Dental hypersensitivity due to hypophosphataenrla? H. Hintze, A. Wenzel and F. Krohoffer* Department of Radiology. Ro yal Dental College Aarhus and 'Private Practice. Randers, Denmark
Received 23 May 1989 and in final form 30 November 1989 Hypophosphataemia usually presents in childhood. This report describes , however, a case of hypophosphataemic rickets diagnosed in an adult female who presented complaining of generalized dental hypersensitivity. Radiographic examination revealed a discrete, generalized hypomineralized zone in the outer dentine which corresponded on histological examination to extensive interglobular dentine. Keywords: Dentine hypersensitivity; hypophosphataemia
Case report An apparently healthy 22-year-old female visited her dentist complaining of pain arising from all her teeth with cold drinks or food. Because of this, she always used warm water for tooth-brushing. The painful sensation could be provoked by drying the teeth with an air-syringe. Clinical examination showed no caries, exposed root surfaces or other dental pathology. Bitewing radiographs revealed approximal caries extending into dentine on the distal surface of the upper left second premolar. In addition, a demarcated radiolucent zone was seen at the dentino-enamel junction of all the teeth on the radiograph (Figure 1). Subsequently a full mouth periapical survey was performed to determine the full extent of the radiolucent zone . These radiographs revealed that the zone was visible within the outermost coronal and radicular dentine in all teeth. The enamel and dentine seemed otherwise to be of normal radiopacity. The pulp horns were of normal size and length. The left upper third molar was found to be in vertical impaction and the left lower third molar mesioangular. The two third molars were removed surgically due to
Figure ] Right bitewing radiograph revealing a well-demarcated radiolucent zone in the outer dentine of all teeth (arrows)
© 1990 Butterworths for 0250-832 x 90/020081-03
1ADMFR
,
Figure 2 Microradiograph of a third molar showing an extensive band of interglobular dentine (between arrows)
their close relationship to the second molars, and immediately fixed in neutral formalin for further examination. Ground sections were produced in the buccolingual direction. Microradiographic examination showed an extensive band of interglobular dentine (defined as unmineralized or hypo mineralized areas between mineralized globules i , 2) in both the coronal (Figure 2) and radicular regions. The mineralized globules were large and circular in profile with extensive interglobular areas. The band of developmentally disturbed dentine was located 100-150 ILm deep to the enamel or cementum: the pulpal half appeared normally mineralized. The remaining portion of the teeth was fractured and the cut and/or fractured surfaces prepared for scanning electron microscopy. The cut surfaces (Figure 3) revealed that the interglobular areas contained a network of fine fibrils (Figure 4) , whereas they appeared empty on the fractured samples (Figure 5). The dentinal tubules usually contained odontoblast processes, but occasionally, at sites corresponding to the opening of the tubules into the interglobular areas, small circular bodies about 0.5 JLm in diameter were found lining the periphery (Figure 6). The patient was referred to her general medical Dentomaxillofac. Radiol., 1990, Vol. 19, May 81
Tooth hypersensitivity due to hypophosphataemia?: Hintze et al.
FlKUre 3 Scann ing electron micrograph of a cut surface of a th ird molar. Interglobular dentine can be seen between the arrows ( x 27)
Figure 6 Scann ing electron micrograph show ing circular bodies (arrows) at the periphery of the den tinal tubul es ( x 690) Table I Biochem ical tests indicat ing slight hypophosphataemia without eviden ce of a vita min 0 deficiency
Normal range
Serum calcium Serum phosphate Serum magnesium Serum sodium A lkaline phosphatase Serum 25-hydroxyvitamin 0 Serum 1,2S-dihydro xyvitamin 0 U rinary calcium Urinary phosphate Urinary hydroxyproline Urinary protein Urina ry glucose
Figure 4 Scanning electron micrograph showing a fibrillar network (marked by arrows) in the intergl obul ar area ( x 1793)
Figure 5 Scann ing electr on micrograph of the interglobular dentine showing an empty lacuna (arro ws) (x 448)
practitioner for biochemical evaluation . The tests were repeated at an interval of about 4 months and showed normal alkaline phosphatase and serum calcium levels. The serum phosphate levels were, however, low (0.46 mmol l" and 0.55 mmol lr") on both occasions (normal level: 0.8-1.5 mmol l"), suggesting a diagnosis of hypophosphataemia. The patient was therefore referred to an endocrinologist for more thorough investigation. There was no personal or family history of generalized bone disease or growth inhibition : the patient 82 Dentomaxillofac. Radiol. , 1990, Vol. 19, May
2.46 mmol l- ' 0.7 mmoW I 0.TI mmoW 1 l 36 mmol l- ' 1191tmoW ' 115nmoll- 1 346pmoW ' 3.7 mmoll - ' 15mmoW ' 190 1tmol Negat ive Negat ive
2.25- 2.60 mmol l" 0.8-1.5 mmol l" 0.75-1 .20 mmol r" 136-148 mmol r" 8O-250ltmolJ " 38.8-93.3 nmol t'" 24-158 pmoW 1 1.2-3.8 mmol l'" 11-63 mmoW ' 60- 190 Itmol
herself did not report the premature exfoliation of primary teet h or the spontaneous dental abscesses, the classical clinical sympto ms of hypophosphataemia in the primary dentition. The clinical examination showed her to be of normal height and lumbar spine curvature , with no signs of growth inhibition or muscle weakness . Radiographs of the thorax , thoracic and lumbar spine and the right hand were normal. Exten sive biochemical analyses showed no evidence of vitamin D deficiency but , rather, increased serum 25hydroxyvitamin D and 1,25-dihydroxyvitamin D levels, normal serum calcium but a low phosphate level (Table I) . The endocrinologist concluded the patient had a slight, non-sex-linked hypophosphataemic rickets. In view of her minimal symptoms, he advised that there was no indication for phosphate supplements but that the patie nt shou ld return if her condition deteriorated. With regard to her generalized tooth hypersensitivity, it was considered that there was no satisfactory treatment available.
