|Year : 2014 | Volume
| Issue : 3 | Page : 355-358
Premature exfoliation of the teeth in a hypocalcemic undernourished African girl
Department of Paediatrics, Aminu Kano Teaching Hospital, Bayero University, Kano, Nigeria
|Date of Web Publication||18-Mar-2014|
Department of Paediatrics, Aminu Kano Teaching Hospital, Kano PMB 3452
Source of Support: None, Conflict of Interest: None
Premature exfoliation of the primary dentition is a rare phenomenon which may be associated with local disorders of the teeth and systemic diseases; however its association with hypocalcemia and malnutrition is uncommon. Nutritional rickets occurs mostly in developing countries despite some of these countries being sunny. Therefore, emphasis is now shifting toward the role of hypocalcemia as the predominant cause of nutritional rickets. Rickets is often described as a disease of growing bones; hence, it should ordinarily not be seen in malnourished children. This, however, has been refuted over the years. Therefore, a case of an undernourished hypocalcemic Nigerian child is reported who presented with premature exfoliation of the central lower incisors with delayed eruption of other primary dentition.
Keywords: Delayed dentitions, hypocalcemia, malnutrition, premature exfoliation of the tooth
|How to cite this article:|
Aliyu I. Premature exfoliation of the teeth in a hypocalcemic undernourished African girl. Med J DY Patil Univ 2014;7:355-8
| Introduction|| |
Premature loss of primary dentition is defined as the loss of a deciduous tooth before the expected time of its natural exfoliation.  It is a rare event and may be associated with a local disorder of the gum like infection (periodontitis) and systemic disorders like hypophosphatasia, Chediak-Higashi syndrome and histiocytosis.  However, commoner causes are often premature eruption of a permanent tooth, dental caries, trauma and premature root resorption. 
Furthermore, concomitant malnutrition and rickets, though thought to be an uncommon occurrence in the past has now been refuted based on several studies. , But its association with premature loss of the teeth is uncommon, which makes this case unique. Therefore, a case of a 27-month-old Nigerian girl who presented with premature exfoliation of the lower central incisors, delayed eruption of the other primary dentition, poor growth and hypocalcemic rickets is reported.
| Case Report|| |
A 27-month-old girl presented with stiffening of the body and excessive crying which had been recurrent since the age of 8 months, but the frequency of which had increased in the weeks before presentation and was not associated with loss of consciousness. She was delivered at term and had remained active with appropriate developmental milestones till the 8th month of life when she took ill with subsequent loss of ability to crawl. Similarly, the language skill became delayed, except for the ability to say only mama and baba. She had her first milk tooth eruption (lower central incisor) at the age of 5 months and second lower incisor at the age of 7 months and upper central incisors erupted at the 8 th month. Afterward tooth eruption stopped but suddenly 2 weeks before presentation, she lost a lower central incisor; and about 4 weeks later, the only remaining lower incisor also exfoliated. There was no history of natal or neonatal teeth, no trauma to the tooth and she was not a known case of epilepsy. She was predominantly breastfed till the age of 5 months when complementary feeding was introduced which was not different from that given to her other siblings and was well tolerated. They lived in a four-bedroom apartment with adequate exposure to sunlight. Her father belonged to middle class and she was the fourth in a family of five children in a consanguineous family setting (parents were first cousins). The mother was a 27-year-old woman and no first-degree relative had similar illness. On presentation, she had carpopedal spasms. She was conscious and small for age weighing 6 kg, which was less than −3 z score for age (−5.13). The mid-arm circumference was 9 cm, the occipito-frontal circumference was 46.7 cm and length was 65 cm (−6.42 z score, stunted), which were all deranged. The head was box shaped with alopecia and patent anterior fontanelle measuring 2 × 2 cm [Figure 1]. The wrists were also swollen with protuberant abdomen. She also had generalized hypertonia at presentation (this normalized after giving medication) with reduced muscle bulk and the deep tendon reflexes (knees and ankles) were increased; the liver, spleen and kidneys were not enlarged. The oral examination showed an exfoliated central lower incisor; however, only a single lower incisor and the upper central incisors were present at first contact, but 4 weeks later, the only remaining lower tooth also exfoliated. She had normal palms and sole skin texture.
