, who was treated with allopurinol for acute UAN during her first pregnancy. She also continued allopurinol treatment during her second pregnancy for prevention of further acute UAN. To the author’s knowledge, this is the first case report of biopsy-confirmed acute UAN during pregnancy. Case report A 20 year old woman, who was 13 weeks pregnant, was admitted with AKI. Past medical history included chronic kidney disease (CKD) and gout since the age of 17. She had an extensive family history of CKD and gout (without diagnosis, despite genetic testing). She had been on daily allopurinol 300mg, but this was stopped 8 weeks prior by her rheumatology team due to concerns about teratogenicity. At that time serum creatinine was at her baseline of 100 μmol/L (normal range 50-120 μmol/L) and serum uric acid had been 740 μmol/L (normal range 140-360 μmol/L). On admission, she felt well and was euvolemic. Serum creatinine was now 352 μmol/L and her serum uric acid level was 1720 μmol/L, with an elevated urine uric acid to creatinine ratio of 1.1. She underwent renal biopsy, which showed significant deposition of uric acid crystals in the renal tubules, confirming a diagnosis of acute UAN. She was given intravenous fluids. The uncertainties of allopurinol use in pregnancy were discussed with her, and she was restarted on allopurinol 200 mg daily. Over the next 3 weeks, serum uric acid decreased to 470 μmol/L and serum creatinine to 116 μmol/L. She was maintained on allopurinol during her pregnancy and delivered a healthy baby girl. She was advised against further pregnancies due to increased risk of maternal and fetal complications. However, three years later she presented at 15 weeks’ gestation. After a discussion regarding the potential teratogenic effects of allopurinol versus the risk of recurrent severe AKI due to acute UAN if it was again discontinued, she chose to continue allopurinol. The pregnancy proceeded without complication. Her daughters are now 8 and 5 years old. They do not have any congenital malformations, though both have mild to moderate learning difficulties. Discussion Allopurinol is approved for the treatment of hyperuricaemia outside of pregnancy, but given it interrupts purine synthesis there is a biologically plausible concern regarding teratogenicity. However, in our patient with long-standing hyperuricaemia it was the discontinuation of allopurinol that precipitated AKI due to the resultant crystal formation when serum uric acid reached very high levels. Biopsy confirmation of acute UAN was vital in this case, given the possibility of missing an alternative diagnosis and the risks of giving empirical allopurinol therapy. Once the diagnosis for her severe AKI was confirmed, it was clear our patient would benefit from uric acid lowering therapy. Our patient had two healthy girls despite using allopurinol from week 16 in her first pregnancy and throughout her second pregnancy. Unfortunately, both girls have mild to moderate learning needs, though it is unprovable whether allopurinol was causative as no study has followed up long term outcomes after foetal exposure during pregnancy.

scholarly journals Auditory temporal abilities in children with history of recurrent otitis media in the first years of life and persistent in preschool and school ages

CoDAS ◽  
2014 ◽  
Vol 26 (6) ◽  
pp. 494-502 ◽  
Author(s):  
Priscila Cruvinel Villa ◽  
Sthella Zanchetta
Keyword(s):  
Otitis Media ◽  
Otitis Media With Effusion ◽  
Temporal Frequency ◽  
Control Group ◽  
First Year ◽  
Normal Range ◽  
Frequency Pattern ◽  
History Of ◽  
First Year Of Life ◽  
Recurrent Otitis Media

PURPOSE: To study the temporal auditory ordering and resolution abilities in children with and without a history of early OME and ROME, as well as to study the responses according to age. METHODS: A total of 59 children were evaluated, and all of them presented pure tone thresholds within the normal range at the time of the conduction of the hearing tests. The children were divided into two groups according to the occurrence of episodes of recurrent otitis media. Then, each group was divided into two subgroups according to age: 7- and 8-year olds, and 9- and 10-year olds. All children were assessed with standard tests of temporal frequency (ordination) and gaps-in-noise (resolution). RESULTS: For the temporal abilities studied, children with a history of otitis media presented significantly lower results compared to the control group. In the frequency pattern test, the correct answers increased with age in both groups. In the identification of silence intervals, the control group showed no change in threshold regarding to age, but this change was present in the group with a history of otitis media. CONCLUSION: Episodes of otitis media with effusion in the first year of life, recurrent and persistent in preschool and school ages, negatively influence the temporal ordering and resolution abilities.

scholarly journals Transitional cell carcinoma arising in a calyceal cyst mimicking a cystic renal tumour

