Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0022672 (acute tubular necrosis)
 2,175 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The combination of fetal hydrops and sacrococcygeal teratoma (SCT), is considered to be lethal. We report two such babies who survived. Case 1 exhibited oliguric acute renal failure (ARF) immediately after birth, and severe respiratory insufficiency despite maximal ventilatory support and vasodilator infusions. Tumor resection on the 2nd day of life resulted in an immediate improvement in pulmonary function as reflected by the ratio of arterial to alveolar oxygen. Renal function returned in a pattern typical of recovery from acute tubular necrosis. Case 2, less desperately ill, developed nonoliguric ARF, in part due to deliberate fluid restriction during the 7 days that followed birth and preceded surgery. This resolved following liberalization of fluid intake that occurred at the time of tumor removal on the 7th day of life. The baby also had respiratory insufficiency that improved after surgery. Respiratory insufficiency may be a severe and life-threatening complication of SCT and hydrops fetalis. Pulmonary function may improve dramatically by removal of the tumor. Why this improvement occurs is unclear. Improvement of respiratory function may result from the elimination of excess tumor blood volume with an improvement of the ventilation-perfusion ratio. Alternatively, the tumor may be a source of vasoactive substances or extremely desaturated blood that leads to pulmonary hypertension and right-to-left shunting. Uncertainties in postnatal fluid shifts and exaggerated fluid compartment volumes demand close attention to details of renal function.
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PMID:The newborn with hydrops and sacrococcygeal teratoma. 176 33

Kelly is a 7-year-old girl with a complex medical history including asthma, mild spastic diplegia, and seizure disorder that is controlled with carbamazepine. She had a significant receptive and expressive language impairment and milder delays in gross and fine motor skills. Kelly is currently repeating first grade in a self-contained classroom; she receives speech, occupational, and physical therapy. At the 7-year-old well child visit, her mother is worried about Kelly's poor progress in school, and she expresses concern about her daughter's hearing. Her pediatrician observes that Kelly is withdrawn, uses minimal language, and is fearful of the examination.Kelly was born full-term by Cesarean section because of placental abruption. She was in the neonatal intensive care nursery for 2 weeks with metabolic acidosis because of acute tubular necrosis. One day after arriving home, she had a cardiopulmonary arrest followed by emergency open-heart surgery for critical pulmonary hypertension. Her postoperative course was significant for renal failure, extracorporal membrane oxygenation, ventilator dependency, tracheostomy, and gastrostomy. By 3 years of age her medical condition stabilized, and the tracheostomy and gastrostomy tubes were removed.A review of Kelly's previous audiological tests revealed a failed otoacoustic emission test at 5 months. An auditory brain stem response test at 8 months recorded normal hearing in the right ear. At 4 years, behavioral audiometry was attempted but not completed because Kelly cried throughout the session. At 5 years, testing with ear inserts showed normal hearing bilaterally.Because of the concerns raised by Kelly's mother at the pediatric visit, she was referred to audiology for a reevaluation. Testing at this time revealed moderate to profound sensorineural hearing loss in both the ears, which was confirmed on subsequent examinations. Kelly was promptly fitted for hearing aids. Her individual education plan was changed to reflect the diagnosis of hearing impairment, and hearing services were implemented in the classroom. On a recent follow-up visit, Kelly was talkative, engaging, and cheerful.
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PMID:Delayed recognition of profound hearing loss in a 7-year-old girl with a neurological condition. 2041 74

Crescentic glomerulonephritis (GN) without immune reactants or deposits (referred to as pauci-immune) is typically characterized by the presence of anti-neutrophilic cytoplasmic antibodies (ANCA). While ANCA-negative patients might be expected to have a more benign course, they often have poor renal outcomes, especially without treatment with steroids and immune-modulating therapy. Pauci-immune crescentic GN can also co-exist with other autoimmune conditions, including rheumatoid arthritis (RA). Here, we describe an ANCA-negative patient with RA who developed dialysis-requiring acute kidney injury (AKI) with findings consistent with focal pauci-immune crescentic GN (i.e., no IgG or immune complex on kidney biopsy). Coexistent conditions included Klebsiella sepsis attributed to pneumonia, rhabdomyolysis, leukocytoclastic immune-mediated skin vasculitis, and positive ANA. He had spontaneous improvement in renal function without immunosuppressive therapy. This crescentic GN was not associated with poor renal outcome as AKI resolved with supportive care and treatment of his infection. The AKI was likely multifactorial with co-existing acute tubular necrosis in the setting of Kebsiella sepsis and rhabdomyolysis, and the crescentic GN was felt more likely to be related to the infection rather than having a primary role. This case highlights the importance of viewing crescentic GN in the context of the clinical picture, as it may not always lead to the need of aggressive immune suppression and is not a universally poor prognostic kidney finding. However, these cases do warrant close follow-up as our patient had recurrent RA disease manifestations over the next 2 years that eventually led to his death from severe pulmonary hypertension.
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PMID:Significance of Crescentic Glomeruli in Acute Kidney Injury with Rheumatoid Arthritis. 3119 27