A rare case of bleeding disorder: Glanzmann's thrombasthenia.

BACKGROUND: Glanzmann's thrombasthenia (GT) is a rare bleeding disorder, which is characterized by a lack of platelet aggregation. It is characterized by qualitative or quantitative abnormalities of the platelet membrane glycoprotein IIb/IIIa. Physiologically, this platelet receptor normally binds several adhesive plasma proteins, and this facilitates attachment and aggregation of platelets to ensure thrombus formation at sites of vascular injury. The lack of resultant platelet aggregation in GT leads to mucocutaneous bleeding whose manifestation may be clinically variable, ranging from easy bruising to severe and potentially life-threatening hemorrhages. OBJECTIVE: To highlight this rare but potentially life-threating disorder, GT. CASE REPORT: We report a case of GT that was first detected because of the multiple episodes of gum bleeding. The patient was an 18-year-old girl who presented with a history of repeated episodes of gum bleeding since childhood. Till the first visit to our hospital, she had not been diagnosed with GT despite a history of bleeding tendency, notably purpura in areas of easy bruising, gum bleeding, and prolonged bleeding time after abrasions and insect stings. GT was diagnosed on the basis of prolonged bleeding time, lack of platelet aggregation with adenosine di phosphate, epinephrine and collagen. CONCLUSION: GT should always be considered as differential diagnosis while evaluating any case of bleeding disorder.

Functional outcome of diaphyseal fractures of femur managed by closed intramedullary interlocking nailing in adults.

OBJECTIVE: To study the effectiveness, advantages, disadvantages and failure rates of closed intramedullary interlocking nailing of daiphyseal fractures of the femur in adults. MATERIALS AND METHODS: A total of 30 cases of diaphyseal femur fractures in adults, who have been treated with closed intramedullary interlocking nailing were studied from 2008 - 2010. Data was analyzed both with regards to the clinical and radiological outcome to evaluate the effectiveness, functional outcome and morbidity associated with the procedure. RESULTS: Average age of the patient was 27.4 years with male preponderance. Road traffic accidents were the most common mode of injury; middle third shaft fractures were most commonly seen (56.66%); comminuted and transverse fractures (63.33%) were the commonest fracture pattern. The union rate was 96.6%. Five patients had superficial infection, four had shortening of limb, and in two cases union was delayed. Excellent to good results were seen in 86.6% cases as per modified Klaus and Klemm criteria. CONCLUSION: Closed intramedullary interlocking nailing has now become the treatment of choice for closed diaphyseal fractures of femur in adults, especially those with high comminution, long spiral, and segmental fractures. Interlocking nail offers the added advantages of early joint mobilization, early weight bearing, early muscle rehabilitation, shortened hospital stay, and most importantly early return to work and prefracture state.

Bone tumors in a tertiary care hospital of south India: A review 117 cases.

BACKGROUND: Bone tumors remain a daunting challenge to orthopedic surgeons. The challenge is heightened in developing countries due to limited diagnostic and therapeutic facilities as well as due to ignorance. The published literature on this subject is sparse in our environment. OBJECTIVE: To determine the pattern of bone tumors including their relative frequencies, age and sex distributions, anatomical sites of occurrence and clinico-pathological characteristics as seen in a tertiary care hospital of south India. MATERIALS AND METHODS: This is a retrospective review of all the histologically confirmed bone tumors seen at JSS Medical College and Hospital, Mysore over an 8 year period: 2002 to 2009. RESULTS: A total of 117 patients (aged 5 to 82 years) with a mean of age of 26.87 years were studied. Seventy-six patients (64.96%) were males and 41 (35.04%) were females. The peak age incidence for primary bone tumors was in the age group of 11-20 years and that for metastatic bone tumors was more than 60 years. Sixty-seven (57.26%) of the tumors were benign. Among these, osteochondroma was the most common, accounting for 26 cases (22.22%) followed by Giant cell tumor (24 cases, 20.51%). Osteosarcoma accounted for 35.14% (13 cases) of all the primary malignant tumors in the study. Lower end of femur was the most common site for primary bone tumors and accounted for 30 cases (25.64%) followed by upper end of tibia and fibula (24 cases, 20.51%). The most common site for metastatic bone tumors was upper end of femur including hip joint followed by spine. CONCLUSION: This study showed that primary bone tumors are mainly benign, occurred predominantly in the second decade of life with a male preponderance. Osteochondroma and osteosarcoma are the most common benign and primary malignant bone tumors, respectively. The most common primary foci for metastatic bone tumor are from the respiratory tract.

