Moyamoya disease presenting with tubular dysfunction in a child: pitfalls in diagnosing an atypical hyponatremic-hypertensive syndrome
Abstract Background Moyamoya disease, a cause of pediatric stroke, has been shown to affect furthermore extra-cranial districts, mostly the kidney arterial site, resulting in steno-occlusive changes. Unilateral renal artery stenosis accounts for 8%-10% out of cases of renovascular hypertension in ch...
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| Format: | Article |
| Language: | English |
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BMC
2023-05-01
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| Series: | BMC Pediatrics |
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| Online Access: | https://doi.org/10.1186/s12887-023-03926-1 |
| _version_ | 1797827328765591552 |
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| author | Maria Luisa Conte Claudio La Scola Francesca Mencarelli Beatrice Filippini Elena Fabbri Valentina Ragnoni Elisa Ravaioli Andrea Pasini Gianluca Vergine |
| author_facet | Maria Luisa Conte Claudio La Scola Francesca Mencarelli Beatrice Filippini Elena Fabbri Valentina Ragnoni Elisa Ravaioli Andrea Pasini Gianluca Vergine |
| author_sort | Maria Luisa Conte |
| collection | DOAJ |
| description | Abstract Background Moyamoya disease, a cause of pediatric stroke, has been shown to affect furthermore extra-cranial districts, mostly the kidney arterial site, resulting in steno-occlusive changes. Unilateral renal artery stenosis accounts for 8%-10% out of cases of renovascular hypertension in childhood, however it rarely underlies a hyponatremic-hypertensive syndrome (HHS). Case presentation We describe an 18-month-old boy with a recent history of polyuria and polydipsia, who presented an acute febrile gastroenteritis with neurological impairment, severe dehydration, hyponatremia, hypokalemia, kidney tubular dysfunction, and elevated aldosterone and renin even with a normal blood pressure. Fluid and electrolytes correction was performed, with complete recovery. An abdominal ultrasound displayed a smaller right kidney. A brain magnetic resonance and an electroencephalogram did not show any relevant abnormalities. Five months later, the child experienced a left-side hemiparesis after a traumatic concussion, and a severe hypertension. A brain tomography documented a cerebral ischemia. Brain and kidney angiographic studies displayed puff of smoke findings of internal right carotid artery branches and a steno-occlusive pattern of right renal artery, respectively. Hence, moyamoya disease with HHS secondary to unilateral renal artery stenosis was diagnosed. After an unsuccessful antiplatelet and antihypertensive pharmacological treatment, the boy underwent a renal angioplasty and a cerebral STA-MCA bypass (direct superficial temporal artery-to-middle cerebral artery bypass), resulting in a significant improvement of both neurological and kidney disease. Conclusions Although the association between unilateral renal artery stenosis and HHS has been previously shown, this is the first report of atypical HHS, with hypertension preceded by tubular dysfunction, recognized in the framework of moyamoya disease. |
| first_indexed | 2024-04-09T12:47:19Z |
| format | Article |
| id | doaj.art-6f199e419fb547bb99122d516822e4c4 |
| institution | Directory Open Access Journal |
| issn | 1471-2431 |
| language | English |
| last_indexed | 2024-04-09T12:47:19Z |
| publishDate | 2023-05-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Pediatrics |