2014 ◽  
Vol 8 (3-4) ◽  
pp. 196 ◽  
Author(s):  
Jeong Ho Kim ◽  
Joo Yeon Song ◽  
Wan Lee
Keyword(s):  
Transitional Cell Carcinoma ◽  
Cell Carcinoma ◽  
Left Kidney ◽  
Transitional Cell ◽  
Renal Cysts ◽  
Transitional Epithelium ◽  
Cystic Tumour ◽  
Viral Hepatitis B ◽  
History Of ◽  
Tomography Scan

Solitary renal cysts are relatively common. The occurrence of transitional cell carcinoma (TCC) in a renal cyst is rare. We present the case of a 59-year-old man with a medical history of viral hepatitis B. During a workup for his hepatitis, a computed tomography scan revealed a large cystic tumour in the upper region of the left kidney. A radical left nephrectomy was performed. Microscopic examination of the cystic tumour revealed a grade 2 TCC. The cyst was lined by transitional epithelium. This is a case of a TCC growing within a renal calyceal cyst.

scholarly journals Gambaran elektrolit serum pada anak dengan diare akut

e-CliniC ◽  
2016 ◽  
Vol 4 (1) ◽  
Author(s):  
Christin V. Wololi ◽  
Jeanette I.Ch. Manoppo
Keyword(s):  
Serum Sodium ◽  
Acute Diarrhea ◽  
Sudden Onset ◽  
Normal Serum ◽  
Normal Range ◽  
Serum Electrolyte ◽  
Medical Record Data ◽  
Electrolyte Disturbance ◽  
Record Data ◽  
Normal Serum Sodium

Abstract: Acute diarrhea is defined as the sudden onset of 3 or more loose stools per day and lasts no longer than 14 days; chronic or persistent diarrhea lasts more than 14 days. Loss of fluid in large quantitiy such as diarrhea results in imbalance of fluid and electrolyte. The first influenced electrolytes are sodium and chloride since both are extracellular electrolytes. This study aimed to obtain the profile of serum electrolytes in children with acute diarrhea. Thus was a descriptive retrospective study using medical record data of patients hospitalized in the Paediatrics Department of Prof. Dr. R. D. Kandou Manado Hospital in the period of January 2015 - December 2015. The results showed that the highest incidence was among males (30 cases; 65.21%). Most cases were aged 1 - < 5 years (23 cases; 50%). Most serum sodium values were in the normal range (36 cases; 78.26%). Most cases had diarrhea without dehydration and had normal serum sodium (20 cases; 43.47%). Most cases had normal serum potassium levels (33 cases; 71.73%). Most inpatients were without dehydration (17 cases; 36.95%). Most cases had normal serum electrolyte levels (39 cases; 84.78%). Most of the diarrhea without dehydration ( 21 cases; 45.65%). Diarrhea was most often in those who were formula-fed (29 cases; 63.04%). Keywords: acute diarrhea, dehydration, electrolyte disturbance Abstrak: Diare akut didefinisikan sebagai onset mendadak 3 atau lebih mencret per hari dan berlangsung tidak lebih dari 14 hari sedangkan diare kronis atau persisten berlangsung lebih dari 14 hari. Bila terjadi kehilangan cairan dalam jumlah banyak secara terus menerus seperti pada diare maka keseimbangan cairan dan elektrolit dalam tubuh tidak dapat dipertahankan. Elektrolit yang pertama terpengaruh ialah natrium dan klorida karena keduanya merupakan elektrolit ekstrasel. Penelitian ini bertujuan untuk mendapatkan gambaran elektrolit serum pada anak dengan diare akut. Jenis penelitian retrospektif deskriptif menggunakan data rekam medik pasien rawat inap di Bagian Pediatri RSUP Prof. Dr. R.D. Kandou Manado periode Januari 2015 - Desember 2015. Hasil penelitian memperlihatkan bahwa insiden tertinggi pada laki-laki sebanyak 30 kasus (65,21%). Usia terbanyak antara 1 tahun - < 5 tahun yaitu 23 kasus (50%). Nilai natrium serum paling banyak pada kisaran normal yaitu 36 kasus (78,26%), paling banyak didapatkan pada penderita tanpa dehidrasi dengan natrium serum normal 20 kasus (43,47%). Kadar kalium serum normal secara keseluruhan berjumlah 33 kasus (71,73%), paling banyak pada penderita tanpa dehidrasi yaitu 17 kasus (36,95%). Kadar serum paling banyak pada kisaran normal yaitu 39 kasus (84,78%), dan paling banyak pada penderita tanpa dehidrasi yaitu 21 kasus (45,65%). Diare paling banyak pada yang diberi susu formula sebanyak 29 kasus (63,04 %).Kata kunci: diare akut, dehidrasi, gangguan elektrolit

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