A 13 year- old girl had RICKETS clinically evident sicne she was 10 years of age. She received multiple doses of vitamin D3 without improvement.This patient manifested an unusal form of hypophosphatemic RICKETS with hypercalciuria. It is recommended that urinary calcium excretion be assessed in all patients with hypophosphatemic RICKETS before the initiation of any therapy

Background: Hypophosphatemic RICKETS is an uncommon metabolic bone disorder which affects all ages and both sex. It is characterized by low concenteration of serum levels, impairement of mineralization of bone matrix and teeth with variable etiology. Dental problems have not been described well in previous studies.Methods: All hypophosphatemic RICKETS patients who came to referral clinic during 2008-2010 enrolled in this study. All patients had low phosphorous leveland high ALP & normal PTH & 25-hydroxy vit D and normal or low serum calcium. After diagnosis by a all patients were examined by dentist for enamel hypoplasia, taurodontism, dental abcesses, dental caries and dentition delay.Findings: Ninteen patients were enrolled in this study. The average age of patients was 10 years (range 3-17).79% patients had regular follow up after diagnosis of background disease. Dental caries and delay in dentition were most prevalent each one (47.7%) followed by enamel hypoplasiahn in 42.1% patients. Other problems were taurdontism in 15.8% patients, dental abcess and gingivitis in 10.9%.Conclusion: Hypophosphatemic RICKETS is a disease with different clinical features that one of them is dentalblem. Dental caries is the most common problem.

RICKETS is defined as a defective mineralization of growing bone matrix. Patients present with seizures, limbs deformity and bone pain as a most common clinical presentation of RICKETS. When we face the children with these presentation, evaluation for RICKETS is necessary.Major causes of RICKETS are vitamin D deficiency, impaired vitamin D metabolism or function, phosphate deficiency or phosphate homeostasis disorder. Infants and children with RICKETS typically present with normal to low-normal serum calcium, low phosphorus and elevated alkaline phosphatase levels. RICKETS can be classified based on serum PTH level to two hypocalcemic and hypophosphatemic groups. In the case of secondary hperparathyroidsm, one should suspect hypocalcemic RICKETS including vitamin D deficiency RICKETS, vitamin D deficiency RICKETS type I, vitamin D deficiency RICKETS type II which coincides with low 25OH D3 level, low 1, 25 (OH) 2 D3 and high 1, 25 (OH) 2 D3 level respectively. In the case of normal or low serum PTH level, one should suspect hypophosphatemic RICKETS including Xlinked hypophosphatemia, hypophosphatemia due to renal tubular acidosis, Fanconi syndrome, and …. Whenever a patient is refered with RICKETS, taking into account conditions in our country in addition to nutritional RICKETS, other cases like vitamin D deficiency RICKETS type I, vitamin D deficiency RICKETS type II, X-linked hypophosphatemia, hypophosphatemia due to renal tubular acidosis, Fanconi syndrome and … should be considered.

Background: To determine daily calcium, Vitamin D intake and serum biochemical findings of RICKETS in adolescent girls.Methods: Atotal of 414 healthy adolescent student girls aged 11-15 years were evaluated from various areas of Tehran, Iran with different socioeconomic status. A randomized, cross-sectional, prospective and descriptive study was undertaken for calculation of daily calcium, phosphorus intake and vitamin D acquirement by sunlight exposure by seven day recall record questionnaire. Serum 25-hydroxyvitamin D, parathyroid hormone, calcium, phosphorus and alkaline-phosphatase levels were measured. The serum abnormal biochemical findings of girls were divided as follows: normal or low calcium with raised alkaline phosphatase, group I; normal or low calcium with raised alkaline phosphatase, normal or raised parathyroid hormone, low 25- hydroxyvitamin D, group II and low phosphorus and 25-hydroxyvitamin D with raised parathyroid hormone, group III.Results: A total of 44 (10.62%) girls of 414 had abnormal biochemical findings, of these 29 (7%) were in group I, 9 (2.17%) in group 11, and 6 (1.45%) in group III.The mean daily calcium intake and vitamin D acquirement by sunlight exposure and dietary intake were 360.85±350.50 mg and 119.2±52.91 Urespectively. All girls had inadequate dietary calcium and vitamin D intake. All had less than 40 minutes sun exposure per day.Conclusion: This survey demonstrated that abnormal biochemical findings of RICKETS can occur even in sunny climates and are is caused by two factors, inadequate calcium intake as the major factor and vitamin D deficiency as a minor factor.

