HIV infection mimicking autoimmune disorder

A 13-yr-old girl born to healthy parents presented with cough, fever, easy fatiguability, photosensitivity and alopecia. She had clubbing and diffuse crackles in the chest on examination. Her CT scan of the chest showed evidence of bronchiectasis with consolidation. Investigations for tuberculosis and collagen vascular disease were negative. In due course she developed features of raised intracranial tension. Her blood for HIV ELISA was positive with CD4 counts of 17/μL. Her CSF, sputum, blood and urine specimen were all positive for Cryptococcus neoformans on culture. HIV was not considered initially because of her atypical presentation. There was no history of sexual abuse, her parents were healthy and she did not receive any blood transfusion in the past.     

Chronic eosinophilic pneumonia in a 13-year-old child

We report a case of a 13-year-old girl with an asymptomatic isoniazid-resistant tuberculosis contact. Six months after the contact had been made, chest radiography showed left upper lobe infiltrates without hilar lymphadenopathy, which led to the start of an antituberculous treatment. Tuberculin skin test remained negative and blood tests showed hypereosinophilia. One month after the onset of the treatment, she presented with asthenia, weight loss, and cough. She was admitted to our unit with a diagnosis of drug-resistant tuberculosis. Blood tests showed the persistence of hypereosinophilia. Chest radiograph and high-resolution lung computed tomography (CT) scan showed alveolar peripheral condensations on both upper lobes without significant hilar lymphadenopathy. Bronchoalveolar lavage (BAL) showed a normal total cell count with 44% of eosinophils. Microbiological analyses were all negative. Chronic eosinophilic pneumonia (CEP) was confirmed after the elimination of other different eosinophilic lung diseases. The patient was highly responsive to high doses of oral corticosteroids. Dyspnoea and cough disappeared within one week and chest CT scan showed regression of the lung infiltrates within one month. No relapse occurred during the following nine months.     

Necrotizing polyarteritis nodosa-like vasculitis in a child with systemic lupus erythematosus

A 10-year-old child presented with prolonged fever, lymphadenopathy, weight loss, oral ulcers, alopecia and parotitis. She later developed arterial thrombosis, poly-serositis, nephritis, myocarditis, sacro-ilitis, autoimmune hemolytic anemia and refractory thrombocytopenia. Though anti-dsDNA was negative, she was diagnosed to have systemic lupus erythematosus (SLE). Terminally, she had pulmonary symptoms and succumbed to her illness. The autopsy showed lupus nephritis-Class II, polyserositis, myocarditis, inflammatory myositis, immune mediated vasculitis involving renal, coronary, pancreatic, adrenal, dermal and intramuscular arteries, and pulmonary hemorrhages and edema.     

Persistent cough in an adolescent

Jessica, a 14-year-old girl with a history of asthma, went to her pediatrician's office because of a persistent cough. She had been coughing for at least 3 months with occasional cough-free periods of less than a few days. The cough was nonproductive and was not accompanied by fever, rhinorrhea, or facial or chest pain. Jessica and her mother observed that the cough increased with exercise and typically was not present during sleep. She has used two metered-dose inhalers--albuterol and cromolyn--without any change in the cough pattern. For the past 5 years, Jessica has had mild asthma responsive to albuterol. She enjoys running on the cross-country team, soccer, and dancing. She is an average student and denies any change in academic performance. She has never been hospitalized or had an emergency department visit for asthma or pneumonia. There has been no recent travel or exposure to a person with a chronic productive cough, tobacco smoke, or a live-in pet. Jessica lives with her mother and younger sister in a 10-year-old, carpeted apartment without any evidence of mold or recent renovation. In the process of taking the history, the pediatrician noticed that Jessica coughed intermittently, with two or three coughs during each episode. At times, the cough was harsh; at other times, it was a quiet cough, as if she were clearing her throat. She was cooperative, without overt anxiety or respiratory distress. After a complete physical examination with normal findings, the pediatrician interviewed Jessica and her mother alone. Jessica's parents had been divorced for the past 6 years. She lived with her mother but visited her father, and his new family with two young children, every weekend. She spoke about this arrangement comfortably and said that she loved her father and mother but didn't like the tension she experienced at her father's home. "I don't like adults arguing when kids are around." When asked why she thought the cough persisted so long, she commented in a neutral tone, "I don't know. It's never been like this before." Jessica's pediatrician prescribed an inhaled steroid with the albuterol. When the cough did not respond after 1 week, he ordered a chest radiograph (normal) and a tuberculin skin test (purified protein derivative-negative), and he added montelukast (a leukotriene inhibitor) and monitored airway resistance with a peak flow meter. The cough persisted, and the peak flow recording showed normal airway resistance. At this time, Jessica's pediatrician suspected a conversion reaction and contemplated the next best therapeutic strategy.     

