The association of consumptive hypothyroidism secondary to hepatic hemangioma and severe heart failure in infancy.

Although hemangiomas are the most common vascular tumors of the liver in infancy, data regarding hypothyroidism and heart failure related to hepatic hemangiomas are limited. Here, we present a 15- day -old girl who presented with prolonged jaundice at the age of 15 days. Because her TSH level was found to be 74 µIU/mL, she was initially diagnosed with congenital hypothyroidism and L-Thyroxine replacement therapy was initiated. On follow-up examination performed two months later, it was observed that her TSH level was not suppressed and a mass was noticed in the right upper abdomen on physical examination. Abdominal ultrasonography revealed multiple masses with sizes of about 3-3,5 cm covering the whole liver. When evaluated with clinical and radiological appearance, oral methylprednisolone at a dose of 5 mg/kg/day and propranolol at a dose of 2 mg/kg were initiated with a diagnosis of hepatic hemagioma/hemangioendothelioma. Consumptive hypothyroidism due to hepatic hemangioma and congestive heart failure were considered in the patient who had findings of heart failure. The dose of L-Thyroxine was increased 2-fold. The patient received intensive care treatment for severe heart failure. Because his findings resolved, he was started to be followed up with propranolol, steroid and L-Thyroxine treatment.

Comparison of granisetron plus dexamethasone versus an antiemetic cocktail containing midazolam and diphenhydramine for chemotherapy induced nausea and vomiting in children.

BACKGROUND: Chemotherapy induced nausea and vomiting (CINV) is one of the most disturbing side-effects in children receiving highly emetogenic chemotherapy. We aimed to assess whether the addition of an antiemetic cocktail containing midazolam and diphenhydramine to granisetron plus dexamethasone combination could ameliorate CINV in this study. PATIENTS AND METHODS: A total of 23 children aged between 1 and 16 years to receive cisplatin containing chemotherapy in our clinic were included in this study from April 2007 to April 2008. 76 cycles in 23 patients were randomly assigned to receive either antiemetic regimen 1 or antiemetic regimen 2. Antiemetic regimen 1 containing granisetron 0, 04 mg/kg plus dexamethasone 0, 2 mg/kg were given in 45 chemotherapy cycles. In 31 cycles, an antiemetic cocktail containing midazolam 0, 04 mg/kg, diphenhyramine 2, 5 mg/-kg in addition to granisetron plus dexamethasone was given. Number of vomiting, severity of nausea, the use of rescue therapy and adverse events were assessed between day 1 and day 5. RESULTS: Complete response for the acute phase was observed 38/45 (84, 4%) cycles in regimen 1 as compared with 28/31 (90, 3%) in regimen 2, antiemetic cocktail regimen (P > 0.05). Complete response for delayed emesis after 24 h of the beginning of chemotherapy was observed in 29/45 (64, 4 %) in regimen 1 and 16/31 (51, 6%) in regimen 2. Antiemetic cocktail was not superior to the granisetron plus dexamethasone combination in controlling emesis in acute and delayed phase. Furthermore, patients receiving antiemetic regimen 2 were noted significantly more side effects. CONCLUSION: Our data showed that antiemetic cocktail containing midazolam and diphenhydramine was not better in controlling acute and delayed emesis. A slightly more toxicity with additional drugs was also observed.

A very rare cause of virilization in childhood: ovarian Leydig cell tumor.

Virilization in childhood results from an adrenal and/or ovarian disorder. These children usually present with hyperandrogenism: acne, deepening voice, abnormal hair growth, cliteromegaly. Ovarian Leydig cell tumor is a very rare cause of hyperandrogenism in childhood. They are usually benign and surgical treatment is usually curative if the tumor is limited to the ovaries. We present the youngest case of ovarian Leydig cell tumor reported in the literature who presented with hyperandrogenism that was satisfactorily resolved after resective surgery. Rare causes of virilization should be investigated with special attention paid to tumoral disease in the differential diagnosis.

The effect of growth hormone treatment on bone mineral density in prepubertal girls with Turner syndrome: a multicentre prospective clinical trial.

BACKGROUND: Patients with Turner syndrome (TS) are treated with GH to increase adult height. Although it is well established that GH promotes longitudinal bone growth, the effects of GH treatment on bone density are less clear. OBJECTIVE: To determine how GH treatment affects trabecular bone mineral density (BMD) in girls with TS at prepubertal ages in a prospective multicentre study. PATIENTS AND METHOD: Twenty-two patients with TS in the prepubertal period with a mean age of 9.8 +/- 2.5 (range 3.6-12.8) years were included in the study. All girls with TS underwent measurement of areal BMD using dual-energy X-ray absorptiometry (DXA) to obtain pretreatment anteroposterior (AP) lumbar spine values at L1-L4. Patients received GH (Genotropin) subcutaneously for 1 year at a dose of 0.05 mg/kg/day. Height and weight were measured at 3-monthly intervals. The AP lumbar spine areal BMD was remeasured using the same technique after 1 year of treatment. Lumbar spine BMD Z-scores and volumetric BMD (vBMD) Z-scores were calculated using national standards. RESULTS: The height SDS of our cases showed a significant increase with GH therapy. The pretreatment lumbar spine (L1-L4) BMD Z-score was -1.2 +/- 1.2 SD and the vBMD Z-score was -0.8 +/- 1.6 SD. There were no significant changes in these values after 1 year of GH treatment. Prepubertal TS girls more than 11 years of age had lower vBMD Z-scores (-1.7 +/- 1.7 SD) than the girls aged less than 11 (-0.1 +/- 1.0 SD) (P < 0.05) at the onset of therapy. No significant changes were observed in these values after 1 year of GH therapy. CONCLUSIONS: Osteopaenia becomes apparent in prepubertal TS patients as they reach pubertal age. BMD evaluation may be necessary in these prepubertal TS girls at diagnosis. Short-term GH therapy in these TS patients does not have a significant effect on bone density when measured at a site with a predominance of trabecular bone.

