Showing papers in "Case Reports in 2011"
TL;DR: Paediatricians need to be aware of the possibility of ARVC in adolescents and young adults presenting with palpitations, fatigue, syncope or cardiac arrest, and the authors present two cases of apparently healthy teenage boys who died suddenly and unexpectedly.
Abstract: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare inherited disease of the heart muscle that causes ventricular tachyarrhythmias and sudden death in young people and athletes. It results in fibrofatty replacement of the right ventricle, and the subepicardial region of the left ventricle. It is the most common cause of sudden cardiac death in young people after hypertrophic heart disease. Diagnosis can be difficult and at present there is no cure for ARVC. Prevention of sudden death is the most important management strategy. Paediatricians need to be aware of the possibility of ARVC in adolescents and young adults presenting with palpitations, fatigue, syncope or cardiac arrest. The authors present two cases of apparently healthy teenage boys who died suddenly and unexpectedly. Postmortem examination of the myocardium was strongly suggestive of ARVC in both cases.
467 citations
TL;DR: A 63-year-old female presented with a 12-week history of worsening proximal pain and stiffness and was treated with intravenous tocilizumab (TCZ;an interleukin 6 blocker), with excellent response with normalisation of her inflammatory markers.
Abstract: A 63-year-old female presented with a 12-week history of worsening proximal pain and stiffness. She was diagnosed with polymyalgia rheumatica and started on corticosteroids. The authors were unable to wean-off her steroid treatment, despite trying various steroid sparing agents on different occasions with no benefit. In August 2010, she was diagnosed with giant cell arteritis with a temporal artery biopsy and ultrasound of the temporal and axillary arteries. An fluorine-18-deoxyglucose positron emission tomography CT showed increased uptake in the aorta and major vessels, in keeping with widespread large vessel involvement. Due to the disease severity, the failure of previous disease-modifying agents and the development of steroid related sideeffects, the authors decided to treat her with intravenous tocilizumab (TCZ;an interleukin 6 blocker). After her first infusion, the patient reported excellent response with normalisation of her inflammatory markers. Prednisolone reduced from 20 mg to 3.5 mg /day after five infusions of TCZ (8 mg/kg).
54 citations
TL;DR: The authors show that a learning-based therapy, designed to exploit experience-dependent plastic mechanisms, can be used to recover stereoscopic visual function in adults with amblyopia, and challenge the long-held dogma that the critical period for visual development and the window for treating amblyopic are one and the same.
Abstract: Disruption of visual input to one eye during early development leads to marked functional impairments of vision, commonly referred to as amblyopia. A major consequence of amblyopia is the inability to encode binocular disparity information leading to impaired depth perception or stereo acuity. If amblyopia is treated early in life (before 4 years of age), then recovery of normal stereoscopic function is possible. Treatment is rarely undertaken later in life (adulthood) because declining levels of neural plasticity are thought to limit the effectiveness of standard treatments. Here, the authors show that a learning-based therapy, designed to exploit experience-dependent plastic mechanisms, can be used to recover stereoscopic visual function in adults with amblyopia. These cases challenge the long-held dogma that the critical period for visual development and the window for treating amblyopia are one and the same.
53 citations
TL;DR: The difficulty of achieving the diagnosis of ROHHAD syndrome and its overlap with other well-established disease entities are highlighted and the mortality and morbidity resulting from the high incidence of cardiorespiratory arrest may be prevented by early ventilatory support.
Abstract: A rare syndrome of rapid-onset obesity with hypothalamic dysfunction, hypoventilation and autonomic dysregulation (ROHHAD) has been recently described. We report the first patient with this syndrome in Southeast Asia and review reported cases to date. Our patient was good health with normal development until the age of 2. He then developed hyperphagic obesity, hypersomnolence, seizures, alveolar hypoventilation, central hypothyroidism, sodium and water dysregulation, gastrointestinal dysmotility, strabismus, disordered temperature and irregular heart rate, altered sweating, delayed puberty, mental retardation and recurrent respiratory tract infections. The cardiomyopathy with heart failure and abnormal cerebral spinal fluid (CSF) neurotransmitter analysis present in our patient have not been reported previously. Tumours of the sympathetic nervous system are known to be associated with this syndrome but had not been found in our patient at the time of reporting. We highlight the difficulty of achieving the diagnosis of ROHHAD syndrome and its overlap with other well-established disease entities. The mortality and morbidity resulting from the high incidence of cardiorespiratory arrest may be prevented by early ventilatory support.
