A case of pediatric secondary hypertension caused by juxtaglomerular cell tumor.
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ABSTRACT:
SUBMITTER: Ruan Y
PROVIDER: S-EPMC10498206 | biostudies-literature | 2023 Sep
REPOSITORIES: biostudies-literature
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A case of pediatric secondary hypertension caused by juxtaglomerular cell tumor.
Ruan Yanan Y Lian Fengyu F Tian Yanjin Y Li Qing Q Huang Xiaoning X
Quantitative imaging in medicine and surgery 20230724 9
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Project description:Juxtaglomerular cell tumor (JGCT) is a rare renal tumor, producing renin and behaving almost in a benign fashion. So far, only three cases have been reported as malignant. We report a rare case with atypical JGCT. A 74-year-old male was referred to our hospital due to hypertension, proteinuria, and hematuria. Abdominal CT revealed a mass measured in 9.7×7.0 cm in the lower portion of the right kidney. Right kidney was removed laparoscopically. Grossly, white to tan tumor with massive hemorrhage and necrosis occupied the lower portion of the right kidney. Microscopically, tumor grew in a solid fashion. Tumor cells were polygonal to ovoid cells with round nuclei and clear to eosinophilic cytoplasm. Mitosis was found in 5 per 10 HPF. Immunohistochemically, tumor cells were stained by vimentin and CD34. Some tumor cells were also positive for renin. Electron micrograph showed near rhomboid crystalline structure in the tumor cells. Because of massive necrosis and mitotic figures, diagnosis of atypical (potentially malignant) JGCT was rendered. Gene mutations for IDH1, PIK3CA, K-ras, N-ras, Braf, and EGFR were not found by MBP-QP system.
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Project description:Study Design Case report. Objective The purpose of this work is to report the case of a giant cell tumor involving the second cervical vertebra in a pediatric patient. Surgical management included a combined posterior and anterior cervical approach. There has been no recurrence in 2 years of follow-up. Case Report A 13-year-old girl presented with scoliosis with incidentally lytic lesion involving the second cervical vertebra. The radiologic investigations and biopsy result indicated a giant cell tumor of the bone. A combined posterior and anterior cervical approach was performed to resect the lesion, reconstruct the spine, and restore stability. Two years of follow-up revealed no recurrence of the lesion with stable reconstruction of the spine. Results The lesion was surgically managed for excision and spinal fusion by combining a posterior occipitocervical arthrodesis with an anterior retropharyngeal cervical approach. The final histopathology result confirmed a giant cell tumor of the bone. Conclusions Giant cell tumor involving the second cervical vertebra is uncommon; this tumor can be managed surgically by using a combined posterior and anterior cervical retropharyngeal approach. The presented case was unique in terms of the tumor location, patient age, and surgical management.
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Project description:ImportanceExtensive population-based studies have explored the prevalence of primary hypertension (HTN) in children and adolescents. However, there is little published data on the characteristics of different types of pediatric HTN and the causes of secondary HTN.ObjectiveTo investigate the characteristics of different types of pediatric HTN and the causes of secondary HTN in a hospital setting.MethodsThe study cohort comprised pediatric inpatients (<18 years of age) discharged with a diagnosis of HTN from Beijing Children's Hospital during 2015-2020. Pediatric patients with HTN were allocated to secondary and primary HTN groups on the basis of comprehensive analyses of their diagnoses, family history of HTN, and findings on physical examination, as documented in their medical records. The Mann-Whitney U test, χ 2 and Fisher's exact test were used to assess differences in characteristics of patients with different HTN types and causes of secondary HTN.ResultsData of 1470 inpatients with HTN from 18 clinical departments were included in the analysis. Among them, 458 (31.2%) had primary HTN, and 1012 (68.8%) had secondary HTN. Compared with patients had primary HTN, children with secondary HTN were younger and had lower body mass indexes and longer lengths of stay. Moreover, children with primary HTN had mostly been managed by the Endocrinology and Cardiology Departments, 75.8% of them having obesity-related comorbidities. In contrast, most patients with secondary HTN had been managed by the Nephrology Department, renal diseases being the leading cause of their HTN (46.3%).InterpretationSecondary HTN is more common than primary HTN in pediatric clinical settings, renal diseases being the leading cause of secondary HTN.