Evaluation of low-dose hCG treatment for cryptorchidism.

The aim of this study was to evaluate the efficiency of low-dose hCG (human chorionic gonadotropin) (500 IU/week for 3 weeks) in the treatment of cryptorchidism and in the assessment of Leydig cell functions. We include 35 male patients who had been diagnosed with cryptorchidism by the pediatric endocrinology specialist in the study. Twenty-one cases (Group I) received 500 IU/week of hCG while 14 patients (Group II) received 1500 IU/m2 three times a week, both for three weeks. The percentage of testis descent was calculated for both groups for the right and left testes. Leydig cell functions were evaluated by the pre- and post-treatment measurement of plasma testosterone level in all cases. A delta testosterone greater than 100 was considered to be a sufficient response. Among our patients, 77% had unilateral and 23% bilateral cryptorchidism. Unilateral cryptorchidism was detected in 80.9% of Group I patients and 71.4% of Group II patients. The pre-treatment percentages for Group I of right- and left-sided cryptorchidism were 81% and 38.1%, respectively, which decreased to 23.8% and 9.5% after treatment. The pre-treatment percentages for Group II of right- and left-sided cryptorchidism were 57.1% and 71.4%, respectively, which decreased to 14.3% and 35.7% after treatment. The success rate of hCG treatment, as defined by the testis descending into the scrotum, was 66.7% for Group I and 57.1% for Group II (p > 0.05). There was no significant difference between the two groups when Leydig cell functions were assessed. In conclusion, it is possible to use low-dose hCG for the treatment of cryptorchidism and the assessment of Leydig cell functions.

Propylthiouracil-induced hypersensitivity syndrome.

Propylthiouracil (PTU) is usually the first choice for the treatment of hyperthyroidism, but it has serious side effects such as hepatitis, cholestatic jaundice, splenomegaly and lupus-like syndrome, in addition to mild and common side effects like granulocytopenia, pruritus, urticaria and maculopapular or papular eruption. Antineutrophil cytoplasmic antibody (ANCA)-positive vasculitis is another serious side effect. A 14-year-old female receiving PTU treatment for hyperthyroidism was referred to our clinic with fever, cough and dyspnea. The PTU dosage was first decreased but pericardial, dermal and joint involvement ascribed to PTU developed later and the drug was discontinued. ANCA-positive vasculitis due to PTU was considered when tests revealed an ANCA-positive state. We suggest that severe multisystemic vasculitis due to PTU should be considered during PTU usage.

LEOPARD syndrome and hypertrophic obstructive cardiomyopathy: a case report.

The LEOPARD syndrome is a rare, autosomal dominant multisystemic disorder characterized by lentiginosis, ocular hypertelorism, abnormal genitalia, growth retardation, sensorineural deafness, and cardiac and electrocardiographic abnormalities. Although it is not cited, hypertrophic cardiomyopathy is often associated with the disease. In this study, we present a nine-year-old boy with LEOPARD syndrome and hypertrophic obstructive cardiomyopathy.

Height improvement by L-thyroxine treatment in subclinical hypothyroidism.

BACKGROUND: Subclinical hypothyroidism(SH) is most commonly an early stage of hypothyroidism. Although the condition may resolve or remain unchanged, within a few years in some patients overt hypothyroidism develops, with low free T4 levels as well as a raised thyroid stimulating hormone (TSH) level. Patients with SH may have subtle hypothyroid symptoms with mild abnormalities of serum lipoproteins and cardiac functions. L-thyroxine in a dosage that maintains serum TSH levels within the normal range is the preferred therapy in these patients. Although short stature is a well-known clinical sign of overt hypo-thyroidism,the effect of SH in growth is not well established. The aim of the present study is to show the effect of treatment on height in these patients. METHODS: In the present study, 2067 patients who were admitted to the Endocrinology Department of SSK Ankara Children's Hospital, Ankara, Turkey, with the complaint of short stature were evaluated and 39 were diagnosed with SH by thyrotropin releasing hormone stimulation test. The anthropometric data of the patients who were subdivided into two groups (prepubertal and pubertal) were analyzed before and after 6-12 months of L-thyroxine treatment. Growth velocity (GV) and GV standard deviation score(GVSDS) of the groups before and after the treatment were statistically analyzed. RESULTS: Both groups showed significant increases in GV and GVSDS by L-thyroxine treatment. CONCLUSION: Patients with short stature have to be evaluated for SH in addition to other potential causes. L-thyroxine treatment in these patients provides significant improvement in height.