47 citations
TL;DR: Skin lesions, which were found to be associated with asymptomatic bilateral hilar lymph nodes and pulmonary nodules, were the sole clinical manifestation of the disease and stopping therapy resulted in clinical and radiological improvement.
Abstract: Sarcoidosis is a rare side effect of interferon therapy that has been reported over the last years. We present the case of a 43-year-old man presenting with systemic sarcoidosis during treatment with pegylated interferon and ribavirin. Skin lesions, which were found to be associated with asymptomatic bilateral hilar lymph nodes and pulmonary nodules, were the sole clinical manifestation of the disease. Stopping therapy resulted in clinical and radiological improvement.
43 citations
TL;DR: A 51-year-old female who was admitted with a large necrotising tumour involving the right breast is presented, who underwent a primary mastectomy with chest wall reconstruction 3 months later she developed local recurrence.
Abstract: Primary osteosarcoma of breast is rare. The authors present a case of a 51-year-old female who was admitted with a large necrotising tumour involving the right breast. CT scan confirmed chest wall invasion along with a solitary lung metastasis. She underwent a primary mastectomy with chest wall reconstruction. Unfortunately 3 months later she developed local recurrence.
41 citations
TL;DR: A full-term male neonate born of a non-consanguineous marriage presented to us on the first day of life with large head and generalised hypotonia and had microphthalmia and white reflex in pupillary areas.
Abstract: A full-term male neonate born of a non-consanguineous marriage presented to us on the first day of life with large head (figure 1) and generalised hypotonia. He had microphthalmia and white reflex in pupillary areas. There was a family history of three neonatal deaths, the parents did not recall any of these babies having had large head.
Figure 1
Large head.
His creatine kinase (CK) level was very high …
37 citations
TL;DR: A case of a girl with the Herlyn–Werner–Wunderlich syndrome who had a prenatal diagnose of right renal agenesis and presented at 13 years old with pelvic pain caused by haematocolpos is reported.
Abstract: The Herlyn–Werner–Wunderlich syndrome is a rare congenital anomaly characterised by uterus didelphys with blind hemivagina and ipsilateral renal agenesis. It usually presents after menarche with progressive pelvic pain during menses secondary to haematocolpos. Awareness is necessary in order to diagnose and treat this disorder properly before complications occur. MRI is the preferred modality for the delineation of uterine malformation. When renal anomalies are encountered, a screening should also be made for congenital abnormalities of the reproductive tract and vice versa. The authors report a case of a girl with this condition who had a prenatal diagnose of right renal agenesis and presented at 13 years old with pelvic pain caused by haematocolpos.
37 citations
TL;DR: In the week preceding the admission, the patient had consumed large quantities of a popular energy drink and the authors believe this may have implicated the development of his coronary event.
Abstract: This report summarises the case of a 19-year-old male, with a history of gastro-oesophageal reflux disease, who presented to hospital with an acute chest pain. An electrocardiographic and biochemical diagnosis of ST elevation myocardial infarction was made; however, subsequent coronary angiography and echocardiography were both normal. In the week preceding the admission, the patient had consumed large quantities of a popular energy drink and the authors believe this may have implicated the development of his coronary event. This is an association that has been suggested previously and this report briefly summarises the evidence supporting the connection.
37 citations
TL;DR: The authors report a 28-year-old man who was brought to the emergency room after sudden onset of tonic-clonic seizures and metabolic acidosis after drinking several cans of a caffeinated energy drink and believe that this clinical picture was caused by caffeine intoxication from an energetic drink causing a syndrome of catecholamine excess.
Abstract: In recent years an increasing number of different energy drinks have been introduced to provide an energy boost. They contain high levels of caffeine and other additives that act as stimulants. Several recent studies present that energy drinks could increase the risk of seizures, acid-base disorders and cardiovascular events. The authors report a 28-year-old man who was brought to the emergency room after sudden onset of tonic-clonic seizures and metabolic acidosis after drinking several cans of a caffeinated energy drink. The authors believe that this clinical picture was caused by caffeine intoxication from an energetic drink causing a syndrome of catecholamine excess. The patient was discharged within a week with no complaints and no neurological signs. Finally, recognising the features of caffeine intoxication and its potential health consequences may be especially relevant when treating younger persons who may be more likely to consume energy drinks.