Discussion The biochemical analyses revealed a normal urinary phosphate but a low serum phosphate -and increased levels of serum 25-hydroxyvitamin D and 1,25dihydroxyvitamin D. The level of urinary phosphate reflects the intake of phosphate as opposed to serum which is a reflection of the renal tubular reabsorption -. In the present case the slight hypophosphataemia was most obviously due to the latter. The slight increase in serum 25-hydroxyvitamin D could be caused by
Tooth hypersensitivity due to hypophosphataemia?: Hintze et al. an increased dietary intake. The level of 1,25-dihydroxyvitamin D, on the other hand, is inversely related to the level of serum phosphate, i.e. the lower the phosphate, the higher the 1,25-dihydroxyvitamin D. The dental features of hypophosphataemia, summarized from previous reports, are elongated pulp horns, multiple periapical infections, premature loss of primary teeth, and widespread areas of globular dentines-o. Very little has been reported regarding the relationship between the presence of interglobular dentine and hypophosphataemia. Nikiforuk and Frasers studied 56 children with three well-defined disturbances of calcium-phosphate homeostasis - vitamin D-dependent rickets (VDDR), X-linked hypophosphataemia (XLH) and hypoparathyroidism. In 20 of 25 normocalcaemic and very hypophosphataemic children with XLH, teeth were available for histological examination: all contained extensive interglobular dentine. Teeth were also available in seven of 10 children with VDDR who were hypocalcaemic and, due to secondary hyperparathyroidism, moderately hypophosphataemic; again, interglobular dentine was present in a mild to moderate degree. In those 15 of the 21 hypocalcaemic and hyperphosphataemic children with hypoparathyroidism studied, the histology revealed severe enamel hypoplasia but none had interglobular dentine. These observations indicate that hypophosphataemia is a necessary condition for the development of interglobular dentine, i.e. a close causal relationship exists", No previous reports have mentioned dental hypersensitivity as a symptom in connection with hypophosphataemia, but the present case illustrates
that a generalized hypomineralization of the dentine may present clinically in this fashion, and should, in the absence of local factors, be considered a possible cause.
Acknowledgements The authors wish to thank Professor Ole Fejerskov, Institute of Oral Anatomy, Dental Pathology and Operative Dentistry, Royal Dental College Aarhus, Denmark, for the microradiography and scanning electron microscopy.
References 1. Seltzer S, Bender lB. The dental pulp. Philadelphia: Lippincott, 1975. 2. Mjor lA, Fejerskov O. Histology of the human tooth. Copenhagen: Munksgaard, 1979. 3. Bijvoet OLM. Relation of plasma phosphate concentration to renal tubular reabsorption of phosphate. Clin Sci 1969; 37: 23-36. 4. Archard HO, Witkop CJ. Hereditary hypophosphataemia (vitamin D-resistant rickets) presenting primary dental manifestations. Oral Surg Oral Med Oral Patholl966; 22: 184-93. 5. Pliskin ME, Brown AM, Baden EE, Kimball HG. Vitamin D resistant rickets of a young adult patient - a review and case report. J Oral Med 1975; 30: 77-SO. 6. Nikiforuk G, Fraser D. Etiology of enamel hypoplasia and interglobular dentin: the roles of hypocalcemia and hypophosphatemia. Metab Bone Dis Rei Res 1979; 2: 17-23. 7. Wulff H. Rational diagnosis and treatment. An introduction to clinical decision-making. Oxford: Blackwell Scientific, 1981. Address: Hanne Hintze, Department of Radiology, Royal Dental College Aarhus, Vennelyst Boulevard, DK-8000, Aarhus C, Denmark.
