A study of self-perception and communication success as perceived by adolescents with cochlear implants and their significant others.

OBJECTIVES: The objectives of this prospective, cross-sectional study were to compare self-perception and communication-success ratings of adolescents with cochlear implant (AWCI) and their caregivers (C-AWCI) and to explore associations with age at CI, implant age, and chronological age. METHOD: Fourteen CI centers across India participated. The Think About it Quiz (TAIQ), Self Assessment of Communication-Adolescent (SAC-A), and Significant Other Assessment of Communication-Adolescent (SOAC-A) were translated into five languages. Data were collected from 173 AWCI aged 10;0-19;6 years and an associated caregiver for each participant. RESULTS: On the TAIQ, self-ratings by AWCI were significantly lower than the ratings by C-AWCI. Peer acceptance correlated with athletic competence for both groups. For the SAC-A versus SOAC-A, there was no significant difference between AWCI and C-AWCI ratings. Except for a negative correlation between peer-acceptance and chronological age for caregiver ratings, no other associations were found between any other ratings and age at CI, implant age, and chronological age. CONCLUSIONS: Caregiver judgments of their adolescents with CI were not in equal agreement with self-ratings by the adolescents across various aspects of performance. Caregivers appeared to underestimate the self-perception issues faced by adolescents with CI but had excellent agreement with their adolescents' self-rating of communication success. The inclusion of activities to improve children's participation in sports could possibly improve peer acceptance.

Tuberculosis control in India: Refocus on nutrition.

Many western societies have eliminated tuberculosis years before the advent of potent anti-tuberculous drugs, as a result of the improved standards of living and good nutrition. But even with the availability of powerful anti-tuberculous drugs, India still has a long road ahead to reach the "End TB by 2025" goal. One of the major reason is that tuberculosis control program in India till now have focused primarily on case detection and medical treatment of active tuberculosis. Drug treatment alone does not completely prevent the occurrence of new infections in the community and also contributes to development of drug resistant strains if used improperly or incompletely. Although the treatment of active cases can reduce the period of transmission of disease, a significant amount of transmission to contacts occurs even before they have been diagnosed and treated. Additionally, this approach cannot prevent re-activation to active TB in the vast pool of persons with latent TB infection. Tuberculosis occurs in those with suppressed cell mediated immunity mainly due to poor nutritional status. Improving the nutritional status of the society by several social interventions hand-in-hand with utilizing the available anti-tuberculous drugs is possibly the only effective strategy. Promising programmatic guidance for nutritional support in TB patients have been formulated by the Central TB division of India but it needs a refocusing of TB control strategies towards nutrition at all levels and strong public health actions for effective implementation.

An outbreak of Kyasanur forest disease in the Wayanad and Malappuram districts of Kerala, India.

Kyasanur forest disease (KFD) is a zoonotic viral haemorrhagic fever and has been endemic to Karnataka State, India. Outbreaks of KFD were reported in new areas of Wayanad and Malappuram districts of Kerala, India during 2014-2015. Investigation of the outbreaks was carried out in these districts during May 2015. The line-list data of KFD cases available with District Medical Office, Wayanad were analysed. Case investigation was carried out to determine the risk factors associated with the outbreak and possible site of contraction infections. Ticks from the forest floor were collected in areas associated with monkey deaths by flagging method to estimate species abundance. Of 102 confirmed cases of KFD reported in Wayanad, 91% were adults aged >15years. About 43% of the cases were from the areas of Poothady Primary Health Centre (PHC) followed by Chethalayam PHC (22%). Most of the affected individuals belong to Kattunayakan tribe, dependent on forest for their livelihood. Those tribes are engaged in trench digging and fire line works in summer months and hence are at a higher risk. In Malappuram, the Cholanaickan tribe, are under high risk of exposure to infected ticks as they live deep in the forest and trap monkeys for game meat. High abundance of Haemaphysalis spinigera and H. turturis, the established vectors of KFD virus was recorded in all affected areas. Incidence of KFD cases/monkey deaths and high abundance of Haemaphysalis vectors in the forest ranges of Wayanad and Malappuram districts indicate that the area has become receptive for KFD outbreaks. Preventive measures (vaccination of high risk groups) coupled with intensive health education should be carried out prior to transmission season.