RICKETS and osteomalacia are characterized by impaired mineralization of bony matrix. In some Asians countries they are common disorders and they may represent a health problem. So we decided to study clinical, biochemical and radiologic features of these disorders in our patients.Methods; In a prospective study during 10 years (1989-1998) we studied all cases of osteomalacia and RICKETS in the rheumatology center of Imam Reza Hospital. Diagnosis was based on the clinical, laboratory and radiographic findings. All patients were treated with Vit D and calcium.Results: We had 797 patients (795 female, 2 male). Their age was between 8-74 years. Most cases were in a second decade. Clinical findings were: bone pain 96.4%, (most common in knee, back and pelvis), muscle weakness 81%, abnormal gait 43%, and bone deformity 19.6% (often genuvalgus and genuvarus). Laboratory findings were: osteopenia 63%, epiphyseal growth plate alterations 74.4%, ground glass appearance 26.8% and looser's zones 26.5%. The response to treatment with Vit. D and calcium was significant. Only four patients were Vit. D resistant.Conclusion: RICKETS and osteomalacia are common disorders in our region. Females especially in growing age are often involved. The most common cause is Vit. D deficiency probably due to inadequate sun exposure. For prevention we suggest that women should be educated for taking adequate sun exposure and Vit. D should be added to dairy products.

Background: RICKETS is a failure in mineralization of growing bone or osteoid tissues. The characteristic early changes may be seen roentgen graphically at the ends of long bones. Clinical manifestations appear late in the course of the disease. Studies have suggested an association between RICKETS and pneumonia. Increased susceptibility to infection in RICKETS may be in part due to impaired immune system, phagocytosis and skeletal changes in the chest cage. The aim of this study was to evaluate the prevalence of RICKETS in children under 5 years of age who had pneumonia. Materials and Methods: Eighty inpatient children younger than 5 years of age with pneumonia (Group I) were screened for evidence of RICKETS clinically, radiologically and biochemically. Simultaneously, 80 age-matched children without pneumoma were selected randomly (Group II) and underwent the same investigations. Results: RICKETS was present in 12.5 percent of cases in Group I and 1.25 percent of cases in Group H. There was a statistically significant difference between the two groups (p<0.02). A high rate of RICKETS was seen in children that had not consumed vitamin D. Conclusion: RICKETS may be an important predisposing factor for pneumonia in children under 5 years of age. Efforts to prevent vitamin D deficiency may result in reduction of pneumonia in these children.

Although the premature tooth exfoliation (PTE) can be associated with some systemic diseases such as changes in immune system or connective tissue disorders, we didn’t find any report on this disorder and hypophosphatemic RICKETS. In bone metabolic disorders, there are a few reports on hypophosphatasia and PTE [1-3]. Although, some factors such as severe oral infection can predispose PTE in HR patients, we recommend a study on prevalence of PTE and its related factors in HR patients in future.The inflammation of the gingiva was evaluated by Gingival Index of Loe and Silness index [4]. It was missed to mention in our article. As we mentioned, dental examination was performed by the same dentist in all patients [5].We agree with Ramezani about the prevalence of taurodontism as a common dental problem with wide range of prevalence in normal population (up to 8% [6]), but as we reported in our paper the prevalence of this disorder was 15% that is very more common than in normal population [5]. It was reported in 0.3% of normal children [7].Finally, for diagnosis of taurodontism, as mentioned in our report, orthopantomogram was performed in all taurodontism suspicious patients [5].

A lack of adequate mineralization of growing bones results in RICKETS. RICKETS may result from the deficiency of either calcium or phosphorus. The term vitamin D-resistant or refractory RICKETS excludes patients in whom RICKETS rapidly responds to moderate amount of vitamin D. Hypophosphatemic RICKETS (HPR) is characterized by renal phosphate wasting, hypophosphatemia and defective bone mineralization. Familial HPR is the most common form of refractory RICKETS. In recent years, though the overall incidence of RICKETS registered has a declined trend; the incidence of non-nutritional RICKETS has surpassed nutritional RICKETS. In this report three cases of vitamin D resistant RICKETS with advanced skeletal changes that were left undiagnosed for many years are presented. The first case was a 10.5 year-old girl, admitted owing to respiratory failure secondary to severe chest deformity. The second and third cases were 8 and 12 year-old girls with HPR who had been referred with advanced rachitic changes including severe bowlegs unresponsive to multiple doses of vitamin D. The final diagnosis was made on the clinical basis, X-ray and laboratory findings including renal phosphate wasting. These cases were confirmed by a dramatic clinical and biochemical response to therapeutic intervention which included oral phosphate, active form of vitamin D and polycitrate to correct metabolic acidosis and hypokalemia in the first case. Presently, all the patients returned to school with minimal problems. The finding of this study suggests that HPR should be considered in any child with rachitic changes particularly in those who do not respond to usual doses of vitamin D and in any older child with severe skeletal deformities.

Rud syndrome consists of mental retardation, epilepsy, ictihyosis, dentition and skeleton defect. We report a case of Rud syndrome that have clinical, radiological and biochemical characteristics of RICKETS. Alteration in epidermal cholestrol metabolism, limited sun exposure or associated defects may explain this condition. All such patients need life -long RICKETS prophylaxis with vitamin D supplements (400 IV/day, A diatary intak alone can not be relied upon.

New Page 1 RICKETS by definition is a condition in which the bone mineralization is defective. Among the large cause of RICKETS (Vitamin D deficiency, gastrointestinal disorders, acidosis, renal tubular abnormalities...), Wilsons disease is a relatively rare cause. There are few cases of Wilsons disease presenting first as RICKETS in the literature. Here we present an Afghan girl with Wilsons disease presenting with intractable RICKETS.