Dentomaxillofac. Radiol., 1990, Vol. 19, May 83
Received 23 May 1989 and in final form 30 November 1989 Hypophosphataemia usually presents in childhood. This report describes , however, a case of hypophosphataemic rickets diagnosed in an adult female who presented complaining of generalized dental hypersensitivity. Radiographic examination revealed a discrete, generalized hypomineralized zone in the outer dentine which corresponded on histological examination to extensive interglobular dentine. Keywords: Dentine hypersensitivity; hypophosphataemia
Case report An apparently healthy 22-year-old female visited her dentist complaining of pain arising from all her teeth with cold drinks or food. Because of this, she always used warm water for tooth-brushing. The painful sensation could be provoked by drying the teeth with an air-syringe. Clinical examination showed no caries, exposed root surfaces or other dental pathology. Bitewing radiographs revealed approximal caries extending into dentine on the distal surface of the upper left second premolar. In addition, a demarcated radiolucent zone was seen at the dentino-enamel junction of all the teeth on the radiograph (Figure 1). Subsequently a full mouth periapical survey was performed to determine the full extent of the radiolucent zone . These radiographs revealed that the zone was visible within the outermost coronal and radicular dentine in all teeth. The enamel and dentine seemed otherwise to be of normal radiopacity. The pulp horns were of normal size and length. The left upper third molar was found to be in vertical impaction and the left lower third molar mesioangular. The two third molars were removed surgically due to
Figure ] Right bitewing radiograph revealing a well-demarcated radiolucent zone in the outer dentine of all teeth (arrows)
© 1990 Butterworths for 0250-832 x 90/020081-03
1ADMFR
,
Figure 2 Microradiograph of a third molar showing an extensive band of interglobular dentine (between arrows)
their close relationship to the second molars, and immediately fixed in neutral formalin for further examination. Ground sections were produced in the buccolingual direction. Microradiographic examination showed an extensive band of interglobular dentine (defined as unmineralized or hypo mineralized areas between mineralized globules i , 2) in both the coronal (Figure 2) and radicular regions. The mineralized globules were large and circular in profile with extensive interglobular areas. The band of developmentally disturbed dentine was located 100-150 ILm deep to the enamel or cementum: the pulpal half appeared normally mineralized. The remaining portion of the teeth was fractured and the cut and/or fractured surfaces prepared for scanning electron microscopy. The cut surfaces (Figure 3) revealed that the interglobular areas contained a network of fine fibrils (Figure 4) , whereas they appeared empty on the fractured samples (Figure 5). The dentinal tubules usually contained odontoblast processes, but occasionally, at sites corresponding to the opening of the tubules into the interglobular areas, small circular bodies about 0.5 JLm in diameter were found lining the periphery (Figure 6). The patient was referred to her general medical Dentomaxillofac. Radiol., 1990, Vol. 19, May 81
Tooth hypersensitivity due to hypophosphataemia?: Hintze et al.
FlKUre 3 Scann ing electron micrograph of a cut surface of a th ird molar. Interglobular dentine can be seen between the arrows ( x 27)
Figure 6 Scann ing electron micrograph show ing circular bodies (arrows) at the periphery of the den tinal tubul es ( x 690) Table I Biochem ical tests indicat ing slight hypophosphataemia without eviden ce of a vita min 0 deficiency
Normal range
Serum calcium Serum phosphate Serum magnesium Serum sodium A lkaline phosphatase Serum 25-hydroxyvitamin 0 Serum 1,2S-dihydro xyvitamin 0 U rinary calcium Urinary phosphate Urinary hydroxyproline Urinary protein Urina ry glucose
Figure 4 Scanning electron micrograph showing a fibrillar network (marked by arrows) in the intergl obul ar area ( x 1793)
Figure 5 Scann ing electr on micrograph of the interglobular dentine showing an empty lacuna (arro ws) (x 448)
practitioner for biochemical evaluation . The tests were repeated at an interval of about 4 months and showed normal alkaline phosphatase and serum calcium levels. The serum phosphate levels were, however, low (0.46 mmol l" and 0.55 mmol lr") on both occasions (normal level: 0.8-1.5 mmol l"), suggesting a diagnosis of hypophosphataemia. The patient was therefore referred to an endocrinologist for more thorough investigation. There was no personal or family history of generalized bone disease or growth inhibition : the patient 82 Dentomaxillofac. Radiol. , 1990, Vol. 19, May
2.46 mmol l- ' 0.7 mmoW I 0.TI mmoW 1 l 36 mmol l- ' 1191tmoW ' 115nmoll- 1 346pmoW ' 3.7 mmoll - ' 15mmoW ' 190 1tmol Negat ive Negat ive
2.25- 2.60 mmol l" 0.8-1.5 mmol l" 0.75-1 .20 mmol r" 136-148 mmol r" 8O-250ltmolJ " 38.8-93.3 nmol t'" 24-158 pmoW 1 1.2-3.8 mmol l'" 11-63 mmoW ' 60- 190 Itmol
herself did not report the premature exfoliation of primary teet h or the spontaneous dental abscesses, the classical clinical sympto ms of hypophosphataemia in the primary dentition. The clinical examination showed her to be of normal height and lumbar spine curvature , with no signs of growth inhibition or muscle weakness . Radiographs of the thorax , thoracic and lumbar spine and the right hand were normal. Exten sive biochemical analyses showed no evidence of vitamin D deficiency but , rather, increased serum 25hydroxyvitamin D and 1,25-dihydroxyvitamin D levels, normal serum calcium but a low phosphate level (Table I) . The endocrinologist concluded the patient had a slight, non-sex-linked hypophosphataemic rickets. In view of her minimal symptoms, he advised that there was no indication for phosphate supplements but that the patie nt shou ld return if her condition deteriorated. With regard to her generalized tooth hypersensitivity, it was considered that there was no satisfactory treatment available.
