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Radio-Renal Syndrome


Presentation

  • Patients typically present absence/hypoplasia of digits, radii and/or ulnae, short stature and mild external ear malformation, as well as kidney agenesis or ectopia. There have been no further descriptions in the literature since 1983.[orpha.net]
  • Nephrologist Referral Referral to a paediatric nephrologist should be made should any of the following features be present at first presentation or at any time during the course of treatment: Age 1 year or 12 years At first presentation Family history[pch.health.wa.gov.au]
  • One patient presented with a Brown-Séquard syndrome and an extradural spinal cord compression from tumor. The radiological and cerebrospinal fluid findings are presented and discussed.[en.wikibooks.org]
  • […] vein to be obstructed for symptoms to be present.[patient.info]
  • Most patients present with both manifestations, but some present with loin pain or hematuria alone. Pain episodes are rarely associated with low-grade fever and dysuria, but urinary tract infection is not present.[en.wikipedia.org]
Physician
  • In-Depth Information The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers.[rarediseases.info.nih.gov]
  • Am Fam Physician. 2005 Dec 15;72(12):2430-2436. to the editor: Posterior reversible encephalopathy syndrome (PRES) is a clinicoradiologic entity with headaches, visual disturbances, and seizures that can be misdiagnosed easily.[aafp.org]
  • Physicians will review a patient’s characteristics, in combination with their experience, to identify candidates appropriate for cryoablation.[galilmedical.com]
  • History and etymology It is named after the German physician Carl Reinhold August Wunderlich. Herlyn-Werner-Wunderlich syndrome Promoted articles (advertising)[radiopaedia.org]
  • These professionals may include pediatricians; surgeons; physicians who diagnose and treat disorders of the skeleton, muscles, joints, and related tissues (orthopedists); physicians who specialize in neurological disorders (neurologists); physicians who[rarediseases.org]
Short Stature
  • Patients typically present absence/hypoplasia of digits, radii and/or ulnae, short stature and mild external ear malformation, as well as kidney agenesis or ectopia. There have been no further descriptions in the literature since 1983.[orpha.net]
  • stature Lumbar malsegmentation, short stature and facial anomalies M MadokoroOhdoSonoda syndrome MardenWalker Syndrome Mastocytosis short stature hearing loss Mastocytosis, cutaneous, with short stature, conductive hearing loss and microtia McDonough[wikidoc.org]
  • stature 60 33 hallmark (90%) Very frequent (99-80%) HP:0003510 16 abnormality of the elbow 60 33 hallmark (90%) Very frequent (99-80%) HP:0009811 17 convex nasal ridge 60 33 hallmark (90%) Very frequent (99-80%) HP:0000444 18 hypoplasia of the radius[malacards.org]
  • Two patients had ureterovesicular junction obstruction, with one studied as part of a routine evaluation for short stature. Four of these five patients required surgery.[pediatrics.aappublications.org]
Hypertension
  • Articles in this issue include: Secondary hypertension; Update on ACE/ARB/DRI; Workup of proteinuria; Diagnosis and evaluation of renal cysts; NSAIDs, COX2’s and the kidney; The PCP/nephrologist partnership in advancing CKD; Nosocomial AKI; Geriatric[books.google.com]
  • […] patients that were refractory to treatment with 5 or more anti-hypertensive drugs at high doses.[revistanefrologia.com]
  • During the European Society of Hypertension meeting this summer, Dr.[dicardiology.com]
  • Hypertension. PRES is commonly seen in the setting of hypertension. The upper limits of autoregulation are not typically reached, but moderate-to-severe hypertension is seen in approximately 75% of patients with PRES.[ajnr.org]
  • When unrecognized, irreversible cytotoxic edema may occur. 2 We report a patient with new onset of seizures with accelerated hypertension. MRI showed characteristic abnormalities of PRES.[aafp.org]
Skeletal Dysplasia
  • dysplasia mental retardation Sketetal dysplasia coarse facies mental retardation Spastic diplegia, infantile type Spinal muscular atrophy, type I, with congenital bone fractures Spondylohypoplasia arthrogryposis popliteal pterygium Spondylohypoplasia[wikidoc.org]
  • dysplasia Sebaceous gland hyperplasia, familial presenile Pentosuria Xanthinuria type 2 Renal hypomagnesemia-6 Deafness, autosomal dominant nonsyndromic sensorineural 3 Hypertrichosis lanuginosa congenita Gestational trophoblastic tumor Multiple endocrine[checkrare.com]
Foot Deformity
  • […] telangiectatica congenita Chondrocalcinosis 2 Plagiocephaly and X-linked mental retardation Paroxysmal nocturnal hemoglobinuria Osteopetrosis autosomal recessive 3 Complement component 2 deficiency Loeys-Dietz syndrome type 4 CHARGE syndrome Hand and foot[checkrare.com]
Hearing Impairment
  • Approximately 25 to 50 percent of individuals with KFS also have hearing impairment.[rarediseases.org]
  • His mother had a hearing impairment from an early age, and a female cousin had a left hypoplastic optic disc, but did not have the Duane anomaly. Figure 4 (A) Left hand of patient 4. (B) Right hand of patient 4.[jmg.bmj.com]
Hematuria
  • […] issue include: Secondary hypertension; Update on ACE/ARB/DRI; Workup of proteinuria; Diagnosis and evaluation of renal cysts; NSAIDs, COX2’s and the kidney; The PCP/nephrologist partnership in advancing CKD; Nosocomial AKI; Geriatric patient with CKD; Hematuria[books.google.com]
  • Loin pain hematuria syndrome ( LPHS ) is the combination of debilitating unilateral or bilateral flank pain and microscopic or macroscopic amounts of blood in the urine that is otherwise unexplained. [1] Loin pain-hematuria syndrome (LPHS) is a poorly[en.wikipedia.org]
  • The most common clinical manifestation of the nutcracker syndrome is intermittent macroscopic hematuria 3 . Repeated episodes of gross hematuria and left flank pain should be present. Hematuria should be from the left ureteric orifice only 4 .[radiopaedia.org]
  • Loin Pain Hematuria Syndrome What Is Loin Pain Hematuria Syndrome? Loin pain hematuria syndrome (LPHS) is a condition that causes severe, long-lasting pain around your flanks. Your flanks include your: back, sides, and the upper part of your belly.[healthcare.utah.edu]
  • Causes of Pulmonary-Renal Syndrome Symptoms and Signs Symptoms and signs typically include Dyspnea Cough Fever Hemoptysis Peripheral edema Hematuria Patients may also have other signs of glomerulonephritis.[msdmanuals.com]
Seizure
  • Children with Dravet syndrome can develop many different seizure types: Myoclonic seizures Tonic-clonic seizures Absence seizures Atypical absence seizures Atonic seizures Focal aware or impaired awareness seizures (previously called partial seizures)[epilepsy.com]
  • Seizures, hypertension, and posterior leukoencephalopathy. In: Delanty N, ed. Seizures: medical causes and management. Totowa, N.J.: Human Press, 2002:251–60. ... 2. Lamy C, Oppenheim C, Meder JF, Mas JL.[aafp.org]
  • Another important association is epilepsy/seizures. The link with a PL has been recognised for some time [11].[remedypublications.com]
  • Other signs and symptoms of hemolytic uremic syndrome may include bruising and seizures.[niddk.nih.gov]
  • Tuberous sclerosis : Tuberous sclerosis is a disease that causes seizures and mental retardation, as well as the formation of tumors in many different organs.[my.clevelandclinic.org]

