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Wiskott-Aldrich syndrome, renal failure, and osteosclerosis
WISKOTT-ALDRICH SYNDROME, FAILURE, AND OSTEOSCLEROSIS
RENAL
A
32-jear-old white man with :I primaq diagnosis of Wiskott-Aldrich syndrome was seen at the Clinical Center Dental Clinic of the National Institutes of Health for ;I routine oral examination hcforc kidne\, transplantation. Wiskot t-Aldrich s! ndrome i\ ‘I rare X-linked rccessivc syndrome characterized clinically bg the triad of cc/ems. thromhocytopenic purpura, and undue susceptibilit); to infection. i Multiple infections and treatment with aminoglycosides resulted in permanent hearing loss and kidney failure in this patient. necessitating kidney transplantation. Abnormal laboratory values at this visit included cle\tated calcium. phosphorus, creatinine, hlood urea nitrogen; increased white blood cell count: and dr:creased red blood cell count. hemoglobin, and hematocrit. Alkaline phosphatase and albumin were uithin normal limits. The patient received hemodialyai\ three times \zeekl!, Radiographic csamination revcalcd ;I radiopaquc mass in the region of tooth 31. which had the radiographic appearance of retained root tips (f:ip. I ). Thib region was as>mptomatic. Within ;I \ear of the examination. the tooth had received ;I tcmpornr> crown. which LV;IS to be replaced 1%ith ;I permanent crown after kidney transplantation. The patient M;I’, seen at the clinic twice in the previous 3 years with fractured posterior teeth with no remaining crow~l. On questioning, the patient reported visiting his private dentist at his home 6 months before the
Fig.
1. I’eriapical
kott-Aldrich complete
film of 32.year-old patient bith syndrome and kidney failure. 6 months extraction of tooth 3 I,
Wisafter
examination. 10 evaluate pain in toolh 1 I 4 rcpor! was obtained from this dentist documenting that the tooth had been extracted with ;I surgical Hap and s~ik huture. with no complications. C‘oniillunic~!tion with the dentist conlirmed that there \+;I\ no prci,ious toot11 fracture and that the entire tooth \+;I\ rcllii)ved dur~ng surgcr!. In addition. :t presurgical radiograph ~1.14 uhtaincd (Fig ? ! The radiopaclue change in the region ol tooth Z I IZ most likelk osteosclerosis in the extraction qite. Bon\changes 111 patlcnts \qith chronic kidney failure ;.Ire well described and range in appearance from radiolucencics and osleomalacia to radiopacities and osteo, sclerosis.-- Ihi\ case illustrates that rapid osscou~ changes ma! occur in patients with kidna failure. In addition. surglc;ll exploration of this area U;I~ no1 ncccssarl bccausc adequate records wcrc maintained hy all health cart professionals caring for this patienr.
Fig.
2. Pre-extraction periapical tilm of tooth Eugene Koval and Charles Getter.
tesq of Drs. Pa.)
3 I. (Cour-llazleton.
RENAL
A
32-jear-old white man with :I primaq diagnosis of Wiskott-Aldrich syndrome was seen at the Clinical Center Dental Clinic of the National Institutes of Health for ;I routine oral examination hcforc kidne\, transplantation. Wiskot t-Aldrich s! ndrome i\ ‘I rare X-linked rccessivc syndrome characterized clinically bg the triad of cc/ems. thromhocytopenic purpura, and undue susceptibilit); to infection. i Multiple infections and treatment with aminoglycosides resulted in permanent hearing loss and kidney failure in this patient. necessitating kidney transplantation. Abnormal laboratory values at this visit included cle\tated calcium. phosphorus, creatinine, hlood urea nitrogen; increased white blood cell count: and dr:creased red blood cell count. hemoglobin, and hematocrit. Alkaline phosphatase and albumin were uithin normal limits. The patient received hemodialyai\ three times \zeekl!, Radiographic csamination revcalcd ;I radiopaquc mass in the region of tooth 31. which had the radiographic appearance of retained root tips (f:ip. I ). Thib region was as>mptomatic. Within ;I \ear of the examination. the tooth had received ;I tcmpornr> crown. which LV;IS to be replaced 1%ith ;I permanent crown after kidney transplantation. The patient M;I’, seen at the clinic twice in the previous 3 years with fractured posterior teeth with no remaining crow~l. On questioning, the patient reported visiting his private dentist at his home 6 months before the
Fig.
1. I’eriapical
kott-Aldrich complete
film of 32.year-old patient bith syndrome and kidney failure. 6 months extraction of tooth 3 I,
Wisafter
examination. 10 evaluate pain in toolh 1 I 4 rcpor! was obtained from this dentist documenting that the tooth had been extracted with ;I surgical Hap and s~ik huture. with no complications. C‘oniillunic~!tion with the dentist conlirmed that there \+;I\ no prci,ious toot11 fracture and that the entire tooth \+;I\ rcllii)ved dur~ng surgcr!. In addition. :t presurgical radiograph ~1.14 uhtaincd (Fig ? ! The radiopaclue change in the region ol tooth Z I IZ most likelk osteosclerosis in the extraction qite. Bon\changes 111 patlcnts \qith chronic kidney failure ;.Ire well described and range in appearance from radiolucencics and osleomalacia to radiopacities and osteo, sclerosis.-- Ihi\ case illustrates that rapid osscou~ changes ma! occur in patients with kidna failure. In addition. surglc;ll exploration of this area U;I~ no1 ncccssarl bccausc adequate records wcrc maintained hy all health cart professionals caring for this patienr.
Fig.
2. Pre-extraction periapical tilm of tooth Eugene Koval and Charles Getter.
tesq of Drs. Pa.)
3 I. (Cour-llazleton.