36 citations
TL;DR: The authors report two ischaemic strokes, which developed because of this arterial occlusion; their presentation differed from each other.
Abstract: Artery of Percheron is a normal variant of the paramedian branches of posterior cerebral artery. This artery supplies the paramedian areas of the thalami and upper midbrain. Occlusion of this artery is rare and results in a multitude of neurological signs and symptoms, which might prompt the physician think of an aetiology other than vascular insults, and therefore change the management plan. The authors report two ischaemic strokes, which developed because of this arterial occlusion; their presentation differed from each other.
TL;DR: Dipylidium caninum is a cestode that requires from the participation of an arthropod in its life cycle, and occurs in dogs and cats, and occasionally in human beings.
Abstract: Dipylidium caninum is a cestode that requires from the participation of an arthropod in its life cycle. This parasitosis occurs in dogs and cats, and occasionally in human beings. Human cases of D caninum infection have been reported in Europe, Philippines, China, Japan, Latin America and the United States; mostly children, one third of them being infants under 6 months old. The diagnosis of this disease is done by the parasitological study of the feces, observing the characteristics of the gravid proglottids. The treatment is performed by administering broad-spectrum anthelmintics. The authors report a case of a rare infection in a Mexican child.
TL;DR: The authors describe this fatal association in two siblings who were never able to receive enteral feeds, remained totally dependent on parenteral nutrition, had repeated episodes of sepsis and died after a very difficult neonatal intensive care course.
Abstract: Hereditary multiple intestinal atresia (HMIA), a presumed autosomal recessive disorder, is an unusual and rare form of recurrent intestinal atresia which can be associated with severe combined immunodeficiency (SCID). The combination of HMIA and SCID is invariably lethal. The authors describe this fatal association in two siblings. The parents are consanguineous and have three other normal healthy children. Both index cases had abnormal antenatal ultrasounds and were symptomatic after birth. The final diagnosis of HMIA with SCID was confirmed in both siblings. They were never able to receive enteral feeds, remained totally dependent on parenteral nutrition, had repeated episodes of sepsis and died after a very difficult neonatal intensive care course. In this article we have reviewed the clinical course and outcome of both cases. The existing literature on multiple intestinal atresia, HMIA and HMIA with immunodeficiency is also reviewed.
TL;DR: The authors report a case of this syndrome presenting with fever, generalised pruritus, macular rash and cholestatic hepatitis during allopurinol treatment.
Abstract: DRESS syndrome (drug rash, eosinophilia and systemic symptoms) is an idiosyncratic drug reaction characterised by rash, fever, lymphadenopathy and internal organ involvement. The authors report a case of this syndrome presenting with fever, generalised pruritus, macular rash and cholestatic hepatitis during allopurinol treatment. This case resolved with drug withdrawal, but the death rate in the setting of hepatic failure can reach 10%. Rapid diagnosis is crucial as prompt withdrawal of the offending drug is the key of the treatment, while the potential role of corticosteroids remains controversial.
TL;DR: The authors present two patients with diarrhoea and malnutrition; one after RYGBP and the other after jejunoileal bypass surgery, subsequently diagnosed with Crohn’s disease.
Abstract: Bariatric surgery for the treatment of severe obesity has increased dramatically in recent years in the USA and parts of Western Europe. The most commonly used technique is the Roux-en Y gastric bypass (RYGBP). Several nutritional and gastrointestinal complications after bariatric surgery have been described during the last 10 years. The authors present two patients with diarrhoea and malnutrition; one after RYGBP and the other after jejunoileal bypass surgery. These patients were subsequently diagnosed with Crohn’s disease.
TL;DR: A 39-year-old male patient displayed signs of acute stroke, which were associated with AAS abuse over the last 3 years, and AAS-induced cardiomyopathy with a thrombus in the left ventricle was discovered to be the aetiology of his stroke and peripheral vascular disease.