Workup

  • […] patient with CKD; Hematuria workup; The kidney in obesity; and Renal transplant in the primary care setting.[books.google.com]
  • See Workup for more detail.[emedicine.medscape.com]
  • Investigative workup revealed normochromic macrocytic anemia with mild anisocytosis (Hb 4.8 g/dl) [Table 1] . Liver function, kidney function, urinalysis, chest roetgenography, and electrocardiography were normal.[ijem.in]
  • Necessary workup includes a midstream clean catch or catheterized urine specimen for urinalysis and microscopy with urine culture as indicated, CBC to assess for leukocytosis, and creatinine to assess for any rise above the patient’s baseline.[auanet.org]
Staphylococcus Aureus
  • In immunocompetent patients, diffuse alveolar haemor-rhage is mainly produced by Leptospirosis and Malaria parasites, influenza A (H1N1) virus and Staphylococcus aureus (1,2).[pneumologia.eu]
Streptococcus Pneumoniae

Treatment

  • The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.[orpha.net]
  • treatment in our hospital— Shijiazhuang Kidney Disease Hospital.[kidney-symptom.com]
  • Nutcracker Syndrome Treatment Nutcracker syndrome treatments can vary based on your age, symptoms, and the severity of your condition.[upmc.com]
  • Although robotic-assisted laparoscopic partial nephrectomy has been implemented as an elective treatment for benign and malignant lesions of the kidney, it has rarely been used in urgent situations as primary treatment.[hindawi.com]

