[Evaluation of peripheral neuropathy in patients with type 1 diabetes mellitus by bedside scoring procedure]

Foot ulceration and lower limb amputation are still common complications of Diabetes mellitus [DM]. Diabetic foot problems account for more hospitalization days than any other diabetic problem. The main etiologic factors are diabetic neuropathy and peripheral vascular disease. An easy, simple to use and low cost method for screening of diabetic peripheral neuropathy will be very useful. From May 2003 to May 2004, all diabetic patients [type 1] referred to endocrine clinic of Namazi Hospital were evaluated for diabetic peripheral neuropathy [DPN], using the bedside scoring procedure. A score of 3-5 indicated mild, 6-8 moderate, and 9- 10 severe DPN. The perception of vibration, temperature, pinprick and ankle reflex was examined. Tuning fork [128 Hz], neuropen, cold and warm water and reflex hammer were used for evaluation. In this study we evaluated 80 patients [34 male and 46 female] with type 1 DM, with the age range of 6-31 years [Mean +/- SD 18.16 +/- 5.22 yr]. The overall prevalence of DPN was 13.75%: mild in 7.5%, moderate in 5% and severe in 1.25% of the patients. The most common physical finding was the absence of ankle reflex. Patients were divided into 2 groups based on the presence of DPN. The prevalence of DPN was significantly associated with age, duration of DM and BMI [P< 0.05]. There was no significant correlation with sex, HbAlc, puberty, parental consanguinity and family history of DM. Bedside scoring procedure is a simple, low cost and easy method for screening of DPN in patients with type 1 DM in outpatient clinic. Examination of ankle reflex is very important. It is concluded that the diabetic patients need better follow-up and more education

Frequency of hypothyroidism in Fanconi anemia

Fanconi anemia [FA] is a rare, autosomal recessive [AR] and multifactorial disorder. A high prevalence of FA observed in Iran is perhaps due to the high rate of consanguineous marriages. This study investigates the extent of short stature in patients with FA, the frequency of hypothyroidism in FA and the correlation between height and hypothyroidism. Eighteen patients with FA referred to the Pediatrics Clinic of Nemazee Hospital were selected based on specific congenital and malformational symptoms and bone marrow results. These patients were evaluated for weight, height, bone age, clinical goiter and thyroid function tests. Eleven out of 18 patients [61%] had overt or compensated hypothyroidism. Short stature was found in 89% of patients with a mean height more than two standard deviation [SD] below the mean height for that age. There was no significant correlation between short stature and overt or compensated hypothyroidism. The mean height standard deviation score [SDS] of patients with normal thyroid function was also more than two SD deviation below the normal mean, revealing that short stature is an inherent feature of FA. In seven cases with goiter, only three had hypothyroidism. The mean +/- SD weight SDS was -1.03 +/- 0.99. The mean +/- SD bone age was 12 +/- 4.5 yr. According to the results of this study, our patients had a considerable prevalence of hypothyroidism and short stature was a common feature of FA. It is recommended that thyroid function test is necessary in these patients

Prevalence of endocrine complications in beta-thalassaemia major in the Islamic Republic of Iran

To identify the prevalence of endocrine dysfunction in Iranians with beta-thalassaemia, we assessed thyroid, parathyroid, pancreatic and adrenal function in 150 beta-thalassaemic patients aged 10-22 years at the Paediatrics Unit, Shiraz University of Medical Sciences. Primary hypothyroidism was found in 6.0% of patients [mean age: 14.6 +/- 1.9 years], hypoparathyroidism in 7.3% [14.5 +/- 3.2 years], type 1 diabetes mellitus in 7.3% [13.9 +/- 2.8 years] and adrenal insufficiency in 1 patient. The relatively high frequency of endocrine dysfunction found in our study may be a result of poor disease control and management in early life when irreversible tissue damage occurs due to iron overload. These findings reinforce the importance of regular follow-up of patients with beta-thalassaemia major for early detection and management of associated complications

Linear growth deficiency in B-thalassemia patients: Is it growth hormone dependent?

beta-Thalassemia major is a serious medical problem. Growth retardation is commonly seen in poly-transfused beta- thalassemia patients. The exact mechanism of short stature in children with thalassemia major is not well understood, however, it is believed to be multi-factorial. To study the growth state and its relationship to growth hormone [GH] deficiency in beta-thalassemia patients. The extent of growth and growth hormone deficiency were evaluated in 150 patients aged 10-22 years [84 males, 66 females] residing in Shiraz. The age, sex, height, weight and serum ferritin levels were recorded using a questionnaire. Growth hormone secretion was determined with L-Dopa provocative test in 138 beta- thalassemia patients. Short stature was present in 64% of patients [63.6% of girls, 69% of boys]. Growth hormone deficiency was present in 57.5% of 138 patients, [53.5% of boys, 46.5% of girls]. Short stature was found in 83.5% of GH deficient and 74.6% of GH sufficient [p=0.22] subjects. There was no difference between the height of GH deficient and GH sufficient patients [p=0.297]. Age at the start of chelating therapy, height deficiency and serum ferritin levels did not differ in GH deficient compared to GH sufficient patients. This study suggests that growth retardation and GH deficiency are common in thalassemic patients and that height deficiency may not be related to GH reserve with the current testing methods for measurement of GH reserve

Prevalence of phototherapy-induced hypocalcemia

Hyperbilirubinemia is the single most common abnormal physical finding in the first week of life and is commonly managed by phototherapy with its inherent complications. A lesser known complication of the phototherapy is hypocalcemia. To study the prevalence of phototherapy-induced hypocalcemia and to compare it between premature and full-term babies. This study was performed on 153 jaundiced neonates [62 premature, 91 full-term] that were managed with phototherapy. These neonates were completely normal on physical examination. Serum calcium was checked on arrival, 48 hours after starting phototherapy and 24 hours after discontinuation of the treatment. The first samples were considered as controls. A comparative study was made between these groups to determine the prevalence of hypocalcemia. Twenty- two neonates [14.4%] developed hypocalcemia. There were significant differences between the prevalence of hypocalcemia in premature [22.6%] and full-term neonates [8.7%] [p= 0.018]. None of the hypocalcemic neonates was symptomatic clinically. Serum levels of calcium returned to normal 24 hours after discontinuation of phototherapy in almost all hypocalcemic neonates. The study shows that neonates under phototherapy are at high risk of hypocalcemia. This risk is greater in premature neonates