Abstract: Acute stroke could be the presentation of unrecognised cardiomyopathy postanabolic androgenic steroid (AAS) abuse. A 39-year-old male patient displayed signs of acute stroke, which were associated with AAS abuse over the last 3 years. Despite the absence of symptoms and signs of congestive heart failure at presentation, AAS-induced cardiomyopathy with a thrombus in the left ventricle was discovered to be the aetiology of his stroke and peripheral vascular disease. Awareness of the complications of AAS led to the prompt treatment of the initially unrecognised dilated cardiomyopathy, and stroke.
TL;DR: A case of late congenital syphilis presenting with Hutchinson’s triad at the age of 7 years is reported, characterised by the presence of interstitial keratitis, eighth nerve deafness and Hutchinson's teeth.
Abstract: Congenital syphilis is an infectious disease caused by Treponema pallidum transmitted by infected mother to her baby during pregnancy. Late congenital syphilis is recognised with 2 or more years after birth. One of the main aspects is observed with the triad of Hutchinson, characterised by the presence of interstitial keratitis, eighth nerve deafness and Hutchinson’s teeth. This manuscript reports a case of late congenital syphilis presenting with Hutchinson’s triad at an age of 7 years. These clinical features are related to syphilis present during pregnancy and at birth, however they commonly become apparent after 5-years of age.
TL;DR: This rare condition is associated with considerable morbidity but may be associated with good fetal outcome and was managed conservatively and postpartum there was complete spontaneous resolution.
Abstract: Physiological enlargement of the breasts occurs at puberty and during pregnancy. It is known as gestational gigantomastia when enlargement in pregnancy becomes excessive, uncomfortable and embarrassing. Gestational gigantomastia may have far reaching effects for the mother and fetus. This rare condition is associated with considerable morbidity but may be associated with good fetal outcome. Our case was very interesting because it was managed conservatively and postpartum there was complete spontaneous resolution.
TL;DR: This research presents a novel probabilistic approach to estimating the response of the immune system to laser-spot assisted, 3D image analysis of central nervous system injuries.
Abstract: The authors describe a new variant of guanosine triphosphate (GTP)- cyclohydrolase deficiency in a young man with severe and disabling major depressive disorder with multiple near-lethal suicide attempts. His cerebrospinal fluid levels showed that the concentration of tetrahydrobiopterin (BH4), neopterin, 5-hydroxyindoleacetic acid and homovanillic acid were below the reference range, suggesting a defect in the pterin biosynthetic pathway and in synthesis of dopamine and serotonin indicative of GTP-cyclohydrolase deficiency. Patient was started on sapropterin, a BH4 replacement protein, for the defect in the above pathway. In addition, the authors started 5-hydroxytryptophan titrated to 400 mg orally twice daily with concomittant carbidopa 37.5 mg orally four times a day, and he responded with remission of suicidal ideation and significant improvement in depression and function.
TL;DR: Two cases of buckwheat allergy that presented to the UK allergy service recently are presented, including a 57-year-old man presented with anaphylaxis after eating home-baked bread prepared using buckWheat flour bought in France and a 63- year-old lady presented with bronchospasm and urticaria after consuming health-food muesli.
Abstract: Buckwheat is commonly consumed in many parts of the world and has recently become more available in the UK. Buckwheat allergy is well recognised in parts of mainland Europe and Asia, typically associated with consumption of specific regional foods. No adult cases of buckwheat allergy in the UK have been reported in the literature. The authors present two cases of buckwheat allergy that presented to our UK allergy service recently. A 57-year-old man presented with anaphylaxis after eating home-baked bread prepared using buckwheat flour bought in France. In the second case, a 63-year-old lady presented with bronchospasm and urticaria after consuming health-food muesli. Sensitisation was confirmed in both cases by positive skin prick testing and specific IgE to buckwheat. Given the growing popularity of foods that may contain buckwheat, including ethnic and health-food ranges, buckwheat allergy is likely to become increasingly common in the UK.
TL;DR: The present case will provide a neuropathologic evidence of correlation between α-synucleinopathy and Parkinsonism of SCA2 as well as shed light on understanding the pathomechanism of Parkinsonism inSCA2.
