A 16-year-old Indonesian boy with uncontrolled hypertension was referred from a remote area in an island to the pediatric nephrology clinic at Cipto Mangunkusumo Hospital, Jakarta, Indonesia. Twenty months prior to admission to our clinic, he experienced palpitation, diaphoresis, and left-sided chest pain without radiation, because of which he was brought to the emergency room. He experienced neither shortness of breath nor blurry vision. His blood pressure was 200/120 mmHg. The internist initiated the administration of 20 mg furosemide per oral (PO) and 12.5 mg captopril PO twice daily, together with 10 mg nifedipine PO 4 times daily. However, the patient’s symptoms and hypertension persisted, due to which his parents turned to herbal treatment and skipped further medical follow-up, although oral medications were continued.\nAs the boy’s palpitations persisted for 14 months, he was taken to another internist who prescribed 30 mg nebivolol PO every morning and 80 mg telmisartan PO every evening. Echocardiography revealed normal cardiac function. Kidney Doppler ultrasound disclosed a suspicion of right adrenal mass and kidney artery stenosis, therefore, he was referred to the pediatric nephrology clinic at our center.\nAt the clinic, he complained of palpitations with diaphoresis. We also discovered that he had lost a weight of 10 kg in the past 4 months. His BMI was 16.5 kg/m2 (undernourished with normal stature), and no skin lesions were noted (). He was hypertensive (blood pressure [BP] 140/83 mmHg) and tachycardic (heart rate (HR) 107 times/minute), with similar BP and HR values in all limbs. Other physical examinations, including neurological examinations and funduscopy, revealed normal findings. Complete blood count, random glucose levels, hepatic enzyme levels, kidney functions, urinalysis, chest X-ray, and electrocardiography were unremarkable. Pheochromocytoma was suspected due to symptoms of catecholamine secretion. However, as the urine catecholamine test was unavailable in Indonesia, the urine sample was sent to a laboratory outside the country. Simultaneously, the patient underwent abdominal magnetic resonance imaging (MRI) ( and ) and 131I-meta-iodobenzylguanidine (MIBG) scintigraphy ( and ) were ordered. 131I-MIBG scintigraphy demonstrated the uptake of pathological tracer in the right suprarenal region corresponding to an adrenal tumor without metastatic lesions. The results of urine catecholamine test, which were received subsequently, confirmed our suspicion of pheochromocytoma (), after which the administration of terazosin was initiated.\nThe boy had always been healthy with no recorded history of high BP, chest pain, or palpitations. He was the first of 3 children in his family. His father had hypertension with obesity. Hereditary diseases were not acknowledged in the family. Since the past 2 years, he had stayed at a boarding school in a remote area. He denied smoking, alcohol use, and the intake of regular medications.\nOne week prior to the surgery, bisoprolol was added to his treatment regimen. Surgery was performed using the posterior retroperitoneoscopic right adrenalectomy approach for removing the tumor ( and ). Administration of 1 mg terazosin PO was maintained until 1 week after the surgery. As his hypertension gradually resolved, terazosin was then discontinued. The results of pathological examination suggesting pheochromocytoma are presented in to . A follow-up examination conducted 6 months after the surgery showed normal and stable BP without the use of any antihypertensive medications, and no other persistent signs associated with pheochromocytoma were noted.
A twenty-four-year-old Irish male with a background history of congenital deafness, intellectual impairment and repair of a posterior urethral valve initially presented to the paediatric nephrology services with nephrotic syndrome. A renal biopsy revealed secondary focal segmental glomerulosclerosis and significant tubuleinterstitial fibrosis. Following introduction of Renin-Angiotensin-Aldosterone system blockade, his proteinuria was controlled.\nDespite this, his chronic kidney disease progressed. Ten years later, he was approaching end-stage kidney disease. A Tenckhoff catheter was inserted without complication. Six weeks later, PD training was initiated. A bicarbonate/lactate buffered solution was used to avoid infusion pain, which he would be unable to verbalise. The following months were complicated by dialysis-associated pericarditis that resolved with intensive haemodialysis for a number of weeks. His baseline PD prescription consisted of seven cycles of two litre exchanges with 1.36% dextrose solution. He was also prescribed an icodextrin-based daytime dwell.\nFive months later, he was admitted with clinical and radiological evidence of a large, rightsided pleural effusion with pleural thickening. The exact duration of the effusion was unclear as he was unable to verbalise symptoms. It was thought to have developed over weeks given the apparent absence of symptoms and the degree of pleural thickening. A chest radiograph four months earlier showed normal lung fields.\nHe was afebrile and systemically well. His serum biochemistry and haematology were unchanged and his inflammatory markers were not raised. ECG and echo were normal. A diagnostic pleural aspirate was performed under ultrasound guidance. This yielded serous fluid and also identified numerous loculations within the collection.\nThe pleural fluid biochemistry was atypical for that seen with pleuroperitoneal leak (). However, as the patient was so well clinically, and numerous pleural fluid cultures were sterile, it was felt that the effusion was not related to infection. It was also felt that the effusion was not due to volume overload given the degree of loculation within the effusion and the absence of other clinical signs of volume overload. The low pleural fluid glucose and high lactate dehydrogenase (LDH) were difficult to interpret as the sampled fluid was walled off within a loculation, probably for many weeks. A clinical diagnosis of pleuroperitoneal leak was made and PD was discontinued.\nThe effusion did not resolve with drainage of the peritoneal cavity, and a chest drain was inserted. This drained minimal serous fluid due to the highly loculated nature of the effusion. A second drain was inserted and intrapleural alteplase administered. This drain became dislodged and was replaced by a third drain with further intrapleural alteplase administration. At this point, however, a chest radiograph confirmed persistence of the large, loculated effusion with a small pneumothorax and a thick pleural rind.\nVideo-assisted thorascopic surgery with decortication was performed. A rapid postoperative recovery ensued and the postoperative chest drains were removed without event. A chest radiograph six weeks later showed a well-expanded right lung, without effusion or pleural abnormality. HD is ongoing without complication.