| spelling | doaj.art-6f199e419fb547bb99122d516822e4c42023-05-14T11:27:54ZengBMCBMC Pediatrics1471-24312023-05-012311610.1186/s12887-023-03926-1Moyamoya disease presenting with tubular dysfunction in a child: pitfalls in diagnosing an atypical hyponatremic-hypertensive syndromeMaria Luisa Conte0Claudio La Scola1Francesca Mencarelli2Beatrice Filippini3Elena Fabbri4Valentina Ragnoni5Elisa Ravaioli6Andrea Pasini7Gianluca Vergine8Department of Pediatrics, Infermi HospitalPediatric Nephrology and Dialysis, Pediatric Unit, IRCCS AziendaOspedaliero-Universitaria Di BolognaPediatric Nephrology and Dialysis, Pediatric Unit, IRCCS AziendaOspedaliero-Universitaria Di BolognaDepartment of Pediatrics, Infermi HospitalDepartment of Pediatrics, Infermi HospitalDepartment of Pediatrics, Infermi HospitalDepartment of Pediatrics, Infermi HospitalPediatric Nephrology and Dialysis, Pediatric Unit, IRCCS AziendaOspedaliero-Universitaria Di BolognaDepartment of Pediatrics, Infermi HospitalAbstract Background Moyamoya disease, a cause of pediatric stroke, has been shown to affect furthermore extra-cranial districts, mostly the kidney arterial site, resulting in steno-occlusive changes. Unilateral renal artery stenosis accounts for 8%-10% out of cases of renovascular hypertension in childhood, however it rarely underlies a hyponatremic-hypertensive syndrome (HHS). Case presentation We describe an 18-month-old boy with a recent history of polyuria and polydipsia, who presented an acute febrile gastroenteritis with neurological impairment, severe dehydration, hyponatremia, hypokalemia, kidney tubular dysfunction, and elevated aldosterone and renin even with a normal blood pressure. Fluid and electrolytes correction was performed, with complete recovery. An abdominal ultrasound displayed a smaller right kidney. A brain magnetic resonance and an electroencephalogram did not show any relevant abnormalities. Five months later, the child experienced a left-side hemiparesis after a traumatic concussion, and a severe hypertension. A brain tomography documented a cerebral ischemia. Brain and kidney angiographic studies displayed puff of smoke findings of internal right carotid artery branches and a steno-occlusive pattern of right renal artery, respectively. Hence, moyamoya disease with HHS secondary to unilateral renal artery stenosis was diagnosed. After an unsuccessful antiplatelet and antihypertensive pharmacological treatment, the boy underwent a renal angioplasty and a cerebral STA-MCA bypass (direct superficial temporal artery-to-middle cerebral artery bypass), resulting in a significant improvement of both neurological and kidney disease. Conclusions Although the association between unilateral renal artery stenosis and HHS has been previously shown, this is the first report of atypical HHS, with hypertension preceded by tubular dysfunction, recognized in the framework of moyamoya disease.https://doi.org/10.1186/s12887-023-03926-1Hyponatremic-hypertensive syndromeKidney tubular dysfunctionUnilateral renal artery stenosisMoyamoya diseaseChildren |
| spellingShingle | Maria Luisa Conte Claudio La Scola Francesca Mencarelli Beatrice Filippini Elena Fabbri Valentina Ragnoni Elisa Ravaioli Andrea Pasini Gianluca Vergine Moyamoya disease presenting with tubular dysfunction in a child: pitfalls in diagnosing an atypical hyponatremic-hypertensive syndrome BMC Pediatrics Hyponatremic-hypertensive syndrome Kidney tubular dysfunction Unilateral renal artery stenosis Moyamoya disease Children |
| title | Moyamoya disease presenting with tubular dysfunction in a child: pitfalls in diagnosing an atypical hyponatremic-hypertensive syndrome |
| title_full | Moyamoya disease presenting with tubular dysfunction in a child: pitfalls in diagnosing an atypical hyponatremic-hypertensive syndrome |
| title_fullStr | Moyamoya disease presenting with tubular dysfunction in a child: pitfalls in diagnosing an atypical hyponatremic-hypertensive syndrome |
| title_full_unstemmed | Moyamoya disease presenting with tubular dysfunction in a child: pitfalls in diagnosing an atypical hyponatremic-hypertensive syndrome |
| title_short | Moyamoya disease presenting with tubular dysfunction in a child: pitfalls in diagnosing an atypical hyponatremic-hypertensive syndrome |
| title_sort | moyamoya disease presenting with tubular dysfunction in a child pitfalls in diagnosing an atypical hyponatremic hypertensive syndrome |
| topic | Hyponatremic-hypertensive syndrome Kidney tubular dysfunction Unilateral renal artery stenosis Moyamoya disease Children |
| url | https://doi.org/10.1186/s12887-023-03926-1 |
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