The patient had calcium gluconate infusion which controlled the spasms and was placed on oral calcium supplement (70 mg/kg/day) which was later increased to 1000 mg/day 8 weeks later. Magnesium, zinc, urinalysis, electrolytes, urea and creatinine, and liver function tests were not remarkable except for an elevated alkaline phosphatase which was 200 U/l (normal 42-110 U/l). Serum calcium corrected for albumin was low [1.2 mmol/l (normal 2.1-2.8 mmol/l)]; this was still low on two separate occasions, while the serum phosphate was normal [4 mmol/l (normal 0.94-1.70 mmol/l)]. Full blood count with peripheral blood film was not remarkable. Serum vitamin D level was adequate [21.5 ng/ml (normal >20 ng/ml)], while 1,25-dihydroxyvitamin D was elevated [50 pg/ml (normal 40-46 pg/ml)]. Thyroid function test was normal and parathyroid hormone assay showed elevated values [70 pg/ml (normal 10-65 pg/ml)]. Abdominal/renal ultrasound scan was also not remarkable. X-ray of the wrists showed widening, though with minimal splaying and fraying of the metaphysis. Based on these findings, the diagnosis of hypocalcemic rickets in a malnourished child with premature exfoliation of the lower central incisors and delayed dentition was made. Administration of initial dose of 1 mcg 1,25-dihydroxyvitamin D 3 showed some improvement; the spasms stopped and the child had attempted crawling, but the swelling on the right wrist increased [Figure 2]. Repeat X-ray of the wrist showed increasin g fraying of the distal end of the ulna and radius [Figure 3]. An X-ray of the face showed presence of unerupted primary dentition [Figure 4]. The dose of 1,25-dihydroxyvitamin D was increased to 3 mcg on the 8th week of treatment. Her serum calcium became normal (2.4 mmol/l). The left and right lateral lower incisors erupted at about the 12th week of therapy [Figure 5] with improvement in her ambulation. She is currently being followed up in the pediatric clinic.
|Figure 3: X-ray of the wrist showing increased soft tissue swelling and metaphyseal splaying and fraying|
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|Figure 4: X-ray of the face showing presence of unerupted primary dentition|
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|Figure 5: Exfoliated lower central incisors and left lateral lower incisor erupting at about the 12th week of therapy|
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| Discussion|| |
There is variable prevalence of vitamin D deficiency in Nigeria. While no single case was documented in a study in Jos, central Nigeria in 1998,  a similar study in 2000 reported 37% of cases due to vitamin D deficiency,  while in Maiduguri mixture of cases of vitamin D deficiency and hypocalcemia as causes of nutritional rickets were documented,  though it is generally agreed that hypocalcemia is the predominant factor in nutritional rickets in Nigeria.
Rickets is a disease of growing bones. Hence a child who is malnourished with arrested growth has less likelihood of developing rickets but a rachitic child may become stunted (rachitic dwarfism), therefore appearing malnourished. Coupled with the risk of infection associated with hypocalcemic rickets, a child with background borderline malnutrition may develop overt protein-energy malnutrition. , Both malnutrition and rickets often cause delayed dentition, which may explain absence of primary dentitions other than the central incisors at the age of 27 months, but its association with premature exfoliation of earlier erupted teeth is a rare and poorly understood phenomenon. Therefore, the dilemma in this case was distinguishing between hypocalcemic rickets and hypophosphatasia. The presence of elevated serum alkaline phosphatase activity and absence of hypercalcemia made hypophosphatasia unlikely, though patients with pseudohypophosphatasia may have a relatively normal serum non-tissue alkaline phosphatase activity.  Furthermore, in rickets, the serum enzyme activity is usually significantly increased, but it was not observed in this case. However, a normal or reduced serum non-tissue alkaline phosphatase activity has been observed in rachitic children with malnutrition.  But this may not completely explain the pattern in this case because the serum zinc and magnesium concentrations were normal, which are also expected to be reduced in malnourished children; therefore, the malnutrition was secondary in this case. Clinical signs of rickets may be subtle when associated with malnutrition. , Hence improved nutrition and ambulation resulted in increased swelling of the wrist with increasing fraying of the distal ends of the ulna and radius.