Kozhikode criteria for diagnosing systemic lupus erythematosus as a hematological disorder.

Systemic lupus erythematosus (SLE) is an autoimmune disease in which cells and tissues undergo damage mediated by tissue-binding autoantibodies. At its onset, it may involve one organ alone or more than one organ simultaneously; over a time, additional manifestations due to the involvement of other organs may occur. Our observations have confirmed that hematological manifestations are the commonest initial presentation in SLE. The criteria used till 2012 was the American College of Rheumatology (ACR) criteria, which is only a classification criterion and not really for diagnosis; if we rely on ACR criteria, the diagnosis is often delayed. Time required for satisfying all four of the eleven criteria is variable and prolonged. Moreover, hematological manifestations are underrepresented in the ACR criteria. Based on the clinical observations made on patients evaluated in a tertiary center in North Kerala, an alternate diagnostic criterion named the Kozhikode criteria was proposed, especially for the diagnosis of SLE when it presents with hematological manifestations alone. The present study was an attempt to validate the same and to look for any association of diet and lifestyle with the disease.

Cowden syndrome with Lhermitte- Duclos disease presenting as ataxia.

Cowden syndrome or multiple hamartoma syndrome is a rare genodermatosis of autosomal dominant inheritance characterized by multiple hamartomatous lesions of ectodermal, mesodermal and endodermal origin. A 45-year-old man presented to us with a history of dural arteriovenous fistula and intracerebral bleed in the past with gradually progressive difficulty in walking. Magnetic resonance imaging (MRI) of the brain showed a heterogeneous lesion in the cerebellum which was diagnosed as adult Lhermitte-Duclos disease which is considered a component of Cowden syndrome. On examination we found florid skin and mucosal manifestations of Cowden syndrome. A family history of thyroid malignancy was also present. Using the Cleveland Clinic web calculator, the patient had an 82% chance of having a phosphatase and tensin homologue (PTEN) mutation.

Unusual Case of ALL with Ovarian Relapse.

Patients with acute lymphoblastic leukemia (ALL) can develop relapse in sanctuary sites like brain, ovary or testis even when the bone marrow is in remission. Pelvic recurrence is rarely reported during the follow up of successfully treated ALL in females. We report here a very unusual case of a large pelvic lump which the patient herself could feel, that was probably an ovarian relapse of ALL, successfully treated with re-induction chemotherapy alone and achieved complete remission.

Invasive mycosis due to species of Blastobotrys in immunocompromised patients with reduced susceptibility to antifungals.

Cases of invasive mycosis due to Blastobotrys serpentis and B. proliferans identified by sequencing in a preterm patient and a rhabdomyosarcoma patient, respectively, are reported. Both species revealed elevated fluconazole and echinocandin MICs by the CLSI broth microdilution method. Additionally, B. serpentis exhibited high amphotericin B MICs, thus posing serious therapeutic challenges.

Cochlear implant programming: a global survey on the state of the art.

The programming of CIs is essential for good performance. However, no Good Clinical Practice guidelines exist. This paper reports on the results of an inventory of the current practice worldwide. A questionnaire was distributed to 47 CI centers. They follow 47600 recipients in 17 countries and 5 continents. The results were discussed during a debate. Sixty-two percent of the results were verified through individual interviews during the following months. Most centers (72%) participated in a cross-sectional study logging 5 consecutive fitting sessions in 5 different recipients. Data indicate that general practice starts with a single switch-on session, followed by three monthly sessions, three quarterly sessions, and then annual sessions, all containing one hour of programming and testing. The main focus lies on setting maximum and, to a lesser extent, minimum current levels per electrode. These levels are often determined on a few electrodes and then extrapolated. They are mainly based on subjective loudness perception by the CI user and, to a lesser extent, on pure tone and speech audiometry. Objective measures play a small role as indication of the global MAP profile. Other MAP parameters are rarely modified. Measurable targets are only defined for pure tone audiometry. Huge variation exists between centers on all aspects of the fitting practice.