Finger clubbing in children with human immunodeficiency virus infection

We investigated the frequency of finger clubbing in 150 HIV-infected children consecutively hospitalized for acute pneumonia in South Africa and described associated clinical, laboratory and radiological features. Clubbing occurred in 30 of 150 (20%) HIV-infected children compared with one of 99 (1%) HIV-negative control patients, p < 0.001. Clubbing was associated with lower presenting heart and respiratory rates and enlarged parotid glands. Total and CD4 + lymphocytes, CD4:CD8 ratio and LDH were lower in children with clubbing, but serum protein and gammaglobulin were higher. No differences in the prevalence or type of microbial pathogens were found between the two groups. Clubbing was associated with a radiological diagnosis of LIP. Children with clubbing had a lower in-hospital mortality rate than those without clubbing (6.7% vs 24.2%, p = 0.035). In geographical areas with high HIV seroprevalence rates, the presence of clubbing in a child hospitalized for respiratory disease should raise the suspicion of HIV infection.     

Bilateral Optic Neuritis in Pediatric Systemic Lupus Erythematosus with Antiphospholipid Antibody Syndrome

Bilateral optic neuritis is an extremely uncommon complication of pediatric systemic lupus erythematosus and sporadic cases are reported in the literature. The authors describe an 11-yr-old girl who presented with fever and progressively increasing pallor for 4 months, headache for 7 days, severe anemia and hepatosplenomegaly. Soon after admission, she developed rapid deterioration of vision, worsening to no perception of light with afferent pupillary defect. Fundoscopy showed bilateral optic neuritis. Investigations revealed autoimmune hemolytic anemia and thrombocytopenia. Anti-dsDNA and anti-phospholipid antibodies were positive. Magnetic resonance venography showed multiple thrombi in the cerebral venous sinuses, for which anticoagulant therapy was initiated. She was managed with intravenous methylprednisolone followed by cyclophosphamide pulse therapy for 6 months along with oral prednisolone. Though she went into remission, visual outcome has been dismal, with development of bilateral optic atrophy, and absence of perception of light.     

Pulmonary sarcoidosis masquerading as tuberculosis

Pulmonary manifestations of sarcoidosis are common and may be initially confused with other common diseases like tuberculosis. We report an 11 yr old girl who presented with chronic cough, low grade fever, recent exposure to tuberculosis and hilar lymphadenopathy. She was provisionally diagnosed as pulmonary tuberculosis and treated accordingly. As she had poor response to anti tubercular therapy, diagnosis was subsequently revised to sarcoidosis by lung biopsy. Treatment with steroids resulted in significant clinical improvement.     

Superior Mediastinal Syndrome Due to Intrathoracic Tuberculosis

A 5-year-old boy presented with fever, cough, breathlessness and facial swelling. He was pale with distended veins over neck and chest, cervical and axillary lymphadenopathy and bilateral expiratory wheeze. Chest radiographs showed superior mediastinal widening. A computed tomography scan of the chest revealed mediastinal lymph nodes compressing superior vena cava and trachea. Bronchoscopy revealed nodular lesions in trachea and bronchi and compression of trachea. Broncho-alveolar lavage revealed acid fast bacilli. Diagnosis of superior mediastinal syndrome (SMS) secondary to tuberculosis was made and child was treated with antitubercular treatment along with oral prednisolone with good response.     