Nutritional rickets was unlikely in this case because this child was reared just like her other siblings who were doing well. Furthermore, the presence of a normal serum phosphate made it unlikely. Therefore the diagnosis of vitamin D dependent rickets type II (also called hereditary vitamin D resistant rickets) is plausible despite the inability to do genetic analysis. This is further buttressed by the presence of alopecia which has been reported in about 67% of cases, , and the elevated 1,25-dihydroxyvitamin D 3 . Since there is no previous report of premature exfoliation of the teeth in hypocalcaemic rickets unlike in familial (X-linked) hypophosphatemic rickets,  the present case is unique.
| Conclusion|| |
The combination of premature exfoliation of the teeth, malnutrition and rickets is a rare event; however, the presence of alopecia in this child makes vitamin D dependent rickets (type 2) the likely cause and the less elevated alkaline phosphatase observed may be due to the associated secondary malnutrition.
| References|| |
|1.||Cavalcanti AL, Barros de Alencar CR, Medeiros Bezerra PK, Granville-Garcia AF. Prevalence of early loss of primary molars in school children in campina grande, Brazil. Pak Oral Dent J 2008;28:113-6. |
|2.||Sharma G, Whatling R. Case report: Premature exfoliation of primary teeth in a 4-year-old child, a diagnostic dilemma. Eur Arch Paediatr Dent 2011;12:312-7. |
|3.||Borum MK, Andreasen JO. Sequelae of trauma to primary maxillary incisors. I. Complications in the primary dentition. Endod Dent Traumatol 1998;14:31-44. |
|4.||Pfitzner MA, Thacher TD, Pettifor JM, Zoakah AI, Lawson JO, Isichei CO, et al. Absence of vitamin D deficiency in young Nigerian children. J Pediatr 1998;133:740-4. |
|5.||Thacher TD, Fischer PR, Pettifor JM, Lawson JO, Isichei CO, Chan GM. Case-control study of factors associated with nutritional rickets in Nigerian children. J Pediatr 2000;137:367-73. |
|6.||Akpede GO, Solomon EA, Jalo I, Addy EO, Banwo AI, Omotara BA. Nutritional rickets in young Nigerian children in the Sahel savanna. East Afr Med J 2001;78:568-75. |
|7.||Aboul-Dahab YW, Zaki K. Studies of rickets and malnutrition in the undernourished child. Am J Clin Nutr 1963;13:98-105. |
|8.||Walter EA, Scariano JK, Easington CR, Polaco AM, Hollis BW, Dasgupta A, et al. Rickets and protein malnutrition in northern Nigeria. J Trop Pediatr 1997;43:98-102. |
|9.||Plotkin H. Hypophosphatasia. Available from: http://www.emedcine.com. [Last accessed on 2013 Feb 17]. |
|10.||Kumari R, Rao YN, Talukdar B, Agarwal S, Puri RK. Serum enzyme abnormalities in protein-energy malnutrition. Indian Pediatr 1993;30:469-73. |
|11.||Sakai Y, Kishimoto J, Demay MB. Metabolic and cellular analysis of alopecia in vitamin D receptor knockout mice. J Clin Invest 2001;107:961-6. |
|12.||Chen CH, Sakai Y, Demay MB. Targeting expression of the human vitamin D receptor to the keratinocytes of vitamin D receptor null mice prevents alopecia. Endocrinology 2001;142:5386-9. |
|13.||Sattur A, Naikmasur VG, Shrivastava R, Babshet M. familial hyposhoshatemic rickets. J Indian Soc Pedod Prev Dent 2010;28:302-6. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]