Outcome of closed proximal phalangeal fractures of the hand.

BACKGROUND: The proximal phalanx (PP) of the fingers is fractured more frequently than the middle or even distal phalanges. The problems of malunion, stiffness and sometimes loss of skin or other soft tissues associated with PP fracture increases the disability. The optimum treatment depends on fracture location, fracture geometry and fracture stability. The objective of the study was to analyse the treatment outcome in a series of closed proximal phalangeal fractures of the hand. MATERIALS AND METHODS: Eighty-four proximal phalangeal fractures in 68 patients were enrolled from 2007 to 2009. The treatment modalities were broadly categorised into two groups, Group A consisted of conservative treatment, and Group B consisted of surgical treatment. Group A included 47 digits treated with closed reduction (CR) with immobilization (n=43), extension block cast and dynamic traction (n=4), while 37 digits were treated in Group B, which included closed or open reduction (OR) and internal fixation (IF) with K-wires (n=31), OR and IF with stainless-steel wiring (n=2), and mini external-fixator (n=4). Belsky's criteria and Gingrass' criteria were used for assessment of finger injuries and to assess the efficacy of conservative and surgical modalities for closed proximal phalangeal fractures of the hand. RESULTS: Average period of follow-up was 1 year (range 10-14 months). The excellent to good results seen in Groups A and B were 89% and 92%, respectively. Six complications were seen in Group A, which included four cases with malunion and two cases with digital stiffness. Three complications were seen in Group B, which included one each of malunion, digital stiffness and extensor lag. Overall, maximum poor results (n=4) were seen with CR and buddy strapping. CONCLUSION: Conservative treatment is an inexpensive method, particularly suitable for stable fractures, and in patients who are poor candidates for surgery, Surgical modalities have distinct advantage of stable fixation, but with added risk of digital stiffness. Percutaneous pinning is a reliable, most commonly used surgical modality and technically easier. Both conservative and surgical modalities have good efficacy when used judiciously.

Acute cholestatic hepatitis along with agranulocytosis: a rare side effect of carbimazole.

Antithyroid drugs have been used for more than 50 years for the management of hyperthyroidism. Most patients tolerate treatment well but some may develop life-threatening side effects such as agranulocytosis, aplastic anemia and cholestatic hepatitis. A 45-year-old female was diagnosed with severe hyperthyroidism. Treatment with Carbimazole 30 mg/day was initiated. Within six weeks following the start of therapy, patient developed potentially life-threatening acute cholestatic hepatitis and agranulocytosis as adverse effects to carbimazole. The patient's symptoms and laboratory abnormalities resolved following withdrawal of offending drug. Agranulocytosis and cholestatic hepatitis together is an extremely rare idiosyncratic side effect of Carbimazole treatment and considered to be dose and age-related. Antithyroid drugs are deceptively easy to use, but because of the variability in the response of patients and the potentially serious side effects, all practitioners who prescribe the drugs need to have a working knowledge of their complex pharmacology.

Placental pathology in pregnancy-induced hypertension (PIH) with or without intrauterine growth retardation.

The foetus, placenta and mother form a composite triad of dynamic equilibrium, and dysfunction of any one of them can affect the others. The aim of the present study was to appreciate qualitatively and to assess quantitatively the pathological features of placentas associated with pregnancy induced hypertension (PIH) with and without intrauterine growth retardation (IUGR) & also IUGR placentas without PIH and to compare them with the normal placentas and to evolve plausible explanation for the outcomes of PIH and IUGR. The study evaluated 40, 51, 58 placentas of PIH with IUGR (category I), PIH without IUGR (category II), IUGR without PIH (category III) respectively and compared them with 118 normal placentas. The basic conclusions of the study were that PIH is common among primigravida and probably the main factor in the genesis of IUGR and reduced placental weight. There was a high prevalence of inflammation, infarction, ischemia, intervillous hemorrhage, and syncytial knots in PIH with IUGR placentas as also in PIH without IUGR (category II) and IUGR without PIH (category III) placentas. Acute atherosis was a characteristic finding of PIH placentas (both category I & II).