Clinically directed selective screening for HIV infection in hospitalized children

BACKGROUND: As HIV infection presents with several manifestations, none of which is specific, several children are subjected to HIV testing. Very few studies have examined the issue of probability of HIV infection with a given clinical manifestation. AIM: To determine the probability of HIV infection when a child is hospitalized with at least one of the selected manifestations. MATERIAL AND METHODS: Children aged 18 mo and above, admitted to a tertiary care center in Mumbai, India with chronic diarrhea, severe malnutrition, persistent cough, generalized lymphadenopathy, oral thrush, hepatomegaly, repeated common infections, generalized dermatitis, chronic parotid swelling, recurrent bacterial infection, disseminated tuberculosis and/ or Pneumocystis carinii pneumonia were enrolled in a prospective study after obtaining informed consent. They were subjected to HIV testing using WHO-UNAIDS strategy II. The data obtained was analyzed using the Statistical Package For Social Sciences (SPSS) software program. RESULTS: Twenty-three (20 PERCENT) of the 115 children enrolled tested positive for HIV. The seropositivity rate for various features ranged from 9.1 PERCENT for chronic diarrhea to 83.3 PERCENT for chronic dermatitis. Oral thrush, generalized dermatitis and generalized lymphadenopathy were the significant independent clinical risk factors for predicting HIV seropositivity. The probability of HIV infection was higher in children who had higher number of risk factors present concomitantly CONCLUSIONS: The probability of HIV infection in a child is dependent upon the nature and number of manifestations present.     

Shaken baby syndrome masquerading as apparent life threatening event

Disseminated cryptococcosis is a rare and often fatal disease in children. The majority of cases usually occur in individuals with defective cell-mediated immunity, most commonly due to HIV infection. The authors here in report an 8-year-old girl from Nepal who presented with fever, cough, headache, lymphadenopathy, hepatosplenomegaly and cutaneous lesions. Lymph node biopsy revealed multiple granulomas composed of histiocytes and epitheliold cells along with numerous yeast forms of cryptococcus. Cultures of CSF, sputum and urine yielded cryptococcus neoformans. Surprisingly,the immune function in terms of T-cell number, CD4 : CD8 ratio, serum immunoglobulins and HIV serology was normal. After the diagnosis of disseminated cryptococcosis was established, the patient was treated with 5-fluorocytosine (100 mg/kg/day) for initial two weeks and amphotericin B (1 mg/kg/day) for 13 weeks. Patient responded well to the treatment with disappearance of presenting symptoms, cutaneous lesions, and lymphadenopathy, though she still had hepatosplenomegaly, which also decreased. Unfortunately, she developed loss of vision in 10th week of therapy. The patient was discharged on oral fluconazole (6 mg/kg/day) and no recurrence was found during the follow-up period of more than 9 months. This is the first case of disseminated cryptococcosis with no detectable immune deficit, from India.     

Barking vocalizations and shaking movements in a 13-year old girl

CASE: Erica is a 13-year old female who was hospitalized for a 4-week history of "barking" noises and 2 weeks of generalized shaking episodes. Four weeks prior to admission, she had a viral upper respiratory infection (URI) with cough which was treated with over-the-counter cough syrup. After resolution of the URI, she developed a persistent cough that turned into a "bark"-like vocalization. Both the mother and patient demonstrated the bark as an "arf" sound like that of a small dog at times, a large dog at others. These vocalizations were unrelenting, occurring 3-10 times per minute only while awake. They were not precipitated by any known factors nor were there alleviating factors. She could not voluntarily suppress the sound.In addition to the vocalizations, episodes of generalized shaking of the extremities began 2 weeks prior to admission. According to Erica's mother, each episode lasted about 10-60 seconds and occurred 30-40 times a day only when she was awake. These episodes were not rhythmic or symmetric, and they were not associated with bowel or bladder incontinence. There was no alteration of consciousness following the episodes. Erica denied any recollection of the barking or shaking.The medical evaluation did not reveal an etiology. It included a complete physical examination, a neurological examination, biochemical laboratory studies, and a negative video EEG study that captured 10 episodes of shaking. Child psychiatry was consulted. Erica was a pleasant, quiet female with slightly constricted affect and a normal speech pattern. She reported that she was a straight-A honors student who had difficulty trusting others; she said that she had no friends, only "associates." She said that she had periods of feeling "sad" and crying easily, but could not identify any recent stressful event. Episodes of barking and shaking diminished during the hospitalization. Erica was discharged home with outpatient psychiatric follow-up.     