Hypertension as a Risk Factor for Haemorrhagic Stroke in Females.

AIM: To study the effect of hypertension as a risk factor for haemorrhagic stroke in women compared to men. METHOD: Confirmed cases of haemorrhagic stroke by Computed Tomography scan were included in this study. The participants underwent detailed clinical examination based on preset questionnaire. Relevant laboratory investigations were done in this group. 100 consecutive cases were studied comparing the baseline characteristics with male patients admitted with haemorrhagic stroke. OBSERVATIONS: Among all the risk factors studied hypertension was the single most important risk factor causing intracerebral bleed. Forty seven point four percentage (47.4%) of females were hypertensive as against 61% in males. Blood pressure recording of females showed lower systolic and diastolic measurement compared to males and the difference was statistically significant with a p value of 0.000. Majority of the female patients were overweight with a significant difference in body mass index compared to males. (p value 0.006). CONCLUSION: Hypertension is an important risk factor for intracerebral bleed. Females developed intracerebral bleed at a lower blood pressure measurement compared to males. Females require better control of hypertension than males to prevent intracerebral bleed.

Emphysematous pyelonephritis in a patient infected with the human immunodeficiency virus.

Emphysematous pyelonephritis (EPN) is a rapidly progressive and life-threatening infection that is seen most commonly in persons with diabetes. The infecting organisms usually consist of mixed flora, including Escherichia coli (68%), Klebsiella pneumoniae (9%) and Proteus mirabilis. Females are affected twice as often as men, and mortality rates can be as high as 80%. Obstructive uropathy, urinary calculi, calyceal stenosis and neoplasms are significant predisposing factors. We report a case of EPN in a patient with the human immunodeficiency virus infection, without diabetes mellitus or urinary tract obstruction, which responded remarkably to conservative management with antibiotics alone.

Hematological Manifestations of SLE at Initial Presentation: Is It Underestimated?

SLE can present with hematological manifestations alone or along with features of other system involvement. With a low index of clinical suspicion or inadequate follow up the diagnosis may be delayed or missed at the time of presentation, in those with hematological abnormalities as the initial manifestation. An observational study was conducted among patients of SLE, in a tertiary referral centre of North Kerala, with the purpose of estimating the proportion of patients with hematological manifestations as the initial presentation of the disease and to study their nature. It was observed that 82% of the patients had hematological manifestations at presentation. It is the most common presenting manifestation of SLE in people of North Kerala. Autoimmune hypothyroidism was one of the common coexisting abnormalities in these patients, which is not included in the American College of Rheumatology (ACR) criteria for diagnosis. Arthritis was uncommon among those who presented with hematological manifestations. A significant number of patients do not satisfy the ACR criteria at the time of diagnosis but do so on follow up. The ACR criteria are weak to diagnose such patients and therefore need revision. We therefore propose an alternative to ACR criteria as "Kozhikode criteria for SLE".

Cerebral vein thrombosis misdiagnosed and mismanaged.

Cerebral venous thrombosis (CVT) should be considered in the differential diagnosis of all unexplained CNS disorders of sudden onset. Etiological factors are often subclinical forms of several common thrombophilic states occurring together, rather than the typical inherited and rare causes. Diagnosis is missed because of the heterogeneity in clinical presentation and etiological factors. In several patients with the so called idiopathic CVT nutritional deficiencies and lifestyle issues are more important factors in pathogenesis, rather than single rarer causes. High index of suspicion is the key to diagnosis. Clinical skill has to be fine tuned to diagnose the problem and to identify all the etiological factors. Radiology is essential for diagnosis but relying on radiology alone will lead to missing several cases and even erroneous diagnosis. It is inappropriate to proceed prematurely to laboratory investigations, forgetting proper clinical evaluation by studying diet, lifestyle, and environment of the patients. Success in managing lies in identifying all the contributory causes and correcting all of them giving excellent outcome almost always. Clinical observations based on case series and sharing of such information alone are the means to arrive at a consensus in diagnosis and management.