Abstract: Clinical phenotype of individuals with spinocerebellar ataxia 2 (SCA2) is characterised by cerebellar ataxia and cognitive impairment Although L-dopa-responsive Parkinsonism is considered as a rare clinical presentation in SCA2, it has been brought to the attention of many neurologists in several studies The authors report an autopsy case of SCA2 with Parkinsonism from a Japanese family using archival materials of our Brain Bank to describe unique neuropathologic findings The individual clinically showed Parkinsonism as a predominant phenotype instead of cerebellar ataxia Besides the classic SCA2 neuropathologic alterations, Lewy bodies and Lewy neurites were present in the brainstem nuclei Genetic analysis revealed shorter abnormal expansion of CAG repeats (less than 39) In contrast, the authors could not find α-synuclein pathology in two SCA2 cases without Parkinsonism The present case will provide a neuropathologic evidence of correlation between α-synucleinopathy and Parkinsonism of SCA2 as well as shed light on understanding the pathomechanism of Parkinsonism in SCA2
TL;DR: A 65-year-old man who was diagnosed with processionary caterpillar dermatitis was treated with topical potassium dobesilate 5% cream twice a day for 2 days and an improvement occurred soon after treatment.
Abstract: Skin contact dermatitis by pine processionary (Thaumetopoea pityocampa) is a public health problem of increasing significance. The authors present here the case of a 65-year-old man who was diagnosed with processionary caterpillar dermatitis. Patient was treated with topical potassium dobesilate 5% cream twice a day for 2 days. An improvement occurred soon after treatment.
TL;DR: Thalidomide, with its antiangiogenic mechanism of action, seems to be promising drug as a treatment option where other modalities have been unsuccessful in treating bleeding gastro-intestinal angiodysplasia.
Abstract: Hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant disorder characterised by epistaxis, cutaneous telangiectasia and visceral arterio-venous malformations (AVMs). It affects approximately one in 5000 people. Control of sustained and repeated haemorrhages from telangiectasias in the nose and gut in patients who may be transfusion dependent is clinically challenging. After repeated endoscopic coagulations, multiple lesions often recur at other sites of gastro-intestinal tract, where endoscopic therapy or surgical resection is not possible. Hormonal therapy has been employed for more than 50 years but has recently been shown to be ineffective. Thalidomide, with its antiangiogenic mechanism of action, seems to be promising drug as a treatment option where other modalities have been unsuccessful. In this article, the authors discuss a novel treatment of bleeding gastro-intestinal angiodysplasia.
TL;DR: A woman with presumed contact dermatitis who was diagnosed with breast cancer in the setting of progressive fatigue and muscle weakness was diagnosed, and treatment simultaneous with treatment of the breast cancer led to regression of DM.
Abstract: Breast cancer most often presents as a palpable mass or with an abnormal mammogram. Much less commonly, breast cancer may present as a paraneoplastic syndrome. Dermatomyositis (DM) is a rare disease most often considered a complement-mediated idiopathic inflammatory myopathy manifested by classic skin findings and proximal muscle weakness. However, DM may also be due to a paraneoplastic syndrome associated with an underlying malignancy. The authors present a case report of a woman with presumed contact dermatitis who was diagnosed with breast cancer in the setting of progressive fatigue and muscle weakness. DM was subsequently diagnosed. Treatment of DM simultaneous with treatment of the breast cancer led to regression of DM. The diagnosis of DM in an adult should raise suspicion of an underlying malignancy. Breast cancer is a common disease that may rarely present with uncommon features that may divert attention from the underlying malignancy.
TL;DR: A 34-year-old male who presented to the emergency department with a week’s history of dull epigastirc pain showed radiological evidence of acute on chronic pancreatitis with thrombosis of the portal vein and multiple intrahepatic abscesses, which could represent an infection of the peripancreatic tissue with F necrophorum further leading to pylephlebitis.
Abstract: Fusobacterium necrophorum is a non-sporulating anaerobic gram negative bacillus and has traditionally been associated with Lemierre’s syndrome. The authors report a 34-year-old male who presented to the emergency department with a week’s history of dull epigastirc pain. Significant medical history included chronic pancreatitis secondary to alcohol use. The patient had radiological evidence of acute on chronic pancreatitis with thrombosis of the portal vein and multiple intrahepatic abscesses. CT-guided drainage of left upper quadrant revealed fluid collection in the pancreatic bed. The fluid culture grew F necrophorum and the patient was treated with tigecycline for 4 weeks. The patient improved symptomatically and his follow-up computerised axial tomography scan 2 months later showed resolving liver abscess, cavernous transformation of the portal vein and stable findings of chronic pancreatitis. This could represent an infection of the peripancreatic tissue with F necrophorum further leading to pylephlebitis.