Follicular carcinoma of thyroid following successful liver transplantation – A report

Dantuluri S, Urs A, Karthik SV. Follicular carcinoma of thyroid following successful liver transplantation – A report. Abstract: Follicular carcinoma of the thyroid is a relatively rare malignancy in childhood even in paediatric solid organ transplant recipients. The risk of developing de novo malignancies after liver transplantation is higher compared to the general population. We report an 18‐yr‐old girl who had successfully undergone liver transplantation five yr earlier for neonatal sclerosing cholangitis complicated by the development of dysplastic nodules. Baseline immunosuppression was with tacrolimus and prednisolone. Mycophenolate mofetil was later added in view of steroid‐resistant episodes of graft rejection. She subsequently suffered from marked obesity and essential hypertension needing antihypertensive medication. Five yr after liver transplantation, she presented with a right‐sided thyroid swelling that was rapidly progressive with no associated lymphadenopathy and normal systemic examination. Ultrasound of her neck revealed a solid lesion in the right lobe of the thyroid gland with ill‐defined margins, and a diagnostic right thyroid lobectomy confirmed the diagnosis of follicular carcinoma with focal capsular and vascular invasion. She underwent total thyroidectomy and currently remains well on thyroxine supplements. Our report highlights the need for high level of suspicion and prompt investigation into any abnormal lesion in the long‐term follow‐up of solid organ transplant recipients.     

A warm antibody mediated acute hemolytic anemia with reticulocytopenia in a four-month-old girl requiring immunosuppressive therapy

We present a four-month-old girl with severe hemolytic anemia and reticulocytopenia. This case is the youngest with hemolytic anemia encountered in our hospital. Findings of autoimmune hemolytic anemia were preceded by diphtheria-pertussis-tetanus (DPT) and oral polio vaccines which were given one month before. At admission, she had heart failure, her hemoglobin (Hb) was 27 gm/L, hematocrit (Hct) 8.5 percent, reticulocyte count 0.2 percent, and gamma and non-gamma Coombs tests were positive. Plasma Hb was 23 percent (N < 3%) and haptoglobin 0 mg/dl. Bone marrow aspiration smear revealed erythroid hyperplasia. No infection, immunodeficiency or malignancy could be established. She received multiple transfusions and did not respond to methyl prednisolone therapy of seven days' duration, but was successfully treated with a combination of immunosuppressive therapy (cyclophosphamide, 6-mercaptopurine, intravenous immunoglobulin and prednisolone, which was added later). This case is interesting in that the disease was preceded by DPT vaccination, was associated with reticulocytopenia and was resistant to steroids.     

Long survivors with Ki-1 lymphoma having t(2;5) (p23;q35). Does the presence or absence of t(2;5) influence the prognosis of patients with Ki-1 lymphoma?

Abstract We experienced three patients with CD30+ diffuse large cell lymphoma having chromosomal abnormalities. The first patient was an 8-year-old girl with bilateral cervical lymphadenopathy. A biopsy of a cervical lymph node revealed diffuse large cell lymphoma (stage III), positive for CD30 and a chromosomal abnormality. t(2;5). She attained a remission and is now in complete remission 108 months after diagnosis, despite frequent relapses. The second patient was a 13-year-old boy with right axillar and supraclavicular lymph-node adenopathy. A biopsy of a cervical lymph node revealed diffuse large cell lymphoma (stage III), positive for CD30 and a chromosomal abnormality, t(2;5). He attained remission and was in continuous first remission 112 months after diagnosis. The third patient was an 11-year-old boy with fever and bilateral cervical lymph node revealed diffuse large cell lymphoma (stage III), positive for CD30 and chromosomal abnormality without t(2;5). He showed a very aggressive clinical course. Only the patients with Ki-1 lymphoma having t(2;5) survived over 100 months from the diagnosis, despite the advanced stage of the disease. These findings and a review of the literature showed that the presence or absence of t(2;5) may influence the outcome of Ki-1 lymphoma.     

Hypereosinophilic syndrome in acute lymphoblastic leukemia with a chromosome translocation [t(5q;14q)]

We report a case of hypereosinophilic syndrome associated with acute lymphoblastic leukemia (L1 type, FAB classification) which showed an abnormal karyotype. An 8-year-old boy was admitted to our hospital with complaints of fever and cough that had persisted for 2 weeks. Peripheral blood examination revealed remarkable eosinophilia (120,000/mm3) and a few lymphoblasts. Bone marrow examination also revealed many mature eosinophils and 20% lymphoblasts that were PAS and peroxidase negative. A direct chromosome analysis of the bone marrow cells demonstrated that 12.5% of the spontaneously dividing cells had an abnormal karyotype of 46XY, t(5;14) (q31;q32). The chest radiogram showed interstitial pneumonia-like densities, and the ECG had the pattern of a right bundle branch block. The therapy consisted of prednisolone, high dose of methylprednisolone, cyclophosphamide, and vincristine. This treatment failed to reduce the eosinophil count. On the 4th day after admission, the patient developed severe dyspnea, complete A-V block, and died. At postmortem, dense infiltrations of eosinophils in various stages of maturation were noted in lungs and liver.     