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Project description:BackgroundHypertensive crisis is a relatively rare condition among infants and usually occurs secondary to an underlying disease. If not managed promptly, it is life-threatening and can lead to irreversible damage to vital organs. While secondary hypertension due to tumours has been reported previously, acute decompensated heart failure is rare, especially in the paediatric population.Case summaryA 2-month-old female infant presented with poor feeding and poor body weight gain. She was extremely ill, and blood gas analysis showed prominent acidosis (pH 6.945). The patient was intubated and referred to our hospital for further care. Her arterial blood pressure (BP) was as high as 142/62 mmHg. Echocardiography showed decreased left ventricular function with an ejection fraction of 19.5% and a left ventricular end-diastolic diameter of 25.8 mm (Z score = 2.71). We promptly started treatment with antihypertensive drugs. She had no congenital heart disease or any lesions that may have caused an increased afterload. There was no palpable mass suggestive of the tumour; however, close examination with abdominal echo and subsequent contrast-enhanced computed tomography confirmed a left kidney mass. Blood tests suggested renin-dependent hypertension due to the tumour causing an excessive afterload. Laparoscopic left nephrectomy improved cardiac function improved as BP decreased.DiscussionBlood pressure measurement is often omitted in daily practice when examining infants because of difficulty in measurement. However, BP may be the only detectable sign in patients with secondary hypertension before decompensated heart failure, and BP should also be measured in infants.
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Project description:We report on the case of a partial nephrectomy for a Juxtaglomerular apparatus (JGA) tumour in a 28 year old female who presented with fatigue and symptomatic hypertension, and a normal serum renin level on pre-operative work-up.
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Project description:Development of pulmonary hypertension (PH) in patients with left side heart disease (LHD) is a predictor of poor prognosis. The use of pulmonary vasodilators in PH associated with LHD (PH-LHD) is controversial. In this study, we describe the practice patterns regarding the use of pulmonary vasodilators in PH-LHD among a group of international pediatric PH specialists. A survey was distributed to the members of three pediatric PH networks: PPHNet, PVRI, and REHIPED. The survey queried participants on the rationale, indications, and contraindications of the use of pulmonary vasodilators in children with PH-LHD. Forty-seven PH specialists from 39 PH centers completed the survey. Participants included PH specialists from North America (57%), South America (15%), and Europe (19%). The majority of participants (74%) recommended the use of pulmonary vasodilators only in patients with combined pre-capillary and post-capillary pulmonary hypertension. Participants required the presence of clinical symptoms or signs of heart failure (68%) or right ventricular dysfunction by echocardiography (51%) in order to recommend pulmonary vasodilator therapy. There was no agreement regarding hemodynamic criteria used to recommend pulmonary vasodilators or the etiologies of LHD considered contraindications for using pulmonary vasodilators to manage PH-LHD. Of the available PH-targeted drugs, most participants preferred the use of phosphodiesterase-5-inhibitors for this indication. In conclusion, the practice of recommending pulmonary vasodilators in PH-LHD is highly variable among international pediatric PH specialists. Most specialists of those surveyed (57% in North America) would consider the use of pulmonary vasodilators in PH-LHD only if pre-capillary pulmonary hypertension and right ventricular dysfunction are present.
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Project description:Background17α-hydroxylase deficiency (17OHD) is an autosomal recessive genetic disease characterized by low renin hypertension, abnormal sexual development, and reduced androgen levels. The morbidity rate of 17OHD is less than 1/10,000, and a lack of knowledge of this condition may lead to misdiagnosis and delayed treatment.Case presentationThis is a case report of a patient suffering from hypertension who was diagnosed with 17OHD. The patient was misdiagnosed for more than 20 years. The patient presented with hypertension, hypokalemia, sexual infantilism, and delayed bone age. The patient had a 46, XY karyotype and a homozygous mutation of the CYP17A1 gene. The mutation site was c.1319G > A (p.Arg440His). After she took Nifedipine Sustained Release Tablets 30 mg once a day in the morning, her blood pressure dropped and is currently under control at about 145/95 mmHg.ConclusionsWith clinicians' increasing awareness of 17OHD, effective treatment based on early diagnosis should correct hypogonadism and avoid the cardiovascular and cerebrovascular complications of hypertension.
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Project description:IntroductionThalamic tumors are rare and uncommonly manifest as movement disorders, including hemidystonia. Despite this association, little is known about the evolution of hemidystonia.Case descriptionWe report on a 11-year-old boy who complained of hypaesthesia and fine motor problems in the left hand. A magnetic resonance imaging showed a large mass in the right thalamus. Stereotactic biopsy revealed a WHO grade 4 astrocytoma, and the patient underwent normofractioned radiochemotherapy with proton-beam radiation and temozolomide. Three months later, a spastic hemiparesis developed on the left side, which progressed over months. Over the following months, the hemiparesis slowly improved, but hemidystonia in the same side developed. This was accompanied with radiological evidence of tumor regression, showing a persistent lesion in the ventral posterolateral and the intralaminar thalamus.ConclusionThis case illustrates the unusual and complex temporal course of appearance and disappearance of hemidystonia along with the regression and growth in glioblastoma involving the thalamus.
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