Discussion The biochemical analyses revealed a normal urinary phosphate but a low serum phosphate -and increased levels of serum 25-hydroxyvitamin D and 1,25dihydroxyvitamin D. The level of urinary phosphate reflects the intake of phosphate as opposed to serum which is a reflection of the renal tubular reabsorption -. In the present case the slight hypophosphataemia was most obviously due to the latter. The slight increase in serum 25-hydroxyvitamin D could be caused by
Tooth hypersensitivity due to hypophosphataemia?: Hintze et al. an increased dietary intake. The level of 1,25-dihydroxyvitamin D, on the other hand, is inversely related to the level of serum phosphate, i.e. the lower the phosphate, the higher the 1,25-dihydroxyvitamin D. The dental features of hypophosphataemia, summarized from previous reports, are elongated pulp horns, multiple periapical infections, premature loss of primary teeth, and widespread areas of globular dentines-o. Very little has been reported regarding the relationship between the presence of interglobular dentine and hypophosphataemia. Nikiforuk and Frasers studied 56 children with three well-defined disturbances of calcium-phosphate homeostasis - vitamin D-dependent rickets (VDDR), X-linked hypophosphataemia (XLH) and hypoparathyroidism. In 20 of 25 normocalcaemic and very hypophosphataemic children with XLH, teeth were available for histological examination: all contained extensive interglobular dentine. Teeth were also available in seven of 10 children with VDDR who were hypocalcaemic and, due to secondary hyperparathyroidism, moderately hypophosphataemic; again, interglobular dentine was present in a mild to moderate degree. In those 15 of the 21 hypocalcaemic and hyperphosphataemic children with hypoparathyroidism studied, the histology revealed severe enamel hypoplasia but none had interglobular dentine. These observations indicate that hypophosphataemia is a necessary condition for the development of interglobular dentine, i.e. a close causal relationship exists", No previous reports have mentioned dental hypersensitivity as a symptom in connection with hypophosphataemia, but the present case illustrates
that a generalized hypomineralization of the dentine may present clinically in this fashion, and should, in the absence of local factors, be considered a possible cause.
Acknowledgements The authors wish to thank Professor Ole Fejerskov, Institute of Oral Anatomy, Dental Pathology and Operative Dentistry, Royal Dental College Aarhus, Denmark, for the microradiography and scanning electron microscopy.
References 1. Seltzer S, Bender lB. The dental pulp. Philadelphia: Lippincott, 1975. 2. Mjor lA, Fejerskov O. Histology of the human tooth. Copenhagen: Munksgaard, 1979. 3. Bijvoet OLM. Relation of plasma phosphate concentration to renal tubular reabsorption of phosphate. Clin Sci 1969; 37: 23-36. 4. Archard HO, Witkop CJ. Hereditary hypophosphataemia (vitamin D-resistant rickets) presenting primary dental manifestations. Oral Surg Oral Med Oral Patholl966; 22: 184-93. 5. Pliskin ME, Brown AM, Baden EE, Kimball HG. Vitamin D resistant rickets of a young adult patient - a review and case report. J Oral Med 1975; 30: 77-SO. 6. Nikiforuk G, Fraser D. Etiology of enamel hypoplasia and interglobular dentin: the roles of hypocalcemia and hypophosphatemia. Metab Bone Dis Rei Res 1979; 2: 17-23. 7. Wulff H. Rational diagnosis and treatment. An introduction to clinical decision-making. Oxford: Blackwell Scientific, 1981. Address: Hanne Hintze, Department of Radiology, Royal Dental College Aarhus, Vennelyst Boulevard, DK-8000, Aarhus C, Denmark.
Dentomaxillofac. Radiol., 1990, Vol. 19, May 83