Reversibility of hyperintense globus pallidus on T 1-weighted MRI follow- ing surgery for a portosystemic shunt in an 8-year-old girl

An 8-year-old Japanese girl with a portosystemic shunt had shown hyperammonaemia since she was 3 years of age. MRI of her brain showed bilateral hyperintense globus pallidus. A portosystemic shunt was evident on US and angiography. She underwent surgical banding of the shunt, after which the lesion and clinical symptoms disappeared. Received: 23 June 1998 Accepted: 30 November 1998     

Intractable wheezing in infants with nasopharyngeal reflux

Two infants with intractable wheezing and moist cough were referred to Chiba Municipal Kaihin Hospital. Their symptoms were persistent even after the usual treatment for respiratory disease. No definite etiological agents were detected. They usually gagged while feeding and barium swallow tests revealed nasopharyngeal reflux and cricopharyngeal incoordination. One of the patients had remarkably high titers of IgE and IgE RAST of cow's milk before she received treatment with thickened formula. She also had peripheral eosinophilia and nasal eosinophilia. These findings were thought to be caused by nasopharyngeal reflux. Four months after therapy commenced, those titers and symptoms were greatly reduced. The clinical and roentgenographic findings in these infants, and their response to therapy, strongly support a causal relationship between nasopharyngeal reflux and wheezing. Therefore, nasopharyngeal reflux should be considered when a baby has intractable wheezing, even when there is no developmental problem.     

Self induced photosensitive epilepsy

The authors report a case of a 12-year-old girl who had rare self induced photosensitive epilepsy. She used to move her right hand over the right eye while simultaneously rubbing the forehead since the age of 8. During these episodes she was lost in herself. Lately these episodes were followed by brief spell of unconsciousness. The EEG examination, in its third attempt, revealed bilateral multiple symmetric spikes on photic stimulation. She admitted that she often induced the episode herself and derived pleasure out of it. She responded well to Sodium valproate     

Moyamoya disease associated with midaortic syndrome

We report a 1-year-old girl who presented moyamoya disease associated with midaortic syndrome. She had been treated for cardiac failure and severe hypertension due to midaortic syndrome until she suffered seizure and repeated cerebral ischemic attack. Cerebral angiography revealed stenosis of the bilateral internal carotid artery at its terminal portion. She was successfully treated with encephaloduroarteriosynangiosis, and ischemic attack ceased postoperatively. This is the first report of moyamoya disease with midaortic syndrome. Although cerebral ischemic attack has been effectively managed by encephaloduroarteriosynangiosis, renovascular hypertension is still difficult to control.     

Control of breathing in the central alveolar hypoventilation syndrome with and without a phrenic pacemaker

Primary central alveolar hypoventilation (CAHV) is a rare disorder described in newborns, children, and adults. We report a 2 9/12 year old child with CAHV of unknown etiology. The evaluation of her ventilatory control system showed abnormalities awake and in the different sleep states. Hypoventilation was found to be more severe during non-REM sleep than during REM sleep and awake state. She had central apnea, an irregular respiratory rhythm in the non-REM sleep too, and diminished ventilatory response to inhaled 5%-6% CO2 in both REM and non-REM sleep. Her ventilation decreased when she was breathing 50% and 100% oxygen. During breathing 15% oxygen she did not arouse in spite a transcutaneous pO2 of 10 mmHg. She was first treated with mechanical ventilation during sleep and has now received bilateral simultaneous phrenic pacemaker support during quiet sleep for about one year. With the phrenic pacemaker she has normal minute volume and transcutaneous blood gases during sleep. During a respiratory infection she needed again mechanical ventilation via her tracheostoma 24 hours a day for one week. This case of a CAHV demonstrates a dysfunction of the central and partially also of the peripheral chemoreceptors. The abnormalities of the ventilation were demonstrable not only in the non-REM sleep but also in the REM sleep and awake state.