Intravesical pressure: A new prognostic indicator in congenital diaphragmatic hernia.

AIMS: To evaluate the usefulness of intravesical pressure as a prognostic indicator in congenital diaphragmatic hernia. MATERIAL AND METHODS: In 25 cases, bladder pressure was measured intraoperatively during repair. RESULTS: Cases were divided into three groups according to the intravesical pressure. Group 1: pressure <10 cm (n.9), Group 2: 10-15 cm (n.11) and Group 3: >15 cm (n.5). Number of ventilated days was tabulated against these groups. Median number of ventilated days for Group 1, with the lowest pressure, was 3 days, while that for Group 2 was 5 and for Group 3, with the highest pressure, was 10 days. This was significant, with a P-value of 0.016. CONCLUSION: Measurement of intravesical pressure is a reliable prognostic indicator in newborns with congenital diaphragmatic hernia. It also helps in predicting postoperative ventilatory requirement.

AIDS associated Kaposi's sarcoma.

Epidemic Kaposi's sarcoma (KS) is the most common malignant neoplasm occuring in HIV patients. Although the prevalence of HIV infection is high in Asian countries, KS is rarely reported. We report a case of fatal KS involving the skin and mucous membranes in a homosexual male.

Primary haemophagocytic syndrome in a young girl.

Haemophagocytic syndrome is a life-threatening systemic illness characterized by an uncontrolled inflammatory response. Patients present with fever, hepatosplenomegaly, jaundice and liver dysfunction, neurological manifestations and often pancytopenla. Bone marrow, lymph node, hepatic or splenic biopsy shows macrophages with Ingested blood cells or their precursors. Laboratory markers include elevated triglycerides and ferritin, low fibrinogen with normal or low erythrocyte sedimentation rate (ESR). Familial haemophagocytic lymphohistiocytosis (HLH) is an autosomal recessive disorder. Secondary haemophagocytic syndrome results from infections, malignancy and collagen vascular disorders. We describe a young girl with primary haemophagocytic syndrome.

A simplified technique of free flap pedicle transfer for reconstruction of defects following head and neck cancer ablation.

Free flaps are being widely used for reconstruction of defects following head and neck cancer ablation. The pedicle of the flap is usually tunneled into the neck wound for anastomosis to the neck vessels. This transfer of the pedicle can be technically difficult to execute and associated with difficulties of torsion and kinking. No effective method has been so far reported to make this procedure easy and safe. A very easy and effective method for the pedicle transfer is described in this report, which has been practiced successfully in our head and neck service in more than 175 free flaps.

Cortical venous thrombosis due to acquired hyperhomocysteinaemia.

A 20-year-old student presented with generalized tonic-clonic seizures and was diagnosed to have cortical venous thrombosis. Her dietary history and the clinical signs of vitamin deficiency prompted further investigations, which detected hyperhomocysteinaemia secondary to vitamin B12 deficiency as a factor contributing to the hypercoagulable state. This case highlights the importance of a balanced diet, as well as the necessity for primordial prevention.

Congenital immunodeficiency disorder.

A 17 year old girl, a 9th standard student with history of recurrent pneumonia and soft tissue "cold abscesses" since neonatal period, presented with fever and cough with yellowish expectoration of 2 months duration. Her clinical and radiological finding along with elevated serum IgE level were consistent with the diagnosis of hyper immunoglobulin E syndrome or Job's syndrome.