Prognosis

  • Prognosis Factors associated with a good prognosis include younger age at diagnosis, absence or a small amount of ascites, and a low serum creatinine level.[patient.info]
  • "Polycystic kidney disease: inheritance, pathophysiology, prognosis, an IJNRD". International Journal of Nephrology and Renovascular Disease : 69. doi : 10.2147/IJNRD.S6939. Retrieved 2015-08-01. External links [ edit ][en.wikipedia.org]
  • Treatment and prognosis If the hemorrhage is self-limiting and the patient is responsive to fluid resuscitation, the patient can be managed conservatively. Angiographic selective embolization may be pursued in haemodynamically stable patients.[radiopaedia.org]

Etiology

  • If the etiology is uncertain, the pulmonary biopsy is indicated. The treatment is based on etiology, especially in case of drug etiology, and can be conservative, supportive, with discontinuation of the incriminated drug.[pneumologia.eu]
  • Wunderlich syndrome is clinically characterized by Lenk's triad : acute flank pain flank mass hypovolemic shock Etiology neoplastic neoplasms are the most common cause among benign neoplasms, angiomyolipoma is the commonest, while among malignancies,[radiopaedia.org]
  • Budd-Chiari syndrome: etiology, pathogenesis and diagnosis. World J Gastroenterol. 2007 May 21. 13(19):2693-6. [Medline]. Darwish Murad S, Plessier A, Hernandez-Guerra M, et al. Etiology, management, and outcome of the Budd-Chiari syndrome.[emedicine.medscape.com]
  • For SVCS caused by an infectious etiology, antibiotics should be the first line of treatment.[springerplus.springeropen.com]
  • Furthermore, Wunderlich’s syndrome is an emergency medical condition that refers to a spontaneous nontraumatic bleeding confined to the perinephric space caused by various etiologies but most frequently by renal angiomyolipoma [ 4 ].[hindawi.com]

Epidemiology

  • Epidemiology The global prevalence of BCS is unknown but is estimated at 1 in 100,000. [ 3 ] The epidemiology and presentation tend to vary in different parts of the world.[patient.info]
  • Introduction Clinical definition calculi (stones) affecting the kidneys; however, these stones can also affect the ureter Epidemiology prevalence 12% in men and 7% in women risk factors hyperparathyroidism hypocitraturia sarcoidosis hyperoxaluria renal[medbullets.com]
  • EPIDEMIOLOGY, MORBIDITY & MORTALITY Perioperative acute renal failure (PARF) faced on the course of surgery or ICU stay is often considered as a complication associated with increased morbidity and mortality.[apicareonline.com]
  • The epidemiology of Budd-Chiari syndrome in France. Dig Liver Dis. 2018 Sep. 50 (9):931-7. [Medline]. Su L, Hu L, Liang P, Wu Y, Sun Q, Gao J.[emedicine.medscape.com]
Sex distribution
Age distribution

Pathophysiology

  • "Polycystic kidney disease: inheritance, pathophysiology, prognosis, an IJNRD". International Journal of Nephrology and Renovascular Disease : 69. doi : 10.2147/IJNRD.S6939. Retrieved 2015-08-01. External links [ edit ][en.wikipedia.org]
  • Based on these arguments, we believe that dissecting one of the multiple bidirectional associations in HF and constructing the so-called cardiorenal syndrome is not justified pathophysiologically.[pharmaceuticalintelligence.com]
  • Pathophysiology and management of abdominal compartment syndrome. Am J Crit Care 2003; 12:367—371 Walker J, Criddle LM. Pathophysiology and management of abdominal compartment syndrome.[apicareonline.com]
  • Studies combining DWI and perfusion-weighted imaging may shed additional light on the pathophysiology of PRES. DEVAKINANDA V. PASUPULETI, M.D. MARYANN MIRANDA, M.D. 2370 South Linden Road Flint, MI 48532 REFERENCES show all references 1.[aafp.org]
  • PTU-induced positive p-ANCA vasculitis was first reported in 1993 and the pathophysiology mecha-nism remains uncertain(26,27).[pneumologia.eu]

Prevention

  • GENERAL PREVENTIVE MEASURES General preventive measures include optimizing oxygen and blood supply, decreasing the demands, and minimizing the renal perioperative insult.[apicareonline.com]
  • Hyperadrenergic POTS treatment is targeted towards preventing release of these transmitters or prevention of their action.[myheart.net]
  • These peptides have shown conflicting results in the prevention of ARF. Large multi-centre trials have demonstrated that atrial natriuretic peptide (ANP) does not prevent death or dialysis in critically ill patients with ARF.[ceaccp.oxfordjournals.org]

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