TL;DR: A case of a patient treated for Wilms’ tumour with IFA who developed rickets with Fanconi syndrome is presented, and selective renal protection against during chemotherapy with I FA may be possible soon.
Abstract: Ifosfamide (IFA) is a powerful chemotherapeutic drug that is active against a variety of paediatric malignancies. However, renal toxicities such as haemorrhagic cystitis and Fanconi syndrome are major hazards that hinder its use in clinical practice. The authors present a case of a patient treated for Wilms’ tumour with IFA who developed rickets with Fanconi syndrome. Patients undergoing IFA treatment must be carefully monitored for the development of iatrogenic complications. Recent studies have improved our understanding of the underlying pathomechanism of IFA induced Fanconi syndrome, and selective renal protection against during chemotherapy with IFA may be possible soon.
TL;DR: The authors emphasise the need to consider chorioangioma as a cause of non-immune fetal hydrops and microangiopathic haemolytic anaemia.
Abstract: Giant choriangiomas are rare placental tumours, associated with a high prevalence of pregnancy complications and a poor perinatal outcome. Neonatal consequences include severe microangiopathic haemolytic anaemia, thrombocytopaenia and hydrops. The associated high perinatal death rate (30–40%) has led to a number of prenatal therapeutic interventions with limited success in most cases. The authors present a case of non-immune fetal hydrops caused by a giant chorioangioma, diagnosed at 27 weeks of gestational age. Despite tocolytic therapy, the baby was born prematurely (28 weeks of gestational age) and required transfusion of blood derivatives, intensive phototherapy and exchange transfusion. She had an uncomplicated recovery and was discharged home in the second month of life. The authors emphasise the need to consider chorioangioma as a cause of non-immune fetal hydrops and microangiopathic haemolytic anaemia.
TL;DR: Screening serological tests for brucella should be used more widely in cases with low index of suspicion, especially in endemic areas, particularly in regions where brucellosis is endemic.
Abstract: Brucellosis is a zoonosis that affects several organs. The spine is the most common site of musculoskeletal involvement. However, multiple-level spinal involvement is rare in brucella spondylodiscitis. The authors report a case of a 56-year-old male shepherd who had developed a spondylodiscitis affecting simultaneously the cervical, thoracic and lumbar regions. The diagnosis was established by using MRI after the brucella-agglutination test was found to be positive. A high degree of suspicion in the diagnosis of brucellar spondylodiscitis is essential to reduce the delay for the treatment. Thus, it should be essentially included in the differential diagnosis of longstanding cervical, thoracic or back pain, particularly in regions where brucellosis is endemic. Screening serological tests for brucella should be used more widely in cases with low index of suspicion, especially in endemic areas.
TL;DR: A 69-year-old female with long-standing severe asthma and on home oxygen, who presented with a 2 cm mobile mass in the left ventricular outflow tract and symptoms of left heart failure and stroke, is reported.
Abstract: Calcified amorphous tumour (CAT) of the heart is a rarely reported non-neoplastic cardiac mass. The authors report a 69-year-old female with long-standing severe asthma and on home oxygen, who presented with a 2 cm mobile mass in the left ventricular outflow tract and symptoms of left heart failure and stroke. During minimal access cardiac surgery, a CAT was found attached to the base of the mitral valve. The tumour was removed and the patient had an uneventful postoperative course. The authors present their experience with this patient and review the current literature on this rare kind of tumour.
TL;DR: The case of a 40-year-old woman with chronic SCLS treated with high-dose intravenous immunoglobulins is reported, after a prophylactic therapy with theophylline and terbutaline was poorly tolerated and failed to decrease the frequency and severity of the attacks sufficiently.
Abstract: The systemic capillary leak syndrome (SCLS), also known as Clarkson’s disease, is a life-threatening disorder of unknown cause. It is characterised by recurrent acute episodes of hypotension, weight gain and generalised oedema with haemoconcentration and hypoproteinaemia caused by paroxysmal capillary hyperpermeability with a shift of plasma fluid from the intravascular to the interstitial space. We report the case of a 40-year-old woman with chronic SCLS treated with high-dose intravenous immunoglobulins, after a prophylactic therapy with theophylline and terbutaline was poorly tolerated and failed to decrease the frequency and severity of the attacks sufficiently.